资源描述
CNⅠ: Olfactory Nerve (嗅神经)
Foster-Kennedy syndrome: Frontal lobe tumors cause optic atrophy on the side of the tumor, papilledema & smell on the opposite side.见于额叶底部肿瘤,表现为病变侧因肿瘤压迫而视神经萎缩,病变对侧因高颅压而视乳头水肿和嗅觉减退
Optic Nerve (视神经)
Clinical Correlation视力障碍&视野缺损
• Optic nerve -Ipsilateral visual loss(同侧全盲)
• Optic chiasm - Bitemporal hemianopsia (双颞侧偏盲)
• Optic tract - Contralateral incongruous homonymous hemianopsia (对侧视野同向偏盲)
• Optic radiations - Quadrantic field defect(象限盲)
1.视辐射全损坏:对侧视野同向偏盲
2.视辐射下部损害(颞叶):对侧视野同向上象限盲
3.视辐射上部损害(顶叶):对侧视野同向下象限盲
• Occiptal lobe - cortical blindness/Contralateral congruous homonymous hemianopia
• 对侧视野同向偏盲,对光反射存在 & macular sparing黄斑回避
Oculomotor Nerve/III (动眼神经)
外侧核:上睑提肌&上直肌(上&内)/下直肌(下&内)/内直肌(内)/下斜肌(上&外)
正中核:内直肌(辐辏运动)
E-W核:瞳孔括约肌&睫状肌(缩瞳&调节反射)
Complete damage symptoms:
1. 上睑下垂Ptosis
2. 外下斜视/分离性斜视Divergent strabismus
3. 瞳孔散大Dilated pupil
4. 调节反射&对光反射消失Loss of light and accommodation reflex
5. 复视Diplopia
Trochlear Nerve / IV (滑车神经)
上斜肌:下&外
Abducens paralysis/ VI (外展神经)
外直肌:外
Internuclear ophthalmoplegia核间性麻痹/medial longitudinal fasciculus syndrome内侧纵束综合征
Reflects disconnection of the oculomotor nucleus from the contralateral lateral gaze centre
When attempting to look the contralateral side, Ipsilateral eye fails to adduct, contralateral eye abduct with nystagmus, convengence reflex is normal.
脑桥侧视中枢&动眼神经核之间内侧纵束上行纤维病变
患侧眼不能内收,对侧眼球外展伴有眼震,辐辏反射正常
2.后核间性眼肌麻痹
脑桥侧视中枢&外展神经核之间内侧纵束下行纤维病变
患侧眼不能外展,对侧眼球内收正常(伴有眼震?),辐辏反射正常
3.一个半综合征one and a half syndrome
一侧脑桥侧视中枢&对侧交叉来的内侧纵束同时受累
患侧眼球不能内收&外展,对侧眼球不能内收,外展时伴有眼震
Uppernuclear ophthalmoplegia核上性麻痹
1.侧向凝视麻痹
1)皮质侧视中枢(额中回后部)受损,双眼向病灶侧共同偏视
Lobe gaze centre injure, fail to attempting to look the contralateral side.
