1、什么是中枢神经系统脱髓鞘疾病？

中枢神经系统脱髓鞘疾病是一组脑和脊髓髓鞘破坏或髓鞘脱失为主要特征的疾病，脱髓鞘是其病理过程中的特征性表现，包括遗传性（髓鞘形成障碍性疾病，脑白质营养不良）和获得性（继发性和原发性免疫介导）两大类。

Central nervous system demyelinating diseases are a group of diseases characterized by destruction of myelin sheaths or demyelination of the brain and spinal cord. Demyelination is a characteristic manifestation of its pathological process, including hereditary (myelinating disorders) , Leukodystrophy) and acquired (secondary and primary immune-mediated) two categories.

2、多发性硬化的诊断标准是什么？

：（1）临床发作次数>2，脑脊液寡克隆阳性可代替一次时间多发性

（2）有客观证据的临床证据的病变数目>2次

CNS白质内同时存在着两处以上的病灶

起病年龄10-50岁

有缓解和复发交替的病史

每次发作>24h，或缓慢进展>1年

排除其他病因
: (1) if the number of clinical attacks is more than 2, oligoclonal positive cerebrospinal fluid can replace one time multiple

(2) The number of lesions with objective clinical evidence was > 2

There are more than two lesions in the white matter of CNS at the same time

The onset age is 10-50 years old

There is a history of alternating remission and recurrence

Each attack > 24h, or slow progress > 1 year

Exclude other causes

3、请简述视神经脊髓炎谱系病（NMOSD）六大核心症状？

视神经炎：急性、单侧或双侧，视力下降或失明伴有眼眶内疼痛，眼底出现视乳头水肿，晚期视神经萎缩

急性脊髓炎：单侧或双侧，初期可以和视神经炎同时出现

最后区综合征，无其他原因能解释的发作性呃逆、恶心、呕吐

急性脑干综合征：累计第四脑室及脑干，表现为头晕、复视、共济失调

症状性发作性睡病、间脑综合征，同时MRI伴有NMOSD特征性间脑病变

大脑综合征伴有NMOSD特征性大脑病变：意识水平下降、认知语言等高级皮质功能减退。

Optic neuritis: acute, unilateral or bilateral, vision loss or blindness with pain in the orbit, papilledema in the fundus, and late optic atrophy

Acute myelitis: unilateral or bilateral, it can appear at the same time as optic neuritis in the initial stage

End zone syndrome, episodic hiccups, nausea, and vomiting for no other reason

Acute brainstem syndrome: cumulative fourth ventricle and brainstem, manifested as dizziness, diplopia, ataxia

Symptomatic narcolepsy, diencephalic syndrome, and MRI accompanied by NMOSD characteristic diencephalopathy

Cerebral syndrome is accompanied by NMOSD characteristic brain lesions: decreased consciousness, cognitive language and other advanced cortical functions.