Provocation of convulsive activity in the EEG by promazine-HCl-induced sleep in children.

The authors report on the convulsive activity provoking effect of Promazine as shown on EEG during sleep. It has successfully been used in infants and children who do not tolerate sleep deprivation. Promazine had smaller effect on the EEG-pattern than other related compounds or barbiturates. The greatest efficacy could be reached in generalised epilepsy, but the method was also helpful in other epilepsies. It is emphasized that in cases of acute neurological symptoms of not epileptical origin the compound is helpful to exclude epilepsy since Promazine induced sleep was never associated with convulsive activity on EEG in these cases.

[Provoking effect of promazine on EEG in childhood]. / Promazin altatás provokáló hatása az EEG-re gyermekkorban.

The authors report on the sleep activation of the EEG-recordings by Promazine a Phenothiazine derivative that can be used also in babies and in those elder children who does not tolerate sleep deprivation. The EEG-s are much less altered then by other related compounds or by Barbiturates. The greatest efficacy could be reached in generalized epilepsies, but the method was helpful in other epilepsy forms too. It is emphasized that in no case of acute neurological symptoms of not epileptic origin showed the Promazine induced sleep a convulsive activity.

Myasthenia gravis in childhood and adolescence. Report on 209 patients and review of the literature.

Authors review the different theoretical and practical problems of childhood and adolescent myastenia gravis, including the heterogeneous group of congenital myasthenia and the big casuistics of the literature. There are reports on 113 cases with childhood myasthenia gravis and 96 cases of adolescent myasthenia. Ratio of these forms ranged 10.76% in childhood myasthenia and 9.14% in adolescent age, resp., 19.9% of the whole patient material. A classification is given concerning juvenile myasthenia: 1. Neonatal (transitory) myasthenia. 2. Congenital (local, non-progressive) form. 3. Congenital form with late generalized symptoms. 4. Myasthenia simulating brain-stem process. 5. Generalized childhood myasthenia. 6. Adolescent type myasthenia (juvenile form). 7. Associated myasthenia, myasthenic syndrome or reaction. A new form of the disease is described in which the congenital myasthenia changes into generalized form in the later course of the disease. Report is given on thymectomies in childhood and adolescence which produce in general excellent results. Thymic pathology and activity are dealt with.

The significance of x-ray examinations of limb musculature in neuromuscular diseases with special regard to childhood diseases.

On the basis of examinations performed on 29 children suffering from various muscular diseases, the authors give a detailed discussion of the application, indication and advantages of muscles x-rays in diagnosing neuromuscular diseases. With the help of x-rays of the musculature an inside view can be obtained in vivo, in a non-invasive manner, of structural changes in the muscles, and the progress of the disease can be followed up objectively. The method opens up new possibilities of diagnosis and promotes a more thorough knowledge of the pathological processes.

Multiple sclerosis in childhood: long term katamnestic investigations.

Sixteen children with multiple sclerosis, 14 with remitting and 2 with progressive course, and their follow up for 4-16 (mean, 9 years) are reported. The disease manifested in eight children at the age of 1.5-9 years, while in the other eight at the age of 12-14 years. The cases were acute, but had a stronger inclination to remission than in adulthood. In addition to the "classical" symptoms known in adult, multiple sclerosis, papilloedema and acute increased intracranial pressure were observed in nine children. Dehydrating treatment was life saving in these cases. Opsoclonus occurred in two patients, and two children had convulsions, one of whom later developed chronic epilepsy. The CSF was pathological in all the cases. Oligoclonal gamma-globulin subfraction was found in three out of the five patients tested. Three patients died. Histological examination was carried out in two of them; it revealed severe perivenous demyelinisation due to inflammation. Cortisone treatment occasionally with azathioprine reduced the duration of exacerbations, but could not prevent renewed ones and caused severe side-effects. Multiple sclerosis is a rare but not exceptional condition in children. The differences between cases in children and adults are age-dependent.

Hyperuricaemia associated with 18q deletion. Atypical Lesch-Nyhan syndrome?

The existence of a clinically typical Lesch-Nyhan syndrome was observed in a male infant with 18q deletion syndrome. Indirect hypoxanthine-guanine-phosphoribosyl transferase activity determination demonstrated a normal value, and thus the possibility of Lesch-Nyhan syndrome linked to the X-chromosome may be excluded. It is assumed that the uric acid metabolism must be under the primary or secondary effect of one or other of the gene loci on the long arm of chromosome 18, since the existence off a hyperuricaemic syndrome was observed in this 18q deletion patient.

[Anticonvulsive treatment and calcium metabolism]. / Antikonvulsivbehandlung und Kalkstoffwechsel.

In 31 children (12 boys and 19 girls), who were treated with at least two anticonvulsants, the calcium metabolism was followed for 2 years. Eight boys and 5 girls (13 cases) showed increased serum calcium and/or alkaline phosphatase values. Administration of vitamin D3 for a period of 3 months resulted only in partial regulation of hypocalcemia in boys, but had no effect whatsoever upon the alkaline phosphatase level. With the boys, the percentage of cases of disturbance of calcium metabolism was higher than with the girls. Roentgenologically detectable changes in long, tubular bones (especially the femur and tibia) could be observed in 6 patients. Among 200 consecutive roentgenograms available to the authors' laboratory, similar pictures were discovered in 3 patients. Two of them were not suffering from epilepsy, while one female patient had been taking antiepileptic drugs for 9 months only. The authors emphasize the importance of making laboratory examinations already before the commencement of antiepliptic medication.

[Tuberous sclerosis--early diagnosis and its significance in juvenile epilepsy]. / Tuberöse Sklerose--Frühdiagnose und ihre Bedeutung bei der epilepsie des Kindesalters

Twenty-three cases of tuberous sclerosis, found in the ten years' material of the authors among 2900 epileptics between o-16 years of age, are presented. 11 patients were treated before the age of 3 years, all showing infantile spasms. The earliest, in most cases congenital extraneural signs were depigmented areas on the skin. They were absent only in one case. They allowed, together with the Westsyndrome to establish the diagnosis if tuberous sclerosis before the appearance of other characteristic symptoms. Sebaceous adenoma, generally accepted as pathognostic for tuberous sclerosis, presented itself in all 12 early cases later then the leukoderma and the epileptic fits. Tuberous sclerosis is one of the important causes of epilepsy in childhood, primarily in early childhood. In this period of life the West-syndrome is the most frequent type of epileptic manifestations of tuberous sclerosis