2)皮质下侧视中枢(脑桥)受损,双眼向病灶对侧共同偏视
2.垂直凝视麻痹
皮质下垂直同向运动中枢(上丘-上半司向上运动,下半司向下运动)受损,双眼垂直运动障碍;
核上性麻痹共同特点:
No Diplopia 无复视
two eyes involved双眼同时受累;
reflex movement exist麻痹性眼肌的反射性运动仍保存(不能随意向一侧运动,但声响可产生转向该侧的反射活动)
Horner’s syndrome
The descending sympathetic tract由颈上交感神经径路损害所致
Lesions result in ipsilateral病变同侧:
1.miosis / Small pupil瞳孔缩小
2.mild ptosis眼睑下垂
3.hemi-anhidrosis / loss of sweating in lesion side损面无汗
4.apparent enophthalmos眼球内陷
Trigeminal Nerve三叉神经
周围分布VS中枢分布
Peripheral:眼支ophthalmic nerve &上颌支maxillary nerve &下颌支mandibular nerve,破坏性症状表现为分布区域痛觉、温觉、触觉均减弱或消失,角膜反射减弱或消失,咀嚼肌瘫痪
Central:三叉神经脊束核(痛温觉),病变后表现为同侧面部呈剥洋葱样分离性感觉障碍,痛温觉缺失而触觉存在(部分三叉神经脊束核),咀嚼肌无力、萎缩、张口时下颌偏斜(三叉神经运动核)
facial nerve 面神经
双侧皮质脑干束调控:上部面肌-额肌&皱眉肌&眼轮匝肌
对侧皮质脑干束调控:下部面肌-颧肌&颊肌&口轮匝肌&颈阔肌
Lower Motor Neuron (LMN) Lesion周围性
下运动神经元损伤:面神经核/周围神经
Upper Motor Neuron (UMN) Lesion 中枢性
上运动神经元损伤:一侧中央前回下部/皮质脑干束
Lesions on the iplilateral: 病灶同侧
Marked facial asymmetry & Atrophy of facial muscles
Eyelid droop眼睑下垂
Uncontrolled tearing角膜干燥反射性导致泪腺分泌
cannot close eye or Bell sign眼睑闭合无力,若用力闭眼,眼球向外上方转动,暴露出巩膜
Smoothing out of forehead and nasolabial folds 鼻唇沟变浅
Drooping of the mouth corner口角下垂
Lips cannot be held tightly together or pursed鼓腮漏气
Difficulty keeping food in mouth while chewing on the affected side
Hunt syndrome
Lesions on the contralateral: 病灶对侧
lower portion of the face muscles paralysis下部面肌瘫痪
Smoothing out of forehead and nasolabial folds 鼻唇沟变浅
Drooping of the mouth corner口角下垂
No eyelid droop无眼睑下垂
Intact folds on forehead 额纹对称
Vestibulocochlear Nerve 前庭蜗神经/位听神经
Glossopharyngeal Nerve & Vagus Nerve舌咽神经&迷走神经
二者有:共同的神经核(疑核&孤束核)、共同的走行、共同的分布
Bulbar palsy真性球麻痹
Pseudobulbar Palsy 假性球麻痹
Impairment of nuclei or cranial nerves of IX & X & XII, bilateral or unilateral
jaw jerk absence下颌反射消失
gag reflex absence咽反射消失
atrophy of tongue 舌肌萎缩
fasciculations in bulbar muscles肌束震颤
Dysarthria 构音困难
dysphonia 发音困难
dysphagia 吞咽困难
normal emotions
disturbance of bilateral corticobulbar tracts (皮质延髓束)
jaw jerk active 下颌反射存在(亢进)
gag reflex active咽反射存在
apparent weakness of muscles of mastication 咀嚼肌无萎缩
spastic dysarthria 痉挛性构音障碍
palatal weakness and difficulty in swallowing 吞咽困难
tongue immobile, pointed and cannot protrude 不能伸舌
emotional lability强哭强笑
bilateral limb upper motor neurone signs 双侧锥体束征
Hypoglossal Nerve舌下神经
只受对侧皮质脑干束支配
Unilateral Hypoglossal Nerve Lesion单侧核性或神经性受损
Unilateral Supranuclear Lesion单侧核上性受损
Deviating toward the side of nerve lesion.舌头偏向神经损伤同侧
Ipsilateral side atrophic同侧舌肌萎缩
Fasciculations 肌束颤动
Deviating toward the contralateral side舌头偏向神经损伤对侧
