Disfunción autonómica en la enfermedad de Parkinson: enfoque práctico / Autonomic dysfunction in Parkinson's disease: A practical approach

RESUMEN Los síntomas por disfunción autonómica son comunes en la enfermedad de Parkinson y están presentes en todas las fases de la enfermedad. Cubren una amplia variedad de síntomas, como manifestaciones gastrointestinales, urinarias, disfunción eréctil e hipotensión ortostática, entre otros, lo que genera discapacidad y deterioro en la calidad de vida. Afortunadamente, con intervenciones no farmacológicas y farmacológicas estos síntomas pueden mejorar sustancialmente, por lo que su evaluación resulta vital para el bienestar del paciente.

SUMMARY Symptoms due to autonomic dysfunction are common in Parkinson's Disease, being present in all phases of the disease. It covers a wide variety of symptoms including gastrointestinal, urinary, erectile dysfunction, and orthostatic hypotension among others, generating disability and deterioration in quality of life. Fortunately, with non-pharmacological and pharmacological interventions, these symptoms can improve substantially, making the evaluation of these symptoms vital for the patient's well-being.

Síndrome de hiperatividade simpática paroxística causada por síndrome da embolia gordurosa / Paroxysmal sympathetic hyperactivity syndrome caused by fat embolism syndrome

RESUMO A hiperatividade simpática paroxística representa uma complicação incomum, com potencial risco à vida, de lesões cerebrais graves, mais comumente de origem traumática. Seu diagnóstico clínico se baseia na manifestação recorrente de taquicardia, hipertensão, diaforese, taquipneia e, às vezes, febre, além de posturas distônicas. Os episódios podem ser induzidos por estímulos ou ocorrer de forma espontânea. É comum que ocorra subdiagnóstico desta síndrome, e o retardamento de seu reconhecimento pode aumentar a morbidade e a incapacidade em longo prazo. Evitar os desencadeantes e a farmacoterapia podem ter muito sucesso no controle desta complicação. A síndrome da embolia gordurosa é uma complicação rara, mas grave, das fraturas de ossos longos. Sinais neurológicos, petéquias hemorrágicas e insuficiência respiratória aguda são as características que constituem seu quadro clínico. O termo "embolia gordurosa cerebral" é estabelecido quando predomina o envolvimento neurológico. O diagnóstico é clínico, porém achados específicos de neuroimagem podem confirmá-lo. As manifestações neurológicas incluem diferentes graus de alteração da consciência, défices focais ou convulsões. Seu tratamento é de suporte, porém são possíveis desfechos favoráveis, mesmo nos casos com apresentação grave. Relatamos dois casos de hiperatividade simpática paroxística após embolia gordurosa cerebral, uma associação muito incomum.

ABSTRACT Paroxysmal sympathetic hyperactivity represents an uncommon and potentially life-threatening complication of severe brain injuries, which are most commonly traumatic. This syndrome is a clinical diagnosis based on the recurrent occurrence of tachycardia, hypertension, diaphoresis, tachypnea, and occasionally high fever and dystonic postures. The episodes may be induced by stimulation or may occur spontaneously. Underdiagnosis is common, and delayed recognition may increase morbidity and long-term disability. Trigger avoidance and pharmacological therapy can be very successful in controlling this complication. Fat embolism syndrome is a rare but serious complication of long bone fractures. Neurologic signs, petechial hemorrhages and acute respiratory failure constitute the characteristic presenting triad. The term cerebral fat embolism is used when the neurological involvement predominates. The diagnosis is clinical, but specific neuroimaging findings can be supportive. The neurologic manifestations include different degrees of alteration of consciousness, focal deficits or seizures. Management is supportive, but good outcomes are possible even in cases with very severe presentation. We report two cases of paroxysmal sympathetic hyperactivity after cerebral fat embolism, which is a very uncommon association.

Prueba de basculación (Tilt Table Testing): Comparación de dos protocolos: isoproterenol versus nitroglicerina / Comparison of the use of isoproterenol or nitroglycerine during the Tilt test

Background: There is debate about the advantages of different protocols usefulness of tilt test for the diagnosis of vasovagal collapse. Aim: To compare the sensitivity, specificity, adverse reactions, complications and time requirements of two different Tilt test protocols. Material and Methods: A Tilt test using isoproterenol in progressive doses (2 μg for 10 min and 5 μg for 5 min posteriorly was performed in 159 patients aged 9 to 84 years (59 males). Another Tilt test using sublingual nitroglycerine in doses of 0.3 mg was performed in 201 patients aged 8 to 87 years (62 males). Also, 20 healthy volunteers were tested. Results: The positivity rates of the tests using isoproterenol and nitroglycerin were 75.5 and 77.6% respectively (NS). The figures for sensitivity were 98.4 and 99.3% (NS). The figures for specificity were 93.2 and 98.4% (NS). The test using isoproterenol requires 15 more minutes. As adverse reactions, 38% of participants experienced palpitations with isoproterenol and 22% experienced headache with nitroglycerin. Conclusions: The Tilt test with nitroglycerin is shorter, simpler, painless, with less personnel involved and has the same diagnostic accuracy than the test with isoproterenol.

