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颈项强直,弯曲时疼痛。
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有脑膜刺激征和脑脊液中白细胞数增高。
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如疾病终止,无瘫痪出现,称为无瘫痪型。
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(三)瘫痪期肌肉瘫痪大都于瘫痪前期的第3~4天开始,随发热而加重,大都经过5~10天。
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一般热退后瘫痪不再进展。
[ { "id": 0, "entity": "瘫痪", "start_offset": 5, "end_offset": 7, "label": "sym" } ]
依据主要病变部位分为下列数型:1.脊髓型最常见,为脊髓前角细胞受损所致,表现为分布不对称、不规则的弛缓性肌肉瘫痪,四肢多见,下肢尤甚。
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感觉存在。
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膀胱肌麻痹时则有尿潴留和尿失禁。
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2.延髓型(脑干型或球型)出现呼吸深浅不匀、节律不齐和各种异样呼吸;心动过速或过缓、血压下降、循环衰竭;侵犯脑神经则产生各种相应症状。
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3.脑炎型偶见。
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急起高热、嗜睡、昏迷和惊厥,可有痉挛性肢体瘫痪。
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4.混合型为脊髓型和延髓型同时存在。
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(四)恢复期瘫痪后1~2周,病肌逐渐恢复功能,常以足趾为起点,然后上升至胫、股部。
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膝腱反射也渐回复。
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(五)后遗症期由于神经组织损害严重,瘫痪不易恢复,受累肌群萎缩,造成躯肢畸形,如马蹄内翻足、脊柱弯曲等后遗症。
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【并发症】少数患者在急性期有心肌损害。
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呼吸肌麻痹和呼吸中枢受累患者因呼吸不畅可并发肺炎、肺不张等。
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尿潴留者易继发泌尿系感染。
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严重瘫痪长期卧床者易有褥疮、骨质脱钙,甚至肾结石。
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尚可有麻痹性肠梗阻和胃扩张等胃肠道并发症。
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【实验室检查】(一)脑脊液检查前驱期脑脊液正常;瘫痪前期细胞数轻度增加,早期以中性粒细胞为主,后期以淋巴细胞为主;热退后细胞数恢复。
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蛋白质在早期多数正常,瘫痪后第2周蛋白质水平常增高,此时细胞数已正常,呈细胞和蛋白质分离现象,对诊断本病有一定帮助。
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蛋白质水平到第4~10周恢复正常。
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(二)病毒分离起病1周内可从咽部和粪便内分离出病毒,早期从血液和脑脊液中有时也能检出病毒。
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需用标准抗血清中和试验鉴定血清型。
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(三)血清学检查前驱期末,特异性IgG抗体出现,补体结合抗体持续时间较短,中和抗体持续较久。
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双份血清抗体滴度呈≥4倍以上增高有诊断意义。
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对顿挫型和无瘫痪型患者,只能依靠病原学检查结果诊断。
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瘫痪患者需要和下列疾病鉴别:(一)急性感染性多发性神经根炎表现为四肢弛缓性瘫痪,常从下肢开始,然后发展到上肢、腰背及躯干。
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病初就有肢体感觉障碍。
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脑脊液中细胞数正常而蛋白质特高。
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(二)其他肠道病毒所致瘫痪,接受过有效灰髓炎疫苗预防的患者如出现类似本病的瘫痪,应进行病原学检查来确诊。
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最初1周强调呼吸道和消化道隔离。
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(二)被动免疫暴露前肌内注射人丙种球蛋白0.3~0.5ml/kg,有一定保护作用。
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(三)主动免疫按计划普遍服用灰髓炎减毒活疫苗。
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在流行发生时,对周围的易感儿及时施行疫苗预防可中断流行。
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(四)急性期的治疗1.前驱期和瘫痪前期治疗患者宜卧床休息以减少麻痹的发生和进展。
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有肌痛者可作局部湿热敷或口服镇痛剂。
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静脉注射适量50%葡萄糖液和维生素C可减少神经系统水肿。
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口服地巴唑可促进神经系统功能的恢复。
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2.瘫痪期治疗注射加兰他敏可促进肌肉的张力。
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将瘫痪的肢体置于功能位,以防止畸形发生。