Without atrophy and fasciculations 无舌肌萎缩和肌束颤动
上运动神经元&下运动神经元 瘫痪鉴别
Cerebral hemisphere大脑半球(额叶、顶叶、枕叶、颞叶、岛叶、边缘叶)
Frontal lobe额叶(主要与随意运动和高级精神活动有关,有运动中枢:中央前回-锥体束,运动前区-锥体外系)
1.Right hemiplegia右侧偏瘫(中央前回运动中枢)
2.head and eyes turned to the left头眼转向对侧损坏病灶(额中回后部侧视中枢)
3.inapproprite social behavior行为异常,personality change人格改变(额极损害)
4.loss of initiative定向力丧失, dementia痴呆
5.release of sucking and grasping reflexes吮吸强握(对随意运动失去控制能力所致,额上回后部近中央前回)
Grasp reflex & groping reflex 强握反射&摸索反射
6.gait apraxia共济失调步态/额叶性共济失调(运动前区的额-桥-小脑束,病变对侧下肢运动笨拙、步态蹒跚)
7.sphincteric incontinence二便失禁(旁中央小叶)
8.Broca’s expressive aphasia运动性失语(优势半球额下回后部/Broca区损害,表现为口语表达障碍,患者能理解语言的意义,但不能用言语表达或表达不完整)
9.agraphia书写不能(额中回后部书写中枢损伤)
10. Foster-Kennedy syndrome: Frontal lobe tumors cause optic atrophy on the side of the tumor, papilledema & anosmia on the opposite side.见于额叶底部肿瘤,表现为病变侧因肿瘤压迫而视神经萎缩,病变对侧因高颅压而视乳头水肿和嗅觉减退
Parietal lobe顶叶(感觉中枢、运用中枢、视觉语言中枢)
1.hemihypoesthesia偏身感觉减退:复合性感觉为主的感觉障碍,如实体觉、位置觉、两点辨别觉、皮肤定位觉丧失,但一般感觉正常;刺激性病变则出现病灶对侧局限性感觉性癫痫发作
2.astereognosis体象障碍(非优势半球受累,自体认识不能autotopagnosia&病觉缺失anosognosia)
3.Gerstmann’s syndrome古兹曼综合征(优势半球顶叶角回损害,dyscalculia失算症/计算不能,finger agnosia手指失认症/不能辨别手指,right&left confusion左右失认症/不能辨别左右,dysgraphia失写症/书写不能)
4.apraxia失用症(优势半球缘上回为运用功能皮质代表区,损害则出现肢体动作运用障碍,分为运动性/观念性/结构性/观念运动性)
Temporal lobe颞叶(与听觉、语言、记忆和精神活动有关,包括听觉中枢、感觉性语言中枢、嗅味觉中枢)
1. contralateral homonymous upper quandrantanopia对侧视野同向上偏盲(颞叶深部的视放射和视束受损)
2. hearing impairments听觉障碍(颞横回听觉中枢),钩回发作(幻嗅&幻味)heterosmia嗅觉异常parageusia味觉异常(颞叶钩回嗅味觉中枢损害)
3.Wernicke’s receptive aphasia感觉性失语(颞上回后部/Wernicke区语言中枢受损,能听见说话声音,能自言自语,但不能理解他人&自己说话的含义),anomic aphasia命名性失语/健忘性失语(颞中、下回后部受损)
Akinetic mutism无动性缄默症
又称睁眼昏迷,患者对外界刺激无意识反应,四肢不能动,可呈不典型去脑强直状态,可无目的睁眼或眼球运动,觉醒-睡眠周期保留或睡眠过度,可伴高热、心律不齐、尿便潴留或失禁等自主神经功能紊乱,肌肉松弛,无锥体束征。
为脑干上部或丘脑的网状激活系统及前额叶-边缘系统损害所致。
• appears alert, but is silent and does not move spontaneously or respond to external stimuli. (貌似清醒,不动,对刺激无反应)
• This arises from bilateral frontal lobe damage, brainstem or the cortico-reticular pathways are interrupted but the motor and sensory pathways spared. (双侧胼胝体前部及额叶损害,网状结构中断,但运动感觉通路正常 )
Internal capsule内囊
2.前肢:额桥束、丘脑前辐射
3.膝部:皮质脑干束
4.后肢:皮质脊髓束(前2/3,上肢靠前,下肢靠后)、丘脑皮质束(后1/3)、视辐射、听辐射
5.损害时:“三偏”综合征:对侧偏盲,偏瘫,偏身感觉障碍Contralateral hemiparesis, hemianopia, hemidysesthesia;
Basal ganglia基底节/basal nucleus基底核(与大脑&小脑协同调节:随意运动&肌张力&姿势&复杂行为活动)
diencephalon间脑(丘脑、下丘脑、上丘脑、底丘脑)
Thalamus丘脑综合征
1.对侧的偏身感觉障碍(感觉传导纤维经丘脑腹后外侧核):impaired of all sensory in contralateral side
所有感觉均障碍、深感觉&精细触觉>浅感觉、肢体&躯干>面部、可出现感觉性共济失调or感觉异常
2.对侧半身自发性疼痛/丘脑痛
