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Biomedical subjects

R A Shakir

Publications and source records attributed to R A Shakir.

At least 19 recordsLinked to original sources
PubMed1992

Progressive myoclonic ataxia without ragged red fibres: Unverricht-Lundborg disease vs Ramsay Hunt syndrome.

We describe eight patients from three families presenting with myoclonus, ataxia, infrequent seizures and minimal intellectual impairment. All were Arabs from different parts of the Arabian peninsula. The new consensus on terminology, genetic and clinical definition of Baltic myoclonus, Ramsay Hunt syndrome and Unverricht-Lundborg disease suggests that our group are best categorised under the term of progressive myoclonic ataxia of the Unverricht-Lundborg type. Moreover, this report reinforces the existence of this syndrome outside Scandinavia.

Adolescent↗
PubMed1991

Congenital malformations before and after the onset of maternal epilepsy.

A total of 222 pregnancies in 56 mothers were studied, 48 pregnancies occurred in 17 mothers before the onset of epilepsy (Group 1). The same group went on to develop epilepsy, receive anticonvulsants and further conceive 43 pregnancies (Group 1a). In the other 39 mothers 131 pregnancies occurred after the onset of epilepsy and while receiving anticonvulsants (Group 2). The incidence of major congenital malformations among live births was significantly higher in Group 2 (14.4%) when compared with all pregnancies in Groups 1 and 1a (1.2%), P less than 0.025 and in Group 1 alone (2.2%) P less than 0.05. There was no significant difference between Groups 1 and Group 1a. Age at first pregnancy in Group 1 was 18.9 years +/- 3.7 SD, which was significantly younger than Group 2, 21.7 years +/- 5.1 SD, p less than 0.05. The age of onset of epilepsy was significantly younger in Group 2 than in Group 1, 15.1 years +/- 6 SD vs 23.3 years +/- 4.6 SD, p less than 0.001. The duration of epilepsy was significantly longer in Group 2 compared with Group 1, 13.8 years +/- 8.7 SD vs 6.1 years +/- 2.6 SD, p less than 0.001. Patients on polytherapy had a significantly higher risk of major malformations compared to those on monotherapy, 20.2% vs 1.3%, p less than 0.001. Our data suggest that epileptic mothers with the highest risk of having offspring with major malformations were those with a long duration of epilepsy combined with exposure to multiple anticonvulsant drugs.

Abnormalities, Drug-Induced↗
PubMed1990

Brainstem auditory evoked potentials in Wilson's disease.

Twelve patients with Wilson's disease, aged 11-25 years, underwent brainstem auditory evoked potential (BAEP) study. The results were correlated to clinical, neuroradiological and laboratory data. Ten had prominent to severe neurological manifestations, suggestive of involvement of one or several CNS structures, and 2 were neurologically free. All had evidence of abnormal copper metabolism, and 8 had CT scan evidence of brain atrophy, or hypodense areas in basal ganglia, or both. The 2 patients without neurological manifestations as well as one with neurological signs had normal BAEP. One patient with neurological signs had increased N1 latency due to cochlear hearing loss, but normal interpeak intervals, while 8 of 10 patients with prominent neurological symptoms and signs had abnormal BAEPs (prolongation of NIII-NV interpeak interval). The value of NIII-NV interpeak interval correlated with the number of different neurological signs (neurological score) attributable to involvement of different CNS structures (r = 0.64 at P = 0.001). Abnormal BAEPs do not seem to be an early finding in Wilson's disease.

Adolescent↗
PubMed1990

Training of Kuwaiti medical doctors and their specialty choices: sociocultural impact.

Kuwait has a comprehensive and well-structured health care system. However, to deliver such care the country relies on expatriate medical doctors. This paper attempts to discuss the sociocultural influences on the training of Kuwaiti medical doctors and their specialty choices in order to gauge future requirements of the Kuwaiti Health Services.

Career Choice↗
PubMed1990

Neurological presentation of neuro-Behçet's syndrome: clinical categories.

In an attempt to categorize the diverse neurological manifestations of Behçet's syndrome, a group of 10 patients was prospectively studied; the diagnosis of Behçet's syndrome was not known in any of them prior to their neurological presentation. A reasonably clear distinction can be made to divide the manifestations into three general categories with some overlap. Category I includes patients with increased intracranial pressure with or without cerebral venous sinus thrombosis. Category II are those presenting with a stroke. Category III are those patients who present with spinal cord involvement and CSF pleocytosis. Brainstem involvement was noted independently of the group. In general, patients with Neuro-Behçet's syndrome showed little ocular involvement. The course of the illness both in morbidity and mortality tended to be better in category I, and mortality was only seen in category II patients. In populations susceptible to the disease, the clinical categorisation proposed may alert the clinician to the possibility of neuro-Behçet's syndrome whenever patients with pseudotumour cerebri, dural sinus thrombosis, stroke in the young, unexplained myelitis or possible multiple sclerosis are encountered.

Behcet Syndrome↗
PubMed1988

Mollaret's meningitis. A variant of recurrent hereditary polyserositis, both provoked by metaraminol.

Mollaret's meningitis is a rare condition with a characteristic clinical and cerebrospinal fluid picture. In many ways it resembles recurrent hereditary polyserositis (familial Mediterranean fever) in its natural history, pattern of attacks, and response to colchicine. Association of the two conditions has been reported, so far, in two patients only. In our patient the symptoms of both conditions were induced by a metaraminol provocative infusion. We have previously introduced this as a specific diagnostic and confirmatory test for recurrent hereditary polyserositis. The possibility that the two conditions represent different manifestations of a single disease is therefore strengthened.

Adolescent↗
PubMed1988

Brainstem auditory evoked potential (BAEP) abnormalities in brucellosis.