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有呼吸肌麻痹时,应保持呼吸道通畅,并给予氧气吸入和各种辅助呼吸。
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呼吸中枢或循环中枢受累时按呼吸衰竭或循环衰竭抢救措施进行。
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3.后遗症期治疗于体温降至正常、病情进入恢复期时就应加强瘫痪肌群的功能锻炼,并作针刺、推拿、按摩、理疗等康复治疗,以促进瘫痪肌群的恢复。
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遗有畸形时,可手术矫治。
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十二、腺泡型软组织肉瘤腺泡型软组织肉瘤(alveolarsoftpartsarcoma)是一临床-病理实体,小儿少见,多见于15岁左右的青少年,女性多于男性。
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肿瘤无明显界限质软和易碎。
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切面呈黄白或灰红色,中央含坏死和出血区,肿瘤光镜下致密的小梁将肿瘤分为大小不同的腔隙腔隙中有界限分明的瘤细胞岛瘤细胞中有坏死和脱落肿瘤在儿童好发于头颈,尤其是眼眶和舌。
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因肿瘤内血管丰富,有时可摸及血管搏动,故一般无症状,而易忽视。
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该肿瘤早期就可向肺、脑、骨骼转移。
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淋巴结转移少见。
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治疗以根治手术切除原发肿瘤及转移灶。
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儿童预后较成人佳,可能因头面部肿瘤易于发现的缘故。
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二、急性咽炎急性咽炎(acutepharyngitis)是咽黏膜、黏膜下组织和淋巴组织的急性炎症。
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单纯性咽炎以学龄前儿童多见,婴儿期较少。
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1.疱疹性咽炎(herpangina)系柯萨奇A组病毒引起。
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急性起病,表现高热、咽痛、流涎、厌食、呕吐等,咽部充血,咽腭弓、腭垂、软腭等处可伴有2~4mm大小的疱疹,周围有红晕,破溃后形成小溃疡.病程1周左右。
[ { "id": 0, "entity": "高热", "start_offset": 7, "end_offset": 9, "label": "sym" }, { "id": 1, "entity": "咽痛", "start_offset": 10, "end_offset": 12, "label": "sym" }, { "id": 2, "entity": "流涎", "start_offset": 13, "end_offset": 15, "label": "sym" },...
2.咽-结合膜热(pharyngo-conjunctivitis)由腺病毒3、7型引起。
[ { "id": 0, "entity": "咽-结合膜热", "start_offset": 2, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "pharyngo-conjunctivitis", "start_offset": 9, "end_offset": 32, "label": "dis" }, { "id": 2, "entity": "腺病毒3、7型", "start_offset": 34, "end_offset"...
以发热、咽炎、结合膜炎为特点,表现高热、咽痛、眼部刺痛、咽部充血、一侧或两侧滤泡性眼结合膜炎,可伴有胃肠道症状、颈部、耳后淋巴结肿大。
[ { "id": 0, "entity": "发热", "start_offset": 1, "end_offset": 3, "label": "dis" }, { "id": 1, "entity": "咽炎", "start_offset": 4, "end_offset": 6, "label": "dis" }, { "id": 2, "entity": "结合膜炎", "start_offset": 7, "end_offset": 11, "label": "dis" }, ...
3.链球菌咽炎(streptococcalpharyngitis)以5岁以上高发。
[ { "id": 0, "entity": "链球菌咽炎", "start_offset": 2, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "streptococcalpharyngitis", "start_offset": 8, "end_offset": 32, "label": "dis" } ]
早期可伴有颈前淋巴结肿大伴触痛扁桃体广泛充血、软腭表面有瘀点、瘀斑提示链球菌感染。
[ { "id": 0, "entity": "颈前淋巴结", "start_offset": 5, "end_offset": 10, "label": "bod" }, { "id": 1, "entity": "颈前淋巴结肿大伴触痛", "start_offset": 5, "end_offset": 15, "label": "sym" }, { "id": 2, "entity": "扁桃体", "start_offset": 15, "end_offset": 18, "label"...
第四章脑积水脑积水(hydrocephalus)系指脑脊液的分泌、循环或吸收过程发生障碍,导致颅内脑脊液增多,引起脑室和(或)蛛网膜下腔异常扩大过量的脑脊液产生颅内压增高,因而扩大了正常脑脊液所占有的空间脑积水在颅缝闭合之前发生,则头颅增大异常显著脑积水称为先天性脑积水,在出生后有明确病因产生的脑积水称为后天性(获得性)脑积水。
[ { "id": 0, "entity": "脑积水", "start_offset": 3, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "脑积水", "start_offset": 6, "end_offset": 9, "label": "dis" }, { "id": 2, "entity": "hydrocephalus", "start_offset": 10, "end_offset": 23, "label":...
由于各种原因引起脑实质本身先发生萎缩而后使脑室和蛛网膜下腔扩大,脑脊液容量相对增加脑积水的范畴。
[ { "id": 0, "entity": "脑室", "start_offset": 21, "end_offset": 23, "label": "bod" }, { "id": 1, "entity": "蛛网膜", "start_offset": 24, "end_offset": 27, "label": "bod" }, { "id": 2, "entity": "脑脊液", "start_offset": 32, "end_offset": 35, "label": "bod" ...
