Random total antiepileptic drug levels and seizure control during pregnancy.

OBJECTIVE: To study the correlation of randomly-tested total antiepileptic plasma levels and seizure control in a retrospectively collected group of pregnant epileptic Saudi women. METHODS: The medical records of 30 Saudi epileptic female patients were reviewed during their subsequent pregnancies (total of 50). The type of antiepileptic drugs used during each pregnancy, the dose of each drug and the corresponding total plasma levels were noted. Antiepileptic drugs assay were carried out randomly during pregnancy either by TDX or a sensitive high performance liquid chromatography method. RESULTS: A total of 50 pregnancies were studied. The most common seizure type in these women was complex partial seizure followed by primary generalized epilepsy, myoclonic seizures and of least occurrence was the simple partial seizure with secondary generalization. All patients were received antiepileptic drugs, including either carbamazepine, phenytoin, valporic acid, phenobarbitone or clonazepam. In a total of 24 pregnancies (48%), the serum levels of antiepileptic drugs were subtherapeutic during the first trimester. Recurrent seizures occurred in a total of 20 pregnancies (40%) especially in the 3rd trimester. CONCLUSION: It is concluded that subtherapeutic serum levels of antiepileptic drugs correlated highly with the increased frequency of seizure in these pregnant women. Monitoring of state of seizure control in epileptic pregnant women should be made regularly during the course of their pregnancies.

Random total antiepilectic drug levels and seizure control during pregnancy.

OBJECTIVE: To study the correlation of randomly-tested total antiepileptic plasma levels and seizure control in a retrospectively collected group of pregnant epileptic Saudi women. METHODS: The medical records of 30 Saudi epileptic female patients were reviewed during their subsequent pregnancies (total of 50). The type of antiepileptic drugs used during each pregnancy, the dose of each drug and the corresponding total plasma levels were noted. Antiepileptic drugs assay were carried out randomly during pregnancy either by TDX or a sensitive high performance liquid chromatography method. RESULTS: A total of 50 pregnancies were studied. The most common seizure type in these women was complex partial seizure followed by primary generalized epilepsy, myoclonic seizures and of least occurrence was the simple partial seizure with secondary generalization. All patients were received antiepileptic drugs, including either carbamazepine, phenytoin, valporic acid, phenobarbitone or clonazepam. In a total of 24 pregnancies (48%), the serum levels of antiepileptic drugs were subtherapeutic during the first trimester. Recurrent seizures occurred in a total of 20 pregnancies (40%) especially in the 3rd trimester. CONCLUSION: It is concluded that subtherapeutic serum levels of antiepileptic drugs correlated highly with the increased frequency of seizure in these pregnant women. Monitoring of state of seizure control in epileptic pregnant women should be made regularly during the course of their pregnancies.

Parkinson's disease. Clinical and electrophysiological evaluation.

OBJECTIVES: Identification of the clinical spectrum and the electrophysiological responses of a Saudi population with Parkinson's disease as opposed to a matched normal population. METHODS: Fifty four subjects (41 males and 13 females) were selected for the study. The patients were clinically evaluated for the occurrence of Parkinson's disease symptoms, as well as other associated medical conditions. All patients had brain computerized tomography scans. Electrophysiological tests were performed on all patients using the Medelec ST 10 Sensor 59394 Model. These tests included somatosensory evoked response of median nerves, brain stem auditory evoked responses and visual evoked responses. The significant differences in these evoked responses between the patients with Parkinson's disease and normal patients were statistically evaluated. RESULTS: Twenty six out of the 40 computerized tomography brain scans which had been carried out showed normal brain morphology and 5 had a clear evidence of cerebrovascular disease while only 9 showed distinctive brain atrophy. The mean values for the brain stem auditory evoked response, the somatosensory evoked response and the visual evoked responses were higher in patients with Parkinson's disease as compared to those who did not have the disease. Significant differences were only seen as prolonged latencies in median nerve somatosensory evoked response, as well as delayed waves I and V on the brain stem auditory evoked response. Inter-wave latencies, however, were not significantly different. CONCLUSION: Parkinson's disease in a Saudi population showed significant differences to somatosensory evoked response and brain stem auditory evoked response electrophysiological data as compared to age-matched controls, however, the clinical characteristics of Parkinson's disease in Saudi patients are not significantly different from those reported for patients elsewhere.

Tumor-like presentation of multiple sclerosis.

