Management of intractable epilepsy.
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Adolescent↗
Biomedical subjects
J H Livingston
Publications and source records attributed to J H Livingston.
Unusual MRI appearance of diffuse subcortical heterotopia or "double cortex" in two children.
Two children with mild epilepsy and learning and behaviour problems had magnetic resonance imaging (MRI) scans showing an almost identical generalised disorders of neuronal migration. Their computer tomography (CT) scans showed abnormal hypodense white matter. The MRI showed a four layered appearance of the cerebral parenchyma extending from the frontal to the occipital region. There was a normal appearance to the white matter in the periventricular region which had an abnormally smooth junction with a thick diffuse layer of heterotopic grey matter. This was surrounded by a thin layer of white matter which had normal digitations with the overlying cortex. The appearance of the overlying cortex was normal. These and other recently described cases broaden the concept of generalised disorders of neuronal migration and illustrate that it is possible to have a generalised cerebral malformation with few clinical consequences.
Brain↗
Vigabatrin in the treatment of epilepsy in children.
1. This study presents the results of the preliminary screening of vigabatrin as add-on therapy in an open, non-controlled multicentre study in children with refractory epilepsy. 2. There were 135 children, with an age range of 2 months-12 years. Main seizure type was partial in 42%, generalized in 29%, Lennox-Gastaut syndrome in 19% and West syndrome in 10%. 3. Vigabatrin was added onto current antiepileptic treatment in an initially recommended dose of 40-80 mg kg-1 day-1. However, the doses were frequently increased when tolerance allowed it, and the final mean dose used was 87 mg kg-1 day-1 (27-600). 4. A 75% to 100% reduction in seizure frequency was observed in 25% of patients (11 patients became seizure free) and 50 to 75% decrease in a further 13%. Efficacy was better in partial seizures, with good to excellent results in 49% of patients. The use of high doses, above 100 mg kg-1 day, was not associated with greater efficacy in this preliminary study. 5. No side effects were reported in 79% of patients. Agitation and insomnia were observed in 8.8% and somnolence in 6%. Other adverse events included ataxia (2.2%), nausea (2.2%) and increased appetite (1%). A moderate and transient decrease in haemoglobin was reported in six patients from the same centre; these patients were all receiving very high doses of vigabatrin (250 to 600 mg kg-1 day-1). 6. Vigabatrin thus appears to be a safe antiepileptic drug that may be effective in the treatment of severe epilepsy in children.
Adolescent↗
Cerebrospinal fluid nucleotide metabolites following short febrile convulsions.
Cerebrospinal fluid (CSF) markers of cerebral energy depletion were measured in 32 infants and children following short (less than 10 minutes) febrile convulsions, and in 19 controls. Specific and sensitive indices of high-energy phosphate compound depletion (hypoxanthine, xanthine and uridine) showed no marked changes. Values for patients and febrile controls were significantly higher than for afebrile controls, which is consistent with increased cerebral metabolism in febrile patients. There were no differences in pH, lactate or creatine kinase levels in the CSF of patients and controls. The results suggest that short febrile convulsions are benign and that in the absence of risk factors for the subsequent development of epilepsy, prophylactic anticonvulsant treatment is not indicated.
Child Development↗
Cerebrospinal fluid nucleotide metabolites following non-convulsive status epilepticus.
The authors studied specific and sensitive indicators of neuronal adenosine triphosphate (ATP) depletion--hypoxanthine, xanthine and uridine levels--in the cerebrospinal fluid (CSF) of nine children during non-convulsive status epilepticus. No evidence of ATP depletion was found and CSF pH and creatine kinase levels were similar to those of controls. Hypoxanthine, xanthine and uridine had a tendency to be low, but this was significant only for xanthine. The authors speculatively link this reduction to a reduction in neuronal protein synthesis. This might be a mechanism whereby non-convulsive status epilepticus could lead to intellectual deterioration and dementia.
Child↗
Epilepsy precipitated by bathing.
A case of epilepsy precipitated by bathing is described and previously reported cases are reviewed. The prognosis for this rare form of reflex epilepsy seems to be good, in that the seizures resolve with age and neurodevelopment remains normal.
Baths↗
The Lennox-Gastaut syndrome.
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Cerebral Cortex↗
Benzodiazepine sensitivity testing in the management of intractable seizure disorders in childhood.
The use of benzodiazepine sensitivity testing in the management of 40 children with intractable seizure disorders was studied. The aetiology and clinical syndromes varied widely with myoclonic, atonic and complex absence seizures predominating. Twenty-five cases had mixed seizure disorders. There was, likewise, a wide range of EEG abnormalities. Seven cases were in non-convulsive status at the time of testing. Diazepam (0.2 mg/kg) was given slowly intravenously and its effect on the EEG was observed. In 21 cases epileptiform activity was abolished. No change was seen in 13 cases and an unusual result was seen in 3. There was a paradoxical response in 3 cases, two of these associated with clinical seizures. Only 1 child in non-convulsive status had a positive result. Following testing, 32 patients went on to long-term oral benzodiazepine treatment. Twenty-one of these patients showed subsequent clinical improvement and 16/21 (76%) had had a positive sensitivity test previously. Eleven of these patients did not improve on long-term treatment. Seven out of the 11 (64%) had had a negative sensitivity test. These results suggest that the benzodiazepine sensitivity test is of value in the long-term management of intractable seizure disorders in childhood, but also emphasise the variability and unpredictability of response to benzodiazepine treatment.
Adolescent↗
Non-convulsive status epilepticus resistant to benzodiazepines.
We describe the failure of an intravenous benzodiazepine to control non-convulsive status epilepticus occurring in six patients with the Lennox-Gastaut syndrome. In one patient the benzodiazepine induced a paradoxical response with clinical and electroencephalographic seizures.
Adolescent↗
Intracerebral haemorrhage after the neonatal period.
Intracerebral haemorrhage is rare in childhood. We have reviewed the last 10 years' experience, in our referral area, of parenchymatous intracerebral haemorrhage in children from 1 month to 16 years of age. There were 27 cases, five of which were intracerebellar and two predominantly intraventricular. The commonest aetiology was vascular malformation (10), followed by haemorrhage into tumour (four), and coagulopathies (five). Clinical features were non-specific, but altered consciousness, headache, vomiting, and focal signs were the most common. Focal signs were, however, rare in the patients with intracerebellar haemorrhage. There was an overall mortality of 54% (14 out of 27). Nine patients were handicapped on follow up, but none severely so. For the diagnosis of intracerebral haemorrhage a high level of clinical suspicion is needed with early use of computed tomography. Maintenance of homeostasis, relief of raised intracranial pressure, and evacuation of haematoma are the aims of management.
Adolescent↗