Estudio de variabilidad de la frecuencia cardiaca en pacientes con sospecha de fallas autonómicas. Utilización del Holter y el Diagrama de Poincare

El estudio de la regularidad de la Frecuencia Cardiaca, a través del Holter de 24 horas se hace desde la década de los años 60 y es bastante efectivo. Sin embargo, desde los años noventa comenzaron a efectuarse estudios cortos de Holter en pacientes sospechados de tener fallas autonómicas de control de la frecuencia cardiaca, especialmente en pacientes con comorbilidades tales como Hipertensión, Diabetes Mellitus, Aterosclerosis etc. De aquí la importancia de realizar un test de Holter de diez minutos, divididos en dos tiempos de 5 minutos, primero en decúbito dorsal y luego en bipedestación, especialmente en pacientes de más de cincuenta años o con comorbilidades presentes. Los resultados se presentan luego en gráficos de Poincare, que incluye el programa operativo del dispositivo, que permite un vistazo de la elipse con sus dos ejes, que representan las acciones simpáticas y parasimpáticas sobre la frecuencia cardiaca. Una variabilidad anormal de la frecuencia cardiaca debe ser luego estudiada más profundamente a fin de reafirmar el diagnóstico y ulteriores pasos en el tratamiento.

The variability of Cardiac Frequency is a valuable monitor of the autonomic function and is currently used as tool for study of changes of regularity through Holter 24 hours. From nighties, several researchers have been oriented to stablish relationship between VCF and autonomic failure, especially in patients with comorbidities, such as Hypertension, Diabetes Mellitus, atherosclerosis etc. Actually is well known that a lost or VCF or a minor variability, even in short traces of Holter in 10 minutes, means an autonomic failure, of baroreflex and quimioreflex resources. Hence, the importance of performing test of ten minutes Holter, five in decubitus position and five in standing, to patients of more than fifty years old, or less if comorbidities are presents, to design a Poincare diagram, which is special to indicate in quick view the prevalence of Sympathetic o Vagal action on cardiac frequency; that conduces to a more deep study of Autonomic failure, such tilt test, extended holter of 24 hours, and others medicals images resources.

Falla autonómica asociada a hipoglucemia / Hypoglycemia associated autonomic failure

La hipoglucemia inadvertida (HI) es una complicación del tratamiento de la diabetes mellitus tipo 1 (DM1) y DM2 tratada con insulina o sulfonilureas, que se caracteriza por una capacidad reducida para percibir el inicio de los episodios de hipoglucemia. En general, coexiste con una insuficiente respuesta hormonal contrarreguladora a la hipoglucemia denominada falla autonómica asociada a la hipoglucemia (FAAH). El desarrollo de HI y de falla contrarreguladora a la hipoglucemia aumentan significativamente el riesgo de hipoglucemias severas. Se han desarrollado escalas de puntuación para identificar, en la consulta clínica, a este grupo de personas con elevado riesgo de hipoglucemias severas. La piedra angular del tratamiento consiste en evitar las hipoglucemias mediante una intervención multifactorial de cuidados clínicos y educación estructurada.

Hypoglycemia unawereness is a complication of type 1 diabetes treatment and of type 2 diabetes treatment treated with insulin or sulfonylureas, characterized by a reduced ability to perceive the onset of episodes of hypoglycemia. In general, it coexists with an insufficient counterregulatory hormonal response to hypoglycemia called: hypoglycemia associated autonomic failure (HAAF). The development of hypoglycemia unawereness and counterregulatory failure to hypoglycemia significantly increase the risk of severe hypoglycemia. Scoring scales have been developed to identify this group of people at high risk of severe hypoglycemia in the clinic. The cornerstone of treatment is to avoid hypoglycemia through a multifactorial intervention of clinical care and structured education.

Pure autonomic failure: a case report / 中华神经科杂志

Pure autonomic failure (PAF) is an α-synucleinopathy featured by slowly progressive autonomic failure. A patient who presented with orthostatic hypotension associated dizziness and syncope, postprandial hypotension, supine hypertension and anhidrosis, was hospitalized. The patient did not show incontinence, urinary retention, constipation, ataxia, and extrapyramidal symptoms. In combination of the description of the patient′s symptoms with PAF related references, the pathogenesis, clinical manifestations, diagnosis and differential diagnosis of PAF and its relationship with other α-synucleinopathies were demonstrated in this report.

