[Achalasia, alacrima without adrenal insufficiency with peripheral and autonomic neurological dysfunction (Allgrove's syndrome)]. / Acalasia, alacrima sin insuficiencia suprarrenal, con disfunción neurológica periférica y autonómica (síndrome de Allgrove).

OBJECTIVE: To present a patient with Allgrove's syndrome and peripheral and autonomic neurological dysfunction. This pathological entity has not been previously reported in national medical literature. BACKGROUND: Allgrove's syndrome is comprised of achalasia, alacrima and adrenal insufficiency. It is a rare disease. Some isolated cases have been informed in the literature. The most extensive series was published by Grant et al in 1993. In this study, 20 patients from various european countries were analyzed. METHOD: We describe a patient with diagnosis suggestive of Allgrove's syndrome and expose the results of a review of the medical literature concerning this syndrome. We emphasize in neurological dysfunction of these patients. Such abnormalities are not actually well defined in this syndrome. RESULTS: A twelve-year-old male patient was admitted to the hospital for dysphagia. A diagnosis of achalasia was performed with endoscopy and esophagogram and confirmed with esophageal manometry. Physical examination showed alacrima and neurological dysfunction. Stimulation of adrenal function with ACTH (Cortosin) was normal. Neurologic examination revealed: peripheral neuropathy, autonomic dysfunction; corticospinal, psychomotor and intellectual impairment. CONCLUSION: Allgrove's syndrome must be in mind in physicians who see children with achalasia. Nevertheless adrenal insufficiency is not always present in cases when diagnosis is performed. It may appear several years afterwards. Neurological dysfunction seems to be the most prominent feature.

Reflejo palmomentoniano.Su significado clinico en enfermedad neurologica. / Palmo-mental reflex. The clinical significance in neurological disease

Se realizo estudio prospectivo, ciego, para establecer la relacion del reflejo palmomentoniano (PM) y enfermedad neurologica con anormalidad en la tomografia axial computada (TAC). Cien pacientes no seleccionados, asistentes a la consulta externa del INNN fueron estudiados mediante investigacion del PM, antecedente de trauma craneoencefalico (TCE) y TAC.Los pacientes se agruparon por diagnostico, las edades fluctuaron entre 3 y 82 anos, el 46% fueron del sexo masculino. Se realizo analisis de correlacion multivariada y el Stepwise entre las diferentes variables. Los resultados del analisis fueron: 1) El PM y TAC anormal fue mas frecuente en neoplasias intracraneales, traumatismos del sistema nervioso y enfermedades heredo-degenerativas y desmielinizantes; 2) El PM resulto mas frecuente en pacientes con antecedentes de TCE y en quienes presentaban TAC anormal; 3) El PM unilateral predomino en pacientes menores de 29 anos, principalmente en menores de 10 anos; 4) EL MP unilateral se asocio frecuentemente con TAC normal; (P < 0.01). Consideramos que existe relacion entre la presencia del PM y anormalidad estructural en la TAC. El PM unilateral se relaciono con posible retardo en la maduracion cerebral. Concluimos que este reflejo es un signo de valor clinico en enfermedad neurologica

Sindrome de Fisher. Presentacion de 4 casos y revision de la literatura. / Fisher's syndrome. Report of 4 cases and review of the literature

El Sindrome de Fisher (oftalmoplejia, ataxia y arreflexia) es considerado variante del Sindrome de Guillain-Barre-Strohl, por las siguientes similaridades: historia de infeccion viral inespecifica, ausencia de reglejos miotaticos, curso clinico benigno y disociacion albuminocitologica en el LCR Se reportan 4 casos con este sindrome (tres del Hospital Universitario y uno del Instituto Nacional de Neurologia y Neurocirugia de Mexico), realizando revision de la literatura. Estos casos representan el segundo reporte de Sindrome de Fisher en nuestro pais