[Serum uric acid concentration and anticonvulsant therapy in childhood].

In antiepileptic treated adults a decrease of serum uric acid concentration was reported. In contrast to these findings we did not find a general decrease of uric acid concentration in 233 studied epileptic children and juveniles treated with antiepileptic drugs. But we found a significant decrease of uric acid concentration in epileptic children and juveniles treated with carbamazepine in monotherapy as well as in combined treatment.

[Neurometabolic diseases. References for classification, clinical aspects and diagnostic procedures].

Diseases caused by neuro-metabolic or neuro-degenerative disorders often lead to diagnostic difficulties, because of their rarity and numerous quantity. The present report tries to combine older classifications according to anatomic aspects with the newer ones underlying biochemical-functional points of view. Then the most important cardinal symptoms in a synoptic demonstration are put in relation to the most considerable neuro-metabolic disorders.

[Moyamoya syndrome. An unusual case report in an 8-year-old boy and review of the literature].

A Moyamoya disease in an 8-year-old german boy is reported. Medical history revealed attacks of hemiparesis and aphasia. The cerebral angiogram showed a typical, bilateral stenosis of the internal carotid arteries, collateral vessels as well as an occlusion of the basilar artery.

Progabide for previously untreated absence epilepsy.

Ten patients suffering from absence epilepsy and showing generalized spike-wave paroxysms in the EEG were treated by progabide monotherapy. Findings concerning clinical data and serial 24 h long-term EEG recordings were compared for the pre-treatment and treatment period. The average total spike-wave duration in 24 h decreased slightly from 810 sec to 699 sec; at follow-up, in 4 cases this trend was found to have reversed.

[Familial episodic ataxia].

5 members of a family with periodic cerebellar dysfunctions are described. This rare disorder occurred in the early childhood and is characterised by episodes lasting minutes to hours with severe cerebellar ataxia, dysarthria, diplopie, nystagmus, vertigo and vegetative symptoms. The examinations between attacks are almost normal.

Longterm follow-up of childhood epilepsy with absences. II. Absence-epilepsy with initial grand mal.

The study deals with 83 patients with absence epilepsy which had started with generalized tonic clonic seizures. Only those patients were included, who could be followed up to an age older than eighteen years. The patient population is heterogeneous; it includes numerous older patients in whom therapy had been instituted at a time when the present standard medication with ethosuximide and valproate was not available.

Longterm follow-up of childhood epilepsy with absences. I. Epilepsy with absences at onset.

The purpose of the follow-up study was to determine whether modern therapy with ethosuximide and/or valproate with/without phenobarbitone and its derivatives improves the longterm prognosis of absence epilepsy as compared to formerly used treatments. The patient population consisted of 194 cases (88 boys, 106 girls) with spike wave epilepsy starting with absences. In each case the diagnosis was confirmed by clinical observation and the typical EEG pattern.

[Adrenoleukodystrophy in a pair of siblings].

The diagnosis of adrenoleukodystrophy (ALD) in two brothers was confirmed by the analysis of long-chain fatty acids in cultured skin fibroblasts. The 23 year old brother was treated for Addison's disease at the age of 7 years. His first symptoms of ALD developed at the age of 22.

Intensive follow-up monitoring in patients with once daily evening administration of sodium valproate.

For 18 previously untreated patients with absence, myoclonic, or grand mal seizures--or combinations--results of clinical and electroencephalographical monitoring are reported. Sodium valproate was given once daily in the evening. Monitoring included repeated 24-48 h EEG recordings and drug blood level measurements.

[Syndrome of ophthalmoplegia, ataxia and areflexia of Fisher in a 14 year old boy (author's transl)].

The syndrome of ophthalmoplegia, ataxia and areflexia first mentioned in 1932 by Collier and in 1938 by van Bogaert was described more precisely in 1956 by Fisher. It represents a specific clinical entity and is considered to be a variant of idiopathic polyradiculoneuropathy (Guillain-Barré). The most striking features are external (sometimes also internal) ophthalmoplegia, hyporeflexia or areflexia and cerebellar ataxia.

[The effect of primidone treatment of thyroid hormones in epileptic children and adolescents (author's transl)].

In primidon-treated patients there are significantly decreased serum concentrations of total and free thyroxin, protein bound iodine and base line serum TSH values. In primidon-treated children T3-resin test values, concentration of thyroxin-binding protein and total cholesterol are identical to those of the control group. Degree of diminution in serum concentration of protein bound iodine, total and free thyroxin and base line TSH was independant of the primidon dose per day.

[Changes in the thyroid hormone system with combined anticonvulsion treatment of epileptic children and adolescents (author's transl)].

Anticovulsant combined treatment produces statistically significant reduction of total thyroxine, free thyroxine and protein-bound iodine. Total cholesterin is significantly raised on average of the total. Basal concentration of thyreotropic hormone is slightly lowered.

[The influence on the thyroid hormone system by long-term anticonvulsive therapy with valproat (author's transl)].

Valproat lowers the serum concentraiton of PBI and T4 in epileptic children. Since this reduction is not so pronounced with PBI as with T4, the difference in comparison with the controls could not be statistically verified with the desired probability of error. The reduction of general thyroxine can be statistically verified when compared with healthy control individuals.

[Influencing of the thyroid hormone system by long-term treatment with 5-5 diphenylhydantion in children and adolescens (author's transl)].

In the context of anticonvulsive maintenance therapy in epileptic children and adolescents, 5-5-diphenylhydantoin leads to a statistically significant decrease in total thyroxin (T4), protein-bound iodine (PBJ) and free thyroxin (FT4). The consequences of DPH therapy are based on the direct and indirect influence of DPH on the thyroid hormone system. DPH competitively pushes the T4 out of its plasma-protein bond.

[Diagnosis of hydrocephalus. Changes in visual evoked potentials in children with progressive hydrocephalus internus].

Visual evoked potentials (VEP) of 12 children with progressive hydrocephalus internus were studied. Depending on the severity of hydrocephalus internus VEP show remarkable changes. First of deformation of VEP occurs, than a slowing of latencies and finally an extreme increase of latencies combined with a further deformation of VEP.