[Continuous partial epilepsy: syndrome and disease]

Abstract

Twenty three patients are described with a Kojewnikow syndrome in order to identify possible etiological factors. According to their history, clinical and E.E.G. features, patients could be classified into two groups; 1) Eleven patients corresponded to the classical description of the Kojewnikow syndrome: variable age at onset of the disease, rare somatomotor seizures, delay of onset of myoclonic jerks often of long duration, myoclonus limited to a small region, normal neurological examination, stable hemiplegia, normal results of psychometric tests, localised E.E.G. alterations. In most of these patients the etiology of the disorder was known, and the symptoms could be related to a localised lesion of the central cortex; 2) the second group (11 patients) presented with completely different characteristics: early onset of fits (age: 2 to 10 years), presence of other seizure types, short delay of onset of myoclonic jerks, high frequency of seizures, localization of myoclonus over large parts of the body, progressive evolution of a motor syndrome, associated neurological signs and disorders of sleep and behavior, progressive mental deterioration, characteristic E.E.G. pattern with long subclinical paroxysms of slow spikes (sharp waves) with variable localization, diffuse cerebral lesions of unknown etiology were found. Only one patient could not be allocated to either group. Several circumstantial data suggest that patients of the second group may suffer from a slow virus infection. Should this hypothesis be confirmed, a reconsideration of the Kojewnikow syndrome from the etiological standpoint would be of great theoretical and practical interest.