Preoperative magnetic resonance imaging. The T2-weighted sagittal image (left) shows a large hyperintense retroclival cystic mass extending into interpeduncular and suprasellar cisterns, causing compression and posterior displacement of brain stem.


Preoperative magnetic resonance imaging. The T1-weighted postgadolinium sagittal image (middle) shows a nonenhancing mass.


Preoperative magnetic resonance imaging. Diffusion weighted image (right) demonstrates a mass lesion in interpeduncular cistern without restricted pattern.


Intraoperative transnasal endoscopic view after fenestration of the cyst into adjacent cisterns.


Postoperative magnetic resonance imaging. The T2-weighted axial.


Postoperative magnetic resonance imaging. Sagittal. Images show cyst shrinkage and decompression of brain stem.


Brain stem degeneration presents in brain MRI. Sagittal.


Brain stem degeneration presents in brain MRI. Transversal. T2-weighted brain MRI indicates gliotic neurodegeneration in the medulla oblongata with predominant loss of pyramidal tracts.


Brain stem degeneration presents in brain MRI. Magnification of inlay (c) uncovers pathologic brain stem formation reminiscent of 'kissing swans.


Brain stem degeneration presents in brain MRI. D; Transversal T1-weighted sectioning of the brain stem (arrow) after Gadolinium administration. No contrast enhancement is detectable.


Brain stem degeneration presents in brain MRI. E; Sagittal T2-weighted cervical spine MRI shows atrophy of the upper cervical spinal cord in addition to medulla oblongata atrophy.


Brain stem degeneration presents in brain MRI. F; Transversal T2-weighted brain MRI indicates putative periventricular rim-sign and global brain atrophy.


Brain stem degeneration presents in brain MRI. G; Brain MRI-angiography reveals normal intracranial vascular status without indication of stenosis.


The audiogram reveals sudden senseurineural hearing loss on the right ear.


The audiogram after the treatment shows no improvement.


The audiogram shows spontaneous recovery of the right ear.


Fundus photographs of a 50-year-old woman, who presented with blurry vision in her right eye 12 h after having coronary angiography. (a) Right eye showing a tiny single juxtafoveal whitish patch consistent with an isolated cotton wool spot.


Fundus photographs of a 50-year-old woman, who presented with blurry vision in her right eye 12 h after having coronary angiography. (b) Left eye fundoscopy was unremarkable.


Upward movement restriction in the left eye.


Coronal computed tomography - left orbital floor fracture with entrapment.


Intact infraorbital rim with entrapped orbital content.


(a) Computed tomography, at admission, demonstrating that localized thick clot in the left Sylvian fissure. Note that it comes with perifocal edema.


(b) Preoperative cerebral angiography demonstrating that the left middle cerebral artery and anterior cerebral artery showed evidence of severe vasospasm.


(c) Three-dimensional digital subtraction angiography, operative view, demonstrating that small aneurysm originates from the left sphenoidal segment of middle cerebral artery (M1) - insular segment of middle cerebral artery (M2) bifurcation (arrow). Note that severe vasospasm was observed in M1 (arrow head).


(a) Intraoperative photograph during direct clipping demonstrating that the left M1-M2 bifurcation aneurysm was exposed. A fibrin cap covers the dome of the aneurysm and the source of intra-Sylvian hematoma was determined to be this small aneurysm.


(b) The ruptured aneurysm was occluded with two clips.


(c) After clipping of the aneurysm, spastic vessels were directly applied with nicardipine which was soaked in oxidized cellulose.


(a) Postoperative cerebral angiography, performed just after the direct clipping of the left middle cerebral artery (MCA) aneurysm, demonstrating that the left M1 was completed dilated.


(b) Magnetic resonance angiography (MRA), on the 3rd day of operation, demonstrating that the left MCA showed evidence of vasospasm.


(c) MRA, on the 18th day of the operation, demonstrating no evidence of vasospasm on the left anterior cerebral artery and MCA.


Electrocardiograme demonstrating symmetrical T wave inversions in the anterolateral leads concerning for ischemia.


(A) Coronary angiogram showing left coronary artery circulation with a non-occlusive lesion in proximal left circumflex artery.


(B) Coronary angiogram showing right coronary artery circulation.


(A,B) Left ventriculography demonstrating apical ballooning and hypokinesis of the apex with normal functioning basal inferior and anterior segments.


Intraoperative views (opening of the dura mater, left side), showing the shunt location and the single draining vein (white arrow).


Which is disconnected with bipolar coagulation (black arrow: disconnected vein).


The draining vein is degenerated with shunt disconnection (black dashed arrow) (d).


Optic disc asymmetry of cup-disc ratio 0.6 in the right eye.


0.4 in the left eye.


Optic nerve head topography on HRT.


Optical coherence tomography of the optic nerve head and peripapillary retinal nerve fiber layer. . Note: Both the HRT and optical coherence tomography were normal except for rim area asymmetry noted on HRT. . Abbreviation: HRT, Heidelberg retinal tomograph.


Humphrey visual field of patient's left.


Right. Eyes.


Echocardiography showed characteristic kinetic disturbances in the apical heart region.


Levocardiography in the right anterior oblique position shows the picture of an octopus pot, which is characteristic for Takotsubo cardiomyopathy.


Diagnostic computed tomography angiogram figure description: bony erosion and remodeling of the left sphenoid and petrous temporal bones are seen, related to the mass (a and b).


On contrasted imaging (c), angiography demonstrates external compression (approximately 50% narrowing) of the cavernous and petrous segment of the left internal carotid artery.


Mandibular and muscular atrophy figure description: contrasted T1-weighted magnetic resonance imaging demonstrates asymmetric of the mandibular rami, with left smaller than right, as well as atrophy of the muscles of mastication.


The axial brain fluid-attenuation inversion recovery (FLAIR) images.


Sagittal T2-weighted images , demonstrating a "heart-shaped" appearance area of hyperintensity located in the tegmentum of the caudal midbrain.


(A) The brain diffusion-weighted image (DWI) of the patient is shown. Note the "heart or V"-shaped lesion showing increased intensity in the tegmentum of the caudal midbrain.


(B) The brain apparent diffusion coefficient map MRI (ADC) of the patient is shown. The "heart or V"-shaped lesion shows low intensity on ADC, consistent with acute infarction.


1.5T Brain MRI, axial T2-weighted images.


Coronal T2-weighted images. Shows the symmetric enlargement and increased signal intensity of both inferior olives (arrow).


Results and evolution in z-scores of individual tests - Case 1. IQ, Intelectual quocient; T1, Time 1; T2, Time 2; T3, Time 3.


Results and evolution in z-scores of individual tests - Case 2. IQ, Intelectual quocient; T1, Time 1; T2, Time 2; T3, Time 3.


Stimulation amplitude of adaptive deep brain stimulation (aDBS) and fluctuations in the beta band (16.60 +- 2.5 Hz) oscillation of the local field potential (LFP) over time for a long period.


A short period Arrow in (b) indicates the timing of levodopa 100 mg /carbidopa 25 mg intake. Conventional DBS (cDBS) (outside the hospital): Stimulation was set at 2.2 mA. ADBS (inside and outside the hospital): Upper stimulation was set at 3.5 mA and lower at 0.7 mA. Strength of beta oscillation = Vin (v) * Gain * 16 (LSB) / 1.2 (v) Vin: input voltage to the analog to digital convertor, Gain = 250, LSB of an analog-to-digital convertor is 16 least significant bits, The analog-to-digital convertor has a voltage range of 1.2 v.


Left optic nerve at presentation, showing diffuse swelling.


Normal right optic nerve at presentation.


Two weeks later, right optic nerve showing very pale swelling.


Electrocardiogram revealed sinus rhythm, low voltages in limb leads, QS waves indicative of pseudoinfarction in precordial and inferior leads, first-degree atrioventricular block, and prolonged QTc.


A four-chamber apical view echocardiogram showing biatrial dilatation, valve thickening, thick ventricular walls (left ventricular wall is 15 mm and interventricular septum is 19 mm), and interventricular septum with speckled appearance, which suggests amyloid infiltrate. . Abbreviations: RV, right ventricle; LV, left ventricle; RA, right atrium; LA, left atrium.


Rectum biopsy: amyloid deposits are confirmed by a positive Congo red stain (arrow), which gives the characteristic salmon pink color (200x).


Phenytoin levels of patient 1. Red dots indicate hospital admissions for increased seizure frequency.


Preoperative picture of the patient showing left-sided tongue atrophy but no deviation.


Image of face showing right sided ptosis.


Course of the ADHD-RS-IV-J score and YGTSS score.


Patient 1 performs ceiling work and injects grouting.


Patient 2 performs ceiling work and injects grouting.


EEG showing repetitive, generalized paroxysms of high amplitude discharges followed by slow wave pattern associated with muscle artifact suggesting myoclonic type of epileptiform discharges.


Cranial MRI. FLAIR axial imaging.


Cranial MRI. T2 coronal imaging. Both images show a tenuous hyper-signal in the left thalamic region (arrows) compatible with vasogenic edema or gliosis. Note the lack of interruption of the hematoencephalic barrier or cytotoxic edema.


Acute hemorrhagic conjunctivitis with bilateral subconjunctival hemorrhages.


External clinical photograph. The slight elevation and boundaries of the subcutaneous nodular lesion are marked by a circle.


Top left: HE (Magnification x200); DNT simple form with oligodendroglial-like cells. Top right: GFAP (Magnification x400); immunostaining GFAP is negative in tumour cells. Bottom left: S100 (Magnification x400); immunostaining S100 is positive in tumour cells. Bottom right: Ki-67 (Magnification x600); immunostaining Ki-67 proliferative index is low <1%.


(a-c) Axial, coronal and sagittal 3D T1 FFE MR images of the lesion for preoperative planning in 2002, showing the lesion's inferior extension in the white matter to within a few millimetres of the superolateral edge of the left lateral ventricle.


Top left: HE (Magnification x200); DNT complex form tumour pattern resembling low grade glioma with multicystic (pilocytic like) pattern. Top right: GFAP (Magnification x400); immunostaining GFAP is negative in tumour cells. Bottom left: S100 (Magnification x400); immunostaining S100 is positive in tumour cells. Bottom right: Ki-67 (Magnification x600); immunostaining Ki-67 proliferative index is low <1%.


(a-c) Stereotactic sagittal T1, sag CET1, axial T2 MR images for Gamma Knife treatment planning demonstrates partial, solid contrast enhancement in the T2 hyperintense, residual DNT in the left postcentral gyrus (2002).


(a and b) Preoperative axial and coronal T2 weighted MR images demonstrate the well-delineated hyperintense mass in the right medial temporal lobe and hippocampus (1994).


Top left: HE (Magnification x100); DNT complex form with specific glioneuronal element. Top right: HE (Magnification x400); DNT complex form with specific glioneuronal element. Bottom left: GFAP (Magnification x200); immunostaining GFAP is negative in tumour cells. Bottom centre: S100 (Magnification x200); immunostaining S100 is positive in tumour cells. Bottom right: Ki-67 (Magnification x600); immunostaining Ki-67 proliferativ index is low <1%.


(a-c) 2001 axial T2, axial and coronal CE T1 MR images post subtotal resection demonstrate residual contrast enhancing DNT.


(a-d) MRI 2013: axial T2, axial and coronal T1 MR images show dramatic decrease in DNT size and contrast enhancement 8 years after GKRS.


(A) Clinical course of the patient. CSF, cerebrospinal fluid; IVIg, intravenous immunoglobulin.


(B) Computed tomography image of the patient's chest. A mediastinal tumor was detected (arrowheads).


