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Demyelinating Diseases. Central nervous syetem : Multiple sclerosis(MS) Neuromyelitis optica(NMO) Peripheral nervous syetem : Acute inflammatory demyelinating polyneuropathy (AIDP).
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Demyelinating Diseases Central nervous syetem: Multiple sclerosis(MS) Neuromyelitis optica(NMO) Peripheral nervous syetem: Acute inflammatory demyelinating polyneuropathy (AIDP)
Multiple Sclerosis Ⅰ.Definition MS is an auto-immune disease.It is characterized by multifocaldemyelination in the white matter of the central nervous system.
Ⅱ.Etiology and pathogenesis 1.Viral infection and auto-immune reaction. 2.Genetic factor: inherited predisposition. 3.Environmental factor.
Ⅲ .Pathology 1.Multiple demyelination in the white matter of the central nervous system. 2.Site:round the lateral ventricle ; optic nerve ;brain stem ;spinal cord ;cerebellum 3.Phase (1)acute phase: (2)chronic phase
Ⅳ.Clinical Features 1.General condition: onset: acute or subacute age:10-50 precipitating factor characteristic of duration:alternative recurrent-remit
3.Characteristic signs (1) internuclear ophthalmoplegia(INO) (2) nystagmus
4.Clinical type Alternative recurrent-remit type Secondary progressive type Primary progressive type Progressive recurrent type Benign type
Ⅴ.Investigation 1.CSF (1)Routine and biological examination cell: normal or slightly high, <15 protein:slightly high (2)IgG:characteristic a.CSF-IgG index: [CSF-IgG/S-IgG]/[CSF-ALB/S-ALB]>0.7 b.oligoclonal bands of IgG(OB):95%
2.Brain evoked potential(BEP)VEP,BAEP,SEP. detect subclinical lesions 3.CT:multiple low density lesion white matter 4.MRI:the most sensitive means a.multifocal long T1 and long T2 signals b.paraventricle,half-oval area,brain stem,cerebellum,spinal cord,corpus callosum c.Enlarged ventricle
2.Brain evoked potential(BEP)VEP,BAEP,SEP. detect subclinical lesions 3.CT:multiple low density lesion white matter 4.MRI:the most sensitive means a.multifocal long T1 and long T2 signals b.paraventricle,half-oval area,brain stem,cerebellum,spinal cord,corpus callosum c.Enlarged ventricle
Ⅶ.Differential Diagnosis 1.Acute disseminated encephalomyelitis(ADEM) 2.Cervical spondylosis 3.Tropical spastic paraplegia(TSP) 4.Brain arteritis 5.Vasodeformity 6.Multiple infarction 7.Cerebral lymphoma 8.SLE,Lyme,etal
Ⅷ.Treatment 1.Corticosteroid and adrenocorticotropic hormone(ACTH) 2.β-IFN 3.Immunoglobin 4.Immunosuppressive therapy: azathioprine;cyclophosphamide; methotrexate;cyclosporine A 5.Plasma exchange(PE) 6.Symptomatic treatment:baclofen
Neuromyelitis Optica(NMO) 1.pathology:demyelination;necrosis 2.Clinical features (1)Acute transverse myelitis: incomplete and asymmetric (2)Optic neuritis 3.investigation: (1)CSF:cell:1/3>50 (2)MRI:long T1 and long T2,>3 segments (3)Oligoclonal band is rare 4.treatment:methylprednisone