The clinical and EEG profiles of 13 cases of eating epilepsy are described. Temporal lobe epilepsy (TLE) formed the seizure type in 12 cases. In all but one case, the seizures were precipitated only by eating a heavy meal. In ten cases EEG showed interictal seizure discharges. EEG recordings were obtained from 12 patients during and after eating. In two patients clinical attacks (TLE) could be induced by eating in the laboratory. There was a good response to conventional anticonvulsants in only 3 cases. Alteration of eating habits helped in controlling reflex seizures in 2 cases. Various mechanisms of reflex precipitation of seizures by eating are discussed.

No abstract

Rippling muscle disease (RMD) has previously been reported as a skeletal myopathy that was attributed to a defect in the sarcomere. Here we report a new form of RMD that is more severe, characterized by fatal arrhythmic cardiomyopathy and delayed bone age. Mortality has previously not been associated with RMD. With this report we hope to raise awareness that a subset of patients with this clinical entity are predisposed to severe cardiac disease.

No abstract

Between 1990 and 1992, 14 children were seen in whom a clinical diagnosis of olivopontocerebellar atrophy (OPCA) had been made. The majority of patients presented with cerebellar ataxia and hypotonia. Five children had a family history of a similar illness in first-degree relatives. All cases had undergone clinical and neurologic examinations, routine laboratory tests and cranial CT. CT features were graded to quantitate the degree of atrophy in each cerebellar hemisphere, vermis and brain stem. All patients had varying degrees of atrophic changes of cerebellum, brain stem and cerebrum. These CT features appear to be distinctive enough to enable the diagnosis of OPCA to be made.