部位不定、性质不定、情绪影响、自主功能受损、止痛剂无效
3.对侧意向性震颤、不自主运动、偏身共济失调;
4.对侧面部表情运动障碍
5.丘脑痴呆,有强迫性哭笑倾向、记忆下降、人格改变、情感障碍;
6.丘脑语言,言语缓慢、重复、发音困难、复述差;
7.精神障碍,如情绪不稳、情感淡漠、情绪低落等
hypothalamus下丘脑(神经内分泌&自主神经系统整合中枢-摄食,体温,情绪,睡眠,生殖,水盐平衡,内脏活动,垂体功能)
epithalamus上丘脑:(缰核.缰连合.后连合.松果体)
Parinaud’s syndrome四叠体综合症: It’s often the result of a pinealoma pressing the epithalamus and subthalamus(上下丘) causing eyes upward or downward gaze paralysis, loss of light reflect action, sensorineural hearing loss, cerebellar ataxia, may accompanied with high ICP.由松果体肿瘤压迫四叠体(上丘,下丘)&中脑导水管所致,表现为对光反射消失(上丘臂受累)、垂直凝视麻痹(上丘受累)、神经性耳聋(下丘受累)、小脑共济失调(结合臂受累),可伴高颅压症状。
Brain stem脑干(中脑、脑桥、延髓)
brain stem---Midbrain中脑
(1).Weber syndrome大脑桥综合症 3对瘫
病变位于一侧中脑大脑脚脚底,大脑后动脉深穿支阻塞,动眼N麻痹,对侧锥体束性偏瘫,包括中枢性面瘫&舌肌瘫痪;
Third nerve palsy:
• Unilateral pupil dilated
• None reaction to light (direct & indirect)
• Associated signs: ptosis上睑下垂(partial or complete),
• external ophthalmoplegia眼外肌麻痹
(2).Benedikt syndrome红核综合征:动眼神经麻痹,对侧肢体震颤、强直(黑质损害)或舞蹈样动作、手足徐动及共济失调(红核损害)
Brain stem---Pons脑 桥
(1):Millard-Gubler Syndrome脑桥腹外侧综合征 6&7&两瘫
小脑前下动脉损伤,损伤了展神经、面神经、锥体束、脊髓丘脑束和内侧丘束,表现为病灶侧展神经及面神经瘫痪,对侧肢体上运动神经元瘫痪,对侧偏身感觉障碍(深&浅,内侧丘系,脊髓丘脑束);
• Anterior inferior cerebellar artery lesion(小脑前下A)
• 6th and 7th cranial nerve damage ipsilateral to side of the lesion(病侧6,7颅N)
• Associated contralateral hemiplegia(对侧偏瘫)
• Associated contralateral hemidysesthesia(对侧偏身感觉障碍)
Foville syndrome:病变累及内侧纵束,除以上症状,还表现为前核间性眼肌麻痹(两眼向病灶对侧共同偏视);
(2): Locked-in syndrome闭锁综合征 醒目上下动,面肢瘫不语
又称去传出状态,表现为不能说话,眼球水平运动障碍但可上下示意,双侧面瘫,吞咽构音障碍,不能耸肩转颈,四肢全瘫,可有双侧病理反射;脑电图多正常;
为桥脑基底部病变使双侧皮质核束&皮质脊髓束受损,网状结构完好,导致桥脑以下脑神经瘫及四肢瘫,多为脑血管病所致。
• a lesion of the ventral pons interrupts, the corticobular and corticospinal pathways, with sparing of the reticular pathways. 双侧脑桥损害,脑干网状结构完好
• The patient is alert but unable to respond with speech or facial or limb movements. Mute and paralysed.下眼睑以下无动,四肢瘫,不语,清醒
• The pathways for eye movement are spared, so the patient can respond with vertical eye movement and blinking.眼球可动及示意回答(可瞬目,面核上部未受损)
• Infarction多为基底动脉脑桥分支双侧闭塞导致脑桥基底部梗死所致
Brainstem--Medulla延髓
(1)Wallenberg syndrome延髓外侧综合征 8&两交感&疑小脑
小脑后下动脉闭塞,眩晕、呕吐、眼震(前庭神经核VIII),交叉性浅感觉障碍(三叉神经脊束核及脊髓丘脑側束); 吞咽、构音障碍,病变同侧软腭咽喉肌及声带瘫痪,咽反射消失(舌咽迷走神经及疑核);共济失调(绳状体及部分小脑),Horner综合征(交感神经下行纤维)。vertigo, anarthria构音障碍 and dysphagia吞咽困难, crossed palsy交叉性感觉障碍, cerebellar ataxia;
cerebellar posterior and inferior artery had obstructed.小脑后下A闭塞
Affected structures and resultant deficits include:
– The vestibular nuclei前庭核. Lesions results in nystagmus眼球震颤, nausea, vomiting, and vertigo眩晕.
