Hereditary neurodegenerative disorders in Nigerian Africans.

Of 2.1 million patients seen in 25 years at the University College Hospital, Ibadan, Nigeria, only 25 suffered from heredodegenerative disorders of the nervous system. Six patients had hereditary ataxia, 10 essential tremor, 4 Huntington's chorea, 2 ataxia telangiectasia, and 3 Charcot-Marie-Tooth disease.

Comparison of the prevalence of Parkinson's disease in black populations in the rural United States and in rural Nigeria: door-to-door community studies.

A door-to-door survey of Parkinson's disease (PD) in Copiah County, Mississippi, using a pretested screening procedure (with a high sensitivity for detecting PD), followed by examination of all positives by a senior neurologist, revealed similar prevalence ratios for blacks and whites. The same procedure was applied in the community of Igbo-Ora, Nigeria, a black population of West Africa. To assure uniformity in the procedures and application of the diagnostic criteria, a neurologist from each survey site visited the other site. Among a black population of 3,521 over age 39 in Copiah County, there were 12 cases of PD, with an age-adjusted prevalence ratio of 341/100,000. The comparable figures for Igbo-Ora were as follows: population over age 39 = 3,412; cases of PD = 2; age-adjusted prevalence ratio = 67/100,000.

Monoclonal gammopathy (Waldenström's macroglobulinaemia) producing specific red cell antibody.

Two cases of Waldenström's macroglobulinaemia have been seen at University College Hospital, Ibadan in the last four years, Case 1 was a 30-year-old soldier who presented with splenomegaly and anaemia, was treated with chlorambucil, and had a complete remission sustained for over two years. Case 2 was a 58-year-old retired civil servant who presented with very severe anaemia and also splenomegaly, and died within three weeks of admission. Both patients had most of the typical features of Waldenström's disease, including retinal changes and serum IgM levels of 4200 and 5500 mg/dl respectively. In both cases an atypical cold antibody was detected in the course of blood cross-matching procedures. In case 1, the antibody agglutinated all adult and cord red cells tested, including the patient's own cells, to a titre of 8000 and above at 4 degrees C. Suprisingly enough, when the patient went into remission and the serum IgM level had fallen to 400 mg/dl, this antibody was no longer detectable and has not reappeared two years later. In case 2, the antibody agglutinated all adult red cells tested to a titre of 2000 at 20 degrees C but not the patient's own red cells. Since cord cells were agglutinated only to a titre of 4 to 20 degrees C it was concluded that the patient had an alloantibody with I-specificity. Therefore in both these patients the monoclonal immunoglobulin produced by the neoplastic lymphoid cell clone had specific activity against red cell antigens.

Pattern and ethnic variations in stroke in Saudi Arabia.

We report our observations in 427 stroke patients (305 Saudis, 122 non-Saudis with an age range of 14 months to 85 years) seen in a tertiary hospital in the eastern province of Saudi Arabia over an 8-year period. Of these patients, 115 (27%) were between 18 and 45 years old, and constituted the "young stroke patients" for this study. The hospital frequency for the young was 5/10,000 inpatients. In general, there was a male preponderance, with a male:female ratio of 2.2:1 and 7:1 for Saudis and non-Saudis, respectively. Ischemic stroke (55%) was more frequent than hemorrhagic stroke (25%), and the stroke was unspecified in 20%. The main etiologic factors were hypertension, diabetes mellitus and cardiac disorders. In the young population, the frequencies of hemorrhagic and ischemic strokes were similar. In this group, the main causes of intracerebral hemorrhage were aneurysms and arteriovenous malformations, while arteriosclerosis and embolism of cardiac origin were responsible for the ischemic strokes. In Saudis, the stroke types were 59% ischemic, and 17% hemorrhagic, as against 45 and 48% in non-Saudis, respectively. Most ischemic strokes were found in Saudis (78%). Intracerebral hemorrhage accounted for 63% of all hemorrhagic strokes, and was more frequent in Saudis but subarachnoid hemorrhage was three times more common in non-Saudis. In the young stroke patients, interethnic comparison showed that individuals from the Far East were nine times more likely to have hemorrhagic than ischemic stroke compared to the others (odd's ratio = 8.7), and the etiology of ischemic stroke remained undetermined in 67% of those from the Indian subcontinent.(ABSTRACT TRUNCATED AT 250 WORDS)

Stroke in Saudi children.

This study reports the clinical features and neuroimaging correlates of stroke in Saudi children seen over a 5-year period at the King Fahd Hospital of the University, Al-Khobar, Saudi Arabia. During the study period, 31 (18 boys, 13 girls; mean age, 26.2 months) of the 20,895 children seen had stroke; the annual stroke incidence was 29.7 per 100,000 in the pediatric population. Ischemic strokes accounted for 90% and hemorrhagic 10% of the cases, respectively. The boys-to-girls ratio for ischemic stroke was 2:1. Cranial computed tomographic scans and magnetic resonance imaging findings were abnormal in 82% and 91%, respectively. The etiologic factor was undetermined in 65% of the cases. Our results suggest that stroke is uncommon in Saudi children. However, further studies evaluating a larger population in different clinical settings are required to provide a more comprehensive picture of stroke in children in this area.

