[Autonomic dysfunction syndromes associated with orthostatic intolerance]. / Síndromes de disfunción autonómica asociados con intolerancia ortostática.

In 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.

Síndrome de Shy-Drager: revisão e apresentação de um caso / Shy-Drager syndrome: revision and presentation of a case

A síndrome de Shy-Drager é uma doença rara descrita em 1960, que consiste em falência progressiva do sistema nervoso autônomo, com parkinsonismo e hipotensão ortostática. Os autores fazem uma revisão, relatando um caso e mostrando aspectos atuais sobre diagnóstico e tratamento.

Sindrome de Shy-drager. A proposito de dos casos / The Shy-Drager syndrome. In connecttion with two cases

Presentamos dos pacientes masculinos, en la sexta decada de la vida, cion un cuadro clinico muy manifiesto del Sindrome Shy-Drager (SSD). El sintoma cardinal de estos enfermos esta dado por caidas frecuentes, por hipotension arterial ortostatica, con inconciencia, sin respuesta vegetativa simpatica (sin frialdad ni taquicardia y con anhidrosis). De forma sumaria el resto del cuadro neurologico estaba expresado por parkinsonismo, impotencia sexual, incontinencia esfinteriana, vesical, disfuncion, cerebelosa, hemiparesia, atrofias musculares, etc. Uno de los pacientes fue autopsiado y se revelo extenso proceso degenerativo cronico neuronal en el sistema nervioso central, asi como en el periferico, donde incluye el sistema autonomico y atrofia muscular neurogena. El estridor laringeo de uno de los enfermos durante el sueno, lo atribuimos a la imposibilidad de la abduccion de las cuerdas vocales en la inspiracion, por atrofia del musculo criacoritenoideo posterior. La respuesta en el caso dos, el tratmiento con indometacina, esteroides y vendaje elastico en los miembros inferiores, fue satisfactoria