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prion_0000 | AIMS: Creutzfeldt-Jakob disease (CJD) is a rare, progressive, fatal neurodegenerative disorder caused by an abnormal glycoprotein known as the prion protein.
The core features include progressive cognitive decline, cerebellar dysfunction, personality changes, and visual disturbances.
Although psychiatric symptoms are r... | [
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"label": "Generic_Prion",
"start": 6,
"end": 31,
"text": "Creutzfeldt-Jakob disease",
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"id": "T1",
"label": "Generic_Prion",
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... |
prion_0001 | Aims: Creutzfeldt–Jakob Disease (CJD) is a rare, fatal neurodegenerative disorder caused by prion proteins, leading to progressive brain damage.
CJD has sporadic, variant, genetic, and iatrogenic forms, with sporadic being the most common, affecting 1–2 people per million annually.
It typically presents with rapid cogn... | [
{
"id": "T2",
"label": "Generic_Prion",
"start": 6,
"end": 31,
"text": "Creutzfeldt–Jakob Disease",
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"start": 6,
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"id": "T3",
"label": "Generic_Prion",
"start": 33,
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... |
prion_0004 | BACKGROUND: TSEs present diagnostic and infection control (IC) challenges.
Creutzfeldt-Jakob Disease (CJD) is the most common human TSE, occurring in 1–2/million/year in the United States, but other zoonotic factors or transmissions remain incompletely understood.
Prompted by the occurrence of four suspected cases from... | [
{
"id": "T2",
"label": "Generic_Prion",
"start": 12,
"end": 16,
"text": "TSEs",
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"start": 12,
"end": 16
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"is_discontinuous": null
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{
"id": "T3",
"label": "Generic_Prion",
"start": 75,
"end": 100,
"text": "Creutzfel... |
prion_0006 | BACKGROUND: Creutzfeldt‐Jakob disease (CJD) is a progressive, irreversible, and fatal disease associated with the misfolding of a protein in the central nervous system.
Several surveillance programs emerged worldwide after the epidemic of the early 2000s.
In Mexico, information about its epidemiology is scarce.
A case ... | [
{
"id": "T21",
"label": "Generic_Prion",
"start": 12,
"end": 37,
"text": "Creutzfeldt‐Jakob disease",
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"id": "T2",
"label": "Generic_Prion",
"start": 39,
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... |
prion_0007 | INTRODUCTION: Creutzfeldt‐Jakob disease (CJD) is a rare, fatal neurodegenerative disorder caused by misfolded prion proteins that accumulate in the brain, leading to spongiform encephalopathy.
It is characterized by rapidly progressive mental decline and other neurological symptoms, typically resulting in death within ... | [
{
"id": "T2",
"label": "Generic_Prion",
"start": 14,
"end": 39,
"text": "Creutzfeldt‐Jakob disease",
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"start": 14,
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"id": "T3",
"label": "Generic_Prion",
"start": 41,
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... |
prion_0008 | BACKGROUND: The unfavorable prognosis of prion diseases highlights the need for a robust diagnostic approach system to accurately distinguish them from other etiologies of dementia.
Correlating biomarkers is crucial for advancing therapies and optimizing patient selection in clinical trials.
METHOD: We prospectively en... | [
{
"id": "T2",
"label": "Generic_Prion",
"start": 41,
"end": 55,
"text": "prion diseases",
"spans": [
{
"start": 41,
"end": 55
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{
"id": "T27",
"label": "Differential_Diagnosis",
"start": 152,
"end": 180,
... |
prion_0011 | PURPOSE: To report on quantitative electroencephalograph (EEG) activity during polysomnography (PSG) in a rare case of confirmed Fatal Familial Insomnia (FFI).
METHODS: Sleep/wake characteristics of a 32-year-old male patient were quantitatively analysed using central EEG recordings during two PSGs (FFI-1 and FFI-2) fi... | [
{
"id": "T32",
"label": "Electrophysio_test",
"start": 35,
"end": 56,
"text": "electroencephalograph",
"spans": [
{
"start": 35,
"end": 56
}
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"is_discontinuous": null
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{
"id": "T33",
"label": "Electrophysio_test",
"start": 58,
"end"... |
prion_0013 | BACKGROUND: Fatal Familial Insomnia (FFI) is a rare autosomal dominant prion disease.
Herein, we present a case of a patient who was diagnosed with FFI, with symptoms initially resembling dementia with Lewy bodies (DLB).
METHOD: A 48‐year‐old Brazilian male with a six‐year history of insomnia presented with rapidly pro... | [
{
"id": "T2",
"label": "FFI",
"start": 12,
"end": 35,
"text": "Fatal Familial Insomnia",
"spans": [
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"start": 12,
"end": 35
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"id": "T3",
"label": "FFI",
"start": 37,
"end": 40,
"text": "FFI",
"... |
prion_0015 | BACKGROUND: Creutzfeld‐Jakob disease (CJD) is an extremely rare neurodegenerative disorder, caused by the deposition of pathogenic protein components, known as prions, in the central nervous system.
It may present a variable combination of symptoms that include rapidly progressive dementia, ataxia, extrapyramidal sympt... | [
{
"id": "T2",
"label": "Generic_Prion",
"start": 12,
"end": 36,
"text": "Creutzfeld‐Jakob disease",
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"start": 12,
"end": 36
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"id": "T3",
"label": "Generic_Prion",
"start": 38,
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... |
prion_0077 | BACKGROUND: Prion diseases are a group of neurodegenerative diseases associated with prion protein.
The disease can be caused by mutations in the PRNP gene, the gene that encodes prion protein.
An octapeptide repeat on the N‐terminus of prion protein plays an important role in normal intercellular function of prion pro... | [
{
"id": "T2",
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"start": 12,
"end": 26,
"text": "Prion diseases",
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"id": "T14",
"label": "Differential_Diagnosis",
"start": 42,
"end": 68,
... |
prion_0081 | Creutzfeldt-Jakob disease (CJD) belongs to a group of prion diseases that may be infectious, sporadic, or hereditary.
The 200K point mutation in the PRNP gene is the most frequent cause of hereditary CJD, accounting for >70% of families with CJD worldwide.
Prevalence of the 200K variant of familial CJD is especially hi... | [
{
"id": "T2",
"label": "Generic_Prion",
"start": 0,
"end": 25,
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"start": 0,
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"id": "T3",
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... |
prion_0109 | Clinical differential diagnosis of early-onset dementia (EOD) includes familial Alzheimer disease (FAD) and hereditary prion disease.
In both disease entities, postmortem brain histopathological examination is essential for unambiguous diagnosis.
Mutations in the genes encoding the presenilins (PS1 and PS2) and amyloid... | [
{
"id": "T2",
"label": "Symptom",
"start": 35,
"end": 55,
"text": "early-onset dementia",
"spans": [
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"start": 35,
"end": 55
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{
"id": "T42",
"label": "Symptom",
"start": 57,
"end": 60,
"text": "EOD",... |
prion_0111 | Since a report in 1996 of 10 cases of Creutzfeldt-Jakob disease (CJD) with onset in a younger than usual age, a pattern of the disease has emerged.
