[Multiple sclerosis: disease entity, subtypes and variants, and diagnostic criteria].
Multiple sclerosis (MS) is the prototypic inflammatory demyelinating disorder of the central nervous system (CNS). The increasing application of new powerful technologies during recent years has yielded new concepts and opened a new horizon. The prototype MS is called classical MS or Charcot variant and is characterized by the presence of the lesions in all parts of the CNS, consistently including cerebrum and/or cerebellum. It takes a relapsing-remitting course initially but, in most of the Caucasian cases, eventually turns into the chronic progressive stage (secondary chronic progressive form). Primary progressive MS takes slowly progressive course from the beginning. Both types of chronic forms are relatively rare in Japanese. Morphologically, the MS lesion is characterized by the key features: perivenular demyelination, inflammation gliosis, and axonal damage. The heterogeneity of MS lesions has been clearly established. Both T-cell and B-cell (antibody) mediated mechanisms are working and the primary target for the autoimmune damage could be myelin and/or oligodendroglia. Axonal damage could be most extensive within the first year after the disease onset. The list of candidate antigens includes myelin basic protein, proteolipid protein, and myelinoligodendroglialglycoprotein, but other non-myelin/oligodendroglial antigens such as S-100 protein also could induce CNS inflammation. Viral and microbial proteins are shown to share epitopes with those candidate antigens and, hypothetically, could induce CNS inflammation when infected to humans. New diagnostic criteria(McDonald et al, 2001) had been proposed and now include MRI criteria for morphological diagnosis and temporal activities. A Criterion for primary chronic progressive form is also included. The MS working group of Japanese MHL is now preparing for a new criteria for Japanese MS patients.
Saida
Takahiko
T
Utano National Hospital, Center for Neurological Diseases.
D000818:Animals; D006801:Humans; D007564:Japan / Q000453:epidemiology; D009103:Multiple Sclerosis / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology; D044469:Racial Groups; D012015:Reference Standards