The neuropathology of progressive autonomic failure of central origin (the Shy-Drager syndrome)
Kakulas, B.A.; Tan, N.; Ojeda, V.J.
Clinical and Experimental Neurology 22: 103-111
1986
ISSN/ISBN: 0196-6383 PMID: 3581499 Document Number: 280511
The neuropathological features of the "Shy-Drager syndrome" have, in the past, been unsettled. The position was recently clarified by Oppenheimer who reviewed the 51 reported patients with progressive autonomic failure in whom neuropathological findings were given. He divided these cases into two groups. Group I included those with lesions of the pigmented nuclei of the brain stem which contained Lewy bodies. These he further subdivided into subgroups IA or IB according to whether or not the patient showed clinical evidence of Parkinson's disease in addition to the common denominator of orthostatic hypotension. In group II he placed those patients in whom multiple system atrophy was found at necropsy. These were the majority. Loss of neurons from the intermediolateral columns of the thoracic spinal cord, thought to be the cause of the postural hypotension, was present in all cases. To illustrate the new classification the necropsy findings in 3 patients taken from the files of the Royal Perth Hospital are described. One of these showed the lesions of Parkinson's disease with Lewy bodies, and thus conformed to Oppenheimer's type IA. The two other patients showed multiple system atrophy and thus belonged to Oppenheimer's type II. All three showed loss of neurons in the intermediolateral columns of the thoracic spinal cord. We believe that Oppenheimer's classification of progressive orthostatic hypotension improves the clinicopathological understanding of the syndrome and is thus useful as well as informative to the practising neurologist.