This includes early neuropsychiatric features and sensory symptoms and neurological signs such as ataxia and involuntary movements later in the course of the disease.
Three... | [
{
"id": "T2",
"label": "Time_point",
"start": 18,
"end": 22,
"text": "1996",
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"start": 18,
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"id": "T3",
"label": "Generic_Prion",
"start": 38,
"end": 63,
"text": "Creutzfeldt-J... |
prion_0121 | The clinical presentation and evolution, neuropathological findings, and genotyping of three members of a Spanish family affected with fatal familial insomnia are reported.
The mother and two of her offspring developed a rapidly evolving disease with insomnia and behavioural disorders as the initial symptoms and died b... | [
{
"id": "T17",
"label": "Autopsy",
"start": 41,
"end": 58,
"text": "neuropathological",
"spans": [
{
"start": 41,
"end": 58
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{
"id": "T18",
"label": "Genetic_test",
"start": 73,
"end": 83,
"text": "ge... |
prion_0122 | This study examined trends in mortality from sporadic Creutzfeldt-Jakob disease in France for 1992-7 by age, genotype at the codon 129 of the prion protein gene, and geographical area.
Case ascertainment was based on notifications by neurologists, neuropathologists, and laboratories; 324 deaths from definite or probabl... | [
{
"id": "T2",
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"start": 54,
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"text": "Creutzfeldt-Jakob disease",
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"id": "T3",
"label": "Duration",
"start": 94,
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"t... |
prion_0141 | A total of 65 cases of Creutzfeldt-Jakob disease with a history of cadaveric dura transplantation in Japan were analysed to clarify the epidemiologic features of such patients and to explore whether other such patients will appear in the future.
The age at transplantation averaged 44.4 years with a standard deviation o... | [
{
"id": "T2",
"label": "Generic_Prion",
"start": 23,
"end": 48,
"text": "Creutzfeldt-Jakob disease",
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"id": "T18",
"label": "Treatment",
"start": 67,
"end": 97,
"... |
prion_0159 | A postmortem case of an atypical form of dural graft associated Creutzfeldt-Jakob disease (CJD) is described.
A 42 year old man developed progressive spastic paresis 163 months after a cadaveric dura mater graft.
He presented with no myoclonus and very late occurrence of periodic synchronous discharges on EEG.
The prio... | [
{
"id": "T2",
"label": "iCJD",
"start": 41,
"end": 89,
"text": "dural graft associated Creutzfeldt-Jakob disease",
"spans": [
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"start": 41,
"end": 89
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"id": "T3",
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... |
prion_0164 | OBJECTIVES: The detection of the protein 14-3-3 in the CSF has been shown to be a reliable and sensitive marker for sporadic Creutzfeldt-Jakob disease (CJD).
Other brain-specific proteins such as neuron specific enolase (NSE), S-100b, and tau protein have also been reported to be increased in the CSF of patients with s... | [
{
"id": "T44",
"label": "Blood_biomarker_test",
"start": 55,
"end": 58,
"text": "CSF",
"spans": [
{
"start": 55,
"end": 58
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"is_discontinuous": null
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{
"id": "T2",
"label": "Generic_Prion",
"start": 125,
"end": 150,
"text": "C... |
prion_0165 | Three members of a family with inherited prion disease are reported.
One additional family member had a progressive neurological disease without details.
Two developed symptoms of ataxia, dementia, myoclonus, rigidity, and hemiparesis, and one had a different phenotype with the combination of lower motor neuron deficit... | [
{
"id": "T2",
"label": "Generic_Prion",
"start": 31,
"end": 54,
"text": "inherited prion disease",
"spans": [
{
"start": 31,
"end": 54
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"is_discontinuous": null
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{
"id": "T20",
"label": "Symptom",
"start": 104,
"end": 136,
"te... |
prion_0170 | OBJECTIVES: To describe the clinical presentation of patients with Alzheimer's disease (AD) or dementia with Lewy bodies (DLB) who were suspected of having Creutzfeldt-Jakob disease (CJD) and to investigate whether current clinical diagnostic criteria cover these atypical forms of AD and DLB.
METHODS: Brains from necro... | [
{
"id": "T2",
"label": "Differential_Diagnosis",
"start": 67,
"end": 86,
"text": "Alzheimer's disease",
"spans": [
{
"start": 67,
"end": 86
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"is_discontinuous": null
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{
"id": "T3",
"label": "Differential_Diagnosis",
"start": 95,
"... |
prion_0200 | OBJECTIVE: To compare conventional and diffusion-weighted MR imaging in terms of their depiction of the abnormalities occurring in Creutzfeldt-Jakob disease.
MATERIALS AND METHODS: We retrospectively analyzed the findings of conventional (T2-weighted and fluid-attenuated inversion recovery) and diffusion-weighted MR im... | [
{
"id": "T23",
"label": "Imaging_sequence",
"start": 22,
"end": 68,
"text": "conventional MR imaging",
"spans": [
{
"start": 22,
"end": 34
},
{
"start": 58,
"end": 68
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"is_discontinuous": true
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{
"id": "T2",
"l... |
prion_0214 | We report the use of diffusion-weighted MR imaging in the early diagnosis and monitoring of the progression of a histopathologically proved case of sporadic Creutzfeldt-Jakob disease.
Ribbon-like areas of hyperintensity in the cerebral cortex on diffusion-weighted images corresponded to the localization of periodic sha... | [
{
"id": "T2",
"label": "Imaging_sequence",
"start": 21,
"end": 39,
"text": "diffusion-weighted",
"spans": [
{
"start": 21,
"end": 39
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"is_discontinuous": null
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{
"id": "T8",
"label": "Imaging_test",
"start": 40,
"end": 50,
"te... |
prion_0217 | Prions have emerged in the past 5 years as serious transmissible infective agents.
Ocular tissue transplantation has come under scrutiny after potential infected tissue was transplanted into healthy patients.
In this review we examine the evidence for the risk of transmission of prions after scleral transplantation and... | [
{
"id": "T2",
"label": "Generic_Prion",
"start": 0,
"end": 6,
"text": "Prions",
"spans": [
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"end": 6
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{
"id": "T3",
"label": "Treatment",
"start": 83,
"end": 112,
"text": "Ocular tissue t... |
prion_0223 | OBJECTIVE: To gain a better understanding of how mutations of the prion protein (PrP) gene are responsible for progressive dementia syndrome and to clarify the correlation between genotype and phenotype, which should help to explain how the prion promotes neurological symptoms. BACKGROUND: Genetic prion diseases are as... | [
{
"id": "T3",
"label": "Differential_Diagnosis",
"start": 111,
"end": 140,
"text": "progressive dementia syndrome",
"spans": [
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"end": 140
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"id": "T5",
"label": "Symptom",
"start": 256,
"... |
prion_0224 | A 47 year old man is described who developed pathology proven Creutzfeldt-Jakob disease (CJD) 38 years after receiving a low dose of human derived growth hormone (hGH) as part of a diagnostic procedure.