Twelve patients with neurobrucellosis and 17 patients with systemic brucellosis without neurological involvement underwent a brainstem auditory evoked potentials (BAEP) study. All neurobrucellosis patients (100%) showed abnormalities in their BAEP recordings, suggestive of brainstem lesions at various levels. On the other hand, only 5 (29%) of the 17 patients with systemic brucellosis had mild unilateral BAEP abnormalities, while the remaining 12 had normal responses. Comparison of pooled data between the systemic brucellosis and neurobrucellosis groups showed highly significant differences in all BAEP parameters. The recording of BAEP is thus considered a sensitive supplementary method to reveal CNS lesions in patients with neurobrucellosis.

Adult↗
PubMed1988

ELISA versus routine tests in the diagnosis of patients with systemic and neurobrucellosis.

Sera from patients in different stages of brucellosis as well as sera and cerebrospinal fluid (CSF) from patients with central nervous system (CNS) brucellosis and controls, were tested by ELISA for Brucella-specific IgG, IgM and IgA. The results were compared with culture findings, micro-agglutination (MA), slide agglutination with Rose Bengal (RB), and Brucella melitensis stained antigens (SA). In sera of patients with acute brucellosis (296), ELISA was positive for IgM (100%), IgG (97%) and IgA (98%), and comparable results were found in sera of patients with subacute brucellosis (44): IgG (100%), IgM (86%) and IgA (100%). However, in patients with chronic brucellosis (40), IgG and IgA were consistently positive (100%) while IgM was only positive in 33% of their sera. The MA and RB showed similar results, being more positive in patients with acute (98%) and subacute (84%) than in chronic (61%) brucellosis. The SA and culture showed significantly lower positive results. In the CSF of patients with CNS brucellosis (45), ELISA was positive in 100%, 20% and 85% for IgG, IgM and IgA, respectively, compared to 13% positive by culture, 25% by MA and 22% by RB. ELISA was negative in the CSF specimens from patients with brucellosis without CNS involvement (66), or meningitis other than Brucella (62), and no meningitis (144). Thus, ELISA with its IgG, IgM and IgA profiles is the test of choice in the diagnosis of patients with brucellosis, especially those with chronic or CNS infection.

Adolescent↗
PubMed1987

Surgery in combined pulmonary and neural cryptococcosis.

A 39-year-old previously healthy patient with combined pulmonary and neural cryptococcosis was successfully managed by pulmonary resection and antifungal chemotherapy. The pulmonary mass was removed under cover of miconazole and 5-fluorocytosine even though the patient had concomitant active meningitis. He recovered dramatically after the surgical procedure and remained well 2 years later. We suggest that, as in our patient, early removal of a pulmonary cryptococcal mass under antifungal cover may in other similar patients obviate the need for protracted antifungal therapy and lead to an excellent response of the neural cryptococcosis.

Adult↗
PubMed1987

Clinical categories of neurobrucellosis. A report on 19 cases.

Brucellosis rarely can present with involvement restricted to the nervous system. We describe a total of 19 cases of neurobrucellosis in whom the clinical presentation lay in three distinct categories. The first was an acute presentation with meningoencephalitis. The disease also presented in a chronic form where the brunt of the illness can either be in the peripheral or the central nervous system (CNS). The chronic peripheral form is that of a proximal polyradiculoneuropathy. The central form is that of diffuse CNS involvement, predominantly with myelitis or cerebellar involvement with or without cranial nerve palsies. Although the two chronic forms, 'peripheral' and 'central', are distinct, some overlap is possible. This was not observed for the acute form. The pathology of the three presentations may be different, being a direct effect of infection in the acute form, and an immune-related process, possibly demyelinating in nature, in the chronic forms. The response to treatment in the acute and chronic forms is also different, being much better in the acute form. Awareness of the condition and performance of the appropriate serological tests will differentiate neurobrucellosis from other chronic CNS infections, especially tuberculosis and neurosyphilis.

Adolescent↗
PubMed1986

Rapid diagnosis of central nervous system brucellosis by ELISA.

The diagnosis of central nervous system (CNS) brucellosis was made by determining Brucella-specific IgG, IgM and IgA in cerebrospinal fluid (CSF) specimens, using an enzyme-linked immunosorbent assay (ELISA) method. A total of 68 CSF specimens including 10 from patients with brucellosis of the nervous system, 4 with Brucella infection but without CNS involvement, 38 with meningitis other than due to Brucella and 16 with no meningitis were studied. Of the 10 CSF specimens from patients with CNS brucellosis, Brucella-specific IgG was detected in all 10, IgM in 7 and IgA in 8. The 10 patients were also seropositive for anti-Brucella IgG and IgM, and 9 were seropositive for IgA. No Brucella-specific IgG, IgM or IgA was detected in the CSF of 4 patients with brucellosis but without CNS involvement, even though the blood of these patients had high titres of anti-Brucella IgG, IgM and IgA. In addition, none of the CSF specimens from the 38 patients with meningitis other than due to Brucella, or from the 16 patients without meningitis, had detectable anti-Brucella antibodies. This study indicates that the Brucella ELISA on CSF is a reliable, rapid, sensitive and specific assay in the diagnosis of CNS brucellosis.

Adolescent↗
PubMed1986

Neurobrucellosis.

Explore the source record for details and available documents.

Anti-Bacterial Agents↗
PubMed1986

Internal carotid artery occlusion in a young female with mitral valve prolapse.

A healthy 28 year old housewife presented with sudden right hemiplegia and aphasia. No predisposing factors could be ascertained apart from posterior cusp mitral value prolapse on two dimensional echocardiography. Extensive investigations confirmed the presence of cerebral infarction and persistent occlusion of the left internal carotid artery near its origin.

Adult↗