The prognostic features of biochemical investigations in tetanus.

Factors determining prognosis in tetanus have been based mainly on clinical grounds and mortality is high especially in the developing countries. The biochemical changes occurring in tetanus before any form of therapy or sedation, were examined to determine screening parameters for earlier institution of intensive care in high risk patients. The results obtained from a prospective study of 73 patients are presented. The blood sugar and serum transaminases correlated well with the severity of the disease. The serum glutamic oxalate transaminase and blood urea were significantly higher in fatal cases. These findings as well as the clinical criteria utilized in determining the severity of the disease in this study are useful in predicting the outcome of the disease. It is suggested that these simple investigations could serve as screening parameters for instituting early intensive care in tetanus.

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.

Obstetric neuropraxia in the Nigerian African.

The results of a prospective study of 34 Nigerian women with obstetric neuropraxia (puerperal paresis of the lower limbs) seen at the University College Hospital, Ibadan, are presented. The height of 29 (84%) was under 62 in (1.58 m). All were younger than 45, and 41% (14) were primiparous. Lumbosacral plexus injury with a foot-drop was the most frequent presenting feature (88%), bilateral involvement was observed in 13 patients (38%), femoral neuropathy was observed in nine (26%) and the ankle tendon jerks were absent in 35%. Spastic paraparesis was not uncommon (15%). Results of electromyographic examination and determinations of conduction velocities were consistent with proximal neuropraxia of the lumbasacral trunk in many of them (88%). The presentation of the fetus was cephalic in 97% of the women. The major predisposing factor was prolonged labor. Among the complications associated with the neuropraxia were hydroureters above the pelvic brim and vesico- and rectovaginal fistulae. Perinatal mortality was high particularly with labor of more than 18 hours. Recovery from the neuropraxia was complete for 76% of the patients. It is concluded that direct pressure on the lumbosacral plexus and nerve trunks by the presenting fetal part is the major factor in the pathogenesis of obstetric neuropraxia encountered in Nigerians.

A clinical study of stroke.

The records of 99 Saudis (68 males and 31 females) admitted to the King Fahd Hospital of the University (KFHU) over a two-year period were reviewed. There was a male to female ratio of 2.2:1. Eighty-five (86%) patients were above 44 years old. All the patients under 44 years old were males. The major predisposing factors identified were hypertension (65%), diabetes mellitus (36%), cardiac disease (20%) and cigarette smoking (29%). The combination of hypertension and diabetes mellitus seemed to carry a higher risk especially in women. Motor dysfunction, encountered in 95 (96%) patients was the dominant clinical feature, and presented mainly as hemiparesis (83 out of 95). Impaired level of consciousness at presentation carried a poor prognosis particularly in the elderly.

Prevalence of headache and migrainous headache in Nigerian Africans: a community-based study.

In a door-to-door survey in a Nigerian town with stable population of about twenty thousand, 18,594 subjects were screened with a questionnaire, which involved a complete census, administered by non-doctor, including primary health care personnel. Migrainous headache was diagnosed on the basis of combination of responses to the questionnaire shown in a pilot study validated by neurological examination to have 92% sensitivity and 99% specificity. Crude life time prevalence ratio of at least one episode of headache unspecified was 51 percent (50% in males and 52% in females). The crude prevalence ratio of migrainous headache was 5.3 per 100 (5 per 100 in males and 5.6 per 100 in females), with peak age-specific prevalence ratios in the first decade in both males and females. Migrainous headache was three times as common in females as in males in the second and third decades. Prevalence of migrainous headache in Nigerian Africans appears less than in Caucasians. No social status was at special risk to developing migrainous headache.

[Cerebrovascular disorders in young adults in Saudi Arabia. A study of 136 cases]. / Accidents vasculaires cérébraux des adultes jeunes en Arabie Saoudite. Etude de 136 cas.

We report our experience of stroke in 136 young adults aged from 18 to 45 years seen in the Eastern Province of Saudi Arabia over a 10-year period. They constituted 25% of all our stroke cases. Thirty-eight percent were Saudi nationals and 62% expatriates. Males largely outnumbered females. The frequency of cerebral infarction (54%) was not very different from that of intracranial hemorrhage (45%). Atherosclerosis and embolism of cardiac origin were the major causes of infarction. The main causes of intracranial bleeding were arterial aneurysms, arteriovenous malformations and hypertension. However, the causes of 29% of ischemic strokes and 44% of hemorrhagic ones remained undetermined. Interethnic comparison of the causes showed that hemorrhages were significantly more frequent in Far East immigrants. Sixty five percent of cerebral infarctions in Asiatic patients remained of undetermined origin. The local variant of sickle cell gene did not seem to play a major role in the pathogenesis of stroke in the Saudi young adult. These data are commented and compared with similar reported data. The influence of the demographic structures of the Saudi population and immigrants communities is analyzed.

