Pathology of Shy-Drager syndrome.

In four patients with the Shy-Drager syndrome, detailed pathological findings in the central nervous system are described. All four patients had striatonigral degeneration, olivopontocerebellar atrophy, pyramidal tract degeneration and ventral horn cell loss. Along with the multisystem degeneration, there was widespread loss of thoracolumbar autonomic neurons, which was demonstrated by comparative cell counts of the intermediolateral horns in two of the four patients. In all four patients, autonomic nuclei of the sacral cord, particularly the Onuf's and intermediolateral nuclei, were severely affected and diffuse cell loss in the nuclei seems to account for disordered bladder, rectal and sexual functions in Shy-Drager syndrome.     

Amyotrophy in Shy-Drager syndrome

Five cases of Shy-Drager Syndrome (SDS) are reported. All patients showed marked muscular wasting often with fasciculation and without sensory loss. Clinical, electromyographic and in one case, pathological findings in the spinal cord indicated a lesion at the level of the anterior born cell. An extensive review of the literature disclosed a significant number of cases of SDS displaying amyotrophy referable to a spinal lesions.     

Vocal cord paralysis in the Shy-Drager syndrome.

Eight out of 12 unselected patients with Shy-Drager syndrome were found to have severe bilateral paresis of vocal cord abduction by fibre-optic laryngoscopy. This commonly presented as increased snoring followed by episodes of inspiratory and expiratory stridor and sometimes by sleep apnoea. Respiratory failure eventually developed in four cases and was reversed by tracheostomy. In another patient tracheostomy relieved severe attacks of sleep apnoea. This complication was not necessarily associated with advanced disease, and it should be considered in all patients with Shy-Drager syndrome as appropriate treatment can lead to a useful extension of life.     

Positron emission tomography in Shy-Drager syndrome

We studied the nigrostriatal dopaminergic pathway in 3 patients with Shy-Drager syndrome, by using positron emission tomography and [18F]6-fluoro-1-dopa to determine whether their parkinsonism correlated with impaired functional integrity of the presynaptic nigrostriatal pathway. One patient had short duration of disease, mild parkinsonism, and a normal positron emission tomographic scan, suggesting pathological changes functionally distal to the nigrostriatal pathway. Two patients with longer duration of disease had more severe parkinsonism and reduced [18F]6-fluoro-1-dopa uptake, suggesting impaired nigrostriatal dopaminergic function with progression of Shy-Drager syndrome.     

Diagnostic difficulties in Shy-Drager syndrome]

The authors describe two cases of Shy-Drager Syndrome in the aspect of diagnostic difficulties. The simple tests for evaluation of automatic system function, especially orthostatic test are stressed.     

Angina pectoris with normal coronary arteries in Shy-Drager syndrome.

A patient with Shy-Drager syndrome who presented with severe angina pectoris is described. Special investigations of his autonomic nervous system showed sympathetic and parasympathetic dysfunction with supersensitive end-organ response. Sympathetic dysfunction manifested as labile hypertension, severe postural hypotension, and inadequate heart rate response to atropine and the Valsalva manoeuvre. These changes in blood pressure were accompanied by severe disabling angina pectoris. Selective coronary angiography showed normal coronary arteries. It is suggested that angina pectoris resulted from the inadequate circulatory response and is another clinical manifestation of the Shy-Drager syndrome.     

Shy-Drager综合症相关因素临床分析

目的 探讨Ｓｈｙ－Ｄｒａｎｇｅｒ综合征（ＳＤＳ）的临床特点及其分析发病相关因素。方法 对７例ＳＤＳ的临床资料进行分析。结果 ７例患者均有发作性晕厥,立位时血压明显下降,而心率变化不大,大部分病人有尿便障碍、性功能障碍和共济失调;卧位休息时多能缓解,它的发生与脑干腹外侧核Ｃ１肾上腺素能元缺失相关,治疗可考虑补偿该类神经元功能。结论发作性晕厥与直立低低血压是ＳＤＳ的特点,肾上腺素能神经元缺失与此密切相     

Vocal cord paralysis in the Shy-Drager syndrome

2 patients with Shy-Drager syndrome with complete bilateral vocal cord paralysis are described. Attention is drawn to the facts that the recurrent laryngeal branch may be affected selectively with preserved parasympathetic function and that bilateral vocal cord paralysis can precede the appearance of both autonomic and extrapyramidal symptoms.     

Shy-Drager syndrome: the transitional variant

The clinicopathological features of a patient with the transitional variant of the Shy-Drager syndrome is described. The only previously reported case of the transitional variant was reexamined and pathological similarities to the present case are reviewed. Both patients exhibited features of Parkinson's disease with Lewy bodies in the substantia nigra and locus ceruleus. Striato-nigral degeneration and olivo-ponto-cerebellar atrophy were evident in both cases. They can thus be considered as transitional forms of the Shy-Drager syndrome with feature of both Parkinson's disease and multiple system atrophy.     

Levodopa therapy for Parkinsonism in the Shy-Drager syndrome

The results of levodopa treatment in five patients with Parkinsonism of the Shy-Drager syndrome are reported. There was no improvement in the neurological signs or functional disability in any patient. Mean values for lying and standing blood pressure increased significantly in three of the five patients during treatment, and were unchanged in the other two patients. The significance of these results is discussed. It is concluded that levodopa is not of therapeutic value in patients with this disorder.