SHY-DRAGER SYNDROME Tomiyoshi Chida 1,2 , Keizo Hirayama 1,3 , Masanori Nagaoka 1 , Hirotaro Narabayashi 1 1Department of Neurology, Juntendo Medical School 2Division of Internal Medicine,Narugo Branch of the University Hospital, Tohoku University School of Medicine 3Department of Neurology, Brain Research Institute, Chiba University pp.433-440
Published Date 1980/4/1
DOI https://doi.org/10.11477/mf.1406204576
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Since the original description of Shy-Drager syndrome (SDS) by Shy and Drager in 1960, many cases have been reported. However, it is still open to question whether Shy-Drager syndrome is disease entity or not, since its pathological findings are similar to those of olivo-pontocerebellar atrophy (OPCA) and striato-nigral degeneration (SND) which of ten have autonomic disturbances in their courses. Some investigators proposed to use the term multiple system atrophy to cover the whole group including SDS, OPCA and SND, mostly based on pathological findings. The authors tried to clarify this problem in view of clinical mani-festations.

In the present study, 8 cases of Shy-Drager syndrome with one autopsied case were analyzed as to the disturbances of the autonomic nervous system and neurological symptoms to examine the lesions attributable to autonomic disturbances and to compare the clinical features of SDS with those of OPCA and SND. The autonomic disturbances were examined by means of Shellong test, cold pressure test, sweat test, instillation test, sub-cutaneous injection of adrenaline, pilocarpine andatropine, and change of plasma renin activity and blood aldosterone by standing. The results were as follows:

1) The disturbances of autonomic nervous system of SDS were severer and more variable than those of OPCA and SND, and the postganglionic fibers of sympathetic nerves as well as the intermedio-lateral nuclei seemed to be degenerated.

2) Sometimes, plasma renin activity and blood aldosterone were not elevated after standing for more than 1 hour. According to the results of the sweat test by subcutaneous injection of pilo-carpine and the pharmacological test by sub-cutaneous injection of adrenaline, damage to the postganglionic fibers of sympathetic nerves was suggested in these cases.

3) Eight cases of SDS showed two characteristic clinical courses. In the first group autonomic dis-turbances were the initial symptoms, followed by parkinsonian symptoms within 5 years. In the second autonomic disturbances were the initial symptom as the first, however, followed by cere-bellar symptoms within 3 years and parkinsonism symptoms much later. These patterns of clinical course seem to be important, since such initial appearance of autonomic disturbances is not usualy observed in OPCA and SND.

Although the pathological findings of SDS are sometimes similar to those of OPCA and SND, its spectrum of autonomic symptoms and clinical course differ from those of OPCA and SND. SDS should be considered as a separate clinical entity, and may not belong to the same groups of diseases as OPCA and SND.

Copyright © 1980, Igaku-Shoin Ltd. All rights reserved.


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