周 围 神 经 病概要1.pptVIP

Clinical features of AIDP(2) 3-5% have complete ophthalmoplegia 15% have autonomic manifestation labile blood pressure, cardiac arrhythmias, bladder dysfunction, constipation, abdominal distension, bloating Neurologic examination Quadriplegia: Muscle weakness and atrophy Muscle stretch reflexes are absent or depressed Cranial nerve paralysis Sensory loss in a stocking-glove distribution Pain in low back, buttocks, thigh Exacerbated by straight leg raising Autonomic dysfunction CSF 90% of cases, protein is elevated by the nadir of illness without leukocytosis. 5% of cases, pleocytosis 10 (10-20) cells/mm3. OB (oligoclonal bands) IgG/24h increasing MBP increasing Special Antibodies : GM1 Electro-diagnostic studies AIDP: Demyelination Axonal AMAN/AMSAN: Axonal Demyelination Sural nerve biopsy demyelinating and remyelinating axonal degeneration Infiltration of inflammatory cells : lymphocytes,macrophages deposition of Ig and complements Course Most patients of AIDP become maximally weak within 11-12 days of onset. AMSN and AMAN usually reach nadir within 6 days. Most patients progress steadily, occasionally, stuttering or stepwise course. The average time to onset of recovery is 4 weeks. Diagnosis Clinical feature: acute progressive quadriplegia CSF: dissociation of protein/leukocytes F-wave, NCV, EMG, MEP Sural nerve biopsy Special antibodies in serum and CSF Differential diagnosis Periodic paralysis Poliomyelitis Myelitis Toxic neuropathy Lyme’s disease Myositis Treatment of GBS （1） Support care Prevent complications Rehabilitation Psychology support immunotherapy Treatment of GBS （2） Immunotherapy Plasma exchange (PE) Iv Ig PE + IV Ig? Corticosteroid? IV Ig＋ Corticosteroid？ Prognosis 80% recovery within 6 months 15% have severe residual disability Mortality rate is 3%-5%. Cause of death: ARDS with or without sepsis, dysautonomia. Chronic inflammatory demyelinating pol