Perisylvian polymicrogyria (PP) is a dysfunction in cortical development with a varying degree of abnormal cortical organization. It may unilaterally or bilaterally involve the opercular and perisylvian regions, as well as the parietal and superior temporal regions. We have described 20 patients with neurologic dysfunction and mental retardation of varying severity. Epilepsy was present in 60% of the patients and usually emerged between six and 10 years of age. Seizures were drug-resistant in only one patient, with the remaining patients either well (33.3%) or partially controlled (58.4%). A pseudobulbar palsy was present in 95% of the patients, while a cognitive deficit of variable severity was found in 80% of the subjects. This study indicates that the clinical and epileptic spectrum of this syndrome is broad but that a PP must be suspected in all the patients with mild or severe mental retardation and with diplegia of facial, pharyngeal and masticatory muscles.
|Translated title of the contribution||Clinical, neurophisiological and neuroradiologic findings in patients with perysilvian polymicrogyria|
|Number of pages||9|
|Journal||SAGGI - Neuropsicologia Infantile Psicopedagogia Riabilitazione|
|Publication status||Published - 1999|
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health