S. Al Rajeh

A Community Survey of Neurological Disorders in Saudi Arabia: The Thugbah Study

We report the findings of a total population survey of Thugbah community in the Eastern Province of Saudi Arabia (SA) to determine its point prevalence of neurological diseases. During this two-phase door-to-door study, all Saudi nationals living in Thugbah were first screened by trained interviewers using a pretested questionnaire (sensitivity 98%, specificity 89%) administered at a face-to-face interview. Individuals with abnormal responses were then evaluated by a neurologist using specific guidelines and defined diagnostic criteria to document neurological disease. The questionnaire was readministered blind by a neurologist to all those with abnormal responses and a 1-in-20 random sample of those without abnormal responses, respectively. The family members of an individual with an abnormal response were also screened to improve accuracy. A total of 23,227 Saudis (98% of the eligible subjects) were screened and those residing in Thugbah on the reference date (22,630) were used to calculate the point prevalence rates. Forty-two percent of those screened were in the first decade of life and only 1.5% were more than 60 years old. There were marginally more females (50.2%) than males (49.8%). Consanguineous marriages especially between first cousins were present in 54.6%. The demographic characteristics of Thugbah community were similar to those in other parts of SA. The overall crude prevalence ratio (PR) for all forms of neurological disease was 131/1,000 population. All subsequent PRs are per 1,000 population. Headache syndromes were the most prevalent disorder (PR 20.7). The PR for all seizure disorders was 7.60, and the epilepsies (6.54) were more frequent than febrile convulsions (0.84). Mental retardation, cerebral palsy syndrome, and microcephaly were common pediatric problems with PRs of 6.27, 5.30 and 1.99, respectively. Stroke, Parkinsons disease, and Alzheimers disease were uncommon with respective PRs of 1.8, 0.27 and 0.22. Central nervous system (CNS) malformations (0.49) such as hydrocephalus and meningomyelocele were more prevalent than spinal muscular atrophy (0.13), congenital brachial palsy (0.13) and narcolepsy (0.04). Multiple sclerosis was rare (0.04). Osteoarthritis and low back pain syndromes were the main non-neurological conditions seen. The major medical diseases that may be neurologically relevant were diabetes mellitus, hypertension, and connective tissue disorders.(ABSTRACT TRUNCATED AT 400 WORDS)

The prevalence of epilepsy and other seizure disorders in an Arab population: a community-based study.

PURPOSE To determine the prevalence of epilepsy and other convulsive disorders and the causes of symptomatic epilepsies in a Saudi Arabian population. METHODS Door to door survey of a restricted area inhabited by 23 700 Saudi nationals. The World Health Organization (WHO) protocol designed to detect neurological disorders was used as screening instrument. All patients with probable seizures were examined by a neurologist and 92% of positive cases were investigated by brain computed tomography (CT) and electroencephalogram (EEG). RESULTS Prevalence rate (PR) for active epilepsy was 6.54 /1000 population (95% confidence interval 5.48-7.60). Twenty-eight percent of the patients had partial seizures, 21% generalized seizures and in 51%, it was not possible to determine if the generalized seizures had focal onset or not. The epilepsy was symptomatic in 32% of the cases: pre or perinatal encephalopathy 23%, head injury 4%, childhood neurological infection 4% and stroke 1%. Febrile convulsions PR was 3.55 /1000 children under the age of 6 years and isolated seizures were documented in only 0.18 /1000 population. CONCLUSIONS The PR of epilepsy in Saudi Arabs is within the range of the values reported in most communities. The causes of symptomatic epilepsies revealed a predominance of perinatal and inherited factors. Isolated and non-convulsive seizures were probably under-recognized due to various social and cultural factors as well as to lack of sensitivity of the questionnaire for non-convulsive seizures.

Thrombosis complicating high dose intravenous immunoglobulin: report of three cases and review of the literature.

High dose intravenous immunoglobulin (IVG) is increasingly used in a broad range of immune mediated diseases. Thrombosis was exceptionaly reported as a complication of this therapy. We describe three cases of thrombotic complications during or soon after IVIG treatment: myocardial infarction in a man and cerebral infarctions in an elderly man, associated with peripheral ischemia in a woman. In addition we review the published cases in the literature and discuss the possible etiologic factors.

