Ketogenic diets in the treatment of epilepsy: short-term clinical effects.

The classical 4:1 ketogenic diet, the medium-chain triglyceride (MCT) diet and a modified MCT diet were used in the treatment of 55 children and four adults with intractable epilepsy. During a three-month treatment period 51 of 63 studies (81 per cent) showed greater than 50 per cent reduction in seizure frequency. This improvement was independent of diet used and type of seizure experienced by patients. The diets were found to be acceptable and of therapeutic benefit to the children, but treatment of the four adult patients with the MCT diet was unsuccessful. There was no correlation between EEG changes and clinical response. The three ketogenic diets were shown to be effective in the short-term management of children whose epilepsy is poorly controlled with anticonvulsant drugs.

Fatal lipid storage myopathy in an infant: case report and autopsy findings.

The clinical presentation, muscle biopsy appearances and autopsy findings in a male infant dying at the age of 13 weeks with a lipid storage myopathy are described. The primary metabolic abnormality was not elucidated but was thought not to be carnitine deficiency.

Treatment of idiopathic polyneuritis by a polyunsaturated fatty-acid diet.

Two children, severely disabled for 6 and 12 months respectively by idiopathic polyneuritis, began to recover within a week of starting on a polyunsaturated fatty-acid diet. Recovery is virtually complete.

Alexander's disease presenting as astrocytoma.

A case of progressive fibrinoid degeneration of astrocytes is described. Symptoms began with convulsions at the age of 6 weeks and cerebral tumour was suspected because of enlargement of the head, increased intracranial tension, and marked proliferation of astrocytes in the brain biopsy which was interpreted as an astrocytoma. Fibres and granules staining deeply with PTAH are a constant feature of the few cases so far described. The aetiology is unknown but an inherited metabolic defect of astrocytes may be responsible.

Neonatal disseminated herpes simplex virus infection with encephalitis treated with cytosine arabinoside.

Herpes simplex encephalitis was diagnosed by immunofluorescence and histology of a brain biopsy on the 19th day of life in a neonate in whom symptoms had begun at 12 days. Treatment with steroid, diuretic and cytosine arabinoside was begun and initially there was dramatic improvement in the symptoms. This improvement was not sustained, however, and the infant developed evidence of severe brain-damage. Disseminated herpes simplex virus infection is discussed and available therapy for this severe disease is outlined.

Sodium valproate in the treatment of intractable childhood epilepsy.

Sodium valproate has been used, in combination with a variety of standard anticonvulsant drugs, to treat 24 children with intractable epilepsy. A reduction of fit frequency greater than 90 percent was obtained in six patients, and a reduction of over 50 per cent in another six, with an improvement in alertness and school performance in the majority of cases. Grand mal, myoclonic and petit mal epilepsy gave the best response, while infantile spasms responded least well. No serious side-effects were encountered.