MRI of the brain revealed change after removal of pituitary tumor and no acute cerebral infarction, cerebral hemorrhage, and inflammatory changes were found on T1 and T2 sequences [T1, T1-Weighted Magnetic Resonance Imaging; T2, T2-Weighted Magnetic Resonance Imaging. T1.


Medical image.


Electroencephalogram recorded. EEG demonstrates delta and theta waves in each lead as background, mingled with sharp waves, spike waves, sharp-slow waves (A).


Electroencephalogram recorded. EEG shows a large number of sharp waves, spike waves, sharp-slow waves, spike-slow waves in the bilateral frontal pole, frontal and anterior temporal areas, paroxysmal or continuous, sometimes accompanied by evolution trend (B-D) (paper speed: 30 mm/s, high-frequency filter (HF): 30 Hz, low-frequency filter (LF): 0.53 Hz, notchfilers: 50 Hz, sensitivity:7 muv/mm. EEG A provided EEG background and the EEG B showed epileptic discharges).


Patient 1 brain MRI revealing the characteristic ARSACS findings. MRI of the index patient reveals atrophy of the superior cerebellar vermis (arrowhead) and thinning of the posterior midbody of corpus callosum (white arrow). T1), bilateral parietal atrophy.


Patient 1 brain MRI revealing the characteristic ARSACS findings. Mostly prominent on the left side (white arrows). T1).


Patient 1 brain MRI revealing the characteristic ARSACS findings. Bilateral hypointense stripes in pons (arrowheads). T2.


Patient 1 brain MRI revealing the characteristic ARSACS findings. . FLAIR).


Patient 1 brain MRI revealing the characteristic ARSACS findings. Bilateral hyperintensity of lateral pons (stars) on coronal images. T2).


Patient 1 brain MRI revealing the characteristic ARSACS findings. A hyperintense rim around both thalami (white arrows). T2).


Family pedigree and sequence chromatograms. The two affected siblings (patient 1: II.1, patient 2: II.3) were homozygous for the identified SACS variant (c.429_430delTT), and both parents were heterozygous carriers.


Fluid attenuated inversion recovery.


T2 turbospin echo sequence. Demonstrating high signal intensity in the dentate nuclei bilaterally (white arrows).


Fluid attenuated inversion recovery imaging at the 7-week follow-up imaging, demonstrating resolution of the previous dentate nuclei high signal; compare with Figure 1.


Diffusion sequence showing resolution of the splenium lesion at follow up. Apparent diffusion coefficient map showing complete resolution of the splenium lesion at follow up imaging; compare with Figure 2.


Enhanced computed tomography findings. . Enhanced abdominal computed tomography showed the tumor (white arrow) adjacent to the common bile duct.


Endoscopic ultrasonography findings. . Endoscopic ultrasonography demonstrated the tumor (white arrow) at the junction of the cystic duct. On Sonazoid-enhanced echo, the tumor was universally enhanced.


Magnetic resonance cholangiopancreatography findings. . Magnetic resonance cholangiopancreatography findings show that the tumor (white arrow) had a slightly high signal on T2 weighted imaging. The remnant cystic duct was dilated by the tumor, which displayed high intensity on T2 weighted imaging (arrowhead).


Macroscopic findings. . Macroscopic findings had two components; the dilated remnant cyst with white bile (arrowhead), and the whitish main tumor with substantial neurofibrotic changes (white arrow).


Pathological findings. . The tumor was stained by anti S-100 antibody (white arrow). The wall of the cystic duct (black arrow) was compressed by the tumor, narrowing the intraductal space (black arrowhead).


Photographs demonstrating ophthalmoplegia secondary to the right third nerve palsy with involvement of the pupil. The center panel demonstrates the extent of ptosis at rest and characteristic "down and out" position of the eye. Remaining images demonstrate eye position during voluntary gaze in each direction relative to the center panel.


Post-procedure average recorded blood glucose (mg/dL) over 1 month after epidural steroid injection (ESI). Each point represents the average blood glucose obtained from the patient's personal glucometer calculated in 3-day bins. Initial value (day 0) reflects the blood glucose level recorded at the treatment center immediately before ESI.


Computed tomography head showed an acute left subdural hemorrhage with significant left-to-right midline shift.


(A and B) An anterolateral (black triangle) and inferior (gray triangle) wall of the LV have necrotic changes with hemorrhage.


(C) The RV (white triangle) also has necrotic changes with hemorrhage. Free wall rupture (black arrow) is noted.


(D) Ventricular septal wall rupture (white arrow) is observed.


(E) The injured myocytes are disrupted in multiple levels (arrows) and interposed by histiocytes and lymphocytes. Groups of myocytes (squares) have increased eosinophilic staining with loss of central nuclei (hematoxylin-eosin, x200). The infiltrating lymphocytes are CD3/CD4 positive helper T cells (CD3, x400) (inlet).


(F) Intact myocytes with myofibrils are red; injured myocytes are contrasted in blue (Masson Trichrome, x200). LV, left ventricle; RV, right ventricle.


Blue color of OMNI microcatheter is visible behind the trabecular meshwork.


Photographs of the patient's face. (A) The face showed marked swelling of right cheek and periorbital area involving eyelid, severe proptosis of the right eye.


Photographs of the patient's face. (B) Nine cardinal gaze photographs showed severe limitation of the right eyeball movement for all gazes except abduction.


CT findings of the patient. (A) Coronal CT scan at presentation showed severe maxillary and ethmoidal sinusitis as well as marked inflammatory swelling of soft tissue in the right cheek and temporal region. Intraorbital soft tissue inflammation and thickening of the lateral rectus muscle of right eye were also observed.


CT findings of the patient. (B) Axial CT scan taken 1 day after sinus surgery showed severe tenting of the posterior part of globe (arrow) and stretching of the optic nerve due to severe proptosis.


Fundus findings of the patient. (A) Fundus photograph of the right eye showed a pale optic disc, cherry red spot in macula, and edema at the posterior pole.


Fundus findings of the patient. (B) Fluorescein angiography of right eye showed a delay of retinal circulation.


Swelling of the cheek and proptosis of the right eye had nearly subsided after 2 weeks of hospitalization but visual loss had not been recovered.


Pre-treatment view: Asymmetry of the face.


Pre-treatment view: Shift of mandibular jaw toward right side while opening.


Facebow transfer.


Indentation marks on flat occlusal table of mandibular denture.


Maxillary palatal cusps contacting the occlusal table.


Post-treatment view: Insertion of definitive complete dentures.


Preoperative and follow-up scalp EEGs. Preoperatively (A) generalized spikes and multiple spike complex were frequently observed with phase reversal at the central region (C3, Cz, C4).


Preoperative and follow-up scalp EEGs. Follow-up EEGs [ 3-months.


Preoperative and follow-up scalp EEGs. 6-months.


Preoperative and follow-up scalp EEGs. 12-months after surgery] showed decreased spike frequency, and some small spikes were found to be limited to one hemisphere. However, large spikes were always bilaterally synchronized. Bipolar montages: blue traces are left (F3-C3, C3-O1, F3-T3, T3-O1), greens are midline (Fz-Cz, Cz-Pz), and reds are right (F4-C4, C4-O2, F4-T4, T4-O2). Sensitivity = 15 muV, Time constant = 0.1, High-cut filter = 60 Hz, AC filter = ON.


Behavioral pure-tone air conduction thresholds at 11 years old.


Behavioral frequency modulated tones thresholds with CI after one-year CI usage.


Pre (Last visit prior to CI) and Post-Operative Word discrimination score.


Brain MRI T1-weighted images of the patient. The positions of the coronal, sagittal, and axial images are indicated by the orange lines. A mural nodule of the pilocytic astrocytoma is shown near the crossing point of the orange lines on the coronal and axial images. A cyst associated with the tumor occupies most of right cerebellum.


Preoperative brain MRI The white arrow (a; axial Flair sequence) points to the tumor invading the cerebellar parenchyma bilaterally. The lesion appears slightly hyperintense. The 4th ventricle is invaded (white arrowhead), causing a hydrocephalus (dilatation of the lateral ventricles in b).


Preoperative brain MRI.


Preoperative brain MRI The tumor expands clearly in the vermis as shown on a sagittal image (c). Early postoperative axial FLAIR slice shows postoperative sequelae at the level of dentate nuclei bilaterally (white arrows in d).


Medical image.


The hydrocephalus is resolved (e). The 4th ventricle is moderately dilated, including in the rostral direction. The superior medullary velum is visible (white arrow in f). The superior cerebellar peduncles are involved (white arrows in g), with a slight extension towards the corpora quadrigemina at the level of inferior colliculi (white arrows in h). Crus cerebri are spared bilaterally (white arrows in i). R: right side.


(A) A 2-min view of the EEG pattern with polygraphy of a left temporal lobe seizure characterized by rhythmic theta and delta activity on left fronto-temporal regions in patient 1. This long-lasting apnea induces a severe oxygen desaturation (SpO2 75%). (A,B) Red arrows highlight the onset of the apnea. Note the ictal marked increase in heart rate. Red channel: ECG; blue channel: thoracoabdominal respirogram.


(B) A more detailed view of the onset of apnea preceding EEG changes and EMG movement-related artifacts by 20 s. (A,B) Red arrows highlight the onset of the apnea. Note the ictal marked increase in heart rate. Red channel: ECG; blue channel: thoracoabdominal respirogram.


(A) Coronal FLAIR MRI of patient 2 (left) showing a hyperintense right amygdala with red arrow pointing to an area of increased signal intensity (red arrow). On the right, the FDG-PET image of the same patient showing right temporal hypometabolism involving the temporal pole and mesial regions.


(B) Coronal FLAIR MRI of patient 4 (on the left) showing a temporo-mesial lesion that on T1 imaging (on the right) present a clear gadolinium enhancement suggesting a tumoral origin.


(A) A 120-s view of a right temporal lobe seizure of patient 4. The apnea was the first ictal sign. The ictal EEG is characterized by a slow rhythmic theta activity on right temporal regions. (A,B) Red arrows point at the beginning of apnea on thoraco-abdominal polygraphic channel. Note the ictal marked increase in heart rate. Red channel: ECG; blue channel: respirogram.


(B) Apnea started 15 s before first EEG changes. (A,B) Red arrows point at the beginning of apnea on thoraco-abdominal polygraphic channel. Note the ictal marked increase in heart rate. Red channel: ECG; blue channel: respirogram.


Magnetic resonance imaging of brain showed hypersignal intensity at bilateral medial thalamus on diffusion weighted imaging.


Fluid attenuated inversion recovery.


T2Weighted. Series.


Magnetic resonance imaging of brain showed hypersignal intensity at central pons on diffusion weighted imaging.


The course of the patient's memory dysfunction using the CERAD (z-values). TMT A/. Trailmaking tests A/. T1, before plasmapharesis; t2, 1 month after plasmapheresis; t3, nearly 6 months after plasmapheresis.


FDG-PET showing mild-to-moderate medial and superior dorsolateral frontal hypometabolism before and normalization after plasmapheresis. The upper and lower row images show the transaxial fluorodeoxyglucose positron emission tomography (FDG-PET) images and the 3D surface projections of the regions with decreased FDG uptake (color-coded Z-score and compared with age-matched healthy controls, respectively).


Unified Parkinson's Disease Rating Scale III evaluation before, during, and after therapy with deferiprone.


(A) T1-weighted axial image shows linear hypointensity at the level of cerebral peduncles in the substantia nigra within a mild area of hyperintensity.


At the same level in the images (B,C), both T2-weighted, a striking hypointensity can be seen in both substantiae nigrae.


