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We report a series of nine children with multiple daily seizures since infancy who underwent functional hemispherectomy that included en bloc resection of the hippocampus and the temporal neocortex. In all cases, the hippocampi were normal by conventional histology despite the fact that these patients had suffered from recurrent seizures over a long period of time. This observation suggests that extremely frequent seizures in childhood are not invariably associated with the development of hippocampal sclerosis.

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The prenatal histories, clinical courses, and neuroradiographic studies of 8 infants who had survived the in utero demise of a homozygous co-twin were reviewed. Three distinct modes of clinical presentation were found: (1) severe neonatal encephalopathy with seizures; (2) a more benign neonatal course with onset of seizures and profound developmental disabilities within the first 6 months of age; (3) late infantile presentation with seizures. Only the third group had milder outcomes. Neuroradiographic studies demonstrated two pathologic patterns: varying degrees of periventricular white matter infarction with migrational abnormalities observed with earlier demise of the co-twin, and multicystic encephalomalacia observed when demise occurred at or near term. Pathophysiology is uncertain and most likely multifactorial. Exsanguination injury to the survivor can occur acutely following co-twin demise, so urgent delivery may be appropriate at or near term.

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This study tested the hypothesis that the duration of fever prior to the onset of a simple febrile seizure may be an important clinical variable with respect to patient outcome. The duration of fever prior to seizure according to patient history was defined as either long (greater than or equal to 24 hours) or short (less than 24 hours). We hypothesized that simple febrile seizures which occur with a history of a fever of long duration (LDF) are more likely to be associated with a significant illness at presentation or a subsequent neurologically abnormal course than are simple febrile seizures which occur with a history of a fever or short duration (SDF). Of 100 cases which met study criteria for simple febrile seizures, nine had a LDF and 91 had a SDF prior to the development of a seizure. No statistical differences in age, sex, maximum fever recorded in the emergency department, duration of seizure, WBC, or electrolytes were found between patients with SDF and LDF (P less than 0.01). Of the nine patients with a LDF, all had either a significant illness at the time of initial visit or a subsequent neurologically abnormal course. Of the 91 patients with a SDF, 88 had a good outcome, while two had a significant illness at the time of visit, and one had a subsequent neurologically abnormal course. These results suggest that children with a history of LDF prior to the occurrence of a simple febrile seizure are more likely to have a serious illness at presentation or a subsequent neurologically abnormal course than are children with seizures which occur with a history of SDF.

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Three patients are described with transient erythroblastopenia of childhood (TEC) associated with a transient neurological disorder. This association may not be uncommon in view of the presentation of these three patients to one small pediatric center over a 2 year period, out of a total of four patients presenting with TEC.

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An 8-year-old boy presented in coma and was found to have a massive posterior fossa hemorrhage on computed tomographic scan. Autopsy disclosed a large cerebellar hematoma within a mixed glioma containing both juvenile pilocytic astrocytoma and oligodendroglioma. It is postulated that the hemorrhage originated from the oligodendroglial component of the tumor.

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Cerebral arterial infarction is a more common cause of neonatal seizures than has been previously appreciated. In 50 full-term newborns with seizures studied, 7 had cerebral infarction which was the second most common definable cause of seizures. We describe these 7 full-term infants with cerebrovascular accidents who presented with focal or generalized seizures. Obstetrical histories were normal in 5 of these patients. Their neurological examinations demonstrated lethargy and generalized hypotonia. Electroencephalograms demonstrated focal abnormalities in 4 infants. Computed tomographic scans in the first week of life showed infarctions in 6 newborns and in another at age 6 months. Neonatal stroke should be considered as a cause of seizures in a full-term newborn in spite of a normal obstetrical history and a nonfocal neurological examination.

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A 19-year-old man had reading-induced epilepsy; generalized seizures were preceded by myoclonus of the jaw. Although reading epilepsy is usually refractory to anticonvulsant therapy, treatment with clonazepam resulted in complete control of the involuntary movements precipitated by reading.

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The principal neuropathologic abnormality observed in three autopsy cases of Menkes steely hair syndrome was widespread nerve cell loss and gliosis, especially severe in the cerebral and cerebellar cortex and in the relay nuclei of the thalamus. Granular stellate cells of neocortical layer IV and the granule cells of the cerebellum are cell classes which were particularly severely depopulated. The degree of reduction of myelinated axons is consistent with axonal degeneration secondary to nerve cell loss. There are also prominent abnormalities in the patterns of dendritic arborization of surviving cortical pyramids and cerebellar Purkinje cells as seen in Golgi impregnations. The deviant neuronal forms are probably due, in part, to failure of innervation by afferent fiber systems during the fetal as well as postnatal epochs.

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Trigeminal neuralgia is a rare symptom in childhood. In the idiopathic variety, no objective neurological deficit is demonstrable, but in the symptomatic or secondary form, deficits of trigeminal function may be found. In the latter circumstance, underlying causative pathology must be looked for vigorously, as emphasized by the present case of symptomatic trigeminal neuralgia in a 5-year-old child, determined to be secondary to an infiltrating embryonal rhabdomyosarcoma.

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