Avoiding or coping with severe hypoglycemia in patients with type 2 diabetes

Hypoglycemia is a major barrier to achieving the glycemic goal in patients with type 2 diabetes. In particular, severe hypoglycemia, which is defined as an event that requires the assistance of another person to actively administer carbohydrates, glucagon, or take other corrective actions, is a serious clinical concern in patients with diabetes. If severe hypoglycemia is not managed promptly, it can be life threatening. Hypoglycemia-associated autonomic failure (HAAF) is the main pathogenic mechanism behind severe hypoglycemia. Defective glucose counter-regulation (altered insulin secretion, glucagon secretion, and an attenuated increase in epinephrine during hypoglycemia) and a lack of awareness regarding hypoglycemia (attenuated sympathoadrenal activity) are common components of HAAF in patients with diabetes. There is considerable evidence that hypoglycemia is an independent risk factor for cardiovascular disease. In addition, hypoglycemia has a significant influence on the quality of life of patients with diabetes. To prevent hypoglycemic events, the setting of glycemic goals should be individualized, particularly in elderly individuals or patients with complicated or advanced type 2 diabetes. Patients at high-risk for the future development of severe hypoglycemia should be selected carefully, and intensive education with reinforcement should be implemented.

Dysautonomia in Narcolepsy: Evidence by Questionnaire Assessment

BACKGROUND AND PURPOSE: Excessive daytime sleepiness and sudden sleep attacks are the main features of narcolepsy, but rapid-eye-movement sleep behavior disorder (RBD), hyposmia, and depression can also occur. The latter symptoms are nonmotor features in idiopathic Parkinson's disease (IPD). In the present study, IPD-proven diagnostic tools were tested to determine whether they are also applicable in the assessment of narcolepsy. METHODS: This was a case-control study comparing 15 patients with narcolepsy (PN) and 15 control subjects (CS) using the Scales for Outcomes in Parkinson's Autonomic Test (SCOPA-AUT), Parkinson's Disease Nonmotor Symptoms (PDNMS), University of Pennsylvania Smell Test, Farnsworth-Munsell 100 Hue test, Beck Depression Inventory, and the RBD screening questionnaire. RESULTS: Both the PN and CS exhibited mild hyposmia and no deficits in visual tests. Frequent dysautonomia in all domains except sexuality was found for the PN. The total SCOPA-AUT score was higher for the PN (18.47+/-10.08, mean+/-SD) than for the CS (4.40+/-3.09), as was the PDNMS score (10.53+/-4.78 and 1.80+/-2.31, respectively). RBD was present in 87% of the PN and 0% of the CS. The PN were more depressed than the CS. The differences between the PN and CS for all of these variables were statistically significant (all p<0.05). CONCLUSIONS: The results of this study provide evidence for the presence of dysautonomia and confirm the comorbidities of depression and RBD in narcolepsy patients. The spectrum, which is comparable to the nonmotor complex in IPD, suggests wide-ranging, clinically detectable dysfunction beyond the narcoleptic core syndrome.

Keishikashakuyakuto Ameliorated Diverse Autonomic Symptoms such as Diarrhea, Abdominal Pain,Urinary Retention and Orthostatic Hypotension in a Case of Pure Autonomic Failure / 日本東洋医学雑誌

Pure autonomic failure (PAF) is a degenerative disorder with diverse autonomic nervous symptoms, but without somatic nervous symptoms. We encountered a patient with PAF who was successfully treated with keishikashakuyakuto. A 61-year-old man complained of diarrhea, abdominal pain, dysuria and orthostatic hypotension. PAF was strongly suspected, based on a low level of plasma noradrenalin at rest and a finding of severe diffuse sympathetic nerve injury on <SUP>123</SUP>I MIBG myocardial scintigraphy. Various Kampo formulas were not effective, or could not be administered continually. Urinary retention was treated with self-catheterization. After the extract of keishikashakuyakuto was administered, his complaints of diarrhea and abdominal pain gradually decreased, and he was able to eat various kinds of food. His daily living activities improved.Moreover, he could urinate by himself, so the self-catheterization was stopped. Five years later, the diagnosis of PAF was clinically confirmed, but his daily living activities did not deteriorate. This suggests that keishikashakuyakuto can be effective for diseases with diverse autonomic nervous symptoms, such as the present case.

