Patricia Campos

A new case of proximal 10q partial trisomy.

We report on a girl with mild phenotypic abnormalities and duplication of chromosome 10q11----10q22. The similarities to two previously reported cases with an identical chromosomal aberration provide further support for the delineation of this entity as a specific, clinically recognisable syndrome.

Encefalopatias epilépticas de la infancia

We present the study of 23 pediatric cases with infantile epileptic encephalopathies at the ambulatory neuropediatric facility of the Hospital Base Cayetano Heredia and ambulatory neurologic facility of Hospital IPPS Guillermo Almenara (Lima, Perú) between January 1984 and October 1988 with 6 to 54 months follow-up. There was one patient with Ohtahara syndrome, 9 with West syndrome and 13 with Lennox-Gastaut syndrome. The clinical and electroencephalographic characteristics were reviewed and related with the outcome. Despite the general characteristics of Ohtahara syndrome our patient had a good prognosis; only two West evolve into Lennox-Gastaut and none of these was West. Although the common features in these syndrome include frequent and intractable seizures and grave prognosis most of our patients had a good control of seizures.We present the study of 23 pediatric cases with infantile epileptic encephalopathies at the ambulatory neuropediatric facility of the Hospital Base Cayetano Heredia and ambulatory neurologic facility of Hospital IPPS Guillermo Almenara (Lima, Peru) between January 1984 and October 1988 with 6 to 54 months follow-up. There was one patient with Ohtahara syndrome, 9 with West sindrome and 13 with Lennox-Gastaut syndrome. The clinical and electroencephalographic characteristics were reviewed and related with the outcome. Despite the general characteristics of Ohtahara syndrome our patient had a good prognosis; only two West evolve into Lennox-Gastaut and none of these was West. Although the common features in these syndrome include frequent and intractable seizures and grave prognosis most of our patients had a good control of seizures.Se presenta una serie de 23 casos de encefalopatias epilepticas de la infancia, vistos en los consultorios externos de Neuropediatria del Hospital General Cayetano Heredia y Neurologia del Hospital IPSS. Guillermo Almenara (Lima, Peru) entre Enero-1984 y Octubre-1988, con un seguimiento entre 6 meses y 4 anos 6 meses para cada paciente. Del total, uno correspondio al sindrome de Ohtahara, 9 fueron West y 13 Lennox-Gastaut. Se revisan las caracteristicas clinico electroencefalograficas de cada uno de los grupos y su evolucion. Con relacion al sindrome de Ohtaharta llama la atencion el buen pronostico encontrado en este paciente; de los 9 West unicamente 2 evolucionaron para Lennox-Gastaut y de estos ultimos ninguno tuvo antecedente de West y contrariamente a lo esperado y teniendo en cuenta los factores de riesgo para mal pronostico, la mayor parte de nuestros pacientes tuvieron un buen control de crisis.

Quiste hidatídico cerebral en niños en el Hospital Nacional Cayetano Heredia. A propósito de un caso.

La enfermedad por Echinococcus granulosus en el sistema nervioso central es de ocurrencia inusual, siendo las localizaciones hepaticas y pulmonar las mas frecuentes. Presentamos la siguiente revision a proposito del unico caso infantil con compromiso neurologico diagnosticado en nuestro hospital.

Enfermedad de Tay-Sachs

La enfermedad de Tay-Sachs es la forma mas comun de neurolipidosis hereditaria, transtorno familiar que se presenta en judios del este de Europa en una proporcion de 1 en 360 casos y en no judios en 1 en 6000 nacidos vivos. Se debe a una deficiencia de la isoenzima BN –acetil hexosaminidasa, que produce una acumulacion de gangliosido GM 2 intraneuronal (1).

Sindrome de Rett: descripción de dos casos

El Sindrome de Rett (SR) fue descrito inicialmente en 1966 por Andreas Rett. Comprende una involucion mental progresiva de inicio antes de los dos anos acompanada de perdida de contacto social y movimientos estereotipados de las manos, apraxia /ataxia de la marcha y crisis de hiperventilacion, presentandose exclusivamente en mujeres (1).

Quiste hidatídico cerebral en niños en el Hospital Nacional Cayetano heredia: a propósito de un caso

Case report of a 12 years old female child who developed in the last seven months focal neurological signs and progressive intracranial hypertension. CT scan showed a giant cystic tumor, and cerebral hydatidosis has been diagnosed. The hydatic cyst was surgically removed without complications. Cerebral hydatidosis is rare, its diagnosis is clinical and tomographic, and the treatment is always surgical.

Procesos expansivos intracraneanos no tumorales correlato clínico - tomográfico

Presentation of clinical-tomographic correlation in 111 cases of non tumoral intracranial expansive processes seen between 1984-1988 in the Hospital Cayetano Heredia (Lima, Peru). Emphasis is given fundamentally to: (1) the importance of stablishing the or-ganicity of partial and late epilepsy; (2) the high incidence rate of inflammatory infectious processes with CNS compromise in underdeveloping countries; (3) the necessity of making public the importance of two parisitic diseases in the differential diagnosis of non tumoral intracranial expansive processes: free living amebiasis, and toxoplasmosis (especially in association with AIDS).