Hallazgos ergométricos en pacientes chagásicos, asintomáticos, con electrocardiograma normal y sin cardiopatía evidenciable / Ergometric Findings in Asymptomatic Chagasic Patients, With Normal ECG and Without Evident Heart Disease / Ergometric Findings in Asymptomatic Chagasic Patients, With Normal ECG and Without Evident Heart Disease Background

Introducción Entre las manifestaciones más precoces de la enfermedad de Chagas se encuentran la disautonomía y la dispersión de los intervalos QT en el electrocardiograma, que han demostrado ser predictores de mayor morbimortalidad. Las nuevas tecnologías diagnósticas que posibilitan el hallazgo temprano de alteraciones endoteliales o disautonómicas deben originar mejores estrategias de diagnóstico y tratamiento que permitan la detección precoz de pacientes con riesgo de evolucionar a la miocardiopatía chagásica crónica o de sufrir muerte súbita. Objetivos 1) Investigar los signos clínicos, electrocardiográficos y ergométricos de pacientes chagásicos asintomáticos, con electrocardiograma normal y sin patología clínica evidenciable. 2) Comparar los datos que presentan durante la prueba ergométrica graduada estos pacientes chagásicos con los de un grupo de características clínicas similares pero con serología negativa para Chagas. Material y métodos Estudio observacional, retrospectivo, en el que se incluyeron 74 pacientes chagásicos y 28 sujetos sanos en los que se efectuó una ergometría por control clínico entre marzo de 2009 y septiembre de 2011. Resultados Los pacientes chagásicos presentaron más a menudo frecuencia cardíaca basal = 60 lpm (27,0% vs. 7,1%; p = 0,02), menor frecuencia cardíaca máxima (129,5 ± 22,8 lpm vs. 145,2 ± 16,5 lpm; p = 0,002), mayor incompetencia cronotrópica con frecuencia máxima < 80% que la preestablecida (47,3% vs. 14,3%; p = 0,001) y mayor frecuencia de arritmia ventricular durante el ejercicio y la recuperación (25,7% vs. 0%; p = 0,003). Durante el ejercicio, mientras que en los pacientes del grupo control el intervalo QTc se acortó normalmente, en la mayoría de los pacientes del grupo Chagas se prolongó en forma notoria (grupo Chagas: 412,1 a 432,8 mseg, p = 0,0005; grupo control: 412,4 a 385,8 mseg, p < 0,0001). Conclusiones Los pacientes chagásicos asintomáticos sin cardiopatía demostrable presentaron durante la ergometría una alta prevalencia de signos de disautonomía, como la incompetencia cronotrópica y la prolongación del intervalo QTc durante el esfuerzo. La prueba ergométrica graduada es un método de diagnóstico incruento, confiable y de relativo bajo costo que puede contribuir al diagnóstico temprano de las alteraciones disautonómicas y de la repolarización ventricular y así identificar a los pacientes chagásicos con mayor riesgo de muerte súbita arritmogénica.

Dysautonomia and QT interval dispersion are one of the earliest manifestations of Chagas disease which have proved to be predictors of increased morbi-mortality. New diagnostic technologies revealing earlier endothelial or dysautonomic disorders should provide better diagnostic and treatment strategies for the premature detection of patients with risk of developing chronic chagasic cardiomyopathy or sudden death. Objectives The aims of this study were: 1) To investigate clinical, electrocardiographic and ergometric characteristics in asymptomatic chagasic patients with normal electrocardiogram and no clinical evidence of the pathology. 2) To compare graded exercise testing results between chagasic patients and subjects with similar clinical characteristics but negative serological tests for Chagas. Methods This retrospective, observational study included 74 chagasic patients and 28 healthy subjects who underwent exercise testing during clinical check-up between March 2009 and September 2011. Results Chagasic patients often presented baseline heart rate £ 60 bpm (27.0% vs. 7.1%, p=0.02), lower maximal heart rate (129.5±22.8 vs. 145.2±16.5 bpm; p=0.002), prevalence of chronotropic incompetence, with maximum heart rate < 80% pre-established value (47.3% vs. 14.3%; p=0.001) and higher rate of ventricular arrhythmias during exercise and recovery (25.7% vs. 0%; p=0.003). While in the control group patients the QTc interval shortened normally during exercise, in most chagasic patients it was noticeably prolonged (Chagas group: 412.1 to 432.8 ms; p = 0.0005; control group: 412.4 to 385.8 ms; p< 0.0001). Conclusions Asymptomatic chagasic patients without verifiable heart disease showed a high prevalence of dysautonomia, such as chronotropic incompetence and QTc interval prolongation during exercise. Stress testing is a noninvasive, reliable and relatively inexpensive diagnostic method that can contribute to the early diagnosis of dysautonomic and ventricular repolarization disorders and thus identify patients with Chagas disease who are at increased risk of arrhythmogenic sudden death.