Hipoglicemia como fator complicador no tratamento do diabetes melito tipo 1: [revisão] / Hypoglycemia as a Limiting Factor in the Management of Type 1 Diabetes: [review]

Os portadores de diabetes melito tipo 1 têm, com freqüência, episódios de hipoglicemia durante a insulinoterapia, que, além do desconforto e de proporcionar situações constrangedoras no dia-a-dia, impedem a obtenção do controle glicêmico ideal. Mais ainda, hipoglicemias induzem deficiente mecanismo de contra-regulação em episódio posterior, com diminuição de liberação de adrenalina e dos sintomas de alarme, estabelecendo a síndrome de hipoglicemia associada à insuficiência autonômica. A ocorrência de hipoglicemias durante algumas atividades de risco, em especial a direção veicular, pode resultar acidentes com o paciente e terceiros, além de lesão de propriedade, motivo pelo qual pessoas com diabetes devem ser orientadas quanto aos cuidados na direção de veículos. Em geral, a recuperação neurológica é total após a correção de coma hipoglicêmico. No entanto, quando esses episódios são repetitivos, especialmente em crianças, podem ter como conseqüência distúrbios cognitivos definitivos. A reversão de quadros de hipoglicemia sem sinal de alerta é difícil, devendo-se evitar meticulosamente sua ocorrência, adequando o tratamento, os alvos glicêmicos, utilizando a monitoração domiciliar e fazendo treinamento para o reconhecimento precoce de hipoglicemias.

Type 1 diabetic patients frequently present hypoglycemic episodes during their insulinotherapy, which, besides the discomfort and constrains does not allow the ideal glycemic control. Further, hypoglycemic events lead to the deficiency of the counter-regulation mechanisms in the subsequent episode, with a decrease in the release of epinephrine and the symptoms of warming, with great risk of severe hypoglycemia. The occurrence of hypoglycemia during some risky activities, specially driving, could result in accidents with the patient and /or third parts including property damage, stressing here the need to advise diabetics against having the necessary caution wheli driving. Generally the connective recovery is total after correcting a hypoglycemic coma. However when these episodes are repetitive, particularly in children, they could result in definitive cognitive disturbances. Hypoglycemic events without a warning signal (hypoglycemic unawareness) are difficult to reverse, thus it is necessary to prevent their occurrence, adjusting the treatment with glycemic targets, using continuous glucose monitoring at home and teaching them how to have an early recognition of hypoglycemia.

Case Report of Epidural Block for A Patient with The Shy - Drager Syndrome / 대한마취과학회지

The Shy-Drager syndrome is a very rare chronic progressive disease characterised by autonomic failure and multiple system atropy. The main clinical manifestations of this syndrome are orthostatic hypotension, urinary and bowel dysfunction, impaired sexual potency and parkinsonean symptoms. A key in the management of anesthesia is the maintenance of cardiovascular atability. These patient are suffered from defective baroreceptor regulation of the blood pressure as a result of autonomic failure. We report a successful epidural anesthesia for vwicolithotomy in a female patient with Shy-Drager syndrome.

Case Report of Epidural Block for A Patient with The Shy - Drager Syndrome / 대한마취과학회지

The Shy-Drager syndrome is a very rare chronic progressive disease characterised by autonomic failure and multiple system atropy. The main clinical manifestations of this syndrome are orthostatic hypotension, urinary and bowel dysfunction, impaired sexual potency and parkinsonean symptoms. A key in the management of anesthesia is the maintenance of cardiovascular atability. These patient are suffered from defective baroreceptor regulation of the blood pressure as a result of autonomic failure. We report a successful epidural anesthesia for vwicolithotomy in a female patient with Shy-Drager syndrome.