医大巡诊

周围神经病与脊髓病

Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)

Prevalence:

0.5/100,000 in children, 2/100,000 in adult.

CIDP and GBS

--Similarity:

Both are widespread polyradiculoneuropathies, usually

with cytoalbuminologic dissociation of the CSF (raised

protein concentration with fewer or no cells);

both exhibit nerve conduction abnormalities characteristic

of a demyelinating neuropathy (reduced conduction

velocity and partial conduction block in motor nerves),

pathologically, both show similar multifocal perivenous

inflammatory infiltrates

Difference

1.mode of evolution and prognosis

CIDP begins insidiously and evolves slowly, either in a

steadily progressive or stepwise manner, attaining its

maximum severity after several months or longer

From the beginning it may be asymmetrical or involve the

arms predominantly

2. CIDP may be distinct immunologically from GBS

3. Difference response to the administration of prednisone

Clinical features

Weakness of the limbs, particularly of the proximal leg

muscles, or numbness, paresthesias, and dysesthesias of

the hands and feet were the initial symptoms

a mixed sensorimotor polyneuropathy with weakness of

the shoulder, upper arm, and thigh muscles, in addition

to motor and sensory loss in the distal parts of the

limbs

Cranial nerve abnormalities were distinctly unusual

There was a systemic condition such as

paraproteinemia, lymphoma, an undifferentiated

reactive adenopathy, or lupus in association with an

inflammatory demyelinating polyneuropathy

The clinical course was monophasic and slowly

progressive in about one-third, stepwise and

progressive in another third, and relapsing in the

remaining third.

Diagnosis

The chronic symmetric sensorimotor loss coupled with

EMG findings of demyelination largely define the illness.

The typical EMG findings are of multifocal conduction

block , prolonged distal latencies (distal block), nerve

conduction slowing to less than 80 percent of normal in

several nerves, loss of late responses, and dispersion of

the compound muscle action potentials, further

reflecting demyelination in motor nerves.

Laboratory Features

The CSF protein is elevated in 80 percent of patients

with CIDP, typically in the range of 75 to 200 mg/dL.

In biopsy material (sural nerve), half of cases are found

to have interstitial and perivascular infiltrates of

inflammatory cells,

onion-bulb formations are conspicuous in the recurrent

and relapsing cases.

Treatment

high doses of gamma globulin (2 g/kg in divided infusions

over 4 or 5 days).

plasma exchanges

Predinison, 60 to 80 mg of prednisone daily that is tapered

over months to the lowest effective dose, typically 25 to 40

mg

azathioprine :for at least 3 months), 3 mg/kg in a single

daily dose,

cyclophosphamide or mycophenolate

脊髓疾病

二便失禁 麻木无力 下肢迟缓性瘫痪 T7 感觉平面 ( 温度觉 ) 膝部音叉震动觉存在

--Bowel and bladder

incontinence

--Numbness and

paralysis

--Flaccid lower extremity

paralysis

--T7 sensation level to

temperature

--Vibration was

perceived at knees.

髓内病变 髓外病变 早期症状 根性痛 感觉障碍 痛温觉障碍 节段性肌无力和萎缩 锥体束征 括约肌功能障碍 椎管梗阻 辅助检查

多为双侧 自一侧进展少见 多见，剧烈分离性， 传导束性自上而下发展 自下向上发展早期出现 少见，局限不明显 早期出现早期出现 晚期出现晚期 早期

纵向定位：确定病变的上界：神经根痛、感觉障碍

平面、反射异常病变下界的确定较困难，但某些体征有

帮助，如节段性感觉障碍

脊髓病变定性 炎症：感染性、非感染性 肿瘤：脊椎、转移、 外伤： 代谢 : 亚急性联合变性 血管病变：脊髓前动脉综合征 先天畸形：脊髓空洞， Arnold-Chiari 畸形 变性 :

脊髓病变定性

急性脊髓炎：横贯性损伤 脊髓空洞症：中央管 脊髓痨：后索、 后角 脊髓亚急性联合变性：后索、侧索 脊髓肌萎缩症、脊髓灰质炎：前角 脊髓前动脉综合征：脊髓前动脉 肌萎缩侧索硬化：锥体束、前角

Central nervous system manifestation of Varicela-Zoster Virus

Neuralgia-----Posterior

horn lesion

Weakness---

-Polyradiculoneuroritis

Reference

Neurologic complications of the reactivation of

varicella-zoster virus NEJM 2000,342:635-645

Chronic Inflammatory Demyelinating

Polyneuropathy NEJM 2005,352:1343-1356