The patient presented with a cerebellar syndrome, which is compatible with iatrogenic CJD.
This is the longest incuba... | [
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"id": "T2",
"label": "Age",
"start": 2,
"end": 13,
"text": "47 year old",
"spans": [
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"start": 2,
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{
"id": "T11",
"label": "Autopsy",
"start": 45,
"end": 54,
"text": "pathology",
"spa... |
prion_0228 | A quantitative assessment of eye movements and a detailed neuropsychological profile were conducted at predementia stage in a patient who later had histological confirmation of sporadic Creutzfeldt-Jakob disease (CJD).
The patient was a middle aged man who presented with abnormal eye movements and poor balance.
Neurops... | [
{
"id": "T11",
"label": "Symptom",
"start": 29,
"end": 42,
"text": "eye movements",
"spans": [
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"start": 29,
"end": 42
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{
"id": "T2",
"label": "sCJD",
"start": 177,
"end": 211,
"text": "sporadic Creu... |
prion_0233 | BACKGROUND AND PURPOSE: Diffusion-weighted imaging can disclose distinct hyperintense lesions in Creutzfeldt-Jakob disease (CJD).
However, these findings and chronologic changes of CJD at diffusion-weighted imaging have not been fully investigated.
Our purpose was to assess the diagnostic value of diffusion-weighted im... | [
{
"id": "T2",
"label": "Imaging_sequence",
"start": 24,
"end": 50,
"text": "Diffusion-weighted imaging",
"spans": [
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"start": 24,
"end": 50
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"is_discontinuous": null
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{
"id": "T4",
"label": "Imaging_finding",
"start": 73,
"end": ... |
prion_0242 | OBJECTIVES: To characterise the nature of cognitive change in Creutzfeldt-Jakob disease (CJD).
METHODS: Case histories are reported of four patients with sporadic (sCJD) and two with familial CJD (fCJD), with postmortem pathological findings in four cases.
The data derived from cognitive examination are examined with r... | [
{
"id": "T22",
"label": "Symptom",
"start": 42,
"end": 58,
"text": "cognitive change",
"spans": [
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"start": 42,
"end": 58
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"id": "T2",
"label": "Generic_Prion",
"start": 62,
"end": 87,
"text": "Cre... |
prion_0281 | Based on in vitro observations in scrapie-infected neuroblastoma cells, quinacrine has recently been proposed as a treatment for Creutzfeldt-Jakob disease (CJD), including a new variant CJD which is linked to contamination of food by the bovine spongiform encephalopathy (BSE) agent.
The present study investigated possi... | [
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"label": "Treatment",
"start": 72,
"end": 82,
"text": "quinacrine",
"spans": [
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"start": 72,
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"id": "T3",
"label": "Generic_Prion",
"start": 129,
"end": 154,
"text": "Creutz... |
prion_0294 | Thalamic degenerations or dementias are poorly understood conditions.
The familial forms are (1) selective thalamic degenerations and (2) thalamic degenerations associated with multiple system atrophy.
Selective thalamic degenerations share clinical and pathologic features with fatal familial insomnia, an autosomal dom... | [
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"id": "T24",
"label": "Anatomic_location",
"start": 0,
"end": 8,
"text": "Thalamic",
"spans": [
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"start": 0,
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"id": "T23",
"label": "Differential_Diagnosis",
"start": 0,
"end": 22,
"text... |
prion_0326 | A conformational change of the prion protein is responsible for a class of neurodegenerative diseases called the transmissible spongiform encephalopathies that include mad cow disease and the human afflictions kuru and Creutzfeldt-Jakob disease.
Despite the attention given to these diseases, the normal function of the ... | [
{
"id": "T15",
"label": "Differential_Diagnosis",
"start": 75,
"end": 101,
"text": "neurodegenerative diseases",
"spans": [
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"end": 101
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"id": "T3",
"label": "Generic_Prion",
"start": 113,
... |
prion_0341 | Sudden onset neurological syndromes are almost always vascular but there is a wide range of causes of subacute onset neurological syndromes.
Most diagnoses will become clear from clinical assessment and, depending on presentation, computed tomography or magnetic resonance imaging of the brain or spine and cerebrospinal... | [
{
"id": "T2",
"label": "Symptom",
"start": 13,
"end": 35,
"text": "neurological syndromes",
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"start": 13,
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{
"id": "T9",
"label": "Symptom",
"start": 117,
"end": 139,
"text": "ne... |
prion_0343 | BACKGROUND: There has been concern that children with variant Creutzfeldt-Jakob disease (vCJD) might be misdiagnosed as cases of Alpers' syndrome, as a spongiform degeneration of the brain is seen in both conditions.
OBJECTIVE: To report a national prospective surveillance study of children with progressive intellectua... | [
{
"id": "T2",
"label": "vCJD",
"start": 54,
"end": 87,
"text": "variant Creutzfeldt-Jakob disease",
"spans": [
{
"start": 54,
"end": 87
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],
"is_discontinuous": null
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{
"id": "T3",
"label": "vCJD",
"start": 89,
"end": 93,
"text": ... |
prion_0359 | OBJECTIVES: The most common familial early onset dementia mutations are found in the genes involved in Alzheimer's disease; the amyloid precursor protein (APP) and the presenilin 1 and 2 (PSEN1 and 2) genes; the prion protein gene (PRNP) may be involved.
METHODS: Following identification of a two-octapeptide repeat ins... | [
{
"id": "T25",
"label": "Differential_Diagnosis",
"start": 28,
"end": 57,
"text": "familial early onset dementia",
"spans": [
{
"start": 28,
"end": 57
}
],
"is_discontinuous": null
},
{
"id": "T1",
"label": "Differential_Diagnosis",
"start"... |
prion_0455 | OBJECTIVE: To investigate whether phosphorylated tau protein (tau-pT181) is increased in variant Creutzfeldt-Jakob disease (vCJD) and if the tau-pT181/tau protein ratio is useful for distinguishing between patients with and without CJD.
METHODS: CSF tau protein and tau-pT181 were measured in 50 patients with sporadic C... | [
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"id": "T5",
"label": "vCJD",
"start": 89,
"end": 122,
"text": "variant Creutzfeldt-Jakob disease",
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"end": 122
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"id": "T6",
"label": "vCJD",
"start": 124,
"end": 128,
"tex... |
prion_0473 | We report the case of a 28 year old man who had received a cadaverous dura mater graft after a traumatic open skull fracture with tearing of the dura at the age of 5 years.