A more cranial axial T2-weighted section shows a marked bilateral hypointensity of the globus pallidus (D,E).


Interictal EEG from Case 4 when JME developed. Awake record at 12 years 7 months old, on no medication. Generalized polyspikes-and-waves are apparent.


Brain MRI scan of a patient with an intragenic tandem duplication in the PAFAH1B1 gene. Axial T2-weighted brain image indicating the occurrence of predominant agyria in the posterior regions, and pachygyria in the anterior regions. The patient was diagnosed with grade 3 lissencephaly.


Brain magnetic resonance images of the patient. A; Marked multiple chronic ischemic lesions in the periventricular white matter (white arrows) and subcortical deep white matter (black arrows) on T2-weighted imaging.


Brain magnetic resonance images of the patient. B; Small, restricted diffusion in the left hippocampus (white arrow) on diffusion-weighted imaging.


(a) Cerebral blood volume scan from a CT perfusion study obtained at the time of admission. A small area of volume deficit is visible in the left PCA territory.


(b) Time-to-peak map from the CT perfusion study showing a large area of reduced perfusion in the left PCA, consistent with salvageable penumbra.


(c) CT angiogram of the common carotid artery. Proximal occlusion consistent with a dissection flap is seen (arrow).


(d) CT angiogram of cerebral vessels showing an early filling defect in the terminal ICA (arrow).


(a) CT scan of the head on hospital day 1, after left pupillary dilation was first observed. Hypodensity in the left PCA territory is seen; no effacement of cisterns was evident.


(b) Axial T2 fluid-attenuated inversion recovery (FLAIR) magnetic resonance image on hospital day 5 showing evolving left PCA infarct.


(c) Coronal T2 magnetic resonance image of head. A left-sided hyperintensity is visible in the retroglobar area (arrow), consistent with congestion of the superior ophthalmic vein.


Painless swelling on the left side of face.


Magnetic resonance imaging brain and carotid angiograph.


Orthopantomography showing a multilocular mixed radiolucent-radiopaque lesion.


Histology with low-grade chronic and floride tubulointerstitial nephritis. Diffuse interstitial infiltrates (arrow). Acute tubulus damage is light to moderate and potentially reversible. * tubular lumen.


Timeline showing the chronological order of symptoms, diagnoses, and treatment. BGA, blood gas analysis; MRI, magnetic resonance imaging.


Preoperative and postoperative visual field examinations of the patient. (a) The arrowed tissue was the appendix.


Preoperative and postoperative visual field examinations of the patient. (b) The arrowed tissue was an enlarged mesenteric lymph node in the ileocecal region.


Preoperative and postoperative visual field examinations of the patient. (c) The arrowed tissue was another enlarged lymph node which was not removed.


Brain MRI (T1-weighted images) showing a severe atrophy of the cerebellum in the sagittal plane (A). The arrows point to the atrophic vermis, whereas the morphology of the brainstem appears normal (arrowhead).


A severe atrophy of the cerebellar cortex is observed in the frontal plane [arrows in panel (B)]. Note that the dentate nuclei are clearly identified on both sides (arrowheads).


Striatum, thalamus, subcortical white matter, and cerebral cortex appear morphologically normal on both sides [axial image in panel (C).


Bedside X-ray chest showing bilateral fluffy opacities suggestive of pulmonary edema.


Post EVD non contrast CT brain showing left-sided cerebellar infarct with a mass effect pushing the brainstem to the right, chinking of fourth ventricle and hydrocephalus. Also seen is air within the ventricle following the EVD.


Overview of patient's chromosome 1 deletion. Chromosome 1 ideogram displaying our patient's 6.9 Mb deletion and the deletion observed in the patient described by Gupta et al. . The OMIM genes common to both deletions include COX20 (FAM36A), HNRNPU, HNRNPU-AS1, EFCAB2, KIP26B, and SMYD3.


Representative MRI images. (A) T2-weighted axial brain MRI done of this patient at 3 days of age.


Representative MRI images. (B) T2-weighted axial brain MRI done on day 2 of the hospital admission showing diffuse increased T2 signal intensity throughout the left hemispheric gray matter.


Representative MRI images. (C) Diffusion-weighted axial brain MRI done on day 2 of the hospital admission showing diffuse left hemispheric cerebral edema.


Midline T2W image showing intrasphenoidal meningo-encephalocel comprised of extension of the infundibular portion of the floor of the 3rd. ventricle and remnants of the pituitary gland herniating into the sphenoid sinus through a defect in the floor of the sella tursica.


T1W image showing CSF existing within the sella, and ,extending into the sphenoid sinus through a possible defect located in the left lateral side of the sinus cavity.


Medical image.


Contrast enhanced T1W images in horizontal, and ,coronal views showing herniation of the glial tissue along with the arachnoidal pouch through the Sternberg's canal (white arrow).


T2W images in horizontal, coronal, and ,sagittal views showing empty sella containing CSF, and ,CSF containing cyst within the sella.


White arrow denoting extension of the glial tissue into the sella via the Sternberg's canal.


Metrizamide CT cisternography in sagittal and coronal views showing CSF containing cavity both within the sella and the sphenoid cell.


Multiplan reconstructions of 3D time-of-flight MRA images in transverse plane at the superior collicular level. The first MR examination.


Multiplan reconstructions of 3D time-of-flight MRA images in transverse plane at the superior collicular level. Follow-up 2 years later. . Note: Development of midbrain atrophy is seen on the follow-up image, with reduction of AP midbrain diameter. . Abbreviations: AP, anteroposterior; MR, magnetic resonance; MRA, magnetic resonance angiography.


Sagittal spin-echo T1-weighted magnetic resonance images on follow-up examination show mild midbrain atrophy with "hummingbird" sign (arrow). . Note: Development of generalized brain atrophy is also seen.


Transverse turbo spin-echo T2-weighted images on follow-up examination. At the basal ganglia.


At the pontine level. . Note: Minor bilateral putaminal hypointensity and generalized brain atrophy are seen.


Audiological assessment: auditory brainstem responses (ABR) were recorded using TDH-39 headphones and monaurally presented alternating polarity click stimuli of 100 mus duration, 11.33 Hz repetition rate and intensity of 90 dB nHL (normalized hearing level). The electrodes were mounted on center forehead (common); A1 left mastoid (active), A2 right mastoid (active) and high center forehead (reference). The responses were compared with our institutional normative values (provided) and demonstrated reproducible right waves I-V of degraded morphology, yet normal amplitude and latency and absent left responses at baseline.


Audiological assessment: auditory brainstem responses (ABR) were recorded using TDH-39 headphones and monaurally presented alternating polarity click stimuli of 100 mus duration, 11.33 Hz repetition rate and intensity of 90 dB nHL (normalized hearing level). The electrodes were mounted on center forehead (common); A1 left mastoid (active), A2 right mastoid (active) and high center forehead (reference). And at a 6-month interval , with additional findings of poorly reproducible right waves I-V and poor wave I morphology at a 6-month interval Due to retrospective nature of the case report, it was impossible to separate the recordings into condensation and rarefaction buffers and to comment on the cochlear microphonic potentials.


Peripheral vestibular assessment:. Video head impulse Test (vHIT), demonstrated reduced gain in all six canals, more marked in the right anterior (asymmetry 17%), and ,both posterior canals.


Ocular vestibular evoked myogenic potentials (oVEMP) were not detected on the left.


Cervical vestibular evoked myogenic potentials (cVEMP) were compared to our normative values and were within normal limits. Tone burst stimuli of 500 Hz was used for both oVEMP and cVEMP, of alternating polarity (specific to the unit's equipment) and 2:1:2 cycle. The scale was 5 ms per division (oVEMP) and 3 ms per division (cVEMP). For oVEMP, the electrodes were placed on forehead (common), A1 centrally below the eye; REF (reference) electrode on the cheek 1 cm below (but not touching) A1. For cVEMP, the electrodes were placed on forehead (common), sternoclavicular joint (inverting/negative), sternocleidomastoid muscle belly (non-inverting/positive).


Gadolinium-enhanced T1 MRI shows left medullary enhancement (arrow.


As extensive nodular circumferential leptomeningeal enhancement (arrows.


Findings correlate with axial T2 FLAIR images also showing lateral brainstem hyperintensity (arrows.


As well as a patchy involvement of the bilateral midbrain (arrows,. And the pituitary infundibulum (arrowhead.


Intraoperative photograph shows the direct swab of a mycotic-appearing mass which resulted in negative smear and culture. Also seen are a number of small colonies (arrowheads), one of which resulted in the positive identification of blastomyces dermatitidis on culture.


T1-weighted gadolinium contrast-enhanced magnetic resonance imaging images of the brain, orbit, and paranasal sinuses showing (a) enhancing soft tissue involving the left ethmoid sinus (red arrow) with its extension into the left orbit involving medial and inferior recti muscles (green arrow), surrounding the optic nerve (yellow arrow) and the left frontal lobe abscess (blue arrow).


(b) Axial image showing enhancing soft tissue involving the orbital apex and infiltrating into the left cavernous sinus, causing its thrombosis (blue arrow).


(c) Biopsy specimen showed growth of Rhizopus on lactophenol cotton blue mount.


(A) Preoperative T1-weighted image (WI) showed a low signal, while fluid-attenuated inversion recovery (FLAIR) scanning showed a high signal and enhanced scanning revealed no signals. An arachnoid cyst was visible in the left temporal region.


(B) At the six-month postoperative review there was no tumor recurrence.


Intraoperative electroencephalogram EEG and deep cortical electroencephalogram (ECoG) monitoring.


Computed tomography of neck; lymphadenomegalies in various dimensions.


Karyorrhectic debris and cellular infiltration of histiocytes and immunoblasts in the absence of polymorphonuclear leukocytes (hematoxylin and eosin stain x200).


Histiocytes and activated B-cells (hematoxylin and eosin stain x200).


Sagittal.


Axial views of a lumbar magnetic resonance imaging (MRI) revealed multilevel lumbar spondylosis and evidence of clumping (arrows) and posterior displacement of nerve roots of the cauda equina within the thecal sac suggesting arachnoiditis.


Demonstrating the four generations of affected members of the family pedigree (y: Years).


Left eye corneal photograph of Case 1. Opacities of granular corneal dystrophy were seen in the corneal stroma.


(A) Subclinical keratoconus findings in the right eye topograph of Case 1 (mother of the proband).


(B) Subclinical keratoconus findings in the left eye topograph of Case 1 (mother of the proband).


Right eye photograph of the proband (Case 4). Sharply demarcated, grayish-white, non-coalescent, star-shaped opacities localized only to the stroma.


(A) Keratoconus findings in the right eye topograph of the proband (Case 4).


(B) Keratoconus findings in the left eye topograph of the proband (Case 4).


Left eye corneal photograph of Case 7. Opacities of granular corneal dystrophy were seen in the corneal stroma.


(A) Subclinical keratoconus findings in the right eye topograph of Case 7 (daughter of the proband).


(B) Subclinical keratoconus findings in the left eye of Case 7 (daughter of the proband).


Axial slice on a T1-weighted image after gadolinium-diethylenetriaminepentacetate administration focusing on the cavernous sinus.


Jugular fossa.


Coronal section of the jugular vein level There is nonenhancement in the right cavernous and sigmoid venous sinus, contiguous with the jugular vein, compatible with sphenoiditis-resultant right cavernous sinus thrombosis and deep venous thrombosis of the right cerebrum and neck.


