Long-term follow-up study of Lennox-Gastaut syndrome in patients with severe motor and intellectual disabilities: with special reference to the problem of dysphagia

A long-term follow-up study of Lennox-Gastaut syndrome (LGS) (>10 years) wa s conducted with 38 patients with severe motor and intellectual disabilitie s (SMID) to clarify the relationship between the rapid development of dysph agia and epileptic seizures, and to elucidate the long-term evolution of LG S in patients with SMID. Those who showed a relatively favourable seizure outcome were compared to t hose with a poor seizure outcome. Poor seizure outcome correlated strongly with: (a) an early appearance of dysphagia and additional deterioration of the already retarded mental function; (b) a predominance of atypical absenc e seizures; and (c) persistent frequent epileptiform discharges during elec troencephalographic evaluations. Neither age at seizure onset nor intelligence level at the time of the last examination was correlated with seizure prognosis. Further, seizure progno sis was not related to the aetiology of LGS. Repeated seizures apparently c aused development of progressive epileptic encephalopathy, in addition to t he underlying severe brain damage. Since development of dysphagia burdens a n already severely handicapped patient with intensive medical care, efforts to reduce the seizures and design a long-term care plan are of great impor tance. (C) 2001 BEA Trading Ltd.