RESEARCH: Multiple System Atrophy

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Schweiz Rundsch Med Prax 2001 Jun 7;90(23):1035-40

Wenning GK, Seppi K.

Universitatsklinik fur Neurologie, Innsbruck. gregor.wenning@...

Numerous studies of the past years have established the clinical features,

course and neuropathology characterizing multiple system atrophy (MSA).

Clinically, two motor subtypes can be classified based on the predominance

of a parkinsonian syndrome refractory to L-dopa and cerebellar ataxia. 80%

of the cases involve MSA-P (the parkinsonian variant of MSA) and 20% MSA-C

(cerebellar variant of MSA). Virtually all of these patients show

disturbances of autonomic and urogenital function, half of the patients also

exhibit pyramidal signs. Neuropathologically, MSA-C is based on an

olivopontocerebellar atrophy (OPCA) and MSA-P on striatonigral degeneration

(SND). However, a combination of OPCA and SND pathologies is observed in

most cases. Recent evidence suggests that a key pathogenetic role may be

played by glial alpha synuclein-containing inclusion bodies, which might

lead to neuronal dysfunction and ultimately to cell loss. There is no

therapy known to be effective in treating the motor disorders of MSA-C. By

contrast, L-dopa replacement is at least transiently effective in about 30%

of patients with MSA-P. Currently, initial efforts are being undertaken

throughout Europe to develop neuroprotective solutions. Experiments are

underway to test whether neurotransplantation by striatal grafting is a

suitable method for inducing a clinically relevant response to L-dopa.

Neurologically, the options for treating orthostatic hypertension and

urogenital disorders are often overlooked.