– The inferior cerebellar peduncle小脑下脚. Lesions result in ipsilateral cerebellar ataxia同侧小脑征损害即cerebellar ataxia.
– The nucleus ambigus 疑核损害of CNⅨ, CNⅩand CNⅪ, Lesions result in ipsilateral laryngeal, pharyngeal, and palatal hemiparalysis同侧软腭麻痹, loss of the gag reflex , dysarthria, dysphagia, dysphonia咽反射消失,构音障碍,吞咽困难,声音嘶哑
– The spinothalamic tracts (脊丘系spinal lemniscus). Lesions result in contralateral loss of pain and temperature sensation from the trunk and extremities.对侧半身痛温觉损害
– The spinal trigeminal nucleus and tract三叉脊束核. Lesions result in ipsilateral loss of pain and temperature sensation from the face (facial hemianesthesia同侧半面麻木).
– The descending sympathetic tract交感N降支损害. Lesions result in ipsilateral(同侧) Horner’s syndrome (i.e., ptosis, miosis, hemianhidrosis, and apparent enophthalmos)眼睑下垂,眼球内陷,半面无汗,瞳孔小
(4)Dejerine syndrome延髓旁正中综合征,病变位于延髓中腹侧,出现舌下神经交叉瘫,表现为:病灶侧舌肌瘫痪和萎缩(舌下神经损害)、对侧肢体中枢性瘫痪(锥体束损害)、对侧肢体深感觉障碍(内侧丘系损害)
(5)Jackson syndrome,延髓橄榄核损害,一侧舌下神经受损和对侧锥体束征,X及XI对脑神经麻痹,无内侧丘系受损所导致的深感觉障碍;
decorticated syndrome:去皮质综合征
对外界刺激无反应、无自发性言语和目的性动作,可无意识睁眼闭眼,光反射、角膜反射存在,上肢屈曲和下肢伸直姿势,病理征(+);保存觉醒-睡眠周期,无意识咀嚼和吞咽(脑干上行网状激活系统未受损)。
见于缺氧性脑病、脑卒中及脑外伤导致大脑皮质广泛损害。
• typically regain spontaneous eye-opening, sleep-wake cycles, brainstem & spinal cord reflexes,but unawareness
• When developed, the patient rove转来转去 his eyes but fails to follow objects or lights or noise.
• remains mute哑.
• Some in coma of “toxic” origin or in globe lesions or cardiac arrest survive.
• If the lesion implicates牵连 the diencephalon间脑, either in midbrain lesion has not recovered ,the term will be described by decerebral rigidity去大脑强直.
脊髓内病变特点:
1.脊髓丘脑束:由内到外→颈.胸.腰.骶(因交叉)
2.薄束(内)楔束(外):由内到外→骶.腰.胸.颈→ 上行传导束
3.锥体束:由内到外→颈.胸.腰.骶→下行传导束 脊髓内病变:痛、温觉障碍由上向下发展(肢体),深感觉障碍由下向上发展(肢体) ,肢体无力由上肢向下肢发展,大小便功能障碍。
Compressive myelopathy/spinal cord compression脊髓压迫症
Definition: some occupied lesions within the vertebral canal lead to compression of the spinal cord, causing a group of diseases called spinal cord compression;
Manifestation:
Manifestation
extradural
extramedullary
Intramedullary
Root pains
Occur early
early
Less frequent
Symptoms develop
Bilateral, symmetrical
Unilateral to bilateral
Bilateral, symmetrical
Motor symptoms
Appear first
earlier
Earlier
Sensory changes
late
early
Early
Sphincter disturbances
later
later
Early
Protein content of CSF
Slightly increased
high
lower
1.急性脊髓压迫症发作acute onset,多出现脊髓休克;
2.慢性脊髓压迫症发作chronic onset,分3期:根痛期、部分受压期和完全受压期;
3.髓外病变时,感觉障碍自下肢远端向上发展至受压节段;髓内病变累及脊髓丘脑束时,导致分离性感觉障碍,自病变节段向下发展,鞍区(S3~5)感觉保留至最后受累,称“鞍区回避”sacral sparing;可用于鉴别髓内外病变;
4.椎管严重梗阻时脑脊液蛋白-细胞分离,细胞数正常,蛋白含量超过10g/L时,黄色脑脊液流出后自动凝结,称弗洛因综合征Froin syndrome;
周围神经脱髓鞘疾病GBS , Guillain-Barré Syndrome: acute inflammatory demyelinating polyneuropathy characterized by acute onset of peripheral & cranial never dysfunction causing progressive symmetric weakness and areflexia反射消失, facial diplegia, oropharyngeal and respiratory paresis, and impaired sensation in hands and feet.
