[Genetic aspects of epilepsy]. / Genetiske aspekter ved epilepsi.

Accumulating evidence suggests a significant proportion of the forms of epilepsy to be genetically determined. Several epilepsy syndromes have been mapped on the human genome, though their molecular basis remains unknown. Technical advances in molecular biology now provide a basis for improving our understanding of the molecular mechanisms involved in genetically determined types of epilepsy Genetic mapping will improve the accuracy of genetic counselling. Improved insight into the molecular biology may help to elucidate the underlying epileptogenic mechanisms and pave the way for new developments in pharmacological control of epileptic seizures. Further advances in research on genetics and epilepsy will require national and international cooperation between epileptologists and geneticists in search of informative families for linkage analysis.

[Progressive myoclonic epilepsy]. / Progressiv myoklon epilepsi.

Progressive myoclonic epilepsy (PME) is a syndrome characterized by myoclonias, epilepsy, progressive dementia and other neurological deficits. PME may be caused by various, rare, incompletely elucidated genetic diseases, and is characterized by age at onset, duration, clinical and pathoanatomical abnormalities. There is ethnic and geographic variation in the frequency of the syndrome. The diseases are frequently autosomal recessive. Research in PME leads to a better understanding of the neurobiological processes of epilepsy. PME should be considered in cases of severe myoclonic epilepsy, progressive neurological disability and poor effect of antiepileptic treatment, and biopsies from skin, mucosa or muscle should be performed. Centralization of treatment of these rare diseases is recommended.

[Steele-Richardson-Olszewski syndrome. Progressive supranuclear paresis]. / Steele-Richardson-Olszewski's syndrom. Progressiv supranukleaer parese.

The signs and symptoms of the Steele-Richardson-Olszewski syndrome (SRO) are described and the therapeutic possibilities are outlined. Two cases are presented. SRO must be suspected when a patient over the age of 40 years presents axial rigidity, dementia, pseudobulbar palsy, and supranuclear ophthalmoplegia.

[Complications of pediatric diseases]. / Komplikationer til børnesygdomme.

A review of all medical reports on patients discharged from the Central Hospital in Viborg from 1974 to 1986 after measles (M), mumps (F), German measles (R), or chickenpox (S) revealed a total of 294 patients suffering from complications, 128 after M, 142 after F, 9 after R, and 15 after S. A total of 373 complications was encountered. Neurologic complications were seen in 166 cases most frequently after F, and superinfection was seen in 105 cases most frequently after M. Sequelae were seen in 3 cases of M, 2 cases of S. and in one case of R. In all cases sequelae were caused by damage in the central nervous system. More widespread use of vaccination is urged.

[Acute non-suppurative encephalitis in children]. / Akut non-suppurativ encefalit hos børn.

From July 1973 to January 1988, 66 children aged from three months to 14 years, 36 boys and 29 girls, were seen at the Central Hospital, Viborg, with acute encephalitis. In 25 cases the encephalitis was caused by mumps, in eight by measles, in six by chicken pox, one by herpes simplex, one by RS-virus, and one by mycoplasma. One case was seen after vaccination, one after infection and in 22 cases the cause of the encephalitis remained unknown. Sequelae were seen in 17 cases. The risk of encephalitis after mumps, measles, and rubella is an important argument for the use of MMR-vaccination and the risk of encephalitis after chickenpox may justify a vaccination campaign against chickenpox.

Myasthenia gravis in the county of Viborg, Denmark.

The epidemiology of myasthenia gravis was studied in a Danish countryside county with 230,760 inhabitants. The incidence rate was 10.4 per million per year and the prevalence rate, 125 per million. The female to male ratio was 1.4 to 1. The diagnoses were delayed from a few hours to 6 years, most frequently on behalf of ophthalmologic diagnoses. A high incidence rate and a considerable diagnostic delay indicate that a number of patients suffering from myasthenia gravis are misdiagnosed, leading to unfortunate psychologic events.

Familial recurrent cranial nerve palsies.

Family cases of recurrent cranial nerve palsies are seldom reported. This paper presents a family with recurrent facial and ocular nerve palsies in 2 brothers. Their father and his sister had Bell's palsies. Examinations provided no explanation. Six previous reports of families with recurrent cranial nerve palsies are summarized. The pedigrees speak in favour of an autosomal dominant mode of inheritance of predisposing factors. The pathogenetic mechanism might be vascular or autoimmune, but is still unknown.

[Outpatient vasectomy]. / Ambulant vasektomi.

PIP: Discussion is presented concerning 89 outpatient vasectomies which were undertaken in a mixed medical-surgical provincial hospital after application for sterilization. Fascial interposition was employed without irrigation of the efferent segment of the ductus deferens. Postoperative control was managed by the general practitioner but control examination for spermatozoa was undertaken in the hospital laboratory following submission of an untreated ejaculate 3 months after the operation. The criterion for sterility was azoospermia. The material was reviewed retrospectively and 7 patients were excluded on account of a departure from the above mentioned procedure. 76% of the remainder submitted samples of ejaculate spontaneously and a total of 86% did so after reminders. 86% of these samples were without spermatozoa on the 1st examination, another 7% on the 2nd occasion and an additional 4% on the 3rd. In 2 patients (2%), recanalization occurred. (author's)^ieng