Subacute sclerosing pan-encephalitis: A review of 17 cases with special reference to clinical diagnostic criteria

Abstract

Seventeen cases of subacute sclerosing panencephalitis are reviewed. The criteria for diagnosis in all cases were the clinical and electroencephalographic findings, together with a paretic Lange curve in the spinal fluid. Confirmation was provided by brain biopsy in 6 cases and by necropsy in 4 cases. Seizures producing periodic spasms of a characteristic kind were present in all but 2 cases. Two features of these spasms (tempo and repetitiveness) are described in some detail because we believe them to be pathognomonic of the disease and a common and important diagnostic clue. Seizures producing periodic antispasms are another clinical feature that was observed only in the later cases of this series after our attention had been drawn to them by Dr. G. Pampiglione. Ocular symptoms (papilloedema, optic neuritis, and nystagmus) and asymmetrical neurological symptoms (particularly hemiplegia) were features in some cases. Electroencephalogram and poly-electromyogram findings are not here considered. These tests, made by Dr. G. Pampiglione in all save 2 cases, will be reported by him in a separate communication.