A clinical suspicion of Creutzfeldt-Jakob disease (CJD) was confirmed by a brain biopsy 5 months prior to death and by autopsy, thus warranting the... | [
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"id": "T1",
"label": "Age",
"start": 24,
"end": 35,
"text": "28 year old",
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"id": "T15",
"label": "Treatment",
"start": 48,
"end": 86,
"text": "received a cadav... |
prion_0486 | Between 1970 and 2003, seven cases of human dura mater-associated Creutzfeldt-Jakob disease (CJD) were identified in the UK.
Furthermore, we identified a case of CJD in a porcine dura graft recipient.
The mean incubation period of the human dura mater cases was 93 (range 45-177) months.
The clinico-pathological feature... | [
{
"id": "T1",
"label": "Duration",
"start": 0,
"end": 21,
"text": "Between 1970 and 2003",
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"id": "T3",
"label": "iCJD",
"start": 38,
"end": 91,
"text": "human dur... |
prion_0504 | An autopsy case of Creutzfeldt-Jakob disease with Kuru-like neuropathological changes which revealed clinically extrapyramidal, pyramidal and psychic symptoms is presented in this report.
On microscopic examination, status spongiosus, neuronal degeneration, proliferation of hypertrophic astrocytes and numerous plaques ... | [
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"id": "T2",
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"text": "Creutzfeldt-Jak... |
prion_0506 | The case of a patient 53 years old that had a picture of cortical impairment and pyramidal disfunction, leading in 6 months to acinetic mutism is reported.
The histopathology lead to the diagnostic of Creutzeldt-Jakob disease, with neuronal loss, gliosis and status spongiosus.
The electron-microscopic examination showe... | [
{
"id": "T1",
"label": "Age",
"start": 22,
"end": 34,
"text": "53 years old",
"spans": [
{
"start": 22,
"end": 34
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Symptom",
"start": 57,
"end": 76,
"text": "cortical impairmen... |
prion_0508 | The clinico-pathological features of 4 cases of Creutzfeldt-Jakob disease, a transmissible virus dementia, are reported.
The onset of the disease varied between 54 and 81 years of age: the course is dramatic and the demise occurs 1-4 months after the onset of symptoms.
The clinical picture includes mental deterioration... | [
{
"id": "T2",
"label": "Generic_Prion",
"start": 48,
"end": 73,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 48,
"end": 73
}
],
"is_discontinuous": null
},
{
"id": "T1",
"label": "Time_point",
"start": 125,
"end": 145,
... |
prion_0509 | A case of Creutzfeldt-Jakob disease treated with amantadine showed considerable initial improvement, followed by a period of almost five years during which the patient remained in a relatively stable condition until he died accidentally.
The diagnosis was confirmed histologically. | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 10,
"end": 35,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 10,
"end": 35
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Treatment",
"start": 49,
"end": 59,
"t... |
prion_0510 | One hundred twenty-four consecutive cases of Creutzfeldt-Jakob disease (CJD) in France, verified by biopsy or autopsy between 1968 and 1977, were analyzed with respect to their clinical characteristics.
The series comprised equal numbers of men and women, with the most frequent age at onset being 60 to 64 years and the... | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 45,
"end": 70,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 45,
"end": 70
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Generic_Prion",
"start": 72,
"end": 75,
... |
prion_0514 | A case suggestive of Creutzfeldt-Jakob disease in an adult with widespread lesions of the cerebral cortex, striatum and the spinal cord is reported.
Anatomopathological studies showed neuronal degeneration, astrocytic proliferation, intense lymphoplasmocytic activity and slight spongiform degeneration.
This inflammator... | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 21,
"end": 46,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 21,
"end": 46
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Anatomic_location",
"start": 90,
"end": 10... |
prion_0521 | The history of a 67-year-old woman with histologically proven Creutzfeldt-Jakob disease (CJD) is presented.
Before typical clinical and neurophysiological signs of CJD developed, magnetic resonance imaging (MRI) showed slightly enhanced signal intensity of the caudate nuclei and putamina in T2-weighted and proton densi... | [
{
"id": "T1",
"label": "Age",
"start": 17,
"end": 28,
"text": "67-year-old",
"spans": [
{
"start": 17,
"end": 28
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Generic_Prion",
"start": 62,
"end": 87,
"text": "Creutzfeldt-J... |
prion_0523 | In the past 3 years there have been five further cases, in addition to one case reported in 1985, of Creutzfeldt-Jakob disease in recipients of human growth hormone in the United Kingdom.
The clinical findings of two of these cases are described, demonstrating a typical presentation with a predominantly cerebellar synd... | [
{
"id": "T3",
"label": "Time_point",
"start": 92,
"end": 96,
"text": "1985",
"spans": [
{
"start": 92,
"end": 96
}
],
"is_discontinuous": null
},
{
"id": "T4",
"label": "Generic_Prion",
"start": 101,
"end": 126,
"text": "Creutzfeldt... |
prion_0524 | The epidemiological and clinical features of Creutzfeldt-Jakob disease have never before been studied in Scotland and Northern Ireland.
Case records for those dying with this diagnosis were obtained for the period 1980-89.
Over the ten year period, 25 definite or probable cases were identified, giving an annual inciden... | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 45,
"end": 70,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 45,
"end": 70
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Time_point",
"start": 214,
"end": 221,
... |
prion_0525 | A 47-year-old man began to suffer from progressive truncal ataxia and mental alterations typical of Wernicke-Korsakoff syndrome.
He showed confusional state, hallucinations, delirium of jealousy and a serious impairment of recent memory.
The symptomatology lasted 13 months, but only in the last weeks was it complicated... | [
{
"id": "T1",
"label": "Age",
"start": 2,
"end": 13,
"text": "47-year-old",
"spans": [
{
"start": 2,
"end": 13
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Symptom",
"start": 39,
"end": 65,
"text": "progressive truncal a... |
prion_0528 | Serial brain CT and 123I-IMP SPECT were examined in a case with Creutzfeldt-Jakob disease (CJD).
A 61-year-old woman had the onset of progressive dementia and gait disturbance in December 1988.
Then, she developed left hemiparesis and dysarthria, and rapidly fell into akinetic mutism within about 2 months.
Brain CT, MR... | [
{
"id": "T48",
"label": "Anatomic_location",
"start": 7,
"end": 12,
"text": "brain",
"spans": [
{
"start": 7,
"end": 12
}
],
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},
{
"id": "T1",
"label": "Imaging_test",
"start": 13,
"end": 15,
"text": "CT",
... |
prion_0530 | We studied the clinical and histopathology findings of the first proved case of Creutzfeldt-Jakob disease in Panama.
A sixty-five-years-old female patient referred from Santiago de Veraguas was admitted to Santo Tomás Hospital with a progressive clinical picture of dementia, incoordination and generalized myoclonia.
Th... | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 80,
"end": 105,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 80,
"end": 105
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Age",
"start": 119,
"end": 139,
"tex... |
prion_0533 | In 1986, we reported two anatomoclinical observations of a familial condition that we called "fatal familial insomnia" (FFI).
