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BACKGROUND: Orthostatic hypotension (OH) is a common problem among the elderly. It is associated with an increase in morbidity and mortality, but its prevalence in Mexico is unknown. METHODS: We conducted a cross-sectional prospective study of intern patients at several Mexican elderly assistance institutions. We carried out a history and took blood pressure readings in a seated position, immediately after standing up, and again after 3 min of standing up. RESULTS: We evaluated 132 patients, mean age 82.3 ± 9.5 years, 74.1% of them female. Thirty-nine (29.3%) subjects had OH. They had a higher prevalence of hypothyroidism, Parkinson's disease, depression and alcoholism. Their Minimental result was 15.45 ± 7.2 vs 16.12 ± 7.9 (p = 0.6) among those without OH, and their quality of life (Minnesota scale) was 12.1 ± 7.3 vs 9.15 ± 7.05 (p = 0.03). They used more ACEI, digoxin and levothyroxin. Hypertension and alcoholism showed respectively a RR of 2.6 (95% CI 0.9-7.6, p = 0.06) and 3.18 (95% CI 0.96-10.48, p = 0.05) to develop OH. CONCLUSIONS: OH was present in 29.3% of the studied population. A third of them had hypertension. The use of different medications does not solely explain OH, so it is necessary to look for different associations. Among those, chronic alcoholism stands out. OH is associated with a poorer quality of life and cognitive performance. OH is asymptomatic in most cases.

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Orthostatic hypotension is a frequent symptom in patients with multiple system atrophy and it has an important impact on their quality of life. We report a case of idiopathic orthostatic hypotension, in a patient with multiple system atrophy (Shy-Drager syndrome), treated with pacemaker implantation resulting in a substantial improvement in the quality of his life.

Hipotensão ortostática é um sintoma freqüente em pacientes com atrofia de múltiplos sistemas e tem importante impacto na sua qualidade de vida. Relatamos um paciente com hipotensão ortostática idiopática e atrofia de múltiplos sistemas (Síndrome de Shy-Drager), tratado com implantação de marca-passo resultando numa evidente melhora na qualidade de vida.

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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.

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Revisão sobre uma síndrome parkinsoniana atípica, a síndrome de Shy-Drager, neuropatia caracterizada por hipotensão ortostática, sintomas disautonômicos (oscilação da pressão arterial, tremores de repouso, bradicinesia, incontinência fecal e urinária) e atrofia de múltiplos sistemas. A paciente apresentou quadro clínico de hipotensão ortostática com quedas frequentes, disfonia, disartria, disfagia e dificuldades para deambulação. Várias doenças metabólicas e neurológicas foram investigadas sem diagnóstico definitivo. O predomínio de sintomas disautonômicos sugeriu a hipótese desta síndrome, que foi confirmada por exame de ressonância nuclear magnética. A doença teve evolução degenerativa de acordo com o descrito na literatura. Trata-se de síndrome rara, mas de morbi-mortalidade significativa que, por não apresentar tratamento definitivo, exige o seu conhecimento, para que, ao menos, possam ser evitadas as complicações inerentes à doença, melhorando, assim, a qualidade de vida dos pacientes.

A case of atypical Parkinson's syndrome, the Shy-Drager Syndrome, with neuropathy characterized by orthostatic hypotension, disautonomic symptoms (oscillation of blood pressure, resting tremor, bradykinesia, fecal and urinary incontinence) and atrophy of multiple systems. The patient had symptomatology of orthostatic hypotension with frequent falls, dysphonia, dysarthria, dysphagia and difficulties in walking. Several metabolic and neurologic diseases were investigated. A precise diagnosis was not reached. The predominance of disautonomic symptons suggests the hypothesis of Shy-Drager syndrome, what was confirmed by magnetic nuclear resonance. The disease had degenerative evolution in compliance with literature. The practitioner should be aware of this rare syndrome.

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INTRODUCTION: Dysautonomic features are the clinical signs and symptoms derived from anomalous functioning of the sympathetic or parasympathetic nervous systems in either the peripheral or central parts. OBJECTIVE: To identify the clinical features related to dysautonomia in a patient with spinocerebellar ataxia with severe functional disability. PATIENTS AND METHODS: We studied a series of cases, including 21 patients with severe disability (confirmed to their bed or wheelchair, unable to walk and totally or partly dependent on other people for essential everyday activities). The patients and their families were closely questioned, and full clinical examination included a test for orthostasia. RESULTS: All patients had some signs of peripheral dysautonomia: all had vasomotor disorders (orthostasia, distal pallor and coldness, Raynaud s phenomenon, etc.), 95.2% (constipation, urinary and rectal incontinence, polachuria, palpitations, tachycardia at rest, etc.), exocrine gland disorders in 71.4% (increased lachrymation, reduced sweating, increased or reduced salivation), 87.5% peripheral tissue nutrition disorders. Similarly, in all patients studied there was evidence of central dysautonomic disorder, with a syndrome of cachexia with bulimia, sleep disorders together with dysregulation of thirst and body temperature. CONCLUSIONS: In patients with type 2 hereditary spinocerebellar ataxia with severe disability there was involvement of the peripheral and central nervous system regulating autonomic function.

