Jacksonian seizures in infancy and childhood.

Summary One-hundred and fourteen cases of jacksonian epilepsy in infants and children are reviewed. Incidence .—The reported frequency of local motor seizures, 7.5 per cent is probably too low because of failure of observation from onset or rapid spread of epileptic discharge. Sex .—Both sexes were about equally affected. Race .—The ratio of Negro to white patients was considerably below the hospital average. Age At Onset .—The greatest incidence was among infants and young children with a progressive decline toward adolescence. Forty-eight per cent were less then 3 years of age. It seems logical to implicate pre-, peri-, and postnatal factors in etiology. A delayed onset of organic epilepsy was usual. Heredity .—Twenty-eight per cent of 95 cases had a history of epilepsy in close relatives (half in parents or siblings). This confirms Lennox's impression of the influence of heredity in organic epilepsy in children. Etiology .—Cause was known in only 34 per cent of cases. Almost two thirds were related to birth injury (13 cases) or congenital or infantile hemiplegia (11 cases). Eleven patients had postnatal acquired disease of the central nervous system of varied etiology. Diffuse surface hemangiomata were demonstrated in 2 cases. Only one patient had an expanding intracranial lesion. The latter also had severe birth injury with hemiplegia and extensive cortical sclerosis. Cerebral neoplasm is a relatively rare cause of jacksonian epilepsy in infants and children. Seizure Analysis .—There was no right or left predominance of jacksonian phenomena. One third had bilateral spread. The commonest site of origin was the face or hand. Only 9 of 60 cases began in the leg. Abnormal sensation prior to movement was common. Nocturnal attacks were fairly frequent. Six patients had febrile seizures before the onset of jacksonian epilepsy. In 4 these were of great frequency. The interval between the febrile and jacksonian seizures was over 2 years in 5 of the 6 cases. One half of this group had a family history of epilepsy. Occasionally, circumstances suggested that jacksonian epilepsy might be a residual of local brain damage from severe and protracted seizures with high fever. In another 6 cases, early jacksonian seizures occurred only with fever and infection. The presence of severe neurological abnormalities prior to the onset of seizures in 3 of these cases confirms the impression that fever and infection can trigger a latent tendency for jacksonian epilepsy. Twenty-five patients had other types of seizures. Five had grand mal epilepsy prior to the onset of local seizures. Five with organic brain involvement had “atypical” petit mal (myoclonic or akinetic). In 4, these preceded the onset of jacksonian epilepsy. Fifteen patients had recurrent paroxysmal phenomena compatible with epileptic discharge without motor component. Hemiplegia .—Twenty-seven patients (23.6 per cent) had hemiplegia. The majority were attributable to prenatal factors or birth injury. Forty-eight per cent were mentally retarded. Postepileptiform paralysis (Todd and Robertson) of varied duration was common. With frequently recurring or prolonged seizures such paralysis may be progressive and ultimately permanent. This was the case in at least 10 patients in our series. This course of events attests to the need for prompt control of jacksonian seizures. Mental Retardation .—Twenty-eight patients (24 per cent) were mentally retarded prior to onset of jacksonian epilepsy. Etiological factors were evident in two thirds. Four patients appeared to develop mental retardation consequent to severe recurrent seizures. Serious behavior disorder was a chief complaint in 4 children. The Electroencephalogram .—In general, the conformance of focal electroencephalogram findings with inferences drawn from pattern of seizure is good, even when only a single tracing is available. An expected electrographic focus is more likely to be demonstrated in a child than in an infant. Spike foci are nonspecific indicators of a region of high cortical excitabity, and not necessarily of underlying structural abnormality. Slow wave foci, recorded with or without accompanying focal spike discharges, more frequently presage proof of underlying structural disorder. Of the 83 children in the series who had electroencephalograms, 18 had neurological lateralizing signs. In 12 of these, a focal process was recorded in the expected locale. In the remaining 65 cases without neurological abnormality, 19 showed focal spike processes in the suspected hemisphere and in 6 others the focus was paradoxical. Fifteen showed generalized spiking. Seven showed slow focal patterns over the central region of the suspected hemisphere. One tracing was normal and the remainder showed intermediate or markedly slow resting frequencies without focalization. Air Encephalograms .—Close observation of patients with local seizures is essential. Encephalography is not routine. Criteria for air studies are increased intracranial pressure, neurological abnormalities or failure of response to adequate antiepileptic therapy. One half of the encephalograms made were considered abnormal. Recognized cause for these abnormalities was present in only 13 of 24 cases. Ten of the 24 patients with abnormal air studies were retarded mentally. Arteriograms were sometimes abnormal with normal pneumoencephalograms and vice versa.