【流行病学】先天性脑积水是最常见的先天神经系统畸形疾病之一,据WHO对24个国家的统计报告,其发病率为0.87‰,男女无明显差别。
[ { "id": 0, "entity": "先天性脑积水", "start_offset": 6, "end_offset": 12, "label": "dis" }, { "id": 1, "entity": "先天神经系统畸形", "start_offset": 17, "end_offset": 25, "label": "dis" } ]
我国1996—2007年31个省市自治区出生缺陷监测资料表明,先天性脑积水的发病率为0.68‰,仅次于神经管畸形。
[ { "id": 0, "entity": "出生缺陷", "start_offset": 20, "end_offset": 24, "label": "dis" }, { "id": 1, "entity": "先天性脑积水", "start_offset": 31, "end_offset": 37, "label": "dis" }, { "id": 2, "entity": "神经管畸形", "start_offset": 51, "end_offset": 56, "label":...
由于后天性脑积水是出生后多种原因引起的一种病理结果,则其确切发病率很难统计。
[ { "id": 0, "entity": "后天性脑积水", "start_offset": 2, "end_offset": 8, "label": "dis" } ]
随着医学科学技术的进步,先天性或后天性脑积水的发病率呈降低趋势。
[ { "id": 0, "entity": "先天性或后天性脑积水", "start_offset": 12, "end_offset": 22, "label": "dis" } ]
根据我国2004—2007年出生缺陷监测网资料,全国先天性脑积水的发病率有明显下降趋势,下降幅度为16.4%,年下降速率为9.0%,且城市较农村下降明显。
[ { "id": 0, "entity": "出生缺陷", "start_offset": 14, "end_offset": 18, "label": "dis" }, { "id": 1, "entity": "先天性脑积水", "start_offset": 26, "end_offset": 32, "label": "dis" } ]
【病理生理】正常情况下,脑脊液在脑室系统和蛛网膜下腔内不断地循环、代谢,其分泌和吸收速度处于动态平衡,从而维持颅内脑脊液容量的相对稳定。
[ { "id": 0, "entity": "脑脊液", "start_offset": 12, "end_offset": 15, "label": "bod" }, { "id": 1, "entity": "脑室", "start_offset": 16, "end_offset": 18, "label": "bod" }, { "id": 2, "entity": "蛛网膜下腔", "start_offset": 21, "end_offset": 26, "label": "bod...
脑脊液的产生主要来自各个脑室特别是侧脑室的脉络丛,约占80%~85%,少数由室管膜上皮渗出,在小儿每分钟产生脑脊液0.3~0.35ml(平均每小时20ml)。
[ { "id": 0, "entity": "脑脊液", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "脑室", "start_offset": 12, "end_offset": 14, "label": "bod" }, { "id": 2, "entity": "侧脑室", "start_offset": 17, "end_offset": 20, "label": "bod" ...
脑脊液在脑室生成后,以约1.47kPa(150mmH2</sub>O)液体静水压循环流动。
[ { "id": 0, "entity": "脑脊液", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "脑室", "start_offset": 4, "end_offset": 6, "label": "bod" } ]
左右两侧侧脑室产生的脑脊液,经室间孔流入第三脑室,与第三脑室产生的脑脊液一起经中脑导水管(又称大脑导水管)流入第四脑室,再与第四脑室产生的脑脊液一起经正中孔和两个侧孔流出而进入蛛网膜下腔的小脑延髓池。
[ { "id": 0, "entity": "侧脑室", "start_offset": 4, "end_offset": 7, "label": "bod" }, { "id": 1, "entity": "脑脊液", "start_offset": 10, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "室间孔", "start_offset": 15, "end_offset": 18, "label": "bod" ...
蛛网膜下腔的脑脊液,向上循环到脑表面,最后通过蛛网膜绒毛(颗粒)被动地吸收入硬膜静脉窦(上矢状窦),这是脑脊液吸收的主要途径。
[ { "id": 0, "entity": "蛛网膜下腔", "start_offset": 0, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "脑脊液", "start_offset": 6, "end_offset": 9, "label": "bod" }, { "id": 2, "entity": "脑表面", "start_offset": 15, "end_offset": 18, "label": "bod" ...
一小部分的脑脊液进入脊髓蛛网膜下腔,由脊髓静脉的蛛网膜绒毛吸收入血液。
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另有少量脑脊液可通过脑室的室管膜上皮、蛛网膜下腔的毛细血管、脑膜的淋巴管以及颅神经出颅处的蛛网膜鞘等结构吸收。
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脑脊液经上述途径不断地回到静脉中去,形成了脑脊液的循环。
[ { "id": 0, "entity": "脑脊液", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "静脉", "start_offset": 13, "end_offset": 15, "label": "bod" }, { "id": 2, "entity": "脑脊液", "start_offset": 21, "end_offset": 24, "label": "bod" ...