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Introducción: las manifestaciones disautonómicas son todos aquellos síntomas o signos clínicos que se derivan de un funcionamiento anómalo del sistema nervioso simpático o parasimpático, tanto en sus porciones periféricas como centrales. Objetivo: Identificar las manifestaciones clínicas relacionadas con disautonomías en el paciente de ataxia espinocerebelosa tipo 2 con discapacidad funcional severa. Pacientes y métodos: Se realizó un estudio de serie de casos en 21 enfermos con discapacidad severa (confinados a la cama o silla de ruedas, que no pueden deambular y dependen total o parcialmente de otra persona para realizar las actividades vitales). Se efectuó un minucioso interrogatorio clínico a enfermos y familiares, así como un examen físico exhaustivo que incluyó la prueba de ortostatismo. Resultados: El 100 por ciento de la muestra presentaron alguna manifestación disautonómica periférica: trastornos vasomotores en el 100 por ciento (ortostatismo, frialdad y palidez distal, fenómeno de Raynaud, etc.), trastornos viscerales en el 95,2 por ciento (constipación, incontinencia vesical y rectal, polaquiuria, palpitaciones,taquicardia en reposo, etc.), glandular exocrino en el 71,4 por ciento (aumento lagrimal, sudoración disminuida, aumento o disminución salival), nutritivo tisular periférico en el 87,5 por ciento. Por otro lado, en el 100 por ciento de los enfermos estudiados se detectaron manifestaciones disautonómicas centrales dadas por un síndrome con predominio de caquexia asociada a bulimia, trastornos del sueño, disregulación de la sed y temperatura corporal. Conclusión: en los enfermos de ataxia espinocerebelosa hereditaria tipo 2 con discapacidad grave se detectó afectación del sistema nervioso central y periférico regulador de la función autónoma(AU)

Introducction: Dysautonomic features are the clinical signs and symptoms derived from anomalous functioning of the sympathetic or parasympathetic nervous systems in either the peripheral or central parts. Objective: To identify the clinical features related to dysautonomia in a patient with spinocerebellar ataxia with severe functional disability. Patients and methods: We studied a series of cases, including 21 patients with severe disability (confirmed to their bed or wheelchair, unable to walk and totally or partly dependent on other people for essential everyday activities). The patients and their families were closely questioned, and full clinical examination included a test for orthostasia. Results: All patients had some signs of peripheral dysautonomia: all had vasomotor disorders (orthostasia, distal pallor and coldness, Raynaud s phenomenon, etc.), 95,2 percent (constipation, urinary and rectal incontinence, polachuria, palpitations, tachycardia at rest, etc.), exocrine gland disorders in 71,4 percent (increased lachrymation, reduced sweating, increased or reduced salivation), 87,5 percent peripheral tissue nutrition disorders. Similarly, in all patients studied there was evidence of central dysautonomic disorder, with a syndrome of cachexia with bulimia, sleep disorders together with dysregulation of thirst and body temperature. Conclusions: In patients with type 2 hereditary spinocerebellar ataxia with severe disability there was involvement of the peripheral and central nervous system regulating autonomic function(AU)

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En los pacientes con intolerancia ortostática, la respuesta hemodinámica al adoptar la posición de pie puede identificar una anormalidad: el síndrome de taquicardia postural ortortostática o una hipotensión ortostática (la cual a menudo puede ser tratada sin requerir de más estudios). Cuando la respuesta hemodinámica al ortostatismo activo es normal, la prueba de inclinación en mesa basculante se hace necesaria para establecer el diagnóstico y seleccionar un tratamiento, en el síncope vasovagal o en el síndrome de taquicardia postural ortostática. Durante la evaluación de la respuesta hemodinámica a la prueba de inclinación es importante la distinción entre el verdadero síncope vasovagal y la llamada respuesta disautonómica que se caracteriza por una disminución gradual y progresiva de la presión arterial que antecede al síncope. La practica clínica actual recomienda el empleo de ß bloqueadores adrenérgicos, fludrocortisona y midodrina para el tratamiento del síncope vasovagal, mientras que los pacientes con el síndrome de taquicardia postural ortostática o con la respuesta disautonómica son mejor tratados con fludrocortisona y midodrina.

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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.

