A population-based study of active epilepsy was conducted in 6-13-year-old mentally retarded children born between 1975 and 1986. The population at risk comprised 48,873 children. Ninety-eight children were identified, 35 mildly and 63 severely retarded. The prevalence was 2.0 per 1000; 0.7 per 1000 for mildly and 1.3 per 1000 for severely retarded children. Sixty-nine children had at least one additional neuroimpairment. Cerebral palsy was found in 42 children with a majority of spastic/dystonic tetraplegias; visual impairment was present in 24 and autism in 24. Thirty-three children had only a mild or no gross motor disability and mild mental retardation, while 23 had IQs
OBJECTIVE: Infants with cleft lip and palate may often have other associated congenital defects although the reported incidence and the types of associated malformations vary between different studies. The purpose of this investigation was to assess the prevalence of associated malformations in a geographically defined population. METHODS: The prevalence of associated malformations in infants with clefts were collected prospectively between 1975 to 1992 on all infants born in greater Stockholm, Sweden. The patient records were also compared with data from the National Malformation Registry and other hospital records if any. RESULTS: Of the 616 cleft infants (367 boys, 249 girls) born during this period, 21% had associated malformations that either required follow-up or treatment. Associated malformations were more frequent in infants who had both cleft lip and palate (28%) than in infants with isolated cleft palate (22%) or infants with isolated cleft lip (8%). Malformations of the upper or lower limbs or the vertebral column were the most common other anomalies and accounted for 33% of all associated defects. Twenty-four percent of associated malformations were in the cardiovascular system and congenital heart disease was the most common isolated associated malformation. Fifteen percent of all associated malformations were multiple and they were frequently associated with mental retardation or chromosomal anomalies. Twenty-two percent of infants with associated malformations were born preterm, compared with an expected 5% incidence of preterm delivery in Sweden. CONCLUSION: A more extensive cleft seems to be associated with a higher risk for associated malformations. Although many associated congenital defects can be detected at a physical examination, the high prevalence of congenital heart disease (16 times that of general population) may justify a routine echocardiographic screening.
All the benzodiazepines (BZDs) in clinical use have the capacity to promote the binding of the major inhibitory neurotransmitter, gamma-amino-butyric acid (GABA), to sub-types of GABA receptors which exist as multi-subunit ligand-gated chloride channels. Thus, the BZDs facilitate the actions of GABA in the brain. The BZDs in use as antiepileptic drugs are diazepam, clonazepam, clobazam, nitrazepam, and lately, also lorazepam and midazolam as emergency therapy. The BZDs have a wide-spectrum of proven clinical efficacy in the prevention of different kind of seizures. Clonazepam and clobazam, as well as nitrazepam in some cases, can be useful as an adjunct treatment in refractory epilepsies. However, the clinical use of BZDs for the prophylactic treatment of epilepsy is associated with two major problems which have limited the long-term use of these drugs: the potential for side-effects, especially sedative effects, and the high risk of development of tolerance. Despite the limitations of BZDs in the prophylactic treatment of epilepsies, these drugs play a prominent role in clinical practice in the emergency management of acute seizures and status epilepticus. Diazepam, clonazepam and lorazepam are all considered first-line agents in the emergency management of acute seizures and status epilepticus. Furthermore, the value of midazolam as an emergency therapy in epilepsy has been increasingly recognized in recent years.
A preliminary survey of the causes of blindness in mentally retarded children in Denmark showed that 50% of the children had optic atrophy, 12.5% had cataract while tapeto-retinal degenerations, other malformation and retrolental fibroplasia each represents 8-9% of the causes of blindness. The survey, was done through written notification and some patients with tapeto-retinal degenerations might have been wrongly diagnosed.
This seventh Swedish population-based cerebral palsy (CP) report comprises 216 children born between 1987 and 1990. The crude live birth prevalence was 2.36 per 1000, indicating a break in the continuous increase since 1970. Excluding 10 postnatally-derived cases, gestational-age specific prevalences were 80 for extremely, 54 for very and 8 for moderately preterms and 1.4 for term children per 1000. Birth weight-specific prevalences were 57 for birth weights or = 2500 g per 1000. The aetiology was considered prenatal in 8%, peri/neonatal in 54% and unclassifiable in 38% of preterms and 33, 28 and 39% of term children. Hemiplegic, diplegic and tetraplegic syndromes accounted for 22, 66 and 7% of preterms and 44, 29 and 10% of term children. Non-walking, mental retardation, epilepsy, severe visual impairment and infantile hydrocephalus were present in 39, 39, 26, 18 and 23% of preterms; and 38, 44, 36, 14 and 5% of term children, respectively. The entire series of 1408 cases born in 1954-90 revealed three distinct trend eras for preterms, clearly related to changes in perinatal care and shifts in type of CP manifestations.
The prevalence of cerebral palsy (CP) in Swedish infants born in the four-year period 1979-82 is reported and related to the prevalence in infants born during the twenty-year period 1959-78. In 1979-82 it was 2.17 per 1,000 livebirths, 1.23 for children born at term and 0.94 for preterms, which means that the rising trend since the beginning of the 1970s persisted. The most pronounced rise, from 0.18 per 1,000 livebirths in the period 1967-70 to 0.67 in 1979-82 was found in the subgroup of preterms with spastic/ataxic diplegia. The severity of motor disability and the relative frequency of mental retardation, infantile hydrocephalus and epilepsy among preterm CP children successively increased over the same period of time. The livebirth prevalence of CP in term infants increased slightly but non-significantly during the period 1967-82. The birthweight-specific prevalence of CP per 1,000 newborns surviving the first week of life increased in all birthweight groups during the period 1967-82, significantly for birthweights below 1,500 g and over 2,500 g. The rising prevalence of CP was concomitant with a parallel fall in perinatal mortality, especially in very preterm infants. In the 1980s, severely multi-handicapped, very preterm children, only exceptionally seen among CP children born in the 1960s and 1970s, has become a matter of concern.
A population-based group of 27 children with total blindness due to retinopathy of prematurity (ROP), born in Sweden from 1980 to 1990, was examined. They constituted all but two of the total of 29 children with total blindness due to ROP known to the national register of visually impaired children when reviewed from 1980 to 1 January 1995. All children had a gestational age of less than 31 weeks and most had had a complicated perinatal period. The retinal disease was discovered late, most often after it had already progressed to bilateral retinal detachment. Repeated vitreoretinal surgery had been performed in most children, but postoperative visual function did not improve. Three-quarters of the group had major neurological impairment (mental retardation, cerebral palsy, or epilepsy) at age 4 to 14 years. There was an impression that extensive ophthalmological efforts delayed neurodevelopmental assessments and examinations as well as adequate habilitation.
For a period of six years, concomitant disorders were registered at annual routine examinations of 226 residents of a central institution for mentally retarded. Only in six patients were no concomitant disorders found. 19.5% displayed concomitant disorders from one type of disease, 30.5% displayed two or more concomitant disorders from two types of disease, and as many as 47.3% displayed concomitant disorders from three or more types of disease. The residents examined showed particularly high occurrences of various deformities, mental disorders, and diseases of the nervous system, sense organs and musculo-skeletal system. The most frequent singular disorders were epilepsy, cerebral palsy and deformities of the back and foot.