RESUMEN
OBJECTIVE: Congenital bilateral perisylvian syndrome (CBPS) is frequently caused by polymicrogyria (PMG). The aim of this study was to correlate the clinical and psycholinguistic aspects with neuroradiological data of patients with CBPS. METHODS: Thirty-one patients were studied. We performed a clinical investigation of the patients and their families, including MRI scanning, neuropsychological tests and language evaluation. RESULTS: The statistical analysis showed that: a) prenatal events are associated with the non-familial type of PMG; b) diffuse PMG is associated with pseudobulbar signs, as opposed to BPPP; c) motor deficit is associated with diffuse PMG; d) epilepsy is equally present in patients with both familial or non-familial PMG, but is more frequently seen in patients with diffuse PMG; e) dyslexia and SLI can be a feature of both the diffuse or BPPP, and either familial or sporadic cases of PMG. CONCLUSIONS: The severity of clinical manifestations in CBPS is correlated with the extent of cortical involvement. Most patients with CBPS have a history of speech delay or language difficulties and no epilepsy. Dyslexia can be found in patients with PMG.
Asunto(s)
Corteza Cerebral/anomalías , Malformaciones del Sistema Nervioso/diagnóstico , Psicolingüística/métodos , Adolescente , Adulto , Anciano , Corteza Cerebral/patología , Corteza Cerebral/fisiopatología , Niño , Dislexia/etiología , Dislexia/patología , Epilepsia/congénito , Epilepsia/etiología , Epilepsia/patología , Salud de la Familia , Femenino , Humanos , Trastornos del Desarrollo del Lenguaje/etiología , Trastornos del Desarrollo del Lenguaje/fisiopatología , Pruebas del Lenguaje/estadística & datos numéricos , Imagen por Resonancia Magnética/métodos , Masculino , Malformaciones del Desarrollo Cortical/complicaciones , Malformaciones del Desarrollo Cortical/patología , Malformaciones del Desarrollo Cortical/fisiopatología , Persona de Mediana Edad , Malformaciones del Sistema Nervioso/clasificación , Malformaciones del Sistema Nervioso/genética , Pruebas Neuropsicológicas/estadística & datos numéricos , Linaje , Estudios Prospectivos , Factores de Riesgo , Síndrome , Adulto JovenRESUMEN
Polymicrogyria (PMG) is a malformation of cortical development characterized by an excessive number of small gyri and abnormal cortical lamination, giving the cortical surface an irregular and gross appearance. The severity of clinical manifestations correlates with the extent of cortical involvement. The objective of the present study was to describe three families with linguistic features of developmental language disorder and reading impairment, and to establish a neuroanatomic correlation through neuroimaging. Subjects have been submitted to a comprehensive protocol including psychological assessment, language evaluation, neurological examination, and neuroimaging investigation. In our families, children usually had the diagnosis of developmental language disorder while adults had the diagnosis of reading impairment. MRI showed perisylvian polymicrogyria in several subjects of each family. Our data support the idea that there is a co-occurrence of developmental language disorder and reading impairment and both conditions may be associated with polymicrogyria.
Asunto(s)
Corteza Cerebral/anomalías , Dislexia/etiología , Dislexia/patología , Malformaciones del Desarrollo Cortical/complicaciones , Malformaciones del Desarrollo Cortical/patología , Adolescente , Adulto , Niño , Femenino , Humanos , Lenguaje , Imagen por Resonancia Magnética , Masculino , Estudios Prospectivos , Lectura , Escalas de WechslerRESUMEN
This is a review that summarizes the work done in our laboratory during the last three years. We have studied four dyslexic brains. They all bear a symmetric anatomical pattern in a structure closely related to the language areas (planum temporale), which is more commonly asymmetric in normal brains. In addition, their microscopic examination shows numerous ectopias and dysplasias in the cerebral cortex. The high incidence of immune disease in dyslexics and their families suggests a more general developmental problem in developmental dyslexia. The hypothesis is raised that fetal effects of testosterone are involved in regulating neurological as well as immunological development, whereby abnormally high testosterone activity would produce a twofold deficit. Finally, strains of immune-defective mice have been found that bear the same cortical abnormalities as seen in the dyslexic brains previously studied. The immune-defective mouse may prove to be an excellent model for the study of the neuropathological basis of developmental dyslexia.