Efficacy of the ketogenic diet in focal versus generalized seizures.

Most reports of the ketogenic diet have focused on its efficacy for generalized seizures. Few data are available regarding its effect on focal seizures. We retrospectively studied patients (mean = 7.5 years of age) with medically intractable epilepsy treated by the ketogenic diet. The predominant seizure types in each patient were classified as generalized (100 patients) or focal (34 patients) based on ictal electroencephalograms (EEGs) or seizure semiology and interictal EEG. A seizure reduction of more than 50% compared with baseline was seen in nine patients (27%) with focal seizures and 46 patients (46%) with generalized seizures at 3 months, in 10 patients (30%) with focal seizures and 46 patients (46%) with generalized seizures at 6 months, and in eight patients (24%) with focal seizures and 42 patients (42%) with generalized seizures at 12 months. Differences were not significant. Outcome tended to be better in patients younger than 12 years of age compared with the older age group, but the difference was significant at 6 months only. Our results suggest that some patients with intractable focal epilepsy may respond favorably to the ketogenic diet and that this option should be considered if epilepsy surgery is not possible.

Benign myoclonus of early infancy: an imitator of West's syndrome.

Benign myoclonus of early infancy is a rare condition characterized by nonepileptic spasms that may resemble the epileptic spasms seen in West's syndrome. The spells in benign myoclonus of early infancy begin before age 1 year and are self-limited. The electroencephalogram (EEG) is invariably normal, and neurologic development is not affected. West's syndrome is characterized by infantile spasms that appear before 1 year of age, an abnormal EEG with hypsarrhythmia, and a poor prognosis. We describe six infants who presented for evaluation of clusters of head, trunk or extremity spasms, eye blinking, brief jerking of upper extremities or trunk, and head nodding episodes. In most, a presumptive diagnosis of West's syndrome was made prior to the referral. One infant had been placed on valproate. Routine EEG recordings or prolonged video EEG monitoring were normal both during and between episodes. After the negative evaluations, the diagnosis of benign myoclonus of early infancy was made in each infant. Subsequently, no infant was treated with anticonvulsants. Follow-up revealed complete resolution of the episodes in all children within 2 weeks to 8 months of onset. All had normal neurologic development. Based on our cases and review of the literature, the prognosis for this disorder is excellent. Care should be taken to recognize this rare entity and avoid unnecessary and potentially harmful antiepileptic therapy.