We now present the pedigree as well as the clinical and neuropathologic findings in five new subjects.
The pedigree includes 288 members from six generations.
Men and women are affected in a pat... | [
{
"id": "T2",
"label": "Time_point",
"start": 3,
"end": 7,
"text": "1986",
"spans": [
{
"start": 3,
"end": 7
}
],
"is_discontinuous": null
},
{
"id": "T3",
"label": "FFI",
"start": 94,
"end": 117,
"text": "fatal familial insomnia",
... |
prion_0536 | Creutzfeldt-Jakob disease (CJD) is presumably caused by a slow infectious pathogen or prion.
The principal clinical features of Creutzfeldt-Jakob disease are dementia, pyramidal and extrapyramidal symptoms and signs, cerebellar dysfunction, and myoclonus.
The patient rapidly deteriorates, declines to a vegetative state... | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 0,
"end": 25,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 0,
"end": 25
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Generic_Prion",
"start": 27,
"end": 30,
... |
prion_0538 | Currently some experts are predicting the incidence of the rare yet rapidly fatal Creutzfeldt-Jakob disease (CJD) is on the rise.
Worldwide, there is about one case per million people per year, but iatrogenic transmission is increasing.
There have been documented cases of CJD transmission through corneal transplants, s... | [
{
"id": "T2",
"label": "Generic_Prion",
"start": 82,
"end": 107,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 82,
"end": 107
}
],
"is_discontinuous": null
},
{
"id": "T3",
"label": "Generic_Prion",
"start": 109,
"end": 112... |
prion_0539 | A focus of Creutzfeldt-Jakob disease is present in Israel among Jews born in Libya.
The present study examines the clinical features in this particular group of patients.
In a country-wide study of Creutzfeldt-Jakob disease, we identified 114 patients; 49 were Libyan immigrants, and 65 (three of whom had Libyan ancesto... | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 11,
"end": 36,
"text": "Creutzfeldt-Jakob disease",
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"start": 11,
"end": 36
}
],
"is_discontinuous": null
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{
"id": "T2",
"label": "Generic_Prion",
"start": 198,
"end": 223,
... |
prion_0540 | Serial magnetic resonance (MR) imagings of two autopsied patients with Creutzfeldt-Jakob disease (CJD) are presented.
Both patients showed a dramatic progression of brain atrophy.
The initial MR imagings were, however, interpreted as normal except for localized mild cortical atrophy in one patient.
When a normal MR ima... | [
{
"id": "T4",
"label": "Imaging_test",
"start": 7,
"end": 39,
"text": "imagings magnetic resonance",
"spans": [
{
"start": 31,
"end": 39
},
{
"start": 7,
"end": 25
}
],
"is_discontinuous": true
},
{
"id": "T6",
"labe... |
prion_0545 | We have encountered a case of Creutzfeldt-Jakob disease associated with myocarditis.
A 77 year-old woman was admitted to a hospital with complaints of rapidly progressive visual disturbance, myoclonus and dementia.
Electroencephalogram revealed a periodic synchronous discharge and Creutzfeldt-Jakob disease was suspecte... | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 30,
"end": 55,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 30,
"end": 55
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Complication",
"start": 72,
"end": 83,
... |
prion_0546 | The case of a 66-year-old woman with atypical Creutzfeldt-Jacob disease (CJD) presents several peculiarities.
Pathologic examination confirmed a rare CJD case with primary generalized spongiform changes of the white matter and only moderate, but typical changes of the gray matter.
Besides an essentially typical clinica... | [
{
"id": "T2",
"label": "Age",
"start": 14,
"end": 25,
"text": "66-year-old",
"spans": [
{
"start": 14,
"end": 25
}
],
"is_discontinuous": null
},
{
"id": "T3",
"label": "Generic_Prion",
"start": 46,
"end": 71,
"text": "Creutzfeldt-J... |
prion_0549 | Sequential measurements of local cerebral glucose metabolism were performed in a case with Creutzfeldt-Jakob disease.
The diagnosis was based on the characteristic clinical symptoms and EEG.
Cerebral glucose metabolism (CMRGlc) was measured by positron emission tomography (PET) and 18F-fluorodeoxyglucose.
CMRGlc was de... | [
{
"id": "T45",
"label": "Anatomic_location",
"start": 33,
"end": 41,
"text": "cerebral",
"spans": [
{
"start": 33,
"end": 41
}
],
"is_discontinuous": null
},
{
"id": "T5",
"label": "Imaging_finding",
"start": 33,
"end": 60,
"text": ... |
prion_0550 | A forty-year-old female presented with an unsteady gait 13 years after receiving an eight-month course of human pituitary-derived gonadotrophin injections as treatment for infertility.
Over the next ten months the patient subsequently developed generalised myoclonic jerks and dementia and finally died.
Neuropathologica... | [
{
"id": "T1",
"label": "Age",
"start": 2,
"end": 16,
"text": "forty-year-old",
"spans": [
{
"start": 2,
"end": 16
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Symptom",
"start": 42,
"end": 55,
"text": "unsteady gait",
... |
prion_0552 | Familial occurrence of three definitive and two possible cases of Creutzfeldt-Jakob disease (CJD) with temporal and spatial separation in the area of focal CJD accumulation in Slovakia is reported.
Incubation period is 51 and 53 years respectively, if spatial and temporal separation of affected siblings is considered, ... | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 66,
"end": 91,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 66,
"end": 91
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Generic_Prion",
"start": 93,
"end": 96,
... |
prion_0553 | The spongiform encephalopathies encompass several diseases affecting humans and animals.
In the United States, the most common of these disorders in humans is Creutzfeldt-Jakob disease.
The most frequent manifestations include dementia, pyramidal tract signs, and extrapyramidal movement disorder.
Several clinically dis... | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 4,
"end": 31,
"text": "spongiform encephalopathies",
"spans": [
{
"start": 4,
"end": 31
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Generic_Prion",
"start": 159,
"end": 184,
... |
prion_0554 | We describe 10 neuropathologically verified patients with Creutzfeldt-Jakob disease who died in Argentina between 1980 and 1987.
Two of the ten cases were Chilean by birth.
Another case visited Chile several times.
Two cases (one Argentinian and one Chilean) regularly consumed sheep brain.
Ages ranged from 42 to 63 yea... | [
{
"id": "T1",
"label": "Autopsy",
"start": 15,
"end": 34,
"text": "neuropathologically",
"spans": [
{
"start": 15,
"end": 34
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Generic_Prion",
"start": 58,
"end": 83,
"text": "C... |
prion_0555 | A 70-year-old man developed a syndrome of progressive nondominant parietal and occipital dysfunction including palinopsia and a visual field defect.
Despite the marked focality of his clinical findings, radiologic studies were normal.
