Congenital Bilateral Perisylvian Syndrome: Familial Occurrence, Clinical and Psycholinguistic Aspects Correlated with MRI (original) (raw)

The aim of this study was to correlate the clinical features, psycholinguistic aspects and neuroradiological data of patients with CBPS. Patients and Methods & Ascertainment of patients and their families We performed a detailed clinical investigation of 31 patients and their families. Family histories were obtained and pedigrees were constructed. Twenty-two patients, belonging to six unrelated kindreds, had a familial recurrence of PMG. The criteria for diagnosis included a clinical presentation of pseudobulbar palsy, epilepsy and / or language disorder without hearing defi cits, mental retardation or oral motor / structural abnormalities, and PMG on imaging studies. We systematically interviewed patients and their family members according to a standard detailed questionnaire, emphasizing the family history of problems with phonation and delayed speech,