Adnan Awada

Stroke Register: Experience from the Eastern Province of Saudi Arabia

A stroke registry was established in the Eastern Province of Saudi Arabia with an estimated population of 750,000 inhabitants of whom 545,000 are Saudi citizens. The register started in July 1989 and ended in July 1993. The Gulf war led to its interruption from August 1990 to August 1991. Four hundred eighty-eight cases (314 males, 174 females) of first-ever strokes affecting Saudi nationals were registered over the 3-year period. The crude incidence rate for first-ever strokes was 29.8/100,000/year (95% CI: 25.2–34.3/100,000 year). When standardized to the 1976 US population, it rose up to 125.8/100,000/year. Ischemic strokes (69%) predominated as in other studies but subarachnoid hemorrhage (SAH) was extremely rare (1.4%). The important risk factors were: systemic hypertension (38%), diabetes mellitus (37%), heart disease (27%), smoking (19%) and family history of stroke (14%). Previous transient ischemic attacks (3%) and carotid bruits (1%) were uncommon. The 30-day case fatality rate was 15%. The study showed that the age-adjusted stroke incidence rate for Saudis in this region is lower than the rates reported in developed countries but within the range reported worldwide. The pattern of stroke in Saudi Arabia is not different from that reported in other communities with the exception of the low incidence of SAH. The risk factors are similar to findings in other studies except for the high frequency of diabetes mellitus in our cases. The lower mortality rate was probably due to the younger age of the population and the availability of free medical services for management of cases.

Headache syndromes amongst schoolchildren in Riyadh, Saudi Arabia

We evaluated 1400 randomly selected Saudi children in grades 1 through 9 to determine the prevalence of migraine and tension‐type headache.

Stroke in Saudi Arabia

In this review, the authors summarize 10 years of work on stroke in Saudi Arabia that has led to more than 25 publications. The incidence and prevalence of strokes were low when compared to those reported from Western countries, but this was mainly due to the younger age of the population. The overall distribution of stroke types was not different from that reported in other communities, with the exception of the low incidence of subarachnoid hemorrhage. However, the relatively high frequency of strokes in the young and strokes related to small artery disease, i.e. lacunar infarcts and cerebral hemorrhage, and the high prevalence of diabetes mellitus as a risk factor were quite distinctive. On the other hand, extracranial large artery disease was quite rare. Undetected and untreated hypertension and a low frequency of cigarette smoking in the stroke-prone age group could be one explanation of these findings.

Pattern of Presentation of Multiple Sclerosis in Saudi Arabia: Analysis Based on Clinical and Paraclinical Features

We studied 89 MS patients comprising 38 males and 51 females seen over a 10-year period. The hospital frequency was 25/100,000 patients. The diagnosis was mainly clinical and was supported by neuroimaging, cerebrospinal fluid analysis and neurophysiological tests. Sixty-five patients (73%) were Saudis and the peak age of onset was in the third decade. Fifty-two patients (58.4%) had clinically definite MS, 17 (19.1%) had laboratory-supported definite MS, 15 (16.9%) were clinically probable MS cases and the remaining 5 (5.6%) had laboratory-supported probable MS. The mean age at onset of Saudi patients (25.9 years) was lower than that of the non-Saudis (29.4 years; p < 0.001). Involvement of the pyramidal system was the commonest mode of presentation. The clinical course was relapsing-remitting in 60.7%, progressive-relapsing in 20.2% and primary progressive in 19.1%. The number of systems involved was significantly associated with the duration of disease (p < 0.001). The demographic features and the variability of clinical presentation of Saudi MS patients is similar to the results from neighbouring countries. Combination of clinical features and paraclinical tests is essential for accurate determination of extent of dissemination and for unmasking clinically silent lesions.

Excessive sweating: an uncommon sign of basilar artery occlusion.

Excessive sweating of the face and, to a lesser degree, of the thorax and limbs, occurred after basilar artery occlusion in a 15 year old Saudi boy. Magnetic resonance imaging (MRI) showed a limited bilateral paramedian infarction of the rostral ventral pons. Basilar artery occlusion in children and adolescents is a rarity. Excessive sweating following such a stroke is rare in any age group. Clinico-anatomical correlations are discussed.

Epilepsy and other convulsive disorders in Saudi Arabia: a prospective study of 1,000 consecutive cases