Clinical manifestation of GBS
n 任何年龄,性别均等
n 起病前1~3周有呼吸或胃肠道感染史Campylobacter infection空肠弯曲菌感染最多见;
n Progressive symmetric limb weakness involves more than one limb, often with paresthesia.首发症状常为四肢对称性无力和感觉异常,常由远端开始,逐渐蔓延至近端;
n facial, ocular, oropharyngeal muscles may be affected, >50% have facial diplegia, and dysphagia and dysarthria. Some require mechanical ventilation;脑神经损伤以双侧面神经损伤最常见,其次为舌咽&迷走;
n Areflexia病理反射阴性
n The degree of sensory impairment varies: or marked diminution in perception of joint position – vibration震动觉 – pain - temperature in stocking-and-glove distribution, sensory modalities are preserved;感觉障碍一般比运动障碍轻或无感觉障碍,表现为肢体远端深感觉异常和手套、袜套样感觉减退,特殊感觉多保留;
n Autonomic dysfunction is commonly associated自主神经症状损害明显, e.g. orthostatic hypotension直立性低血压, unstable BP, tachyarrhythmia, bradyarrhythmia, resting tachycardia, disturbed sweating is frequent in severe cases, and easy to die
n 病情进展迅速,4周内可停止进展,1~2个月后开始恢复;
Froin sign/“phenomenon of protein-cell dissociation” 蛋白细胞分离现象
Cell count is usually normal. Protein content is elevated in most patients but may be normal in the first few days after onset, it may increase from 2 weeks after onset. Protein(0.8-8g/L).
【变异型】
n Miller-Fisher Syndrome
characterized by gait ataxia, areflexia, and ophthalmoparesis, papillary abnormalities are sometimes present;
三大特点是共济失调、腱反射消失、眼外肌麻痹;有时可见瞳孔改变;
多由呼吸道感染诱发,持续数周后好转,脑脊液蛋白含量升高,没有肢体瘫痪,血清中抗神经节苷脂GQ16抗体滴度升高,NCV多正常,H反射多延迟;
n Acute motor axonal neuropathy (AMAN)急性轴索性运动神经病
多caused by infection with campylobacter jejuni or paraenteral injection of gangliosides神经节苷脂;
急性发作,24~48小时内即出现四肢无力的迟缓性瘫痪,少感觉障碍,病情严重,多累及呼吸机,需气管切开;20~30%患者血清有GM1 or GD1神经节苷脂抗体;
【诊断】
1.病前1-3周有感染史
2.急性或亚急性起病,4周内进展的对称性四肢弛缓性瘫痪和脑神经损害,轻微感觉异常
3.脑脊液蛋白-细胞分离现象
4.肌电图检查,早期见F波或H反射延迟或消失,神经传导速度减慢,远端潜伏期延长,动作电位波幅正常或下降
5.排除其他疾病
【鉴别诊断】
1. Acute poliomyelitis急性脊髓灰质炎:
n acute, generalized disease caused by destruction of motor neurons in the spinal cord
n It is distinguished by asymmetry of flaccid paralysis, signs of meningeal irritation, fever, and CSF pleocytosis
n no dysfunction of sense( anaesthesia; hyperaesthesia)
2. Acute myelitis急性脊髓炎:
n a transverse myelitis interrupting both motor and sensory tracts at one level, usually thoracic.
n It usually begins with localized back or radicular pain followed by abrupt onset of bilateral paresthesia in the legs, an ascending sensory level
n Urinary bladder and bowel involvement occurs early and is p
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