Myoclonus and ataxia began 6 weeks after onset of his illness at which time brain bio... | [
{
"id": "T1",
"label": "Age",
"start": 2,
"end": 13,
"text": "70-year-old",
"spans": [
{
"start": 2,
"end": 13
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],
"is_discontinuous": null
},
{
"id": "T5",
"label": "Symptom",
"start": 54,
"end": 100,
"text": "nondominant parietal... |
prion_0556 | Progressive aphasia without dementia (primary progressive aphasia) is increasingly recognized as an important neurobehavioral syndrome.
Clinical diagnosis of progressive aphasia is difficult early in its course, and the differential diagnosis is usually said to include Alzheimer's and Pick's diseases.
We report a 61-ye... | [
{
"id": "T1",
"label": "Symptom",
"start": 12,
"end": 19,
"text": "aphasia",
"spans": [
{
"start": 12,
"end": 19
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Differential_Diagnosis",
"start": 28,
"end": 36,
"text": "deme... |
prion_0557 | An autopsy case of Creutzfeld-Jacob disease with widespread amyloid plaques is reported.
A 45-year-old Japanese man, whose father had died of a similar disease, had a 5-year illness characterized by progressive cerebellar signs.
Mental changes and brain-stem signs developed in the late stage.
Myoclonus frequently occur... | [
{
"id": "T1",
"label": "Autopsy",
"start": 3,
"end": 10,
"text": "autopsy",
"spans": [
{
"start": 3,
"end": 10
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Generic_Prion",
"start": 19,
"end": 43,
"text": "Creutzfeld-Jaco... |
prion_0559 | Creutzfeldt-Jakob disease is a 'slow virus' illness usually presenting as a rapidly progressive dementia.
Although rare, its laboratory and accidental transmission has given it great theoretical importance.
However, the infective agent remains mysterious and it is not known how, or even whether, natural transmission oc... | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 0,
"end": 25,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 0,
"end": 25
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Symptom",
"start": 76,
"end": 104,
"text... |
prion_0561 | Two familial Italian cases of Creutzfeldt-Jakob disease are reported.
Clinical picture and life history are presented and compared: 1) with the findings in familial cases reported in other countries, 2) with the findings (age at onset, disease duration) in sporadic Italian cases. | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 30,
"end": 55,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 30,
"end": 55
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Age",
"start": 222,
"end": 234,
"text"... |
prion_0562 | We have presented an unusual case of CJD in which the magnetic resonance images obtained four months before death revealed high signal intensity in the caudate nuclei bilaterally.
These findings correspond to severe involvement of this region at autopsy.
This radiologic pattern has not generally been included in the di... | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 37,
"end": 40,
"text": "CJD",
"spans": [
{
"start": 37,
"end": 40
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Imaging_test",
"start": 54,
"end": 79,
"text": "magnetic res... |
prion_0563 | The authors present an analysis of the clinical course of 6 cases of Creutzfeldt-Jakob disease (patients were aged from 27 to 59 years).
The diagnosis was established during the life of the patients.
In the neurological status dementia and syndromes of pyramidal and extrapyramidal lesions predominated.
Neuropathologica... | [
{
"id": "T2",
"label": "Generic_Prion",
"start": 69,
"end": 94,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 69,
"end": 94
}
],
"is_discontinuous": null
},
{
"id": "T3",
"label": "Age",
"start": 110,
"end": 134,
"text"... |
prion_0564 | We reported an autopsy case of panencephalopathic type of Creutzfeldt-Jakob disease (CJD), one of whose early clinical signs, visual loss, had been documented by magnetic resonance imaging (MRI).
The patient was a 59-year-old woman, who showed disturbance of visual acuity, memory and calculation at the early stage.
Abo... | [
{
"id": "T1",
"label": "Autopsy",
"start": 15,
"end": 22,
"text": "autopsy",
"spans": [
{
"start": 15,
"end": 22
}
],
"is_discontinuous": null
},
{
"id": "T32",
"label": "Anatomic_location",
"start": 31,
"end": 49,
"text": "panencep... |
prion_0565 | Frequent serial EEG investigations of three patients with neuropathologically confirmed Creutzfeldt-Jakob disease lasting 13, 24 and 68 weeks revealed typical periodic activity of short duration with stereotyped bilateral sharp waves at the 7th, 8th, and 12th week, respectively, after the onset of symptoms.
During the ... | [
{
"id": "T1",
"label": "Electrophysio_test",
"start": 16,
"end": 34,
"text": "EEG investigations",
"spans": [
{
"start": 16,
"end": 34
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Generic_Prion",
"start": 88,
"end": 113,
... |
prion_0566 | We present the clinical findings in affected members of a large kindred with Gerstmann-Sträussler-Scheinker disease.
Sixty-four patients exhibited progressive ataxia, dementia, and parkinsonian features.
Inheritance appears to be autosomal dominant.
Impaired smooth-pursuit eye movements, defective short-term memory, cl... | [
{
"id": "T1",
"label": "GSS",
"start": 77,
"end": 115,
"text": "Gerstmann-Sträussler-Scheinker disease",
"spans": [
{
"start": 77,
"end": 115
}
],
"is_discontinuous": null
},
{
"id": "T3",
"label": "Symptom",
"start": 147,
"end": 165,
... |
prion_0567 | A 2-year-old girl had a craniopharyngioma removed in 1964.
She received human growth hormone (HGH) twice a week from July, 1972, until July, 1976.
In March, 1984, a subacute dementing illness developed with neurological signs that included pronounced cerebellar ataxia.
A clinical diagnosis of Creutzfeldt-Jakob disease ... | [
{
"id": "T1",
"label": "Age",
"start": 2,
"end": 12,
"text": "2-year-old",
"spans": [
{
"start": 2,
"end": 12
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Time_point",
"start": 53,
"end": 57,
"text": "1964",
"spans":... |
prion_0568 | While the incidence of Creutzfeldt-Jakob disease is very low worldwide, sufficient cases are appearing in neurological units to warrant increasing nursing information.
In this article epidemiology, etiology, clinical manifestations, and diagnosis, specific nursing diagnoses and suggested areas of intervention with spec... | [
{
"id": "T2",
"label": "Generic_Prion",
"start": 23,
"end": 48,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 23,
"end": 48
}
],
"is_discontinuous": null
}
] |
prion_0569 | A family cluster of the ataxic form of Creutzfeldt-Jakob disease with one probable and two autopsy proven cases that occurred in a single generation between 1974 and 1982 is reported.
The clinical characteristics of the cases are closely similar to those of kuru patients, with a fair correlation between the prominent t... | [
{
"id": "T11",
"label": "Symptom",
"start": 24,
"end": 35,
"text": "ataxic form",
"spans": [
{
"start": 24,
"end": 35
}
],
"is_discontinuous": null
},
{
"id": "T1",
"label": "Generic_Prion",
"start": 39,
"end": 64,
"text": "Creutzfe... |
prion_0570 | In an electron microscopic study of brain biopsy material from a case with Creutzfeldt-Jakob disease intranuclear vacuoles containing membrano-vesicular structures were found.