产生脑积水可能有三种情况:①脑脊液产生过多,这种情况极少;②脑脊液吸收发生障碍脑脊液循环发生障碍脑积水病例属于这类。
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【分类与病因】(一)分类临床上习惯于将脑积水分为交通性(非阻塞性)和非交通性(阻塞性)两种类型,这是根据解剖学的分类。
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由于脑室系统内的循环通路阻塞引起的脑积水称为非交通性脑积水,在临床上以此型脑积水多见。
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(二)病因脑积水的原因很多,主要为脑脊液循环通路的阻塞。
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常见原因为中脑导水管阻塞、颅内肿瘤的压迫及各种原因引起的蛛网膜粘连先天性脑积水系因脑脊液循环阻塞非交通性脑积水。
[ { "id": 0, "entity": "蛛网膜", "start_offset": 28, "end_offset": 31, "label": "bod" }, { "id": 1, "entity": "蛛网膜粘连", "start_offset": 28, "end_offset": 33, "label": "sym" }, { "id": 2, "entity": "先天性脑积水", "start_offset": 33, "end_offset": 39, "label": ...
1.脑室系统内的阻塞(1)先天性畸形1)室内孔闭锁。
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2)中脑导水管狭窄、分叉、胶质增生和隔膜形成中脑导水管的阻塞先天性脑积水最常见的原因。
[ { "id": 0, "entity": "中脑导水管", "start_offset": 2, "end_offset": 7, "label": "bod" }, { "id": 1, "entity": "中脑导水管狭窄、分叉、胶质增生和隔膜形成", "start_offset": 2, "end_offset": 22, "label": "sym" }, { "id": 2, "entity": "中脑导水管", "start_offset": 22, "end_offset": 27, ...
3)Dandy-Walker畸形:由于第四脑室正中孔及侧孔先天性闭塞而引起脑积水。
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6)其他:无脑回畸形,软骨发育不良,脑穿通畸形,第五、六脑室囊肿等,均可发生脑积水。
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2.脑室系统外的阻塞(1)出血:如蛛网膜下腔出血后引起的纤维增生。
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(2)炎症:如化脓性、结核性或其他类型脑膜炎,由于增生的纤维组织引起脑底部蛛网膜粘连脑脊液循环通路。
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(3)脑膜癌。
[ { "id": 0, "entity": "脑膜癌", "start_offset": 3, "end_offset": 6, "label": "dis" } ]
(4)颅内手术后。
[ { "id": 0, "entity": "颅内手术", "start_offset": 3, "end_offset": 7, "label": "pro" } ]
3.脑脊液的分泌和吸收障碍(1)脑脊液产生过多脉络丛乳头状瘤。
[ { "id": 0, "entity": "脑脊液", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "脑脊液", "start_offset": 16, "end_offset": 19, "label": "bod" }, { "id": 2, "entity": "脑脊液产生过多", "start_offset": 16, "end_offset": 23, "label": "sy...
2)维生素缺乏。
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(2)脑脊液吸收障碍静脉窦压力增高静脉窦血栓颈静脉血栓上腔静脉阻塞先天性蛛网膜颗粒发育不良。
[ { "id": 0, "entity": "脑脊液", "start_offset": 3, "end_offset": 6, "label": "bod" }, { "id": 1, "entity": "脑脊液吸收障碍", "start_offset": 3, "end_offset": 10, "label": "sym" }, { "id": 2, "entity": "静脉窦", "start_offset": 10, "end_offset": 13, "label": "bod...
【临床表现】临床症状并不一致,与脑积水病理变化出现的年龄、病情的轻重和病程的长短有关。
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(一)头颅改变先天性脑积水。
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头颅进行性异常增大,与全身的发育不成比例伴囟门扩大、颅缝裂开以及颅骨变薄变软先天性脑积水的特有体征。
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婴幼儿患脑积水时行头颅透光试验可呈阳性,枕部局限性透照光圈增大提示为Dandy-Walker畸形,头颅广泛透光则见于严重脑积水。
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(二)颅内压增高的表现在婴幼儿,颅内压增高的一般症状多不明显,但可见囟门隆起,张力增高,头皮静脉怒张脑积水进展较快时,亦可出现反复呕吐。
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其中头痛常在卧床休息较久时加重,故常有早晨头痛而起床活动后消失的现象,可能是活动促使脑脊液通过狭窄部位所造成。
[ { "id": 0, "entity": "头痛", "start_offset": 2, "end_offset": 4, "label": "sym" }, { "id": 1, "entity": "脑脊液", "start_offset": 42, "end_offset": 45, "label": "bod" } ]
当脑积水发展缓慢、脑室扩大颅内压增高较慢时,可以只表现为头痛、个性和情绪的改变,或者出现展神经麻痹而使眼球内斜颅内压增高症。
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