Congenital Bilateral Perisylvian syndrome
What are the symptoms?
Patients have paralysis of the face, throat, tongue and the chewing process, with dysarthria (speech difficulties) and drooling. Most have cognitive deficit and epilepsy. Fixed deformity of the ankle joints (arthrogryposis) has been described in some patients. Seizures usually begin between the ages of four to twelve years and are poorly controlled in about sixty per cent of patients. The most frequent seizure types are atypical absences, tonic or atonic drop attacks and tonic-clonic seizures, often occurring as Lennox-Gastaut syndrome. A minority of patients (twenty-six per cent) have partial seizures.
Background
| What are the causes? ![]()