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1、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 cell
2、s); 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
3、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 respons
4、e 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
5、 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 p
6、olyneuropathy 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 ty
7、pical 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
8、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 conspic
9、uous 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
10、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
11、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
12、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,