Multiple sclerosis patients may present with clinical data suggestive of cerebral tumor, however, most of the lesions do not show expansive signs in computerized tomography of brain or magnetic resonance imaging. We report in this paper, 2 patients who had shown expansive radiological signs suggestive of neoplasm. Cerebral biopsy was an important diagnostic procedure in these 2 cases which revealed the diagnosis of demyelinating disease.

Parkinson`s disease. Clinical and electrophysiology evaluation.

OBJECTIVE: Identification of the clinical spectrum and the electrophysiological responses of a Saudi population with Parkinson`s disease as opposed to a matched normal population. METHODS: Fifty four subjects (41 males and 13 females) were selected for the study. The patients were clinically evaluated for the occurrence of Parkinson`s disease symptoms, as well as other associated medical conditions. All patients had brain computerized tomography scans. Electrophysiological tests were performed on all patients using the Medelec ST 10 Sensor 59394 Model. These tests included somatosensory evoked response of median nerves, brain stem auditory evoked responses and visual evoked responses. The significant differences in these evoked responses between the patients with Parkinson`s disease and normal patients were statistically evaluated. RESULTS: Twenty six out of the 40 computerized tomography brain scans which had been carried out showed normal brain morphology and 5 had a clear evidence of cerebrovascular disease while only 9 showed distinctive brain atrophy. The mean values for the brain stem auditory evoked response, the somatosensory evoked response and the visual evoked responses were higher in patients with Parkinson`s disease as compared to those who did not have the disease. Significant differences were only seen as prolonged latencies in median nerve somatosensory evoked response, as well as delayed waves I and V on the brain stem auditory evoked response. Inter-wave latencies, however, were not significantly different. CONCLUSION: Parkinson`s disease in a Saudi population showed significant differences to somatosensory evoked response and brain stem auditory evoked response electrophysiological data as compared to age-matched controls, however, the clinical characteristics of Parkinson`s disease in Saudi patients are not significantly different from those reported for patients elsewhere.

Outcome of pregnancies in epileptic women: a study in Saudi Arabia.

We studied the outcome of 79 pregnancies in 44 Saudi women who had epilepsy. Their mean age was 28+/-6.5 years and the number of pregnancies studied varied from one to six. Nineteen subjects had generalized seizures, 16 had partial seizures and nine were unclassified. The commonest drug prescribed was carbamazepine and the majority of the women (61%) were on monotherapy. The seizures were controlled in 53 pregnancies (67%). Spontaneous vertex deliveries were the commonest. The indications for intervention by lower segment Caesarean section, forceps or ventouse were foetal distress, pre-eclamptic toxaemia (PET), eclampsia, breech presentation and prolonged labour. The most frequent adverse outcome in the babies was low birth weight (<2.5 kg) in nine pregnancies. The frequency of congenital malformation was 2.5%. Low birth weight was associated with prematurity, PET, congenital malformation and polytherapy. Avoidance of polytherapy appears to be the most feasible intervention in reducing the frequency of low birth-weight children by epileptic mothers.

Motor terminal latency index in carpal tunnel syndrome.

We determined the motor terminal latency index (MTLI) of the median nerve across the carpal tunnel in 41 upper extremities of 31 patients with carpal tunnel syndrome. Changes in motor nerve conduction velocity (MNCV), motor terminal latency (MTL), sensory action potential and the amplitude of the compound muscle action potential recorded from the abductor pollicis brevis muscle were all suggestive of proximal and distal segment involvement of the nerve across the carpal tunnel. There was no correlation between forearm MNCV and MTL (r = 0.40), although MTLI was correlated with MTL (r = 0.67) but not with MNCV, indicating a disproportionate conduction across the carpal tunnel.

Terminal Latency Index: A distinctive diagnostic criterion for carpal tunnel syndrome.

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Motor terminal latency index in carpal tunnel syndrome

We determined the motor terminal latency index [MTLI] of the median nerve across the carpal tunnel in 41 upper extremities of 31 patients with carpal tunnel syndrome. Changes in motor nerve conduction velocity [MNCV], motor terminal latency [MTL], sensory action potential and the amplitude of the compound muscle action potential recorded from the abductor pollicis brevis muscle were all suggestive of proximal and distal segment involvement of the nerve across the carpal tunnel. There was no correlation between forearm MNCV and MTL [r = 0.40], although MTLI was correlated with MTL [r = 0.67] but not with MNCV, indicating a disproportionate conduction across the carpal tunnel

Dementia in Saudi Arabia: experience from a university hospital.

