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      Evaluación del funcionamiento del sistema nervioso autonómico en pacientes con polineuropatía de Charcot Marie Tooth tipo 1 Translated title: Evaluation of the functioning of the autonomic nervous system in patients with Charcot Marie Tooth 1 polyneuropathy


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          Se realizó este estudio para evaluar las alteraciones clínicas y electrofisiológicas del sistema autonómico en pacientes con la enfermedad de Charcot Marie Tooth 1 y conocer la sensibilidad de índices electrofisiológicos y clínicos disponibles para identificar y cuantificar disautonomías asociadas a esta enfermedad. Se hizo un estudio analítico de casos y controles. Se obtuvo una muestra de 15 pacientes con polineuropatía sensorimotora desmielinizante hereditaria tipo 1 y 20 controles pareados. A todos los pacientes se les aplicó un formulario clínico para disautonomía. También se monitoreó la frecuencia cardíaca, se observó la variabilidad en reposo y durante la maniobra de Valsalva, las respiraciones profundas y el ortostatismo. Se calcularon los coeficientes de variación y los índices de Valsalva (VS), de espiración/inspiración y máximo/mínimo al ortostatismo. Se halló que el 60 % (9 casos) de los pacientes padecían de frialdad en los pies y las manos, urgencia para la micción y digestiones lentas con llenado fácil durante el acto de comer. El coeficiente de Inspiración/espiración fue estadísticamente menor en los pacientes que en los controles (p = 0,01). No se encontraron diferencias estadísticamente significativas en el resto de los indicadores de VFC (índice Max/Min, RR medio, desviaciones estándar, y MSSD media) registrados en pacientes y controles. Se concluyó que los pacientes con CMT tienen en su cuadro clínico manifestaciones de disautonomía y que el índice de respiraciones profundas evidenció una lesión ligera parasimpática cardiovascular.

          Translated abstract

          A study was conducted to evaluate the clinical and electrophysiological alterations of the autonomic nervous system in patients with Charcot Marie Tooth 1 disease and to know the sensitivity of electrophysiological and clinical indexes available to identify and quantify dysautonomies associated with this disease. An analytical case-control study was undertaken. It was obtained a sample of 15 patients with type 1 hereditary motor and sensory demyelinating polyneuropathy and 20 matched controls. All the patients were applied a clinical questionnaire for dysautonomy. Heart rate was monitored, and it was observed the variability at rest and during Valsalva's maneuver, deep breaths and orthostatism. The variation coefficients, the Valsalva's indexes, and the expiration/inspiration and maximum/minimum coefficients on orthatism were calculated. It was found that 60 % (9 cases) of the patients suffered from coldness in hands and feet, urgency for miction, had slow digestions and they easily filled up when eating. The expiration/inspiration coefficient was statistically lower in patients than in controls (p = 0.01). No statistically significant differences were found in the rest of the VFC indicators (Max/Min index, mean RR, standard deviations and mean MSSD) registered in patients and controls. It was concluded that the patients with CMT have manifestations of dysautonomy in their clinical picture and that the index of deep breaths evidenced a mild parasympathetic cardiovascular lesion.

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          Most cited references28

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          The value of cardiovascular autonomic function tests: 10 years experience in diabetes.

          Five simple, noninvasive cardiovascular reflex tests have been used to assess autonomic function in one center over the past 10 yr. Seven hundred seventy-four diabetic subjects were tested for diagnostic and research purposes. In 543 subjects completing all five tests, abnormalities of heart rate tests occurred in 40%, while abnormal blood pressure tests occurred in less than 20%. Their results were grouped as normal (39%), early (15%), definite (18%), and severe (22%) involvement. Six percent had an atypical pattern of results. Two hundred thirty-seven diabetic subjects had the tests repeated greater than or equal to 3 mo apart: 26% worsened, 71% were unchanged, and only 3% improved. The worsening followed a sequential pattern with first heart rate and later additional blood pressure abnormalities. Comparison between a single test (heart rate response to deep breathing) and the full battery in 360 subjects showed that one test alone does not distinguish the degree or severity of autonomic damage. These tests provide a useful framework to assess autonomic neuropathy simply, quickly, and noninvasively.
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            Principios de Neurología

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              The valvue of cardiovascular autonomic function test: 10 years experience in diabetes


                Author and article information

                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Revista Cubana de Medicina
                Rev cubana med
                ECIMED (Ciudad de la Habana )
                April 2005
                : 44
                : 1-2
                : 0
                [1 ] Hospital Clinicoquirúrgico Lucía Iñiguez Landin Cuba
                [2 ] Instituto Nacional de Neurología y Neurocirugía Mexico
                [3 ] Facultad Mariana Grajales



                SciELO Cuba

                Self URI (journal page): http://scielo.sld.cu/scielo.php?script=sci_serial&pid=0034-7523&lng=en
                MEDICINE, LEGAL

                Social law,Medicine,Internal medicine
                Charcot Marie Tooth,polineuropatía hereditaria,disautonomía,hereditary polineuropathy,dysautonomy


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