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.

Lyme borreliosis from a patient's view-point.

The paper describes my own case of Lyme borreliosis, thus from the patient's point of view, stressing the clues which might have led to an earlier diagnosis. Intense antibiotic treatment is of value, also when given first some 15 years after the probable time of the infection, as, at least in my case, it stopped the progress of all symptoms and removed some of them, particularly muscle pains and skin abnormalities. Long-standing, well-established neurological symptoms, however, remain unchanged, but their previous slow, steady progress has stopped.

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.

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.

A new type of muscular dystrophy in two brothers: analysis by use of DNA probes suggests autosomal recessive inheritance.

X-chromosome-specific DNA probes were used to study a new type of muscular dystrophy (MD) presented by two boys in a family in which there was no previous history neuromuscular disease. Clinical investigations showed evidence of myogenic myopathyia, but its exact nature could not be established. The results of the DNA analysis exclude DMD, BMD and EMD.

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.

Selenium supplementation in X-linked muscular dystrophy. Effects on erythrocyte and serum selenium and on erythrocyte glutathione peroxidase activity.

The selenium concentrations in serum and erythrocytes and the erythrocyte glutathione peroxidase activity were determined in 15 boys with the Duchenne type and in 5 boys with the Becker type of X-linked muscular dystrophy before and during long-term selenium and alpha-tocopherol supplementation and compared with values in unsupplemented controls. The purpose of the treatment was to improve the muscular strength. Twelve of the 20 patients had pretreatment levels of selenium in serum that were within the 95% confidence limit of the unsupplemented control children.

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.

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.

Haemangio-endothelioma of the orbit.

A case of infantile orbital haemangio-endothelioma was successfully treated with corticosteroids. Roentgenological and histological findings before and after treatment are described. It is suggested that an haemangio-endothelioma may be transformed into a cavernous angioma.

Bilateral teratoma of testis in two brothers with 47,XXY Klinefelter's syndrome.

Two brothers, the only children of healthy, unrelated parents with normal karyotypes, had 47,XXY-Klinefelter's syndrome and bilateral testicular teratomas. The oldest XXY-boy had, in addition, congenital hydrocephalus caused by congenital stenosis of the aqueduct of Sylvius.