OBJECTIVES: To describe the pattern of presentation, the types of dementia and the associated conditions in Saudi patients. MATERIALS AND METHODS: Hospital-based study using DSM-IV and ICD 10 criteria for consensus diagnosis of cases from clinical information and results of investigations. Dementia subtypes were made according to NINCDS-ADRDA, NINDS-AIREN and ICD 10 criteria while CDR was used for severity grading. RESULTS: A total of 77 demented patients (49 males, 28 females) were studied. The hospital frequency was 19.3/100,000 patients. The mean age at presentation was 74.6 years and age at onset was below 65 years in 17 patients. The types of dementia were: Alzheimer's disease (51.9%), vascular dementia (18.2%), mixed cases (15.6%), dementia with Parkinson's disease (7.8%) and treatable dementia (5.2%). Only 3 patients were in the severe clinical stage and infections were important causes of deterioration. CONCLUSION: The hospital frequency appears to be low probably because of the relatively young population. The pattern of dementia with preponderance of AD is similar to that in western countries and intervention directed at the risk factors for stroke could reduce the burden of vascular dementia.

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.

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.

Epilepsy classification and factors associated with control in Saudi adult patients.

There is insufficient information on the epilepsies in Saudi Arabia. The objectives were to classify the patients according to seizure types and epilepsies as well as to determine the factors associated with control. In a hospital-based study, clinical information, electroencephalographic and neuroimaging findings were utilized to classify the cases into seizure types and epilepsies according to ILAE criteria and to determine the factors statistically associated with control. In the study there were 826 patients (454 males and 372 females; mean age = 28.7 years; >80% below 30 years at onset). The seizure types were: generalized tonic-clonic (43.8%), partial seizure secondarily generalized (41.9%), myoclonic (8.4%), simple partial (1.3%), complex partial (1.3%) and absence (0.4%). About 15% of the classifiable epilepsies were symptomatic. Most symptomatic epilepsies occurred in people over the age of 50 years. One-year remission rate was 80% and the factors associated with control were: compliance, monotherapy, therapeutic drug level and seizure type. The results showed that epilepsy predominantly affected young adults and confirmed the association of partial epilepsy with clinical and CT abnormalities. The 1-year remission rate was comparable with reports from other studies as well as the factors associated with control in our patients.

Severe hyponatremia as poor prognostic factor in childhood neurologic diseases.

We determined the duration of hospital stay and neurologic sequelae in 72 patients divided into three groups according to the serum sodium level on admission. The mean duration of stay in those with normal sodium (Group I) was 18 days compared with 37 and 69 days for those with mild (Group II) and moderate to severe deficits (Group III) respectively. The differences were statistically significant (F=327.2, P<0.01). Thirty-one out of the 35 patients in Group I recovered fully; all the Group II subjects had residual deficit and all the 14 patients in Group III were either left with severe deficit (10) or died (4). Pediatric neurological cases having severe hyponatremia should be considered as a high risk group for poor outcome in spite of cautious correction.

Incidental cerebral venous thrombosis in a patient with multiple sclerosis.

Cerebral venous thrombosis (CVT) is rarely reported in patients with multiple sclerosis. We recently managed a 32 year-old Saudi female patient who was managed for a relapse of her multiple sclerosis but in whom CVT was incidentally found in the course of investigations. She was asymptomatic with regards to the CVT and she had no identifiable predisposing factor apart from her recent confinement which was uneventful. The case underscores the importance of looking for other lesions that may be 'clinically silent' in MS patients presenting with relapse as well as emphasising the advantage of neuroimaging in revealing unsuspected but potentially fatal disorders.

Consanguineous marriages in a Saudi population and the effect of inbreeding on prenatal and postnatal mortality.

Consanguineous marriages are strongly favoured in the Saudi population. A population-based study of consanguineous marriages was conducted in the Riyadh area. The prevalence rate of consanguineous marriages was 51.3% with an average inbreeding coefficient of 0.02265, which is high compared with many other countries. The most important variables affecting inbreeding were the regional background of the family (p < 0.001) and the level of education, which was inversely associated with consanguineous marriage (p < 0.001). Perinatal and postnatal mortalities were not significantly different between consanguineous and non-consanguineous families.