T2-weighted image axial section at the mid sphenoid level (d). There is mucus in the right sphenoid sinus (white arrow), right mastoiditis, and a bright signal in the right sigmoid sinus and jugular fossa indicating nonflow void in the venous sinus and jugular vein.


Illustration of the three-dimensional anatomical location of the electrodes (patient 1). (A) Frontal view of the electrodes (in yellow) with the different contacts (white) and three-dimensional view of STN (in blue), and SNr (in purple). This representation is superimposed on the coronal T2 preoperative MRI of patient 1 to illustrate the location of the STN and SNr. Each electrode had four contacts with the length of 1.5 mm separated by 0.5 mm intervals and numbered from 0 to 3 in the right hemisphere. Contact 0 is the deepest contact (green).


Illustration of the three-dimensional anatomical location of the electrodes (patient 1). (B) Frontal view of the right STN and SNr. In (B-D), the STN and SN were represented in transparency to show the location of the contacts and visualize the "laughter" contact 0 localized within the ventral and medial STN. LV, lateral ventricle; TH, thalamus; TL, temporal lobe.


Illustration of the three-dimensional anatomical location of the electrodes (patient 1). (C) Anterolateral view of the right STN and SNr. In (B-D), the STN and SN were represented in transparency to show the location of the contacts and visualize the "laughter" contact 0 localized within the ventral and medial STN. LV, lateral ventricle; TH, thalamus; TL, temporal lobe.


Illustration of the three-dimensional anatomical location of the electrodes (patient 1). (D) Superior view of right STN and SNr. In (B-D), the STN and SN were represented in transparency to show the location of the contacts and visualize the "laughter" contact 0 localized within the ventral and medial STN. LV, lateral ventricle; TH, thalamus; TL, temporal lobe.


Illustration of the three-dimensional anatomical location of the electrodes (patient 2). (A) Frontal view of the electrodes (in yellow) with the different contacts (white) and three-dimensional view of the STN (in blue), and SNr (in purple). This representation is superimposed on the coronal T2 preoperative MRI of patient 2 to illustrate the location of the STN and SNr. Each electrode had four contacts with the length of 1.5 mm separated by 0.5 mm intervals and numbered from 0 to 3 in the left hemisphere. Contact 0 is the deepest contact (green).


Illustration of the three-dimensional anatomical location of the electrodes (patient 2). (B) Frontal view of the left STN and SNr. In (B-D), the STN and SNr were represented in transparency to show the location of the contacts and visualize the "laughter" contact 0 localized within the SNr. LV, lateral ventricle; TH, thalamus; TL, temporal lobe.


Illustration of the three-dimensional anatomical location of the electrodes (patient 2). (C) Anterolateral view of the left STN and SNr. In (B-D), the STN and SNr were represented in transparency to show the location of the contacts and visualize the "laughter" contact 0 localized within the SNr. LV, lateral ventricle; TH, thalamus; TL, temporal lobe.


Illustration of the three-dimensional anatomical location of the electrodes (patient 2). (D) Superior view of the left STN and SNr. In (B-D), the STN and SNr were represented in transparency to show the location of the contacts and visualize the "laughter" contact 0 localized within the SNr. LV, lateral ventricle; TH, thalamus; TL, temporal lobe.


White arrows in coronal fluid-attenuated inversion recovery (FLAIR) image.


Axial T2-weighted image. Show right temporal cavernous malformation (CM). Cortical atrophy and ventriculomegaly of the brain are also seen.


(c,d) FLAIR images show total removal of the CM by a transcortical approach.


(a) Intraoperatively, we place the four-contact subdural strip on the parahippocampal area.


(b) Frequent, very small-amplitude spikes and spike-waves are seen, mostly from contact 1 (arrows) before removal of the cavernous malformation (CM).


Angio-CT showing the occlusion of the right internal carotid artery (arrow). The "flute beak" aspect is suggestive of carotid artery dissection.


A; Brain MRI scan (coronal FLAIR sequence) shows a high signal intensity area on the right hemisphere, including also the tongue area of the primary motor cortex (arrow).


B; Brain MRI scan (axial diffusion-weighted image) shows restricted diffusion with high signal intensity in the same area (arrow).


T2 FLAIR axial images show a large anterior cranial fossa mass (bifrontal) with surrounding vasogenic edema with a mass effect upon the overlying medial frontal lobes bilaterally with downward compression and mass effect on the caudate (A, B).


A large, avidly enhancing midline anterior cranial fossa mass with a maximum size of 5.7x4.2x5.6 cm is seen on axial and coronal sections (C, D).


(a) Catheter angiography from PBD 29 showing severe supraclinoid left ICA vasospasm.


(b) Balloon inflation angioplasty of vasospasm segment.


(c) Improvement of vasospasm s/p balloon angioplasty of left ICA.


Case #2 MRI showing typical appearance of cavernoma in the 3rd ventricle. [ Sagittal T2.


Case #2 MRI showing typical appearance of cavernoma in the 3rd ventricle. Axial T2.


Case #2 MRI showing typical appearance of cavernoma in the 3rd ventricle. Sagittal T1 with contrast.


Case #1 (a) Initial Catheter angiography AP projection of left ICA.


(b) Initial Catheter angiography lateral projection of left ICA.


Case #2 (a) Catheter angiography from PBD 22 showing a dominant left A1 segment with vasospasm.


(b) Improvement of vasospasm s/p balloon angioplasty of left A1 segment.


Hyperintense signal in T2-weighted images in the central pons suggestive of central pontine myelinolysis.


T2-weighted magnetic resonance imaging (3 Tesla) with a cortical solid-cystic mass lesion in the medial and superior temporal gyrus of the anterior left temporal lobe.


T1-weighted magnetic resonance imaging + gadolinium (3 Tesla) shows a contrast-enhancing nodule in the cystic lesion together with calcification or hemosiderin products in the cyst wall.


The dysembryoplastic neuroepithelial tumor component is composed of myxoid (arrow) and multicystic (*) areas.


Note the dysplastic neurons (circles) and perivascular lymphoid infiltrates (arrow) in the ganglioglioma component.


Magnetic resonance imaging findings in the patient: regions of cortical dysplasia (arrows); nodules of heterotopic matter (interrupted arrows).


Postoperative MRI. Volume of surgical intervention (described in the text of the article).


MRI findings at onset of neurological disturbances. . Notes: Brain MRI images from June 2015 showed. T2 hyperintense lesions in left temporal lobe, and ,brainstem (circles).


MRI findings at onset of neurological disturbances. T1 hyperintense lesions in basal ganglia (thin arrows), known as calcifications.


MRI findings at onset of neurological disturbances. Leptomeningeal contrast enhancement at midbrain-pons junction (arrow). . Abbreviation: MRI, magnetic resonance imaging.


Preoperative nonenhanced computed tomography (CT) scan demonstrating a paramedian right occipital well-circumscribed, inhomogeneously hyperdense, lesion with sharply demarcated erosion of both tables of the skull in axial.


Sagittal view.


Preoperative 3D CT scan showing the bone defect.


Preoperative contrast-enhanced T1-weighted magnetic resonance imaging in sagittal.


Coronal.


View demonstrating the hypointense lesion obstructing the confluence of the superior longitudinal and transverse sinuses, confirmed by MRV.


Intraoperative images: the lesion, exposed through a longitudinal median parieto-occipital incision and skeletonization, bulged from the eroded outer bone table, harvested by a fibrous capsule.


Under microscopic magnification, the capsule was incised evidentiating the cyst content, which had a variegated appearance with some dark areas suggestive for previous subacute intralesional hemorrhage and other areas with the classical pearly aspect of epidermoid cysts.


The lesion was removed piecemeal.


After evacuation of the cystic contents, the fibrous capsule was separated from the borders of the bone and dissected from the intact dura mater.


The sclerotic and thickened bony borders were drilled away, highlighting blood flow restoration in the confluence of sinuses.


The bony defect was reconstructed by acrylic resin cranioplasty.


Postoperative CT scan showing complete removal of the lesion and reconstruction of the bone defect by acrylic resin material cranioplasty in axial.


In 3D CT scan.


Postoperative contrast-enhanced T1-weighted MRI in sagittal.


View, demonstrating the complete exeresis of the lesion with resolution of venous sinuses obstruction, as confirmed by MRV.


Photomicrograph of the specimen shows a cystic formation with a fibrous wall, outlined by squamous epithelium and containing keratinic material arranged in lamellae. HE, x100), foreign body granulations with moderate vascularity.


Intralesional hemorrhage can be observed at higher magnification. HE, x200; foreign body granulation in the circle).


The preoperative December 2014 magnetic resonance imaging parasagittal study documented a massive central-left sided L2-L3 lumbar disk herniation resulting in severe thecal sac and left L2 and L3 foraminal/lateral recess root compression respectively. Notably the right mild/moderate sequestrated L4-L5 disc was asymptomatic.


The preoperative December 2014 magnetic resonance imaging axial study documented a massive central-left sided L2-L3 disk herniation resulting in severe thecal sac and left L2 and L3 foraminal/lateral recess root compression respectively.


The immediate preoperative April 2015 parasagittal magnetic resonance imaging study documented the same massive central-left sided L2-L3 lumbar disk herniation seen on the magnetic resonance imaging from December of 2014. It still resulted in severe central-left sided thecal sac and left L2 and L3 foraminal/lateral recess root compression respectively.


The immediate preoperative April 2015 axial magnetic resonance imaging study documented the same massive central-left sided L2-L3 lumbar disk herniation seen on the magnetic resonance imaging from December of 2014. It still resulted in severe central-left sided thecal sac and left L2 and L3 foraminal/lateral root compression respectively.


A 68 old man patient with unilateral leg motor weakness 9 days after the onset of herpes zoster in his left L4 dermatome.


Psoas compartment block was performed with local anesthetic and triamcinolone 40 mg.


HMRS study of brain. (a) T2 sequence showed hyperintensities in both basal ganglia.


HMRS study of brain. (b, c) Showed concentrations of glutamate by multivoxel imaging (TR 4200 TE 103.8). The inner green square identified a single voxel.


HMRS study of brain. (d) Magnetic resonance spectroscopy curve showed elevated glutamate levels (arrowhead).


Radon transformation of the glutamate concentrations in the multi-voxel regions of interest in Figure 1. This revealed a bilaterally symmetric involvement of basal ganglia.


(a) Some neuronal dysplastic elements (arrows) between glial neoplastic cells in a H and E, x40. (b) Microcalcifications in a H and E, x10. (c and d) Neuronal component was immunoreactive for neurofilament protein (c) and NeuN (d). (e) Glial component was positive for GFAP. (f) The Ki-67 (MIB-1) proliferation index was 1.


MRI features. FLAIR images ,. Evidence of bilateral involvement of the entire temporal lobe (cortex and white matter), from the temporal pole up to the hippocampus, with signs of laminar necrosis The edema is significantly more prominent 3 days after SE onset.


MRI features. FLAIR images ,. The edema is significantly more prominent 3 days after SE onset. F Cortical involvement outside the temporal lobe.


MRI features. FLAIR images ,. C, f The left pulvinar shows hyperintensity, which increased 1 month later, with involvement of the contralateral nucleus (black arrowheads).


MRI features. D-f FLAIR images 1 month later. Evidence of bilateral involvement of the entire temporal lobe (cortex and white matter), from the temporal pole up to the hippocampus, with signs of laminar necrosis.


MRI features. , compared with 1 month later. F Cortical involvement outside the temporal lobe.


MRI features. White arrows), in particular of the left precuneus (black arrows), is far more evident in the second MRI.


MRI features. Contrast-enhanced T1-weighted image ,. White arrowheads), is easily recognizable after gadolinium.