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Shy-Grager syndrome presents as a combination of parkinsonism and autonomic failure (orthostatic hypotesion, anhidrosis, pupillary failure, loss of bladder and bowel sphincter function and iris atrophy). This article introduce to the automatic diagnosis

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Presentamos dos pacientes masculinos, en la sexta década de la vida, cion un cuadro clínico muy manifiesto del Síndrome Shy-Drager (SSD). El síntoma cardinal de estos enfermos está dado por caídas frecuentes, por hipotensión arterial ortostática, con inconciencia, sin respuesta vegetativa simpática (sin frialdad ni taquicardia y con anhidrosis). De forma sumaria el resto del cuadro neurológico estaba expresado por parkinsonismo, impotencia sexual, incontinencia esfinteriana, vesical, disfunción, cerebelosa, hemiparesia, atrofias musculares, etc. Uno de los pacientes fue autopsiado y se reveló extenso proceso degenerativo crónico neuronal en el sistema nervioso central, así como en el periférico, donde incluye el sistema autonómico y atrofia muscular neurógena. El estridor laríngeo de uno de los enfermos durante el sueño, lo atribuimos a la imposibilidad de la abducción de las cuerdas vocales en la inspiración, por atrofia del músculo criacoritenoideo posterior. La respuesta en el caso dos, el tratmiento con indometacina, esteroides y vendaje elástico en los miembros inferiores, fue satisfactoria (AU)

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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

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A síndrome de Shy-Drager é uma doença degenerativa do sistema nervoso central, de etiologia desconhecida, caracterizada por falência progressiva do sistema autonômico e atrofia de múltiplos sistemas. É relatado o caso de uma paciente do sexo feminino que aos 41 anos de idade foi estabelecido o diagnóstico de síndrome de Shy-Drager. Provas funcionais autônomas, exames laboratoriais e exame físico, principalmente minucioso exame neurológico, evidenciaram claramente a falência do sistema nervoso autônomo e sinais de atrofia multissistematizada. Seräo ressaltados aspectos clínicos-evolutivos que caracterizaram os 8 anos da enfermidade, de investigaçäo da funçäo autonômica e de diagnóstico diferencial - já que a doença foi confundida durante a fase inicial com outras enfermidades sistêmicas e do sistema nervoso

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A sindrome de Shy-Drager e uma doenca degenerativa do sistema nervoso central, de etiologia desconhecida, caracterizada por falencia progressiva do sistema autonomo e atrofia de multiplos sistemas. E relatado o caso de uma paciente do sexo feminino que aos 41 anos de idade foi estabelecido o diagnostico de sindrome de Shy-Drager: Provas funcionais autonomas, exames laboratoriais e exame fisico, principalmente minucioso exame neurologico, evidnciaram claramente a falencia do sistema nervoso autonomo e sinais de atrofia multissistematizada. Serao ressaltados aspectos clinicos-evolutivos que caracterizaram os 8 anos da enfermidade, de investigacao da funcao autonomica e de diagnostico diferencial-ja que a doenca foi confundida durante a fase inicial com outras enfermidades sistemicas e do sistema nervoso.

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Presentamos los resultados de administrar un péptido vasoactivo como el octreótido en un paciente portador del síndrome de Shy-Drager, enfermedad que cursa con hipotensión ortostática severa. En este trabajo se describen los efectos de la prueba de inclinación o Tilt test. Durante el estudio basal, nuestro paciente desarrolló hipotensión ortostática severa, con el octreótido se observó un aumento significativo de la presión arterial diastólica sobre todo durante la inclinación a 60o. Este incremento es probablemente secundario a la vasoconstricción esplácnica producida por el octreótido, evitando la caída de la presión al redistribuir la volemia. El octreótido estaría indicado en casos refractarios para prevenir síntomas (AU)

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Report of an early case of Shy-Drager syndrome in a 67 year-old woman patient. Autonomic failure was diagnosed by functional evaluation as well as laboratory tests. MR imaging disclosed a prominent putamina hypodensity in T2-weighted images at high field strength due to iron increased depositing in this basal ganglia. MR imaging evidences confirm Shy-Drager syndrome diagnosis, and contributes for differential diagnosis of idiopathic hypotension (pure autonomic failure) in special in SDS early cases.

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É relatado o caso de uma paciente de 67 anos de idade com quadro inicial da síndrome de Shy-Drager. O diagnóstico foi possível por provas funcionais autonômicas e exames laboratoriais. A ressonância magnética cerebral (contraste baseado na densidade de prótons e em T2) objetivou preminente hipodensidade putaminal em T2, secundária ao aumento do depósito do ferro nesta regiäo. Esse achado da RM confirma o diagnóstico da síndrome de Shy-Drager e permite diferenciá-la da hipotensäo ortostática idiopática, particularmente na fase de início da SSD quando os sinais de comprometimento do SNC säo discretos ou estäo ausentes

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A programmable atrial pacemaker was implanted in an elderly man, 75 years-old, with incapacitating orthostatic hypotension due to automatic dysfunction (Shy-Drager) unresponsive to traditional therapy. Initially, a temporary programmed atrial pacing was implanted paced at 96 beats/minute during the day, reducing the rate at night to 60 betas/minute. An increase in cardiac output was achieved. Favourable results encouraged us to implant a permanent programmed pacemaker. The manufacturer designed a simple, safe programmer operable by the patient which only alters the rate from night (60) to day (96). A marked improvement in symptoms has been observed during a follow-up of nine months without complications. It is recommended that this sort of treatment be considered in elderly patients with symptomatic orthostatic hypotension due to autonomic dysfunction with inadequate compensatory tachycardia who have not responded to traditional therapy.

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