PIP: A total of 2001 married Saudis living in Riyadh (1365 women and 636 men aged 20-45 years) were included in this study carried out between January and June 1993. The proportion of subjects surveyed was based on the population of each district: North (19.6%), East (39.6%), South (20.6%), and West (20.1%). All eligible subjects were interviewed using a structured interview format in the district primary health centers and antenatal clinics. Data were collected on types of marriages and relationships of spouses, region of origin, the level of completed education, and fertility. Consanguinity was found in 1022 of the marriages (51.3%). Marriages between second cousins or closer relatives accounted for 41.1% of all unions. In another 10% of the marriages the women were married to a relative beyond the level of second cousin. The mean coefficient of inbreeding was 0.02265. 364 (63.0%) of 578 subjects who were either illiterate or had completed primary education only were married to a relative, compared with 658 (46%) of 1423 who had acquired higher education. The odds ratio was 1.98 (p 0.0001). There was a significant association between the level of education and consanguinity (p 0.001). The proportion of subjects married to relatives was highest in those from the southern region of the kingdom (69%) followed by those from the northern region (60%). The lowest frequency of consanguinity was found among those from the eastern region (41.5%). The abortion/miscarriage rate among women married to a second cousin or closer relative was 6.9% compared with 7.4% for nonconsanguineous marriages. The stillbirth rate was also comparable. Total prenatal losses were essentially the same among consanguineous (8.3%) and nonconsanguineous couples (8.9%). The rate of neonatal death and total postnatal fatality was 2.7% for consanguineous families and 2.2% for the nonconsanguineous group. Education programs should be established to increase awareness of the consequences of consanguineous mating, but without offending age-old traditional practices with their perceived social benefits. Genetic counseling and prenatal diagnosis should also be available for consanguineous couples.

Centro-temporal benign epilepsy in Saudi children.

A review of 36 interictal EEGs compatible with the diagnosis of benign childhood epilepsy with centro-temporal spikes (BECTS) was made in this study. These children constituted 31% of the children with focal epileptic EEG activity observed in the same period. The dipolic spikes occurred either as a single focus (78%) or as two independent foci with a greater preponderance to the right (22%). The onset of seizures before the age of 5 years (Group I) is 53% and between 6 and 10 years (Group II) is 47%. Clinical evaluation showed that 60% of the patients in Group I and 75% in Group II had lateralized seizures, although all of them were initially diagnosed as nocturnal generalized tonic-clonic seizures. A follow-up study showed 31% complete recovery, 47% seizure-free under medications, 8% occasional seizures, 8% frequent seizures, and the rest 6% had a single seizure without medication. It is therefore concluded that the syndrome is common in Saudi Arabia and is usually unrecognized by the general physicians. The follow-up of our patients so far confirms the excellent prognosis.

Clinical and molecular pathological features of severe childhood autosomal recessive muscular dystrophy in Saudi Arabia.

The clinical, biochemical and histochemical features of 14 patients (nine females and five males) with severe childhood autosomal recessive muscular dystrophy (SCARMD) seen at a tertiary hospital in Riyadh from 1982 to 1993 are described. Onset was at 3 to 9 (median 3) years and four of five children aged > 12 years lost ambulation. Five of the eight pairs of parents were closely consanguineous. The mean creatine kinase was 20 times the upper normal limit. Histochemistry of muscle showed dystrophic features in all cases, and dystrophin was positive in all cases examined (N = 6). Three patients (two girls and a boy) were deficient in adhalin, the 50-kDa dystorphin-associated glycoprotein. A boy aged 13 years had rapidly progressing disease. Another boy of the same age (from a family characterized by early onset and slower progression) had normal dystrophin and adhalin. The clinical features conformed with previous observations from Sudan, North Africa and Qatar in the Arabian Peninsula. The disease is common in Saudi Arabia and seems to be more prevalent than Duchenne muscular dystrophy.

Binswanger disease: The King Khalid University Hospital experience.

Two hundred and twenty-one Saudi patients admitted for stroke in King Khalid University Hospital between 1982 and 1987 were evaluated clinically and by laboratory and radiological investigations. Twelve patients were found to have leukoraiosis on brain CT and a clinical picture compatible with Binswanger disease (subcortical arteriosclerotic encephalopathy). Arterial hypertension was present in all cases, seizure disorders in 25%, and dementia in 83%. The features of these cases are compared with similar cases reported from other places. The importance of control of hypertension in prevention of Binswanger disease is emphasized.