MRI features. Diffusion-weighted image. The high signal in the apparent diffusion coefficient map demonstrates it is vasogenic (h, i).


MRI features. Apparent diffusion coefficient map. Performed 3 days after SE onset. The high signal in the apparent diffusion coefficient map demonstrates it is vasogenic (h, i).


Polymerase chain reaction-direct sequencing. . Notes: Chromatogram showing the germline mutation in the case included in the present study. Arrow indicates the missense variant c. C952T>C (p. C318R) in exon 5 of GATA3.


Pedigree. . Notes: Arrow indicates the proband. Males are represented by squares and females by circles. The filled circle indicates the affected female. A diagonal line through a square or circle indicates a deceased person.


Age of nonsmoking patients diagnosed with lung squamous cell carcinoma at Kobe University Hospital from 2011 to 2017. . Note: Of 263 patients diagnosed with lung squamous cell carcinoma at Kobe University Hospital, 16 were nonsmokers.


Chest X-ray. . Note: Left pleural effusion, pleural thickening, and scoliosis were detected. . Abbreviations: R, right; P-A, posteroanterior.


Cranial magnetic resonance imaging (MRI) of a patient with anti-CV2 autoimmune encephalitis. (A-C) T2WI axial images.


Cranial magnetic resonance imaging (MRI) of a patient with anti-CV2 autoimmune encephalitis. (D-F) FLAIR axial images.


Cranial magnetic resonance imaging (MRI) of a patient with anti-CV2 autoimmune encephalitis. (G) FLAIR coronal scan.


Cranial magnetic resonance imaging (MRI) of a patient with anti-CV2 autoimmune encephalitis. (H) Enhanced MRI.


Cranial magnetic resonance imaging (MRI) of a patient with anti-CV2 autoimmune encephalitis. (I) Diffusion-weighted MRI. The arrows indicate abnormal signals in the white matter area of the bilateral lateral ventricles and bilateral internal capsules. No significant enhancement was observed.


Graphic of respiratory chain enzyme activities in the muscle biopsy of the two patients described in Case 1 and Case 2, as compared to normal controls. There is a mild reduction in complex I + III.


(A) Sanger sequencing of DNA from skeletal muscle from case 1 showing homoplasmic m.13345G>A mutation.


(B) MtSNP Database mtSAP alignment of 11 mammals showing that the residue Ala337 and the adjacent aminoacids are very well-conserved (all 61 mammals in the database have alanine at this position).


(C) A human cryo-EM structure of complex I (PDB:5XTD), with ND5 highlighted in blue. Frontal (right) and lateral (left) view. Ala337 is buried inside the protein structure and its position is indicated with black/white circles.


(D) Pathogenicity prediction score of different tools. PROVEAN, Rhapsody, PolyPhen-2, EVmutation and SIFT.


(E) A337 is shown in the above structure with PyMOL version 2.3.4. (Schrodinger, LLC. ) with its closest interactors Leu457 and Val96. Dotted lines mark the quantified distances between the side chain atoms. The color codes for hydrophobicity, whereby red is strongly hydrophobic and white is strongly hydrophilic. This indicates that Ala337 is in a strongly hydrophobic environment. The plugin color_h was used on the basis of a previously established hydrophobicity grading.


MRI showing transverse view of the brain. Hemimegalencephaly of the right side of the brain can be easily identified. A cephalhematoma can also be seen on the right.


EEG recording shows predominantly right-sided spike, polyspikes, and wave discharges.


T1-weighted MRI after gadolinium administration. A fistula (black arrows) from a sigmoid diverticulitis (white arrows) to a presacrally localized abscess (white asterisks) is shown.


T1-weighted MRI after gadolinium administration. Shown is the presacrally localized abscess (black asterisk) with complete staining of the border. There is diffuse staining of the sacral bone (plus symbols) without involvement of the intervertebral discs, which is suggestive of osteomyelitis. Furthermore, there is diffuse meningeal staining (white arrows) of the conus medullaris and cauda equina. A rectal device (white asterisk) is in situ.


CT scan of the abdomen showing multiple intraspinal fluid and air collections (white arrows) and a presacral abscess (black asterisk).


Non-contrast brain CT 24 h after symptom onset.


A supine hemilithotomy position in authors' institute is routinely maintained with a boot immobilization and patients' knee flexed over 90. instead of a calf supporter or a suspension knee strap, making the posture of the non-operated limb similar to squatting.


Intraoral photograph bitten tongue and autoextraction of 73, 72, 71, 81, and 82.


Photograph of maxillary and mandibular casts showing primary teeth.


(a) Extraoral photograph of bite guard.


(b) Intraoral photograph showing modified bite guard.


(a) Intraoral photograph showing erupted 53, 63 and avulsed 52.


(b) Twelve months intraoral photograph.


Brain MRI with and without contrast performed on arrival to our hospital. A; Axial FLAIR images show multiple, extensive and bilateral areas of abnormal signal intensity in the white matter.


Brain MRI with and without contrast performed on arrival to our hospital. B; Axial T1 post-gadolinium images show numerous large, confluent, and patchy ring-enhancing lesions.


Ascending colon mass-related with Takotsubo Cardiomyopathy. A) Large malignant-looking mass of the ascending colon on colonoscopy.


Ascending colon mass-related with Takotsubo Cardiomyopathy. B) Large focal density within the proximal ascending colon on CT abdomen and pelvis with contrast.


Rhizopus arrhizus associated lethal midline granuloma/rhino-orbital cerebral mycosis. " Destruction of the sinus, nose, face, palate, and orbit; defects of the left sided external nose, soft tissues, and the front wall of the maxillary sinus; and yellow pus was seen within the left maxillary sinus. (A,B) Show patient onset before and after debridement with biopsies.


Rhizopus arrhizus associated lethal midline granuloma/rhino-orbital cerebral mycosis. " Destruction of the sinus, nose, face, palate, and orbit; defects of the left sided external nose, soft tissues, and the front wall of the maxillary sinus; and yellow pus was seen within the left maxillary sinus. (C) Reveals the patches dark discoloration (short arrow), light discoloration (medium arrow), and perforation (long arrow) of the hard palate that represent inflammation, ischemia, and necrosis, respectively.


Rhizopus arrhizus associated lethal midline granuloma/rhino-orbital cerebral mycosis. " Destruction of the sinus, nose, face, palate, and orbit; defects of the left sided external nose, soft tissues, and the front wall of the maxillary sinus; and yellow pus was seen within the left maxillary sinus. (D) Shows widespread destruction in the left side of the palate (arrow).


Rhizopus arrhizus associated lethal midline granuloma/rhino-orbital cerebral mycosis. " Destruction of the sinus, nose, face, palate, and orbit; defects of the left sided external nose, soft tissues, and the front wall of the maxillary sinus; and yellow pus was seen within the left maxillary sinus. (E) Reveals pale gray wool-like mycelium grown on the necrotic nose tissue (arrow).


Rhizopus arrhizus associated lethal midline granuloma/rhino-orbital cerebral mycosis. " Destruction of the sinus, nose, face, palate, and orbit; defects of the left sided external nose, soft tissues, and the front wall of the maxillary sinus; and yellow pus was seen within the left maxillary sinus. (F) Shows complete remission with scars 4 months after antifungal therapy.


18F-FDG PET/CT scan of a 51-year-old Caucasian female with symptoms of central pontine myelinolysis. Scan is showing localized FDG uptake in the pons, with normal and symmetrical activity in the rest of the brain.


MR-scan T2-weighted image showing a hyperintensive region in the central part of pons. In addition, hyperintensive region was present symmetrically in the caput nucleus caudatus, putamen, and the lateral part of thalamus.


(99m)Tc-TRODAT-1 SPECT showed low fixation of the dopamine transporter in the striated nuclei bilaterally.


Three generation pedigree with a total of 10 affected family members. Colored shapes indicate affected individuals based on testing, history or both. Patient is indicated by arrow.


Head CT without contrast demonstrating hemorrhage in the left occipital pole.


FIESTA MRI revealing the course of the right third nerve as it meets the arachnoid cyst.


Intraoperative images demonstrating a frontotemporal approach to the middle fossa. Internal carotid artery and optic nerve (a).


Intraoperative images demonstrating a frontotemporal approach to the middle fossa. Arachnoid cyst bounded inferiorly by the internal carotid artery (b).


3-month postoperative MRI demonstrating resolution of T2 FLAIR signal abnormality consistent with complete surgical resection of the right parasellar arachnoid cyst.


Bone window.


Noncontrast.


Postcontrast. CT brain.


T1 FLAIR pre.


Postgadolinium.


Medical image.


Intraoperative photograph of the tumour breaching dura.


The tumour had extended through the skull.


Hematoxylin, and ,eosin stain (high-power view).


Hematoxylin and eosin stain (low-power view).


PAS positive.


FLI-1 positive.


MIC-2 positive.


Ki67 30% positive by visual estimation.


Generalized atrophy and elbow contracture was detected in our case.


Perseveration in Luria alternating series test (drawing of the patient's 35-year-old brother).


T2-weighted MRI of the patient demonstrating prominent frontotemporal atrophy.


ENG in the patient with pseudo-BPPV (patient 2). UW = 0% < 25% revealed normal vestibular functions.


A; Axial T2 brain MRI showing a wedge-shaped segmental hyperintense lesion in the left paramedian pons.


B; Axial T1 brain MRI showing a wedge-shaped segmental hypointense lesion in the left paramedian pontine region.


C; Sagittal T2 brain MRI showing a wedge-shaped segmental hyperintense lesion extending from the basis pontis to the tegmentum pontis, indicating subacute infarction.


A; Both the common carotid artery and the internal and external carotid arteries were normal and patent, and the circle of Willis and its major branches were well demonstrated.


B; Both vertebral arteries were well visualized and normal.


A; Axial T2 brain MRI showing a segmental hyperintense lesion in the left paramedian pons.


B; Sagittal T2 brain MRI showing a segmental hyperintense lesion extending from the ventral to the dorsal pons, indicating chronic paramedian pontine perforator infarction.


Multimodal imaging of the right eye at the first presentation. Infrared reflectance, and ,optical coherence tomography (OCT) of the macula.


Multimodal imaging of the right eye at the first presentation. Fundus photograph show a large pigment epithelial detachment (PED) at the macula with surrounding small and intermediate drusen. There was a small satellite PED which coalesced into a single large PED 6 days later.


Multimodal imaging of the right eye at the first presentation. (c) Enhanced-depth imaging OCT scan through the foveal center shows a large PED with serous subretinal fluid at its apex.


Evolution of the pigment epithelial detachment (PED) and subretinal fluid (SRF) of the patient. (a) Baseline enhance-depth imaging optical coherence tomography.


Evolution of the pigment epithelial detachment (PED) and subretinal fluid (SRF) of the patient. (b) 1 month after the first intravitreal injection (bevacizumab), the PED was partially resolved, but SRF increased.


Evolution of the pigment epithelial detachment (PED) and subretinal fluid (SRF) of the patient. (c) 1 month after the second injection (aflibercept), partial resolution of both PED and SRF is shown.


Evolution of the pigment epithelial detachment (PED) and subretinal fluid (SRF) of the patient. (d) 1 month after the third injection (aflibercept), both PED and SRF were completely resolved. The patient's visual acuity improved to 20/30.


Multimodal imaging of the right eye at the first relapse of the pigment epithelial detachment (PED). Fundus photograph.


Multimodal imaging of the right eye at the first relapse of the pigment epithelial detachment (PED). Infrared image.


