Pathology of childhood epilepsies
Heinze-Joachim Meencke, MD
Neurologische Klinik, Universitätsklinikum Rudolf-Virchow, Standort Charlottenburg, Freie Universität Berlin, Spandauer Damm 130, D-1000 Berlin 19, West Germany
Infantile spasms (West syndrome, Lennox-Gastaut syndrome), childhood absence epilepsy (pyknolepsy) and juvenile myoclonic epilepsy (impulsive petit mal) all belong to those epilepsies that have somewhat imprecisely also been called childhood epilepsies.
These generalized epilepsies have common biologic parameters and common features in the symptomatology of seizures. There are, however, clearly defined differential criteria. The comparable genetic background, the marked age dependency, and electroencephalographic (EEG) criteria belong to the common biologic parameters. Common features of seizure symptoms are bilaterality, preponderance of trunk motor symptoms, suddenness of commencement and termination, and lack of vegetative phenomena as well as sensory and psychic experiences. These common features contrast with the differential criteria, which have, in each case, a distinctive age of onset and a specific syndrome-related symptomatology. Furthermore, there are differences in the natural course and the therapeutic response of the different generalized epilepsies.
There are several neuropathologic reports about epilepsies in general, with a broad range of nonspecific findings concerning etiology; but there are only a few syndrome-related neuropathologic studies. The discrepancy between clinical relevance and frequency of generalized epilepsy syndromes, and the small number of pathologic studies is striking (Table 1).
Nearly 40 studies of infantile spasms have been made, with a total of about 160 cases, but there are only four studies of the Lennox-Gastaut syndrome with 17 cases, three reports of childhood absence epilepsy with 14 cases, and two reports of juvenile myoclonic epilepsy with four cases.
A lack of sufficient neuropathologic data leads to the assumption that the brain. . .