[Multiple system atrophy: the beginning of a new era in the history of neurodegenerative diseases].
Multiple system atrophy (MSA) belongs to the neurodegenerative diseases of the nervous system, but it is different from them in many aspects: it has no familiar form and no genetic factor was identified in the pathomechanism. Its neuropathology is unique too, because oligodendroglial cells are harbouring the main pathological burden. It was described in MSA that there is no elective neuronal degeneration in neurodegenerative disorders: the glial cells show the same pathochemical and structural abnormalities as found in the neurones. The discovery of the glial cytoplasmic inclusions, as a pathognostic marker for MSA, has directed attention to the glial cells in other neurodegenerative disorders. As a result of this, there are several neurodegenerative diseases nowadays in which glial inclusions were described, similar to the neuronal inclusions in their structural and biochemical properties and some of them became the diagnostic marker of the disease. In our review we summarize the clinical features, the history and the neuropathology of MSA and we discuss its special features.
Papp
Mátyás
M
Semmelweis Egyetem, Altalános Orvostudományi Kar, Neurológiai Klinika, Budapest. matyi@neur.sote.hu
Kovács
Tibor
T
D006801:Humans; D002479:Inclusion Bodies; D019578:Multiple System Atrophy / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology / Q000473:pathology; D019636:Neurodegenerative Diseases / Q000175:diagnosis; D009457:Neuroglia / Q000473:pathology*