[Tolerability and adverse effects of propofol in the Wada test]. / Tolerabilidad y efectos adversos del propofol en la prueba de Wada.

INTRODUCTION: The Wada test consists of the selective and reversible inhibition of a cerebral hemisphere by intracarotid injection of amobarbital in order to evaluate the laterality of language and memory. However, there are other anesthetic drugs such as propofol, as an alternative for the test. OBJECTIVE: The objective of the study was to describe the tolerability and adverse effects (AE) of the use of propofol for the Wada test, during the presurgical study of patients with drug-resistant epilepsy. METHODS: Consecutive patients with a diagnosis of drug-resistant structural epilepsy were selected who underwent the Wada test during the pre-surgical study in the period from June 2012 to May 2019. The patients were retrospectively evaluated. The AE were described according to the Mikuni classification, modified by Curot. The variables of sex, age, epileptic foci laterality, language laterality, lesional substrate, etiology and dose of administered Propofol were analyzed for any statistical significance. RESULTS: A total of 74 patients, 40 men (54%), were studied. Forty-seven patients (63.5%) had at least one AE. The mean dose of propofol was 9.23 mg. The most frequent AE were tearing, sweating and red eye, corresponding to group I (57%). One patient developed convulsive status epilepticus, an important AE not previously described during the Wada test. CONCLUSION: Performing the Wada test with propofol causes frequent mild adverse effects, which do not prevent its completion. We describe a case of convulsive status epilepticus as the only serious AE.

TITLE: Tolerabilidad y efectos adversos del propofol en la prueba de Wada.Introducción. La prueba de Wada consiste en la inhibición selectiva y reversible de un hemisferio cerebral mediante la inyección intracarotídea de amobarbital con el objetivo de evaluar la lateralidad del lenguaje y la memoria. Existen otros fármacos anestésicos, como el propofol, como alternativa para la prueba. Objetivo. El objetivo del estudio fue describir la tolerabilidad y los efectos adversos (EA) del uso de propofol para la prueba de Wada durante el estudio prequirúrgico de pacientes con epilepsia farmacorresistente. Pacientes y métodos. Se seleccionó a pacientes con diagnóstico de epilepsia estructural farmacorresistente consecutivos, quienes se sometieron a la prueba de Wada durante el estudio prequirúrgico en el período de junio de 2012 a mayo de 2019. Los pacientes fueron evaluados de manera retrospectiva. Los EA se describieron según la clasificación de Mikuni, modificada por Curot. Se analizaron las variables de sexo, edad, lateralidad del foco epiléptico, lateralidad del lenguaje, sustrato lesional, etiología y dosis de propofol administrada en busca de significación estadística. Resultados. Se estudió a un total de 74 pacientes, de los cuales 40 eran hombres (54%). Cuarenta y siete pacientes (63,5%) tuvieron al menos un EA. La dosis media de propofol fue de 9,23 mg. Los EA más frecuentes fueron lagrimeo, sudoración y ojo rojo, correspondientes al grupo I (57%). Un paciente desarrolló estado epiléptico convulsivo, EA importante no descrito anteriormente durante la prueba de Wada. Conclusión. La realización de la prueba de Wada con propofol ocasiona frecuentes efectos adversos leves, los cuales no impiden su finalización. Describimos un caso de estado epiléptico convulsivo como único EA grave.

[TK2 mutations and late onset myopathy: first description in a Mexican patient]. / Mutacion del gen TK2 y miopatia de inicio tardio: descripcion del primer caso mexicano.

TITLE: Mutacion del gen TK2 y miopatia de inicio tardio: descripcion del primer caso mexicano.

Polirradiculoneuropatía crónica inflamatoria desmielinizante. Experiencia de 10 años en un centro mexicano / Chronic inflammatory demyelinating polyradiculoneuropathy 10 years’ experience in a mexican centre

La polirradiculoneuropatía crónica inflamatoria desmielinizante (CIDP) es una neuropatía autoinmunecrónica adquirida potencialmente tratable. Existen pocos estudios en la bibliografía acerca de la epidemiología, las características clínicas de presentación y el pronóstico funcional a largo plazo. Objetivo. Describir las formas clínicas y neurofisiológicas de los pacientes con CIDP al inicio y su seguimiento al cabo de un año. Pacientes y métodos. Estudio descriptivoy retrospectivo de pacientes hospitalizados en nuestra unidad desde 1995 hasta 2005. Los casos se definieron deacuerdo a los criterios del Grupo para la causa y tratamiento de las neuropatías (INCAT). Se documentaron las características demográficas, formas clínicas de presentación, hallazgos de neurofisiología, líquido cefalorraquídeo y pronóstico funcionalal año. Se realizó un análisis descriptivo estadístico. Resultados. Se incluyó a 26 pacientes (12 varones, 46,15%, y 14 mujeres, 53,84%) con edades comprendidas entre los 15 y los 71 años (40,17 ± 15,7 años). La CIDP se asoció a otras enfermedades autoinmunes en el 20,8% de los pacientes. Las características predominantes al inicio de la enfermedad fueron la paresia y las parestesias simétricas distales en las cuatro extremidades, hiperproteinorraquia y desmielinización con degeneración axonal. La prednisona se administró en el 43% de los casos. Al año, permanecieron asintomáticos cinco pacientes (22,72%), con mejoría parcial en 13 (59,09%) y sin mejoría en cuatro (18,18%). Conclusión. La forma de presentación clínica inicial más frecuente de la CIDP en nuestra población es la de cuadriparesia y parestesias simétricas distales, hiperproteinorraquiay desmielinización con degeneración axonal, que se relacionan con buen pronóstico funcional a un año

