医医医医 医医医医医医医医医
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