Multimodal imaging of the right eye at the first relapse of the pigment epithelial detachment (PED). Enhance-depth imaging optical coherence tomography show a relapse of shallow serous PED without subretinal fluid.


Multimodal imaging of the right eye at the first relapse of the pigment epithelial detachment (PED). (d) Fundus fluorescein angiography demonstrates the pooling of the PED beginning at the inferotemporal area.


Multimodal imaging of the right eye at the first relapse of the pigment epithelial detachment (PED). (e) Indocyanine green angiography shows hypocyanescence blockage of the PED. No abnormal hypercyanescence plaque of choroidal neovascularization was identified. The choroidal hyperpermeability was less intense than at the baseline visit.


Enhanced-depth imaging optical coherence tomography shows relapses of the (PED). (a) 7 days after receiving 8 mg intravenous dexamethasone, the second relapse of the large PED is shown.


Enhanced-depth imaging optical coherence tomography shows relapses of the (PED). (b) After 1 month of observation, subretinal fluid (SRF) developed.


Enhanced-depth imaging optical coherence tomography shows relapses of the (PED). (c) After receiving the fifth intravitreal aflibercept injection, PED and SRF completely disappeared.


Enhanced-depth imaging optical coherence tomography shows relapses of the (PED). (d) 4 months after the last injection, a shallow PED recurred but was spontaneously resolved.


Enhanced-depth imaging optical coherence tomography shows relapses of the (PED). 7 months.


Enhanced-depth imaging optical coherence tomography shows relapses of the (PED). 11 months.


Enhanced-depth imaging optical coherence tomography shows relapses of the (PED). 22 months after the last injection, there were no further recurrences of the disease.


Pelvis X-rays showing left iliac wing reconstruction with tibial autografts and stabilization with screw-rod type osteosynthesis.


Bone window CT scan in sagittal view obtained at the time of admission showing no osteolytic changes of the calvarial bone.


Preoperative axial.


Coronal. Postgadolinium T1-weighted MRI images, showing a strongly enhancing right frontoparietal tumor with sagittal venous sinus invasion. Note the dural tail indicating a meningeal tumor.


Intraoperative photograph of the bone flap showing extradural lobulated tumor tissue tightly attached to the inner calvarial bone.


Hematoxylin and eosin-stained tumor specimen showing densely packed, small round cells with scanty clear cytoplasm and regular vesicular and hyper chromatic nuclei; magnification, x200.


Postoperative coronal CT scan showing near total tumor removal with craniotomy defect.


Axial T2 weighted magnetic resonance image (MRI) demonstrating lesion in the left hypoglossal foramen being T2 hyperintense with no gadolinium contrast enhancement.


Follow-up T2 weighted 3D magnetic resonance image (MRI) 12 months after initial presentation demonstrating compression at the level of the brainstem.


Post-operative (4 months) T2 flair axial magnetic resonance image (MRI) showing absence of lesion.


Agarose gel electrophoresis patterns of the PCR-based open reading frame typing (POT) using the Cica Geneus Pseudo POT KIT. PCR of reaction mixtures 1 and 2 was carried out for strain 1 and 2. Lane 1: strain 1 obtained from the patient's cerebrospinal fluid. Lane 2: strain 2 obtained from a cleaning sponge brush. The sizes of the bands in the M lane are 600, 500, 450, 400, 350, 300, 250, 200, 150, and 100 bp (from the top). PC, positive control; NC, negative control, used as an in-house ladder marker for detection PCR of Pseudomonas aeruginosa. Lines 1 and 2 showed the same band patterns, and the POT numbers of both strain 1 and 2 were the same values. The results from POT 1 and 2 values indicated these strains did not carry a metallo-beta-lactamase, such as IMP and VIM.


Hip and pelvis radiograph of the patient following his admittance. Standard hip and pelvis anteroposterior radiograph depicting the slippage of the left capital femoral epiphysis on the initial presentation of the patient at the accident and emergency department.


Magnetic resonance imaging scan of the patient following his admittance to our department. (a). Proton-density weighted image sequence-spectral attenuated inversion recovery coronal view.


Magnetic resonance imaging scan of the patient following his admittance to our department. (b). T1-weighted image sequence coronal view (T1W).


Magnetic resonance imaging scan of the patient following his admittance to our department. (c). MDIXON weighted sequence axial view. The slippage of the left capital femoral epiphysis seems to be milder following the application of a simple skin traction for a period of approximately 3 h.


Radiographs after the removal of the hip spicacast at 8 weeks following reduction. Hip and pelvis anteroposterior radiographs (a.


Radiographs after the removal of the hip spicacast at 8 weeks following reduction. Standard, b. Frogleg) after the removal of the hip spicacast at 8 weeks after the reduction of the slipped capital femoral epiphysis. The fracture has healed and the affected left epiphysis has the same size and shape as the contralateral. No signs of avascular necrosis or any other pathology can be identified.


Initial presentation of non-germinatous germ cell tumor causing obstructive hydrocephalus. Gadolinium-enhanced T1-weighted head MRI in the axial.


Initial presentation of non-germinatous germ cell tumor causing obstructive hydrocephalus. The FLAIR image in the axial plane (b) revealed some edema in the midbrain.


Initial presentation of non-germinatous germ cell tumor causing obstructive hydrocephalus. Coronal.


Initial presentation of non-germinatous germ cell tumor causing obstructive hydrocephalus. Sagittal. Axes performed at presentation showed a pineal region tumor compressing the tectum and causing obstructive hydrocephalus.


Rapid growth of non-germinatous germ cell tumor displacing the left substantia nigra and rostral-caudal fibers from midbrain. Gadolinium-enhanced T1-weighted head MRI in the axial.


Rapid growth of non-germinatous germ cell tumor displacing the left substantia nigra and rostral-caudal fibers from midbrain. Diffusion tensor imaging showed that the rostral-caudal fibers (blue color) from the midbrain were displaced anteriorly and laterally on the left and laterally on the right at the pineal.


Rapid growth of non-germinatous germ cell tumor displacing the left substantia nigra and rostral-caudal fibers from midbrain. The tumor extended caudally into the midbrain on the left exerted pressure medially and anteriorly (c).


Rapid growth of non-germinatous germ cell tumor displacing the left substantia nigra and rostral-caudal fibers from midbrain. Midbrain. Levels.


Rapid growth of non-germinatous germ cell tumor displacing the left substantia nigra and rostral-caudal fibers from midbrain. Coronal.


Rapid growth of non-germinatous germ cell tumor displacing the left substantia nigra and rostral-caudal fibers from midbrain. Sagittal. Axes performed before initiation of treatment showed a heterogeneously enhancing mass arising from the pineal region and measuring 34 x 28 x 23 mm. It caused 10 mm of midline shift and extended anteriorly into the left thalamus.


Second-look neurosurgical resection achieved additional cytoreduction. Gadolinium-enhanced T1-weighted head MRI obtained 1 day after surgery in the axial.


Second-look neurosurgical resection achieved additional cytoreduction. As well as T2-weighted image in the axial plane , demonstrated tumor cytoreduction and relief of compression on the tectum and the midbrain.


Second-look neurosurgical resection achieved additional cytoreduction. Coronal.


Second-look neurosurgical resection achieved additional cytoreduction. Sagittal. Axes.


A photograph of the needle placement in the mental foramen under ultrasound guidance (10-12 MHz linear transducer). (A) In the actual patient.


A photograph of the needle placement in the mental foramen under ultrasound guidance (10-12 MHz linear transducer). (B) Anterior view in a human skull model, which is empirically depicted. The transducer (white box) was moved like (1) for identifying the mental foramen and mental foramen was positioned at the end of transducer (2).


A photograph of the needle placement in the mental foramen under ultrasound guidance (10-12 MHz linear transducer). (C) An ultrasound image of the radiofrequency needle in the mental foramen (10-12 MHz linear transducer, long-axis in-plane technique). The cleft within the hyperechoic line indicates the mental foramen. The radiofrequency needle has passed through the mental foramen and is indicated with arrow heads.


Fluoroscopic images during pulsed radiofrequency treatment of the left mental nerve of the mandibular branch. (A) Anteroposterior view.


Fluoroscopic images during pulsed radiofrequency treatment of the left mental nerve of the mandibular branch. (B) Lateral view. Fluoroscopic images show that the needle was in the mental foramen. No vascular uptake of radio-contrast agent was observed.


The treatment procedures and corresponding leukocyte counts in the patient's cerebrospinal fluid. Monocytes were predominant among CSF white cells; they had a proportion of approximately between 60 and 90%. The CSF protein level was also elevated to 95.00-125.20 mg/dl. mNGS. P, metagenomic next-generation sequencing of cerebrospinal fluid detected Sarocladium strictum positive; Culture. P, Sarocladium strictum was positive in cerebrospinal fluid culture.


Bilateral hypointensities in T1 in the striatum, more prominent on the right side.


Rare small subcortical white matters hyperintensities on T2 (mainly periventricular and frontal) on Magnetic Resonance Imaging of the brain.


(C) Single Photon Emission Computerized Tomography of the Dopamine Transporter (DAT) with 123I-ioflupane showing a significant low uptake of DAT, mostly in the right striatum.


Embolia cutis medicamentosa. A reticulate, nonblasnching, nonindurated, coalesced areas of mottled erythemato-violaceous patches over the right superior gluteal distribution with extension to the lateral aspect of the thigh, and right lumbar paraspinal area, with satellite lesions measuring in its maximum diameter 23 cm x 18 cm.


Magnetic resonance imaging.


Magnetic resonance imaging. T2-weighted sagittal spine magnetic resonance imaging revealed a longitudinally extensive altered signal intensity from T7 level to conus level with. (T2-weighted axial magnetic resonance imaging at T10 level) centromedullary cord involvement.


Resection of the frontal/parietal/temporal mass. Post-operative MRI contrasted T1 and T2 scans showed resection of the meningiomas in the left frontal/parietal/temporal convexity with expected post-operative changes (red arrows in A,C, not shown in B). The occipital lobe mass was visible from the sagittal view (white arrow in A).


Latest imaging. Her most recent MRIs (16 months after her last surgery) show multiple enhancing extra-axial masses, stable compared to her immediate post-operative MRIs. Here is a stable 1.8 cm (superior-inferior) meningioma arising from the left posterior falx, adjacent to the prior resection cavity (red arrows in A-C). No recurrence observed.


Initial presentation of the patient.


CT scan on the horizontal plane; the arrows indicate the length to measure.


Partial improvement in ptosis and ophthalmoplegia after 2 weeks.


Complete recovery from ptosis and ophthalmoplegia after 10 weeks.


Electroencephalogram of the brain.


Magnetic resonance image scan of the brain.


(a) T1-weighted imaging with gadolinium enhancement at the initial presentation showing a large right sphenoidal tumor.


(b) T1-weighted imaging after Gamma Knife radiosurgery for recurrent tumor showing a residual tumor around the right anterior clinoid process.


(c) T1-weighted imaging during the occurrence of ocular neuromyotonia (ONM) showing a rapid growth of the posterior part of the tumor (arrow).


(d) Heavily T2-weighted imaging during the occurrence of ONM showing the right oculomotor nerve involvement (arrowhead).


(e) ONM symptom. The position of the eyes in the normal status (upper) and ONM (lower).


(a) An intraoperative view of the right oculomotor nerve after tumor removal (arrow).


(b) T1-weighted imaging with gadolinium enhancement after surgery showing that most of the tumor was removed.


(c) Eye movement after surgery showing a resolution of the right ONM with only a slight disturbance of the inward movement of the right eye.


Wide field fundus photograph shows marked swelling of optic nerves and a large chorioretinal coloboma in the right eye inferiorly.