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic, but potentially treatable, acquired autoimmune neuropathy. A review of the literature shows that few studies have been conducted on its epidemiology, presenting symptoms and long-term functional prognosis. Aim. To describe the clinical and neurophysiological forms of patients with CIDP at the outset and their follow-up at one year. Patients and methods. We conducted a descriptive,retrospective study of patients who were hospitalised in our unit between 1995 and 2005. The cases were defined inaccordance with Inflammatory Neuropathy Cause and Treatment (INCAT) group criteria. Data gathered included demographic characteristics, forms of clinical presentation, neurophysiological findings, cerebrospinal fluid and functional prognosis at one year. A statistical descriptive analysis was performed. Results. The sample consisted of 26 patients –12 males (46.15%)and 14 females (53.84%)– between 15 and 71 years of age (40.17 ± 15.7 years). CIDP was associated with other autoimmune diseases in 20.8% of the patients. The predominant features at the outset of the disease were paresis and distal symmetrical paresthesias in the four limbs, high protein levels in cerebrospinal fluid and demyelination with axonal degeneration. Prednisone was administered in 43% of the cases. At one year, five patients remained asymptomatic (22.72%), there was apartial improvement in 13 (59.09%) and no improvement was seen in four cases (18.18%). Conclusions. The most frequent initial form of clinical presentation of CIDP in our population is quadriparesis and distal symmetrical paresthesias, high protein levels in cerebrospinal fluid and demyelination with axonal degeneration, which are related to a good functional prognosis at one year

[Chronic inflammatory demyelinating polyradiculoneuropathy. 10 years' experience in a Mexican centre]. / Polirradiculoneuropatía crónica inflamatoria desmielinizante. Experiencia de 10 años en un centro mexicano.

INTRODUCTION: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic, but potentially treatable, acquired autoimmune neuropathy. A review of the literature shows that few studies have been conducted on its epidemiology, presenting symptoms and long-term functional prognosis. AIM: To describe the clinical and neurophysiological forms of patients with CIDP at the outset and their follow-up at one year. PATIENTS AND METHODS: We conducted a descriptive, retrospective study of patients who were hospitalised in our unit between 1995 and 2005. The cases were defined in accordance with Inflammatory Neuropathy Cause and Treatment (INCAT) group criteria. Data gathered included demographic characteristics, forms of clinical presentation, neurophysiological findings, cerebrospinal fluid and functional prognosis at one year. A statistical descriptive analysis was performed. RESULTS: The sample consisted of 26 patients--12 males (46.15%) and 14 females (53.84%)--between 15 and 71 years of age (40.17 +/- 15.7 years). CIDP was associated with other autoimmune diseases in 20.8% of the patients. The predominant features at the outset of the disease were paresis and distal symmetrical paresthesias in the four limbs, high protein levels in cerebrospinal fluid and demyelination with axonal degeneration. Prednisone was administered in 43% of the cases. At one year, five patients remained asymptomatic (22.72%), there was a partial improvement in 13 (59.09%) and no improvement was seen in four cases (18.18%). CONCLUSIONS: The most frequent initial form of clinical presentation of CIDP in our population is quadriparesis and distal symmetrical paresthesias, high protein levels in cerebrospinal fluid and demyelination with axonal degeneration, which are related to a good functional prognosis at one year.

[Brachial diplegia as a variant of amyotrophic lateral sclerosis (Vulpian-Bernhart syndrome): the first case reported in Mexico and a review of the literature]. / Diplejía braquial como variante de esclerosis lateral amiotrófica(síndrome de Vulpian-Bernhart): descripción del primer casomexicano y revisión de la bibliografía.

INTRODUCTION: Brachial diplegia refers to an atypical clinical variant of amyotrophic lateral sclerosis (ALS), which is defined as a lower motor neuron disease in the upper limbs, with no significant functional complications in other regions. CASE REPORT: A 65-year-old female who presented a clinical picture of brachial diplegia that slowly progressed over a period of 72 months. Neurophysiology studies revealed a chronic denervation process. Throughout a follow-up lasting one year the clinical course remained stable. CONCLUSIONS: Brachial diplegia in females is a rare form of presentation of ALS and they probably have a higher survival rate than males.

Diplejía braquial como variante de esclerosis lateral amiotrófica (síndrome de Vulpian-Bernhart): descripción del primer caso mexicano y revisión de la bibliografía / Brachial diplegia as a variant of amyotrophic lateral sclerosis (vulpian-bernhart syndrome): the first case reported in Mexico and a review of the literature

La diplejía braquial se refiere a una variante clínica atípica de esclerosis lateral amiotrófica (ELA),que se define como una alteración de la neurona motora inferior de las extremidades superiores, sin una complicación funcional significativa de otras regiones. Caso clínico. Mujer de 65 años que presentó un cuadro lentamente progresivo de diplejíabraquial durante 72 meses. Los estudios de neurofisiología revelaron un proceso de denervación crónico. La evolución clínica fue estable durante 1 año de seguimiento. Conclusión. La diplejía braquial en mujeres es una presentación rara de ELA y probablemente tengan una sobrevida mayor con relación a los hombres

Brachial diplegia refers to an atypical clinical variant of amyotrophic lateral sclerosis (ALS), whichis defined as a lower motor neuron disease in the upper limbs, with no significant functional complications in other regions. Case report. A 65-year-old female who presented a clinical picture of brachial diplegia that slowly progressed over a period of 72 months. Neurophysiology studies revealed a chronic denervation process. Throughout a follow-up lasting one year the clinical course remained stable. Conclusions. Brachial diplegia in females is a rare form of presentation of ALS and they probably have a higher survival rate than males