Prevalence of mental retardation among children in Saudi Arabia.

This survey determined the prevalence and regional distribution of mental retardation among children in Saudi Arabia. Data were analysed from a population-based national survey conducted during 1996-99, in which 60,630 children aged up to 18 years were screened using IQ tests and questionnaires completed by physicians. The prevalence of mental retardation was 8.9 per 1000 children, a rate similar to that reported in other countries. Moderate or severe retardation was classified in 70.9% of these children. Of the mentally retarded children in the 0-18 years age range, 83.2% were not attending school. Special educational programmes are needed to improve the quality of life of mentally retarded children.

Prevalence of mental retardation among children in Saudi Arabia

This survey determined the prevalence and regional distribution of mental retardation among children in Saudi Arabia. Data were analysed from a population-based national survey conducted during 1996-99, in which 60,630 children aged up to 18 years were screened using IQ tests and questionnaires completed by physicians. The prevalence of mental retardation was 8.9 per 1000 children, a rate similar to that reported in other countries. Moderate or severe retardation was classified in 70.9% of these children. Of the mentally retarded children in the 0-18 years age range, 83.2% were not attending school. Special educational programmes are needed to improve the quality of life of mentally retarded children

Sotos syndrome (cerebral gigantism): a clinical and radiological study of 14 cases from Saudi Arabia.

Fourteen children (of Arab ethnic origin) with Sotos syndrome are described. They were referred to King Khalid University Hospital, Riyadh between July 1992 and June 1997. Their phenotypic characteristics were compared with established diagnostic criteria. There was a male:female ratio of 1.3:1 and a high rate of consanguinity (36%) among parents. At birth, 54% were large and about one-third showed increased height and occipitofrontal head circumference (OFHC). The neonatal histories revealed respiratory and feeding problems in 21%, followed later by delayed motor milestones and speech development in 57%. During childhood, weight, height and OFHC increased further to > 97th centile in 71%, 71% and 93%, respectively. A seizure disorder affected 43%, and 75% had mental retardation (IQ < 70). A non-specific EEG abnormality was found in half of those with seizures. Cranial CT/MRI showed ventricular dilatation in 15% and one patient had corpus callosum dysgenesis. Abdominal ultrasound revealed hydronephrosis in two patients. Radiological cephalometric measurements showed relative prognathism in cases of Sotos syndrome compared with controls (p = 0.003). The study highlights the importance of considering Sotos syndrome in children who present with psychomotor delay.

Childhood neuromuscular disorders: a decade's experience in Saudi Arabia.

The study describes the pattern of childhood neuromuscular disorders seen in a decade (1982-1992) at King Khalid University Hospital, Riyadh, Saudi Arabia. Similar data are scanty outside Europe and North America, and lacking in Saudi Arabia. Eighty-four children (< or = 16 years) were assigned to an entity of neuromuscular disease following review of the clinical, biochemical and neurophysiological data, and after re-examination of the histological and histochemical features of the muscle biopsies. Of the 84 ascertained cases, 40 (48%) had different forms of muscular dystrophy (MD), 26 (31%) had one of the various types of spinal muscular atrophy (SMA) and two (2.4%) hereditary motor and sensory neuropathy type I. The rest were miscellaneous cases including one (1.2%) with dermatomyositis. Of the dystrophies, severe childhood autosomal recessive muscular dystrophy (SCARMD) was more prevalent (30%) than Duchenne type (25%), conforming with observations from North African countries known to have a high incidence of consanguineous marriages. Family history of other cases of SCARMD included three males and three females, one of whom died at 15 years, and consanguinity was evident in 63%. Congenital MD, inherited in an autosomal recessive pattern, was also common (30%). A history of consanguinity was present in 55%. Of the 26 cases of SMA, type I (Werdnig-Hoffman disease) was the most prevalent (69%). Consanguinity was ascertained in 65% of SMA families and histories revealed another 14 affected siblings. Autosomal recessive forms seem to constitute the bulk of neuromuscular disorders in Saudi Arabia.

Rett syndrome in Saudi Arabia: report of six patients.

Six girls (five Saudis and one Sudanese) aged between 3.5 and 12 years demonstrated the classic features of Rett syndrome (RS), including developmental regression with dementia, loss of acquired speech and hand function, and stereotypic hand movements. Myoclonic seizures were observed in two of them. Electroencephalography showed diffuse slowing in three cases generalized epileptic discharges in one, and normal findings in two. Cranial computed tomography revealed normal findings, except in the 12-year-old girl who had mild bilateral frontal lobe atrophy. Except for mildly elevated blood ammonia in three patients, associated with slightly elevated blood lactate in two of them, results of neurometabolic screening tests were normal. There are, as yet, no laboratory markers pathognomonic for RS. Hence, adherence to current diagnostic criteria is essential. To prevent unnecessary and costly investigations, especially in developing countries, wider knowledge of the clinical features of the syndrome is warranted.

Chorea as a presentation of herpes simplex encephalitis relapse.

Three infants, ages 3 months to 3 years, presented with chorea as the initial manifestation of herpes simplex encephalitis (HSE) relapse. Patient 2, treated with repeated 10 day courses of 30 mg/kg/day of acyclovir, had no clear improvement in neurological status. Patient 1, treated with a repeated 10-day course, improved only to have another HSE relapse 4 years later. Patient 3 clearly improved soon after a 3-week course of acyclovir at conventional dosages. A fourth patient (Patient 4) who relapsed with chorea after what was thought to be HSE, and who did not respond to repeated acyclovir treatment, was negative for herpes simplex virus indicators on brain biopsy and DNA testing. We recommend treating all patients suffering from HSE with a minimum 3-week course of acyclovir at 30-35 mg/kg/day in 3 divided doses.

Antecedents and outcome of simple and complex febrile convulsions among Saudi children.

Of 215 Saudi children seen with their first febrile convulsion (FC) at the King Khalid University Hospital, Riyadh, between January 1984 and December 1988, the index FC was simple for 133 children and complex for the remaining 82. History of adverse antecedent factors, particularly perinatal asphyxia, birth injuries and pre-existing neurological deficits, were significantly more associated with complex FC, as was occurrence of first FC before the age of 12 months. Recurrence of FCs and development of epilepsy were also more common among the group of children with complex FC. Complex FCs were less benign in the present study population than has been reported in some Western studies.