Search

Shy-Drager syndrome (SDS) is a subtype of multiple system atrophy (MSA) with the clinical predominance of autonomic failure. The differential diagnosis between SDS and Parkinson's disease (PD) can sometimes be difficult clinically. The features favoring a clinical diagnosis of SDS are marked orthostatic hypotension, erectile impotence in males, urinary symptoms, nocturnal stridor, rigidity and akinesia without tremors, levodopa unresponsiveness, cerebellar signs, cerebellar atrophy on brain CT scans and MRI.

Shy-Drager syndrome is characterized by severe orthostatic hypotension and other autonomic dysfunctions, cerebellar ataxia, parkinsonism, and upper and lower motor neuron symptoms. The disease starts in the 4th to 6th decades with severe dizziness, blackout or syncopi on standing up, and the duration of the illness is 6-7 years in the majority of the cases. The pathological lesions responsible for the autonomic dysfunctions are in the hypothalamus, locus saeruleus, Edinger-Westphal and other nuclei in the brain stem, intermediolateral column and Onuf's nuclei in the spinal cord. Oppenheimer has postulated a concept of multiple system atrophy to such widely distributed degenerative neurological disorders. However, olivopontocerebellar atrophy of Dejerine and Thomas, striatonigral degeneration and this syndrome can be distinguished clinically as well as pathologically.