NEURORADIOLOGICAL FINDINGS IN THE NORTHERN EPILEPSY SYNDROME

Neuroradiological findings are presented of 19 patients with the north ern epilepsy syndrome, a recessively inherited childhood epilepsy with associated mental deterioration. The first signs of cerebellar-brains tem atrophy already appeared in young adulthood, with atrophy tending to increase with age. Findings of cerebral atrophy were detected later : four of the seven patients under 30 years of age displayed central o r cortical atrophy, while all 12 patients over 30 were affected. The d egree of mental deterioration correlated well with the severity of cer ebral atrophy. One half of the patients with either central or cortica l atrophy were moderately to severely retarded, whereas all patients w ith both central and cortical atrophy were moderately to profoundly re tarded.