Aetiology of status epilepticus in Ibadan: a neuropathologic study.

In this neuropathologic study of 41 cases diagnosed as status epilepticus (SE) over a 10-year period at the University College Hospital, Ibadan (UCH), we found that the commonest cause was infection of the central nervous system (17 cases). The other aetiologic factors were: metabolic derrangement/toxic (14 cases) and cerebrovascular disease which was the most frequent cause in subjects above 12 years of age. It was encountered in 6 cases. Space occupying lesions which involved the frontal lobes were found in 4 cases. The conditions associated with the disease at death were: cerebral oedema, pulmonary oedema; pulmonary consolidation and pulmonary embolism. Our findings highlight the importance of looking for treatable conditions in patients presenting with this grave condition in this environment.

Diseases of peripheral nerves as seen in the Nigerian African.

The anatomical and aetiological diagnoses of peripheral nerve disease excluding its primary benign and malignant disorders, as seen in 358 Nigerians are presented. There is a male preponderance and the peak incidence is in the fourth decade. Sensori-motor neuropathy was the commonest presentation (50%). Guillain-Barré syndrome was the commonest identifiable cause (15.6%), accounting for half of the cases with motor neuropathy. Peripheral neuropathy due to nutritional deficiency of thiamine and riboflavin was common (10.1%) and presented mainly as sensory and sensori-motor neuropathy. Diabetes mellitus was the major cause of autonomic neuropathy. Isoniazid was the most frequent agent in drug-induced neuropathy. Migraine (20%) was not an uncommon cause of cranial neuropathy although malignancies arising from the reticuloendothelial system or related structures of the head and neck were more frequent (26%). In 26.5% of all the cases, the aetiology of the neuropathy was undetermined. Heredofamilial and connective tissue disorders were rare. Some of the factors related to the clinical presentation and pathogenesis of the neuropathies are briefly discussed.

Clinical study of unilateral peripheral facial nerve paralysis in Nigerians.

The clinical feature of isolated unilateral peripheral facial nerve paralysis (PFP), seen in 153 consecutive Nigerians over a 14-year period at the University College Hospital (UCH), Ibadan, are presented. The hospital incidence rate was 2.67 per 10,000 with a mean annual rate of 11 per 100,000. Although males (61%) were more frequently affected than females (39%), the peak incidence for both sexes was in the third decade, and 53% of the cases were between 20 years old and 39 years old. Bell's palsy (ninety-three cases) was the most common type encountered. Hypertension (eleven cases) was associated with PFP only in patients above 50 years old. Herpes zoster infection (six cases) and otogenous (eight patients) were not uncommon. Although conjunctivitis (8%) was the most frequent complication, post-paralytic motor features in the form of synkinesia (eight cases), hemispasmas or contractures, and autonomic disturbances such as the crocodile-tear phenomenon (three cases) and auriculo-temporal syndrome (one case) were rare.

Werdnig Hoffman's disease (spinal muscular atrophy type I): A clinical study of 25 Saudi nationals in Al-Khobar.

We describe the clinical features of 25 cases of Werdnig Hoffman's disease (spinal muscular atrophy (SMA) type I) seen propectively over a two-year period at the King Fahd Hospital of the University (KFHU), Al-Khobar. The hospital incidence rate was 1.93 per 1,000 live births (95% confidence limits, 0.80-3.06/1,000). The estimated prevalence rate for the community was 0.92/10,000 with 0.59-1.25 per 10,000 children as its 95% confidence limits. The male to female ratio was 2:3. Reduced fetal movements were reported by six mothers; 8 children (32%) had symptoms at birth, and 24 (96%) had symptoms by the time they were six months old. Other features apart from hypotonia, muscle weakness, and absent deep tendon reflexes included head lag with inability to achive head control at six months (88%), respiratory problems consisting of difficulty with breathing or frequent chest infections (44%), and difficulty with feeding (40%). Wasting with fisciculations of the tongue was seen in 64%. Death occurred within six months of presentation in 75% of the cases. The parents were consanguineous in 64% of the cases. This high consanguinity rate was probably the major cause for the high population prevalence rate.

Pattern of degenerative ataxias in the eastern province of Saudi Arabia.

The patterns of degenerative ataxias as seen over a six year period at the King Fahad Hospital of the University, Al Khobar in the Eastern Province of Saudi Arabia are reported. Twenty two cases were seen, giving a hospital frequency of 7/100,000 patients. Early onset ataxias were the most common, particularly Friedreich disease (9/22). Uncommon types such as ataxia with hypogonadotropic hypogonadism and ataxia with oculomotor apraxia were encountered. There were six late onset ataxias: "pure cerebellar" ataxias were the most common. These data are discussed and compared with other reports, keeping in mind that these disorders are evelotive and their nosologic patterns can change with their progression.

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.