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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:

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slide1

医大巡诊

周围神经病与脊髓病

chronic inflammatory demyelinating polyradiculoneuropathy cidp
Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)
  • Prevalence:

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

cidp and gbs
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
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
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
slide6
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
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 demyelinationin motor nerves.
laboratory features
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
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
slide15
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

slide16

--Bowel and bladder incontinence

--Numbness and paralysis

--Flaccid lower extremity paralysis

--T7 sensation level to temperature

--Vibration was perceived at knees.

slide20
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

髓内病变髓外病变
slide21
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

slide23
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:
slide24
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
slide29

Neuralgia-----Posterior horn lesion

Weakness---

-Polyradiculoneuroritis

reference
Reference
  • Neurologic complications of the reactivation of varicella-zoster virus NEJM 2000,342:635-645
  • Chronic Inflammatory Demyelinating Polyneuropathy NEJM 2005,352:1343-1356