Fundus photograph shows optic nerve swelling and a large chorioretinal coloboma in the right eye.


Pre-operative MRI brain demonstrating a heterogeneous, enhancing posterior fossa lesion with surrounding edema (blue arrow). 1a: Axial T2-weighted; 1b: Axial FLAIR; 1c: Axial T1-weighted pre-contrast; 1d: Axial T1-weighted post-contrast; 1e: Sagittal T1-weighted post-contrast; 1f: Coronal T1-weighted post-contrast.


RBANS part a, visual summary of the patient's performance. The patient's scores are plotted in the standard assessment chart showing low score across the board (grey area highlights the percentile rank below 1. Percentile) with relative strength in attention skills (forth column from the left). RBANS = Repeatable Battery for the Assessment of Neuropsychological Status. The columns numbered 1 to 5 represents the areas of assessment in RBANS part a. Columns from left to right: 1 = Immediate Memory; 2 = Visuospatial / Constructional; 3 = Language; 4 = Attention; 5 = Delayed Memory.


Sagittal FLAIR (a) showing (blue arrows) high signal affecting inferior olivary nuclei, and gliosis and malacia at the surgical bed with presumed damage to central tegmental tract and dentate nuclei.


Axial T2-weighted (b) showing high signal medulla bilaterally, at the level of the inferior olivary nuclei. Post-operative pseudomeningocoele is also evident.


Coronal T2-weighted (c) further highlights post-operative damage to cerebellum involving central tegmental tract and dentate nuclei with gliosis and hemosiderin deposition.


Language fMRI with word, noun-verb and stories paradigms was performed for language localization and lateralization. The underlying BOLD technique is susceptible to the artifact arising from the shunt. This demonstrated normal left hemispheric language activation in the frontal lobe, Broca's area, and the superior temporal gyrus, Wernicke's area.


Diffusion tensor imaging was performed. A fractional anisotropy map was obtained and tracts were manually seeded. There was some degradation secondary to the shunt, however overall tracts, especially the corticospinal tract, remain intact. Coronal.


Diffusion tensor imaging was performed. A fractional anisotropy map was obtained and tracts were manually seeded. There was some degradation secondary to the shunt, however overall tracts, especially the corticospinal tract, remain intact. Sagittal. Views.


Clinical, therapeutic, and radiological course. Abbreviations: CSF cerebrospinal fluid; d, days; EEG, electroencephalography; GE, gadolinium-enhancement; IVIG, intravenous immunoglobulin; JCV-PCR John Cunningham virus-polymerase chain reaction; MP, methylprednisolone; MRI, magnetic resonance imaging; NCSE, non-convulsive status epilepticus.


Neuroimaging. Brain CT in the postoperative course after the patient developed nausea, vomiting, and generalized weakness. The red arrows point at revealing wide-spread bilateral hypodensities in the subcortical white matter of the frontal, parietal, and occipital lobe (A,B). Brain MRI findings on day 14 of month 1 of the first nivolimab course. T1-contrast enhanced images on the same level as image [ (red arrow).


Neuroimaging. Brain CT in the postoperative course after the patient developed nausea, vomiting, and generalized weakness. The red arrows point at revealing wide-spread bilateral hypodensities in the subcortical white matter of the frontal, parietal, and occipital lobe (A,B). Brain MRI findings on day 14 of month 1 of the first nivolimab course.


Neuroimaging. Brain CT in the postoperative course after the patient developed nausea, vomiting, and generalized weakness. Fluid-attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI) showing multiple bilateral hyperintensities in gray cerebellar matter [(C), red arrows.


Neuroimaging. Brain CT in the postoperative course after the patient developed nausea, vomiting, and generalized weakness.


Neuroimaging. Brain CT in the postoperative course after the patient developed nausea, vomiting, and generalized weakness. MRI FLAIR images showing bilateral thalamic hyperintensities with corresponding T1-contrast enhancement left [, red arrow.


Neuroimaging. Brain CT in the postoperative course after the patient developed nausea, vomiting, and generalized weakness. FLAIR MRI images showing confluent cortical hyperintensities.


Neuroimaging. Brain CT in the postoperative course after the patient developed nausea, vomiting, and generalized weakness. T1 contrast-enhancement showing pial gyriform pattern of enhancement [, red arrows.


Photograph of 2-year-old neonate showing a giant occipital encephalocele.


Computed tomography scan showing calvarial defect of 22 mm x 15 mm in occipital bone.


Computed tomography scan showing cerebrospinal fluid filled sac herniating through the calvarial defect along with part of cerebellum sac.


Lumbosacral spinal sagittal post-gadolinium T1-weighted MRI.


T2-weighted MRI.


Axial post-gadolinium T1-weighted MRI. Showing a posterior epidural abscess extending from L4 to S1 with cauda equina compression. Note the ring enhancement.


Operative view demonstrating an encapsulated epidural collection with purulent material inside (a).


Appearance of the abscess wall adhering to a filamentous foreign body (textiloma) (b).


Hematoxylin-eosin-stained tissue showing histopathologic features of the granulomatous lesion that contain polynuclear and multinucleated giant cells around cut fibers (arrows) (Medium power magnification).


Schema of the patient's left hemisphere, cortical tubers (yellow regions), intracranial electrodes (light blue disks), results of extraoperative functional mapping of anterior language area (red shaded), posterior language area (white shaded), motor area of face/larynx/tongue (green loop), hand (orange loop), and hand sensory area (purple loop), ictal onset electrodes (pink stars), and resection areas (yellow dot loops: 1-3). We implanted 92 electrodes [2 grid electrodes (8 x 5, 4 x 5), 3 strip electrodes (10, 8, 8), and 1 depth electrode (6)]. One cortical tuber was present below and over the anterior language area. Another cortical tuber distributed posterior to the posterior language area. Ictal onset electrodes partially overlapped with the anterior and posterior language areas. The resections were performed from the resection areas of 1, 2, to 3. During the resection surgery, the patient presented with speech difficulty after the resection of site A, and retardation in responsive naming, paraphasia, and phonological recall after the resection of site B.


1, fixing left eye moderate RXT; 2, fixing right eye moderate LXT; 3, fixing right eye more LXT + slight hypotropia + left ptosis; 4, fixing right eye (slight adduction) LXT + slight left hypotropia + left ptosis; 5-7, fixing right eye further in left gaze significant left hypotropia and ptosis; 8, fixing right eye in abduction + left eye upshot.


1, fixing right eye left XT + ptosis; 2, occluding RE; 3-5, fixing left eye RXT + slight right hpotropia + slight right ptosis; 6, 7, fixing left eye in primary position + significant right hypotropia; 8, fixing left eye in attempted dextrodepression + significant right hypotropia.


Ocular rotations in different gaze positions.


Sagittal fluid-attenuated inversion recovery image delineates corpus callosum hypogenesis. The rostrum, genu, and splenium are absent while remnant of the body seems as a short thin line. Note polymicrogyria in the frontal lobe.


Corpus callosum is absent in this coronal T2-weighted section through thalami. Lateral ventricles are slit like and small in caliper. They are upturned (black arrow) and there are Probst bundles (arrowhead), secondary to callosal hypogenesis. Note bilateral periventricular nodular gray matter heterotopia (white arrows).


Axial T1-weighted image shows bifrontal parasagittal polymicrogyria (black arrows) and bilateral periventricular nodular gray matter heterotopia (white arrows).


Subsequent axial T2-weighted images clearly delineate bilateral periventricular gray matter heterotopia (white arrows). Note bifrontal parasagittal polymicrogyria (a and b).


Axial T2-weighted image demonstrates abnormal cerebellar foliation and fissuration with loss of the normal architecture in the inferior aspect of the cerebellar hemispheres, consistent with cerebellar dysplasia.


Baseline FEES showed a silent aspiration and moderate residues at both, the pyriform sinuses and vallecula for liquids.


MRI Brain with contrast revealed a mass adjacent to the brainstem at the level of the medulla hypointense on axial T1-weighted sequences.


With heterogeneous enhancement on the post-contrast sequence.


Edema noted on T2 enhancing.


FLAIR. Sequences.


The planning target volume (PTV) consisted of the resection cavity (outlined in orange) plus a 3 mm margin (outlined in red) as identified on the T1 post-contrast sequence (a). 100% of the dose was prescribed to this volume.


A 3-arc volumetric modulated arc therapy technique with 6-MV photons was used to cover the volume with the 100% isodose line (5400 cGy) (in yellow) covering the PTV target (resection cavity + 3 mm margin) (in red) on the planning CT head (b).


Axial T1-weighted post contrast MRI sequence at the level of the brainstem 24 months after resection demonstrated no residual or recurrent gross disease.


NCCT brain showing SAH in the interhemispheric fissure and bilateral sylvian fissure.


Preoperative CT angiography was showing an anterior communicating artery aneurysm.


MRI brain showing multiple focal infarcts.


Fundus on postoperative day 3: Normal vessels.


Disc margins. Well-defined disc with attenuation of blood vessels fundus picture on 15th postoperative day.


Axial.


Coronal. T1-weighted magnetic resonance imaging showing contrast-enhancing dural-based lesion with parenchymal invasion along the right frontal lobe (arrow).


Axial ,. Along the falx cerebri.


Coronal. Right temporal lobe.


Sagittal. T1-weighted magnetic resonance imaging showing contrast-enhancing dural-based lesions through the frontal sinus, and . Right sphenoid wing. (arrows).


Sagittal. T1-weighted magnetic resonance imaging showing a contrast-enhancing sinonasal mass with intracranial extension through the cribriform plate into the anterior cranial fossa, maxillary, and sphenoid sinuses.


Sagittal T1-weighted magnetic resonance imaging showing contrast-enhancing dural-based lesions posterior to C2 vertebral body.


Posterior to T9-T10 disc space.


Along the posterolateral dura at the L3-L4 level. (arrows).


MRI of the cerebellum showing the new cerebellar infarct.


The old insular lesion. Indicated with arrows.


Preoperative magnetic resonance imaging (MRI) imaging. MRI of pelvis demonstrating soft tissue mass centered within the right hemigluteal region. Sagittal T2-weighted image.


Preoperative magnetic resonance imaging (MRI) imaging. MRI of pelvis demonstrating soft tissue mass centered within the right hemigluteal region. Axial T1-weighted postcontrast image. Is shown with arrowheads indicating location of the mass.


Myxopapillary ependymoma. The tumor is composed of many papillary structures formed by vessels (arrow) encircled by basophilic myxoid material (asterisk) and collars of cuboidal tumor cells (arrowhead).


Myxopapillary ependymoma. Which demonstrate strong GFAP positivity.


Postoperative magnetic resonance imaging (MRI) imaging. MRI of pelvis taken 6 months postoperative demonstrating no evidence of tumor recurrence or metastatic disease to the pelvis. Sagittal T1-weighted postcontrast image.


Postoperative magnetic resonance imaging (MRI) imaging. MRI of pelvis taken 6 months postoperative demonstrating no evidence of tumor recurrence or metastatic disease to the pelvis. Axial T1-weighted postcontrast image. Shown above.


External examination of extraocular eye movements. . Notes: Images demonstrating complete ophthalmoplegia of the right eye with ptosis prior to surgery or amphotericin B treatment.


External examination of extraocular eye movements. , followed by images obtained after 10 months showing full levator function with restoration of right extraocular eye movement in each position of gaze.


Magnetic resonance imaging of brain plain T2-weighted sequence showing hyperintense signal in.


Dorsal midbrain. Dorsal pons.


Magnetic resonance imaging of brain.


