Child neurology--from my point of view.

My own way to pediatric neurology started with basic training in pediatrics at a time when neurology barely existed, but certainly not pediatric neurology. By chance, I happened to be involved with a family with interesting muscular symptoms and this awoke my interest in neurology in general, and particularly in muscle diseases and genetically determined diseases. I am deeply indebted to American pediatric neurology, since I got a wonderful chance to study pediatric neurology in the United States, particularly in Boston. To work within this field has always given me pleasure. The combination of strictly logical neurologic reasoning and the way to reach it through improvising and playing with the child is particularly exciting. For many of these diseases we have no definite cure, and some of them cause the death of the child, a serious situation for all involved. In these situations, I have come to know many parents for whom I feel a deep respect, affection, and admiration. Children and parents have taught me much, particularly the necessity of always being at eye level with the patient or the conversation partner, both literally and figuratively.

A trial of selenium and vitamin E in boys with muscular dystrophy.

The administration of selenium and vitamin E was tried in a group of 20 boys with muscular dystrophy. Muscular strength was measured at intervals of 6 months. The boys were treated for 1 year (selenium 6 micrograms/kg for 6 months and 20 micrograms/kg for 6 months), followed by 1 year of no treatment. The whole series was completed in 16 boys, nine of whom had classical Duchenne muscular dystrophy and the rest who had more benign variants. No boy showed any side effects. The decrease of muscle strength was slightly more rapid during the second year (no treatment) than during the first year (with treatment) of the trial. The difference was, however, slight and could conceivably be explained by the increase of age. No boy showed any practically usable increase of muscle strength during the year of treatment. The minimal muscle strength required for walking is presented.

Friedreich's ataxia in 13 children: presentation and evolution with neurophysiologic, electrocardiographic, and echocardiographic features.

Thirteen children with Friedreich's ataxia were reviewed. The clinical presentation and evolution of the disease was compared to that observed in large series--based mainly on adult patients--and the few studies in children. The mean age of onset (5.3 +/- 2.7 years) was lower than that reported in the former studies. Progressive unremitting ataxia of all four limbs was the earliest and most consistent finding, whereas dysarthria and loss of joint or vibration sense occurred with less frequency than that reported in adult series. The tendon jerks were absent or reduced in the lower limbs in almost all children. The universal absence of lower limb reflexes was shown to be too rigid to be obligatory for the diagnosis of early cases of Friedreich's ataxia. Electrophysiologic investigations revealed typical findings, ie, normal or low-normal motor conduction velocities and absent sensory responses. Electromyography showed more features of denervation in the lower limbs than in the upper limbs. Cardiac symptoms and signs were minimal, whereas electrocardiographic abnormalities occurred in 92% of patients, presenting mostly as significant T-wave changes. Concentric symmetric thickening of both the interventricular and left ventricular posterior walls was the major echocardiographic finding.

Dermatomyositis and polymyositis in childhood.

Personal experience of 30 years work with 40-50 cases of dermatomyositis and polymyositis in childhood is reviewed, stressing the clinical findings of skin eruptions on the knuckles, elbows and knees (except in the 10 per cent of patients without any skin involvement), weakness of particularly proximal muscles and tightness of tendons. Special diagnostic procedures are reviewed as well as treatment. The basic treatment is corticosteroids, which must be started at a high dose and as soon as possible be given at intervals of 48 hours to diminish the side-effects. In many, though not all, patients the treatment must be continued for years. The parents should always know that extended treatment may be necessary. Physiotherapy should be started cautiously and be slowly increased, close co-operation between the physician and the physiotherapist being necessary. If the patient does not respond to corticosteroids, antimetabolites may be added. Most patients survive with no or only minor sequelae.

The chronically Ill, handicapped, dying child and his family: need for total care and support.

It is stressed that the best possible medical care is the basic need and an absolute pre-requisite for the total care of a family with a chronicall ill, handicapped or dying child. However, this is not enough. The family will need something more than just strict medical care. This paper is concerned with the "something more," stressing the family's need for measures helping them to work with the crisis reaction, elicited by the information they have received about the child's diagnosis and prognosis. The methods of bringing the family help is discussed in relation to the time-schedule of the disorder. The importance of how the first information is given is particularly stressed.

Neonatal convulsions treated with continuous, intravenous infusion of diazepam.

Eight patients born at term in the years 1974-76, with neonatal convulsions due to severe perinatal asphyxia, were treated for 6-11 days with continuous intravenous infusion of diazepam. Doses of 1.0-1.5 mg per hour (mg/h) were usually required to stop the convulsions. In one infant 2.75 mg/h was needed. During the treatment, all infants had measurable serum concentrations of diazepam, half of them above 35 mumol/1. The convulsions stopped in all eight infants, and did not return after discontinuation of the infusion. Side-effects were noted in all infants, but they were all able to breathe adequately. At follow-up the psychomotor development was normal in all cases and there were no signs of neurological disorders, except in one patient, in whom mild epilepsy was observed. Continuous infusion of diazepam should be given in doses of at least 1 mg/h (corresponding to around 0.3 mg/kg h) to stop convulsions in full-term infants efficiently and should be increased under close supervision and with monitoring of respiration and heart rate until treatment is effective.