To our knowledge this finding has not been previously reported in human spongiform encephalopathies.
It may represent a specific alteration asso... | [
{
"id": "T9",
"label": "Anatomic_location",
"start": 36,
"end": 41,
"text": "brain",
"spans": [
{
"start": 36,
"end": 41
}
],
"is_discontinuous": null
},
{
"id": "T1",
"label": "Autopsy",
"start": 42,
"end": 48,
"text": "biopsy",
... |
prion_0571 | We report three patients with both spongiform encephalopathy and cerebellar amyloid plaques; one showed kuru-like plaques and was diagnosed as having Creutzfeldt-Jakob disease (CJD), and two had multicentric plaques and were diagnosed as having Gerstmann-Sträussler-Scheinker disease (GSSD).
Evaluation of these cases an... | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 35,
"end": 60,
"text": "spongiform encephalopathy",
"spans": [
{
"start": 35,
"end": 60
}
],
"is_discontinuous": null
},
{
"id": "T32",
"label": "Anatomic_location",
"start": 65,
"end": 7... |
prion_0573 | A case of Creutzfeldt-Jakob disease (CJD) is reported in a 28-year-old woman who had received a cadaveric dural graft 19 months earlier after resection of a cholesteatoma.
The circumstances of the case point to the graft as the most likely source of the disease.
Cadaveric dura should be added to the list of materials t... | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 10,
"end": 35,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 10,
"end": 35
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Generic_Prion",
"start": 37,
"end": 40,
... |
prion_0574 | In a retrospective study of Creutzfeldt-Jakob disease (CJD) in Italy from 1972 to 1986, we found 79 cases which fulfilled the diagnostic criteria for CJD.
The annual mortality rate was 0.09 cases per million inhabitants.
In this series the female to male ratio was 2.59, a value significantly higher than that found in I... | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 28,
"end": 53,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 28,
"end": 53
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Generic_Prion",
"start": 55,
"end": 58,
... |
prion_0575 | The concentration of cerebrospinal fluid (CSF) lactic acid was determined in three patients with biopsy proven Creutzfeldt-Jacob disease (CJD).
When compared with twenty demented patients diagnosed as having either Alzheimer's disease or multi-infarct dementia, the CSF lactic acid values of the CJD patients were signif... | [
{
"id": "T8",
"label": "Blood_biomarker_test",
"start": 21,
"end": 40,
"text": "cerebrospinal fluid",
"spans": [
{
"start": 21,
"end": 40
}
],
"is_discontinuous": null
},
{
"id": "T7",
"label": "Blood_biomarker_test",
"start": 42,
"end"... |
prion_0576 | A 77-year-old man suffered intermittent hemiconvulsions of unknown etiology on the left side for a period of about 5 weeks.
At the autopsy, there was marked neuronal loss, severe proliferation of astrocytes and spongiform changes in the right cerebral cortex.
The cerebral white matter showed loosening with astroglial p... | [
{
"id": "T2",
"label": "Age",
"start": 2,
"end": 13,
"text": "77-year-old",
"spans": [
{
"start": 2,
"end": 13
}
],
"is_discontinuous": null
},
{
"id": "T4",
"label": "Symptom",
"start": 27,
"end": 55,
"text": "intermittent hemiconv... |
prion_0577 | The author presents a case of a patient with Creutzfeldt-Jakob disease, a rapidly progressive encephalopathy that is characterized by early behavioral changes.
Because of the nature of this disease, the electroencephalogram (EEG) is helpful in establishing a diagnosis. | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 45,
"end": 70,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 45,
"end": 70
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Differential_Diagnosis",
"start": 74,
"end... |
prion_0578 | A 27-year-old man presented disturbances of gait and language, quickly followed by intellectual deterioration, tetraplegia, anarthria and myoclonus.
Histological examination of a cerebral biopsy showed not only cortical changes consistent with the diagnosis of Creutzfeldt-Jakob disease, but also many amyloid-plaques wi... | [
{
"id": "T2",
"label": "Age",
"start": 2,
"end": 13,
"text": "27-year-old",
"spans": [
{
"start": 2,
"end": 13
}
],
"is_discontinuous": null
},
{
"id": "T4",
"label": "Symptom",
"start": 28,
"end": 48,
"text": "disturbances of gait"... |
prion_0579 | We present a definite case of Creutzfeldt-Jakob disease (CJD) with visual evoked response (VER) and CSF oligoclonal band abnormalities and discuss the neurobiological significance and diagnostic value of these findings. | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 30,
"end": 55,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 30,
"end": 55
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Generic_Prion",
"start": 57,
"end": 60,
... |
prion_0580 | A 46-year-old female is described with prolonged, progressive dementia and a brain biopsy consistent with Creutzfeldt-Jakob disease (CJD).
She had neither myoclonic jerks nor an electroencephalogram with periodic spikes and suppression.
Five of her close relatives were also demented.
The nosology of CJD was discussed i... | [
{
"id": "T1",
"label": "Age",
"start": 2,
"end": 13,
"text": "46-year-old",
"spans": [
{
"start": 2,
"end": 13
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Symptom",
"start": 62,
"end": 70,
"text": "dementia",
"spans... |
prion_0582 | A clinically atypical, neuropathologically verified case of Creutzfeldt-Jakob disease is described in a 32-year-old New Zealand woman with idiopathic hypopituitarism who had been treated in late adolescence (1970 to 1973) with human growth hormone processed from pooled cadaveric pituitary glands. | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 60,
"end": 85,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 60,
"end": 85
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Age",
"start": 104,
"end": 115,
"text"... |
prion_0583 | A fourth histologically-confirmed American case of Creutzfeldt-Jakob disease (CJD) related to human growth hormone (hGH) therapy is reported.
Like kuru, the illness was dominated by cerebellar signs and relatively little mental deterioration.
The diagnosis was strongly supported premortem by the presence of two abnorma... | [
{
"id": "T11",
"label": "Autopsy",
"start": 9,
"end": 23,
"text": "histologically",
"spans": [
{
"start": 9,
"end": 23
}
],
"is_discontinuous": null
},
{
"id": "T1",
"label": "Generic_Prion",
"start": 51,
"end": 76,
"text": "Creutzf... |
prion_0584 | An adolescent girl with idiopathic hypothalamic dysfunction and hypopituitarism was treated with human growth hormone between 1969 and 1979, dying of parainfluenza pneumonia 2 months after her last hormone treatment.
Although she had no signs of progressive neurologic disease, reexamination of autopsy material revealed... | [
{
"id": "T1",
"label": "Treatment",
"start": 84,
"end": 117,
"text": "treated with human growth hormone",
"spans": [
{
"start": 84,
"end": 117
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Duration",
"start": 118,
"end": 139,... |
prion_0585 | The case histories are presented of two patients with Creutzfeldt-Jakob disease.
The clinical diagnosis was confirmed on autopsy.