Fluid attenuated inversion recovery sequences showing hyperintense signal in dorsal pons. No postcontrast enhancement in dorsal pons.


Papanicolaou staining x20 of cervical lymph node fine-needle aspiration cytology showing lymphocytes, macrophages, foci of necrosis (shaded arrow), and macrophage engulfing karyorrhectic debris (open arrow) which is characteristic of Kikuchi-Fujimoto disease.


Positron emission tomography/computed tomography of brain showed increased focal uptake of tracer in. Left frontal lobe.


Anteriosuperior to anterior cingulate gyrus. Left frontal and left temporal lobe.


Magnetic resonance imaging of brain. T2-weighted sequence.


Fluid-attenuated inversion recovery sequence showing complete resolution of hyperintensities.


Image of Becky demonstrating evidence of gait disturbance.


X-ray image of lumbar spine post contrast injection showing disruption of dorsal flow of contrast material and demonstrating impingement of the spinal cord.


(a) Wide clipping and skin preparation for surgery.


(b) Exposure of the L4 - 7 levels.


Pedigree chart of the family. The arrow represents the proband. Squares represent males, and circles represent females. The diagonal lines represent deceased family members. Black squares or circles indicate the members with FHM. White squares or circles represent members without FHM.


Radiological and histopathological information of patient II-1. CT.


Radiological and histopathological information of patient II-1. GRE T2-weighted MRI. Showed multiple cavernous malformation lesions across the left lateral ventricle and bilateral cerebral hemisphere in the proband, patient II-1. The red arrows show the location of lesions.


Radiological and histopathological information of patient II-1. Histopathological examination revealed vascular malformation associated with bleeding ( ), calcification ( ), and iron deposits ( ) in the proband, patient II-1, at. X100.


Radiological and histopathological information of patient II-1. X100.


Pedigree of a Chinese family. Affected patients were diagnosed with CCMs upon T2-weighted MRI or SWI of the brain.


Diffusion weighted image 48 h after admission demonstrating a bilateral thalamic.


Midbrain. Infarction.


AP CXR status post pacemaker placement.


Axial 1.5 T MR, CT and CTA images demonstrate bilateral SCA infarct and left vertebral artery dissection. A-d Sequential axial FLAIR MR images demonstrate bilateral cerebellar hemisphere hyperintensity superiorly with relative sparing inferiorly (not shown) and involvement of the colliculi in the midbrain and the superior cerebellar peduncles.


Axial 1.5 T MR, CT and CTA images demonstrate bilateral SCA infarct and left vertebral artery dissection. E-h Sequential axial diffusion MR images demonstrate restricted diffusion in both cerebellar hemispheres in the same distribution of the FLAIR images.


Axial 1.5 T MR, CT and CTA images demonstrate bilateral SCA infarct and left vertebral artery dissection. I-l Sequential axial non-contrast CT images demonstrate patchy areas of hypoattenuation bilaterally in the superior aspects of the cerebellar hemispheres. M-n Select axial CTA images demonstrate decreased caliber of left vertebral artery with arterial lumen filling defect (arrow). As well a filling defect is present in the basilar terminus extending to the P1 segment of the left PCA (arrowhead). MR magnetic resonance, FLAIR fluid-attenuated inversion recovery, CT computer tomography, CTA computer tomography angiography, SCA superior cerebellar artery, PCA posterior cerebral artery.


12-month-old boy presented to our outpatient department with a history of global developmental delay and infantile spasms since age of 6 months diagnosed as a case of pachygyria. (a and b) T2-weighted axial images show hyperintensities in periventricular (single arrow) and bilateral frontoparietal region with frontal predominant cortical thickening (double arrow) with paucity of sulci favoring cortical dysplasia with pachygyria.


12-month-old boy presented to our outpatient department with a history of global developmental delay and infantile spasms since age of 6 months diagnosed as a case of pachygyria. T2-weighted axial images show "tigroid-like stripes"(single arrow) along with frontal predominant cortical thickening (double arrow).


12-month-old boy presented to our outpatient department with a history of global developmental delay and infantile spasms since age of 6 months diagnosed as a case of pachygyria. Fluid-attenuated inversion recovery image shows hyperintensities in bilateral subcortical white matter (thin triple arrow) suggesting myelination abnormality.


12-month-old boy presented to our outpatient department with a history of global developmental delay and infantile spasms since age of 6 months diagnosed as a case of pachygyria. T2-weighted sagittal images. Show radial stripes (single arrow).


12-month-old boy presented to our outpatient department with a history of global developmental delay and infantile spasms since age of 6 months diagnosed as a case of pachygyria. : show scattered dots in form of hyperintensities on a normal background white matter (double arrow) - "leopard-like appearance" along with a normal appearing cerebellum.


12-month-old boy presented to our outpatient department with a history of global developmental delay and infantile spasms since age of 6 months diagnosed as a case of pachygyria. Awake electroencephalogram of the child shows background high amplitude polymorphic delta wave activity with multifocal spikes.


Brain MRI. T1 axial slice. (A) There is marked ventriculomegaly. The maximum width of the frontal horns of the lateral ventricles is 5.8 cm; the maximal internal diameter of the skull at the same level is 13.9 cm; the calculated Evans index is 0.42 (normal value: < 0.3).


T1 sagittal slice. (B) The anterior commissure- posterior commissure line is drawn, and its length is 4.0 cm; this line is an important landmark for stereotactic targeting in GPi-DBS.


T2 axial slice). (C) The third ventricle is also dilated, and there is prominent flow void artifact (white straight arrows); this artifact means that CSF flow velocity is high and there is no obstruction. Magnetic susceptibility artifacts due to ventriculoperitoneal catheter and valve (white stars).


Postoperative GPi-DBS exams. Skull radiography frontal view.


Brain CT coronal slices). The lead tracts (white curved arrows) avoid the right ventriculoperitoneal shunt catheter (white arrowheads) and the enlarged frontal horns of the lateral ventricles.


Superimposed fused images between preoperative T2 MRI and postoperative CT. Axial.


Coronal) confirmed the lead placement on the GPi bilaterally (white straight arrows).


(a) Head CT scan showing diffuse and thick SAH on admission.


(b) 3D image of left IC angiography showing a paraclinoid aneurysm.


(c) The aneurysm was successfully obliterated with coiling.


(d) Head CT scan on day 3 showing most of the SAH was washed out.


Neuroimages obtained at the onset of deafness. (a) MR angiogram showing severe vasospasm in the right MCA and moderate vasospasm in the left MCA.


Neuroimages obtained at the onset of deafness. (b) DW image showing high signal intensity at the right insular cortex and the left superior temporal gyrus, indicating acute infarction due to vasospasm.


Neuroimages obtained at the onset of deafness. (c) ASL images show bilateral superior temporal gyri.


Results of neuropsychological tests. The patient was examined thoroughly twice in 2017. Results are listed as z-scores in relation to normal results. The dashed line indicates the cut off for pathological scores adjusted for age. Trail Making B was also performed, but the patient was not able to finish the test. Furthermore, the patient was not able to complete the visual puzzles test in the follow up examination in 09/17. Tests included: *Wechsler Adult Intelligence Scale - Fourth Edition;. Wechsler Memory Scale - Fourth Edition and #Regensburger verbal fluency test.


Timeline of symptoms, clinical findings, imaging biomarker, and treatment. Criteria for the clinical diagnosis of Dementia with Lewy bodies are indicated in red.


(a and b) T2-weighted Brain MRI (coronal plane). T2 Coronal MRI thin-section 3D CISS images showing the right PICA crossing the cerebello-pontine cistern in close contact with the right VII and VIII nerves. The arrow indicates the PICA loop.


(a-c) Axial MRI thin-section 3D CISS images: the right PICA makes a loop in the cerebello-pontine cistern.


(d) Close contact of the PICA and VII nerve. The arrow indicates the close contact between the vessel and the VII-VIII nerve complex.


Intraoperative views during microvascular decompression. We observed close contact between vascular structures and the facial nerve, which could explain the irritative symptoms presented by the patient. *VII-VIII nerve complex; arrow-head - loop of PICA; IX - glossopharyngeal nerve.


Patient's skin and teeth manifestations. . Notes:. Hyperpigmented lesions on the back, and ,abdomen.


Patient's skin and teeth manifestations. Stage 4 hypopigmented lesion on the lower limbs.


Patient's skin and teeth manifestations. Conoid and missing teeth.


Cardiac catheterization. . Notes:. Right pulmonary artery.


Cardiac catheterization. Left pulmonary artery. Black arrows point to collateral vessels; white arrows point to right and left pulmonary arteries.


Pseudophakic pupillary block glaucoma, initial presentation (see text).


Pseudophakic pupillary block glaucoma, ultrasonogram. (a) Shows chain of low amplitude spikes in A-scan due to sterile vitritis (see text).


Pseudophakic pupillary block glaucoma, ultrasonogram. (b) Shows a bright anterior echo from intraocular lens (short arrow) followed by multiple reverberations (long arrow) in B-scan.


Pseudophakic pupillary block glaucoma: White pupillary membrane unearthed on mydriasis, still attached to iris superiorly but relieved inferiorly.


Pseudophakic pupillary block glaucoma: Slit lamp section shows pupillary membrane and superior iris bombe.


MRI imaging of the brain reveals diffusion restriction in the right medulla oblongata (red arrow) and an old infarction of the left cerebellum (white arrow).


Sicca keratopathy of the right eye seen as superficial punctate fluorescein staining of the corneal epithelium.


A list of all the signs and symptoms presented by our patient suffering a right-sided dorsolateral medullary infarction.


Straight left upper border of heart.


Left ventricular (LV) apical aneurysm.


Fundus photograph (A) at presentation demonstrates stable findings following macular translocation for age-related macular degeneration, with geographic atrophy along the inferior arcades and no visible heme, fluid, or exudates. No ophthalmoscopic evidence for active choroidal neovascularization is visible (orange arrow, A).


Early.


Mid-phase. Fluorescein angiograms at presentation demonstrate a small leaking juxtafoveal choroidal neovascularization lesion (orange arrow). A window defect corresponding to the inferior atrophy is also noted.


Magnetic resonance imaging T1 GE 3D axial sequence without intravenous contrast, immediately after virus infusion. The intraparenchymal injection of gadolinium before infusing the virus with the MEMS cannula (this cannula has two independent channels) is pushed out from the tumor as the virus is infused from a second channel.


Diffuse midline glioma showing strong nuclear H3K27M mutant protein (immunohistochemistry, X200).


Magnetic resonance imaging scan of brain in T2 sequence showed non-enhancing altered signal intensity in both thalamic and left medial temporal lobe suggestive of encephalitis.


Magnetic resonance imaging brain in T2 fluid attenuation inversion recovery sequence showed bilateral symmetrical hyperintensities in both thalamic regions.


Magnetic resonance imaging scan of brain T2 fluid attenuation inversion recovery sequence showed altered signal intensity in both thalamic and splenium of corpus callosum region.


Pedigree of the family with SCA40. Squares indicate males; circles indicate females; an arrow indicates the propositus. Slash marks indicate subjects who are deceased. Roman numerals indicate generations, and Arabic numbers indicate subjects. SCA40, spinocerebellar ataxia type 40.


Flat pons, not full and mild cerebellar atrophy in T2-weighted MRI.


CCDC88C gene mutation sequencing results of II-2, II-3, III-4, III-5, IV-1, and IV-2.


MRI follow up. (A) March 2018: FLAIR sequences showed a left temporo-parieto-occipital and a right temporal hyperintensity (A1, A2, A3); no gadolinium enhancement (A4, A5).