Shy-Drager Syndrome : a functional protocol study of the Autonomic Nervous System in 3 cases / Sindrome de Shy-Drager : protocolo de estudo funcional do sistema nervoso autonomo em 3 casos clinicos

AUTOR(ES)
DATA DE PUBLICAÇÃO

1984

RESUMO

This study was motivated by the desire to better understand the autonomic functions in subjects clinically diagnosed as Shy-Drager syndrome. This syndrome was selected because it presents a disorder in the central autonomic structures of CNS. This degenerative disease represents a multiple disorders of CNS which include dysautonomia associated with pyramidal, extra-pyramidal and cerebellar symptoms. The objectives of this investigation were twofold: 1st: To develop a protocol to aid iri the localization of therlesion of the cardiovascular reflex arc responsible for orthostatic hypotension in neurologic diseases; 2nd: To develop a clinical protocol to evaluate the autonomic nervous system regarding the clinical diagnosis of the Shy-Drager, syndrome. This research can by divided into two parts. The first part is composed by a large review of the literature on physiology and pathology of the autonomic nervous system. Physiological aspects of blood pressure in supine and upright positions were discussed as well as the physiopathology of postural hypotension. Clinical and anatomical criteria for Shy-Drager syndrome were-described and nosological boundaries were established in relation to idiopathic orthostatic hypotension and "parkinsonian" autonomic failure. The second part is constituted by a clinical description of 3 cases of Shy-Drager syndrome observed in the Neurological Clinics, Civils Hospital, af The Louis Pasteur University of Strasbourg, France, from 1975 to 1978. The patients were investigated from the autonomic nervous System standpoint which included: a- hemodynarnical and pharmacological trials; b- EEG during tilting; c- laboratory tests ( renine and dopamine-B-hydroxilase activity and urine adrenalin and noradrenalin during postural changes ); d- central metabolism of dopamin and serotonin by probenecid test; e- pulmonary ventilation control during tilting and sleeping and CO2 and occlusion pressure test; f- EEG study of sleep organization, and g- EMG study of anal and urethral sphincters functioning and motor conduction velocity. Each of these tests was considered in separate as well as its own contribution to the topographic diagnosis of lesion in the Cardiovascular reflex arc. Taking into account the results it may be concluded that: 1st: There is a sympathetic and parasympathetic dysfunctions in alI cases. This is secondary to central dysfunction of cardiovlscular reflex arco This hypothesis was substantiated by pulmonary ventilation dysfunction during tilting and sleeping; 2nd: The decrease of central doparnin and serotonin turn-over was correlated with lesions in nigrostriatal and autonornic tracts of CNS and respansible for the clinical picture of the patients; 3rd: The vascular supersensitivity for vasoactive drugs is due to a failure of the CNS to buffer blood pressure fluctuations; 4th: The decrease or absence REM sleep in all cases was correlated with central doparnin and serotonin rnetabolisrn dysfunction detected by the probenecid testi 5th: The Shy-Drager syndrorne is probably due to hypofunction of central arninergic systerns of CNS; 6th: The abnorrnalities of peripheral motor neuron, clinical and/or EMG displayed, constituted a motor systern desorganization in the Shy-Drager syndrorne

ASSUNTO(S)

sindromes medula espinhal sistema nervoso central

ACESSO AO ARTIGO

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