A clinical and genetic study of 56 Saudi Wilson disease patients: identification of Saudi‐specific mutations

Wilson disease (WD) is a hereditary disorder, with recessive transmission and genetic heterogeneity. Several mutations of ATP7B, the gene underlying WD, were reported in many ethnic groups. In this study, mutation screening in ATP7B of 56 Saudi Arabian WD patients was undertaken. The clinical data of all patients were recorded. The entire ATP7B coding sequence, including intron–exon boundaries were screened for mutation by the polymerase chain reaction (PCR)‐based mutation detection technique and DNA sequencing. Thirty‐nine patients were symptomatic at presentation and 17 subjects were pre‐symptomatic siblings of affected patients. Fourteen patients had neurological, 11 patients had mixed (hepatic and neurological), and 14 patients had hepatic presentations. Family history suggestive of WD was present in 72% of cases and 68% had consanguineous parents. Genetic analysis showed disease‐causing mutations in three exons (exons 8, 19 and 21) of the ATP7B gene in 28 patients (50%). Mutations in exons 21 (18 cases) and 19 (one case) were unique for Saudis. This large series of Saudi patients with WD has shown wide variability in the genomic substrate of WD. There is no correlation between genotype and clinical presentation.

Stroke and pregnancy

Objective: To study the controversial relationship between pregnancy and stroke. Old studies and those from developing countries suggest that pregnancy considerably increases the risk of stroke. Methods: The records of 946 consecutive cases of stroke observed in Saudi patients in 10 years were reviewed. Cases which occurred during pregnancy or during the first 15 days postpartum were included in the study. Results: Twelve patients (1.3%) were pregnant or in postpartum at stroke onset. They constituted 27% of women of childbearing age. Cerebral infarction was diagnosed in nine cases and cerebral hemorrhage in three. Four patients died. Conclusion: The risk of stroke is only marginally increased (× 1.5) in pregnant compared with non‐pregnant Saudi women. Many factors including peripartum cardiomyopathy, patent foramen ovale, changes in clotting factors, peripartum angiopathy and vasoactive steroid hormones, could be responsible for this increased risk.

The prevalence of migraine and tension headache in Saudi Arabia: a community-based study

The prevalence of migraine and tension headache was determined in a 2‐stage, door‐to‐door community survey in Thugbah, Saudi Arabia. Out of 22630 subjects surveyed, 2742 individuals had headaches thus yielding a crude prevalence of 12.1% (95% CI = 11.7–12.5%) and with age‐adjustment, it rose to 15.9% (95% CI = 15.4–16.4%). There was female preponderance overall and the peak frequency was in the 3rd decade. The age‐specific rates rose from 2.4% in the first decade to 37.2% in the 7th decade. The prevalence of tension‐type headache (PR 9.5%; 95% CI = 9.1–9.9%) was higher than migraine (PR = 5.0%; 95% CI = 4.7‐5.3%). The low headache prevalence in this community compared to findings in western countries could be ascribed to the young age of the population and/or could possibly reflect the influence of traditional life styles and cultural factors in the Kingdom.

Non-traumatic cerebral hemorrage in Saudi Arabs: a hospital-based study of 243 cases

This is a retrospective study involving 243 Saudi Arabs with non-traumatic cerebral hemorrhages confirmed by CT scan. Intracerebral hemorrhages accounted for about 20% of all strokes in this population. THe male to female ratio was 2.74 which is slightly higher than those reported from elsewhere. Peak frequency was around 60 years. Hypertension was the most common cause accounting for 64% of the cases, hemopathies/coagulopathies for 10% and arteriovenous malformations (AVMs) for 6%. THe cause was undetermined in 21% of patients. Amyloid angiopathy was probably underrepresented due to lack of autopsy correlation. The etiologies were, however, age-related with a clear predominance of AVMs and coagulation disorders before the age of 40 years. Hypertensive hemorrhages were located in the putamen in 46% of cases compared to 20% in non-hypertensive patients while 52% of non-hypertensive hemorrhages were lobar in location compared to 20.5% in hypertensives. Early mortality (19%) in our series was relatively low compared to reports from the industrialized world.

Angiographic patterns of carotid ischemic cerebrovascular disease in Saudi Arabia

The angiographic findings in 100 Saudi patients with carotid ischemic cerebrovascular disease (CVD) (transient ischemic attacks (TIA) or strokes) were retrospectively reviewed. These patients were representative of all patients with similar disorders in the terms of sex, frequency of diabetes mellitus and ischemic heart disease. They were, however, significantly younger, smoked more, had more cervical bruits and less atrial fibrillation. Analysis of the data showed that a significant stenosis (> 70%) of the carotid artery origin was found only in 12% of TIA cases, 4% in the lacunar infarction cases and 6% of the large infarction cases on the symptomatic side and only in one case of TIA in the asymptomatic side. These frequencies were significantly lower than those found in similar studies performed in western countries. This study suggests that stenoses and occlusions of extracranial carotid artery plays a smaller role in the pathogenesis of ischemic CVD in Saudis than in Caucasians. The low consumption of cigarettes among elderly and females may be one of the explanations.