A 51-year-old man and a 63-year-old woman developed--after a period of non-specific prodromal symptoms--a rapidly progressive dementia accompanied by cerebellar, pyramidal and extrapyramidal... | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 54,
"end": 79,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 54,
"end": 79
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Autopsy",
"start": 121,
"end": 128,
"t... |
prion_0586 | Decreased perfusion of the left frontal and left temporoparietal cortex has been shown in [123I] HIPDM planar and single photon emission computed tomographic images of a patient with Creutzfeldt-Jakob disease (CJD) that was proven by brain biopsy and subsequent autopsy.
An EEG showed diffuse, periodic discharges most p... | [
{
"id": "T24",
"label": "Imaging_finding",
"start": 0,
"end": 19,
"text": "Decreased perfusion",
"spans": [
{
"start": 0,
"end": 19
}
],
"is_discontinuous": null
},
{
"id": "T1",
"label": "Anatomic_location",
"start": 32,
"end": 39,
... |
prion_0587 | This is a report of a Chinese male, aged 67, who had been ill for 6 months and who was admitted with left hemiparesis and dementia.
The diagnosis of Creutzfeldt-Jakob disease (CJD) was confirmed by serial electroencephalographic (EEG), computed tomographic (CT) and neuropathological studies.
This is the first formal re... | [
{
"id": "T1",
"label": "Age",
"start": 36,
"end": 43,
"text": "aged 67",
"spans": [
{
"start": 36,
"end": 43
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Duration",
"start": 66,
"end": 74,
"text": "6 months",
"spans"... |
prion_0588 | A patient with Creutzfeldt-Jakob disease is described.
Many alternative psychiatric diagnoses were considered, and the true situation only became apparent late in the clinical course. | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 15,
"end": 40,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 15,
"end": 40
}
],
"is_discontinuous": null
},
{
"id": "T3",
"label": "Differential_Diagnosis",
"start": 72,
"end... |
prion_0589 | The hippocampus is generally believed to be only rarely affected in Creutzfeldt-Jakob disease.
In a systematic study of the hippocampus in 6 cases of Creutzfeldt-Jakob disease, the stratum moleculare-lacunosum showed a definite spongiform change with gliosis in 5 cases.
The stratum radiatum et pyramidale showed spongif... | [
{
"id": "T1",
"label": "Anatomic_location",
"start": 4,
"end": 15,
"text": "hippocampus",
"spans": [
{
"start": 4,
"end": 15
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Generic_Prion",
"start": 68,
"end": 93,
"text": "C... |
prion_0591 | This is a report of the case of a patient with Creutzfeldt-Jakob disease, whose electroencephalograms and polysomnograms were repeatedly recorded throughout the course of the illness with details of the alterations of periodic synchronous discharges.
In the advanced stage of the disease, the appearance of peculiar paro... | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 47,
"end": 72,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 47,
"end": 72
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Electrophysio_test",
"start": 80,
"end": 1... |
prion_0593 | The clinical and EEG findings in patients in the literature with Creutzfeldt-Jakob disease (CJD) were reviewed and compared with findings in 36 patients with CJD at the Massachusetts General Hospital (MGH).
Twenty-one of the 36 MGH cases had histopathology, all with findings consistent with CJD.
EEGs in 18 patients stu... | [
{
"id": "T1",
"label": "Electrophysio_test",
"start": 17,
"end": 20,
"text": "EEG",
"spans": [
{
"start": 17,
"end": 20
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Generic_Prion",
"start": 65,
"end": 90,
"text": "Creutz... |
prion_0594 | Two Chinese cases of Creutzfeldt-Jakob disease (CJD) are described which show the typical clinical, EEG and neuropathological changes of a subacute spongiform encephalopathy.
One case was sporadic and the other was probably familial.
This is the first formal report of CJD occurring in China. | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 21,
"end": 46,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 21,
"end": 46
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Generic_Prion",
"start": 48,
"end": 51,
... |
prion_0595 | We report the coincidence of pathologically confirmed sporadic CJD in two unrelated schoolteachers who shared a school wing for 9 months.
The first developed ataxia, tremulousness, and dementia 5 months after his last contact with his colleague.
Diagnosis of CJD was made 2 months later by brain biopsy.
Eight months lat... | [
{
"id": "T18",
"label": "Autopsy",
"start": 29,
"end": 43,
"text": "pathologically",
"spans": [
{
"start": 29,
"end": 43
}
],
"is_discontinuous": null
},
{
"id": "T1",
"label": "sCJD",
"start": 54,
"end": 66,
"text": "sporadic CJD",... |
prion_0598 | A case of Creutzfeldt-Jakob disease, Heidenhain's form is reported.
The first clinical manifestations were cortical blindness and visual agnosia.
The patient here concerned, a woman aged sixty three, during the clinical course of the disease showed mental deterioration and pyramido-extrapyramidal manifestations.
She di... | [
{
"id": "T1",
"label": "Generic_Prion",
"start": 10,
"end": 35,
"text": "Creutzfeldt-Jakob disease",
"spans": [
{
"start": 10,
"end": 35
}
],
"is_discontinuous": null
},
{
"id": "T10",
"label": "sCJD",
"start": 37,
"end": 54,
"text"... |
prion_0599 | We describe a kindred with 7 confirmed and 2 probable cases of subacute dementia accompanied by myoclonus.
The inheritance pattern is consistent with autosomal dominance and shows anticipation.
The pathological changes involve marked gliosis with neuronal loss of the dorsomedial and midline thalamic nuclei, with lesser... | [
{
"id": "T7",
"label": "Symptom",
"start": 72,
"end": 80,
"text": "dementia",
"spans": [
{
"start": 72,
"end": 80
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Differential_Diagnosis",
"start": 96,
"end": 105,
"text": "my... |
prion_0601 | In this consecutive series of 230 patients with neuropathologically verified Creutzfeldt-Jakob disease (CJD), the disease was found to affect men and women with approximately equal frequency in a peak plateau between the ages of 55 and 75 years (mean, 61.5 years).
Familial cases accounted for 4 to 8% of the total serie... | [
{
"id": "T1",
"label": "Autopsy",
"start": 48,
"end": 67,
"text": "neuropathologically",
"spans": [
{
"start": 48,
"end": 67
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Generic_Prion",
"start": 77,
"end": 102,
"text": "... |
prion_0602 | Magnetic resonance (MR) imaging and CT of three patients with Creutzfeldt-Jakob disease (CJD) showed bilateral cortical atrophy and no apparent white matter changes.
Serial examinations revealed the progressive nature of the atrophy, findings compatible with the patients' clinical deterioration.
At autopsy some white m... | [
{
"id": "T16",
"label": "Imaging_test",
"start": 0,
"end": 31,
"text": "Magnetic resonance (MR) imaging",
"spans": [
{
"start": 0,
"end": 31
}
],
"is_discontinuous": null
},
{
"id": "T2",
"label": "Imaging_test",
"start": 36,
"end": 38,... |
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