【医学课件】周围神经病与脊髓病

周围神经病与脊髓病 中国协和医科大学 Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) nPrevalence: 0.5/100,000 in children, 2/100,000 in adult. CIDP and GBS n-Similarity: nBoth are widespread polyradiculoneuropathies, usually with cytoalbuminologic dissociation of the CSF (raised protein concentration with fewer or no cells); n both exhibit nerve conduction abnormalities characteristic of a demyelinating neuropathy (reduced conduction velocity and partial conduction block in motor nerves), npathologically, both show similar multifocal perivenous inflammatory infiltrates Difference 1.mode of evolution and prognosis nCIDP begins insidiously and evolves slowly, either in a steadily progressive or stepwise manner, attaining its maximum severity after several months or longer nFrom 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 nWeakness of the limbs, particularly of the proximal leg muscles, or numbness, paresthesias, and dysesthesias of the hands and feet were the initial symptoms na 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 nCranial nerve abnormalities were distinctly unusual nThere was a systemic condition such as paraproteinemia, lymphoma, an undifferentiated reactive adenopathy, or lupus in association with an inflammatory demyelinating polyneuropathy nThe clinical course was monophasic and slowly progressive in about one-third, stepwise and progressive in another third, and relapsing in the remaining third. Diagnosis nThe chronic symmetric sensorimotor loss coupled with EMG findings of demyelination largely define the illness. nThe 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 nThe CSF protein is elevated in 80 percent of patients with CIDP, typically in the range of 75 to 200 mg/dL. nIn biopsy material (sural nerve), half of cases are found to have interstitial and perivascular infiltrates of inflammatory cells, nonion-bulb formations are conspicuous in the recurrent and relapsing cases. Treatment nhigh doses of gamma globulin (2 g/kg in divided infusions over 4 or 5 days). nplasma exchanges nPredinison, 60 to 80 mg of prednisone daily that is tapered over months to the lowest effective dose, typically 25 to 40 mg nazathioprine :for at least 3 months), 3 mg/kg in a single daily dose, ncyclophosphamide or mycophenolate 脊髓疾病 n二便失禁 n麻木无力 n下肢迟缓性瘫痪 nT7感觉平面(温度觉) n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 n早期症状 n根性痛 n感觉障碍 n痛温觉障碍 n节段性肌无力和萎缩 n锥体束征 n括约肌功能障碍 n椎管梗阻 n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 n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 n炎症：感染性、非感染性 n肿瘤：脊椎、转移、 n外伤： n代谢: 亚急性联合变性 n血管病变：脊髓前动脉综合征 n先天畸形：脊髓空洞，Arnold-Chiari畸形 n变性: 脊髓病变定性 n急性脊髓炎：横贯性损伤 n脊髓空洞症：中央管 n脊髓痨：后索、 后角 n脊髓亚急性联合变性：后索、侧索 n脊髓肌萎缩症、脊髓灰质炎：前角 n脊髓前动脉综合征：脊髓前动脉 n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- -Polyradiculoneu