A Neurological Syndrome Associated with Orthostatic Hypotension

Shy, G. Milton

doi:10.1001/archneur.1960.03840110025004

Cited by 683 publications

(203 citation statements)

References 33 publications

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“…They are diseases of late middle age with general signs of dysfunction of the autonomic nervous system, e.g., bowel and bladder disturbances, inability to sweat, and im- potence. The Shy-Drager syndrome (304) identifies a distinct group of patients who have orthostatic hypotension and signs of extrapyramidal tract involvement (Parkinson's disease); there are widespread but nonspecific degenerative lesions of the central nervous system. Patients with the Shy-Drager syndrome or idiopathic orthostatic hypotension often have abnormal cardiovascular function also at rest supine.…”

mentioning

confidence: 99%

Cardiovascular Adjustments to Gravitational Stress

Blomqvist

Stone

1983

Comprehensive Physiology

198

163

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mentioning

confidence: 99%

Cardiovascular Adjustments to Gravitational Stress

Blomqvist

Stone

1983

Comprehensive Physiology

198

163

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“…Another type of autonomic failure was reported in 1960 in a landmark paper by Shy and Drager 9 . In contrast to Pure Autonomic Failure, this more severe condition is manifested by severe orthostatic hypotension, progressive urinary and rectal incontinence, loss of sweating, iris atrophy, external ocular palsy, impotence, rigidity, and tremors.…”

Section: Primary Disorders Of Autonomic Failurementioning

confidence: 92%

A review of the classification, diagnosis, and management of autonomic dysfunction syndromes associated with orthostatic intolerance

Grubb¹,

Jorge²

2000

Arq. Bras. Cardiol.

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Syncope, the transient loss of consciousness and postural tone, is one of the oldest of recorded medical problems. Indeed, Hippocrates, the father of medicine, recorded the first description of syncope, and it is from the Greek that we derive the medical term for fainting (syncoptein -to cut short). Both a sign and a syndrome, syncope may result from a wide variety of causes. Over the last decade, considerable attention has been focused on one particular cause of syncope, the phenomenon previously called vasovagal syncope. Research into the nature of this disorder has demonstrated that it is only one aspect of a much broader group of disturbances of the autonomic nervous system that may lead to hypotension, orthostatic intolerance, and ultimately syncope. Indeed, recent discoveries have caused us to reevaluate our entire classification of autonomic disorders, and to develop a new system that better reflects our current knowledge. Because it is the cardiologist and the clinical cardiac electrophysiologist who are now frequently called upon to recognize and treat these disorders, this review is designed to acquaint the reader with these conditions, their diagnosis, and management. The autonomic nervous systemBecause these nervous disorders all result from a disturbance in normal autonomic function, it would seem appropriate to briefly review some aspects of the structure and operation of the nervous system.The human nervous system has two basic components: the central nervous system, made up of the brain and the spinal cord 1 , and the peripheral nervous system, which is comprises groups of neurons called ganglia, and of peripheral nerves that lie outside the brain and spinal cord. Although anatomically separate, the two systems are functionally interconnected. The peripheral nervous system is further divided into somatic and autonomic divisions. The somatic division is principally concerned with sensory information about the environment outside the body as well as muscle and limb position. The autonomic division (usually called the autonomic nervous system or ANS) is the motor system for the viscera, the smooth muscles of the body (especially those of the vasculature), and the exocrine glands. It is composed of three distinct parts: the sympathetic, parasympathetic, and enteric nervous systems. The sympathetic nervous system helps control the reaction of the body to stress, and the parasympathetic system works to conserve the body's resources and to restore equilibrium to the resting state. The enteric system controls the function of the gut. The organ systems governed by the ANS are, for the most part, independent of volitional control (although they sometimes can be affected by volitional or emotional inputs) and include the cardiorespiratory organs, the gastrointestinal and genitourinary tracts. The autonomic system is vital to the maintenance of internal homeostasis and achieves this by mechanisms that regulate blood pressure, fluid and electrolyte balance, and body temperature.Although representative of one ...

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“…Aunque la mayoría de los sujetos identificados son adultos mayores, esta alteración puede ocurrir en cualquier etapa de la vida, inclusive en la infancia. DISFUNCIÓN AUTONÓMICA E INTOLERANCIA ORTOSTÁTICA En 1960, en un artículo ya clásico, Shy y Drager informaron sobre otro tipo de falla autonómica (17). En comparación con la falla autonómica, esta condición se manifiesta por hipotensión ortostática severa, incontinencia fecal y urinaria progresiva, pérdida de la sudoración, atrofia del iris, parálisis de los músculos extraoculares, impotencia, rigidez y temblor; en etapas tardías pueden existir fasciculaciones musculares y atrofia de la musculatura distal.…”

Section: Disfunción Autonómica Primaria Desórdenes Crónicosunclassified

Síndromes de disfunción autonómica asociados con intolerancia ortostática.

Grubb¹,

Vesga²,

Guzmán³

et al. 2003

biomedica

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Los síndromes de disfunción autonómica asociados con la intolerancia ortostática son una de las alteraciones cuya fisiopatología fue reconocida hace sólo pocos años, lo que ha llevado a un mejor reconocimiento clínico. Entre las manifestaciones clínicas, el síncope y el presíncope asociados con ortostatismo son muy frecuentes. El espectro de la presentación clínica de estos síndromes varía desde la fatiga crónica hasta el síncope neurocardiogénico recurrente. En este artículo se revisa la fisiopatología y clasificación actual de los síndromes de disfunción autonómica asociados a intolerancia ortostática, así como las causas de disautonomía primaria y secundaria, y su enfoque diagnóstico y terapéutico.Palabras clave: intolerancia ortostática, disfunción autonómica, función autonómica, síncope mediado neuralmente, síncope vasovagal. Recent advances in diagnosis of autonomic disfunction syndromes associated with orthostatic intoleranceIn recent years increased interest has focused on the nature and pathophysiology of orthostatic intolerance and syndromes associated with autonomic disorders. Understanding the pathophysiology underlying these syndromes has led to the recognition of several distinct clinical entities with overlapping features and the associated need to reclassify many of the previously unrecognized syndromes. Among the clinical manifestations, syncope and near syncope are frequently associated with orthostatic intolerance. In addition, however, a wide spectrum of symptoms have been described ranging from chronic fatigue to recurrent neurally mediated vasodepressor reactions. The present review focuses on the pathophysiology and classification of syndromes of autonomic dysfunction associated with orthostatic intolerance. Primary and secondary causes of dysautonomia as well as therapeutic approach to these frequently unrecognized syndromes is presented.Key words: orthostatic intolerance, autonomic failure, autonomic function, neurally mediated syncope, vasovagal syncope. El sistema nervioso autónomo (SNA) como eje central del control cardiovascular modula los reflejos relacionados con el control de la presión arterial y la frecuencia cardíaca, lo que permite mantener un gasto cardíaco adecuado en respuesta al ortostatismo. El estudio fisiopatológico y la caracterización clínica de los síndromes de disfunción autonómica que se manifiestan con intoleracia ortostática han sido un reto para los investigadores de las áreas básicas y clínicas debido a su frecuente presentación y a la falta de herramientas terapéuticas que permitan su adecuado manejo.

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A Neurological Syndrome Associated with Orthostatic Hypotension

Cited by 683 publications

References 33 publications

Cardiovascular Adjustments to Gravitational Stress

Cardiovascular Adjustments to Gravitational Stress

A review of the classification, diagnosis, and management of autonomic dysfunction syndromes associated with orthostatic intolerance

Síndromes de disfunción autonómica asociados con intolerancia ortostática.

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