Abstract The pattern of epilepsy and other convulsive disorders in 1,000 consecutive Saudi nationals is described. These disorders were common with a hospital frequency rate of 8 per 1,000. Men were more frequently affected than women and 60% of the patients were under 10 years old at the onset of their illness. The epilepsies were the commonest type (74%). Febrile convulsions (20%) presented mainly between the ages of one and five years. Isolated seizures (3%) and acute symptomatic convulsions (3%) were uncommon. In the epileptic group, generalised seizures (71%) were more frequent than partial (29%) and complex partial seizures occurred mainly in those above 21 years old. Absences (4%), infantile spasms (3%) and atonic seizures (3%) were uncommon. No specific etiology of the epilepsy was determined in the majority of the cases (63%). The identified major etiologic factors of the epilepsies were perinatal encephalopathy (21%), cerebral trauma (11%), sequelae of meningitis or encephalitis (2%), brain tumors (0.5%), and vascular lesions such as stroke and arteriovenous malformation. Perinatal encephalopathy accounted for 40% of the epilepsies in children less than 5 years old, and trauma for 20% of those above 20 years old. A family history of epilepsy in close relations was obtained in 23% of the cases, and the consanguinity rate among the parents was 53%. The high incidence of associated perinatal encephalopathy found in this study suggests that perinatal factors play a major role in the pathogenesis of epilepsy in Saudi Arabia. The high frequency of cerebral trauma was also striking. Although consanguinity of the parents appeared not to be a major factor in the genetics of convulsive disorders in this environment, it might have potentiated the tendency of familial aggregation of convulsive disorders in this community. Consanguinity may be an important factor in the production of some of these disorders but its precise role has not been determined.

Cavernous Angioma Presenting as Pregnancy‐Related Seizures

Summary: Purpose: To determine the reason that one‐quarter to one‐third of epileptic women experienced an increased number of seizures during pregnancy. The cause of this increase is not always clearly understood and the principle emphasis of the literature is on the pregnancy‐associated changes of anticonvulsant pharmacokinetics.

A novel deletion mutation within the carboxyl terminus of the copper-transporting ATPase gene causes Wilson disease

In patients with Wilson disease (WD), an autosomal recessive disorder, toxic accumulation of copper results in fatal liver disease and irreversible neuronal degeneration. ATP7B, the gene mutated in WD, contains 21 exons and encodes a copper-transporting ATPase. In this study, all exons of the ATP7B gene of nine WD patients were screened for alterations by conventional mutation detection enhancement (MDE) heteroduplex analysis, followed by direct sequencing of the regions that showed heteroduplex formation. For the first time, a novel deletion mutation (4193delC) in exon 21, causing a frameshift leading to premature truncation of the protein was detected in four of nine patients. The 4193delC removes several signals within the carboxyl terminal domain that may disrupt trafficking of ATP7B protein through trans-Golgi network at the cellular level.

Molecular analysis of the SMN and NAIP genes in Saudi spinal muscular atrophy patients

In this study we examined the deletion of the SMN and NAIP genes in 14 Saudi families (16 patients and 38 relatives of the patients, including parents and siblings) and six healthy Saudi volunteers. The homozygous deletions of exons 7 and 8 of the telomeric SMN gene and exon 5 of the NAIP gene were found in seven out of eight spinal muscular atrophy (SMA) type-I patients. In seven SMA type-II patients, exons 7 and 8 of telomeric SMN were deleted in six cases and exon 5 of NAIP was deleted in three cases. Three patients with SMA diagnosis did not show either of the above deletions. All control Saudi volunteers and all but two family members of the patients had both normal SMN and NAIP genes. Our results show that the incidence of NAIP deletion is higher in the more severe SMA cases and the dual deletions of the SMN and NAIP genes are more common in Saudi SMA type-I patients compared to patients of other ethnic groups.

Community survey of neurological disorders in Saudi Arabia: Results of the pilot study in Agrabiah.

A pilot study of the Agrabiah area in Al-Khobar was undertaken to field test study methodologies and identify possible limitations and constraints to a planned community survey for neurological disorders in the Eastern Province of Saudi Arabia. The survey used a pre-tested questionnaire administered by trained personnel to all subjects living within 50 blocks randomly selected from the 198 inhabited ones in the area. Subjects with abnormal responses on screening were then evaluated by neurologists using specific guidelines and criteria to establish the diagnosis of neurological disease. One thousand four hundred and eighty-five subjects (98.3% of all eligible subjects) were screened: 227 (15%) had abnormal responses. Of the 202 subsequently evaluated by neurologists, 178 had definite neurological disease. The overall crude prevalence rate (PR) per 1000 population for neurological morbidity was 120.5 (95% confidence limits [CL] 103.5 to 136.5). Headache syndromes (PR 99.7, CL 83 to 114.7) were common. The other common disorders were seizures (PR 10.2, CL 5.1 to 15.3), peripheral nerve disorders (PR 2.7), and stroke (PR 2.0). Mental retardation and cerebral palsy were the main pediatric problems with PRs of 1.4 and 0.7 respectively. Our results show that a community survey for neurological disorders is feasible in Saudi Arabia and the modified questionnaire was a good screening instrument (sensitivity 94.7%, specificity 96.8%). However, the findings on the pattern and prevalence of neurological disorders need to be viewed with caution, particularly against the background of the scope of the study and the small number of subjects assessed. Cultural practices, local time and social events, and climatic conditions significantly affected community participation and the coverage achieved by the study. These factors should be considered when planning community surveys in Saudi Arabia and other environments with similar sociocultural settings.