Abstract
First described in 1969 by Graham and Oppenheimer, multiple system atrophy (MSA) is increasingly recognised as distinctive neurodegenerative disease. Clinically, it is characterised by autonomic failure, parkinsonism, cerebellar ataxia and pyramidal signs in any combination. The motor disorder is dominated by parkinsonism in 80% of patients (MSA-P) or by cerebellar ataxia in the remaining 20% (MSA-C). Although the clinical syndrome is often highly characteristic in advanced stages clinicopathological and epidemiological data indicate that multiple system atrophy is underdiagnosed in movement disorders clinics as well as in the general population. It remains to be determined whether the recent consensus diagnostic criteria for multiple system atrophy (Gilman et al. 1998) will improve patient recognition. Physical manoeuvres and/or drug therapy may considerably improve orthostatic dysfunction and urogenital complaints, unfortunately these measures are not always implemented. The motor disorder of multiple system atrophy cannot be treated effectively in most patients although transient relief may be obtained by L-Dopa. The latter should therefore be administered to all MSA patients with parkinsonian features. Alternative therapeutic strategies such as neuroprotection and neurotransplantation are currently being explored in experimental multiple system atrophy models.