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Biomedical subjects

A Mrabet

Publications and source records attributed to A Mrabet.

At least 19 recordsLinked to original sources
PubMed2006

[Presentation of a cavernous angioma with an oculomotor nuclear syndrome: a case report].

INTRODUCTION: The nuclear syndrome of the oculomotor nerve, first described in 1981, is characterized by ipsilateral third nerve palsy and elevation paresis of the contralateral eye. CASE REPORT: A 59-year-old man suddenly developed diplopia and headache. Neurological examination showed nuclear ophthalmoplegia of the left third nerve without any other neurological abnormality. MRI findings demonstrated a typical, popcorn-like, smoothly circumscribed left posterior lesion. Surgical resection was not performed because of the deep localization. Spontaneous bleeding developed. CONCLUSION: This neuro-ophthalmological complication of a cavernous angioma, as seen in our patient, has rarely been described in the literature. The clinical presentation and anatomic elements of the nuclear oculomotor syndrome are discussed. This exceptional localization provides a useful illustration of anatomoclinical correlations.

Brain↗
PubMed2005

[Dyke-Davidoff-Masson syndrome: a report of two cases].

Dyke-Davidoff-Masson syndrome is a condition characterized by seizures, facial asymmetry, contralateral hemiplegia or hemiparesis and mental retardation. We report the clinical and imaging features in two patients with epilepsy revealing a Dyke-Davidoff-Masson syndrome. Brain MRI showed unilateral loss of cerebral volume with hypertrophy and hyperpneumatization of the paranasal sinuses and mastoid cells. Ipsilateral fronto-parietal polymicrogyria was present in one patient.

Adolescent↗
PubMed2003

Mutations in MTMR13, a new pseudophosphatase homologue of MTMR2 and Sbf1, in two families with an autosomal recessive demyelinating form of Charcot-Marie-Tooth disease associated with early-onset glaucoma.

Charcot-Marie-Tooth disease (CMT) with autosomal recessive (AR) inheritance is a heterogeneous group of inherited motor and sensory neuropathies. In some families from Japan and Brazil, a demyelinating CMT, mainly characterized by the presence of myelin outfoldings on nerve biopsies, cosegregated as an autosomal recessive trait with early-onset glaucoma. We identified two such large consanguineous families from Tunisia and Morocco with ages at onset ranging from 2 to 15 years. We mapped this syndrome to chromosome 11p15, in a 4.6-cM region overlapping the locus for an isolated demyelinating ARCMT (CMT4B2). In these two families, we identified two different nonsense mutations in the myotubularin-related 13 gene, MTMR13. The MTMR protein family includes proteins with a phosphoinositide phosphatase activity, as well as proteins in which key catalytic residues are missing and that are thus called "pseudophosphatases." MTM1, the first identified member of this family, and MTMR2 are responsible for X-linked myotubular myopathy and Charcot-Marie-Tooth disease type 4B1, an isolated peripheral neuropathy with myelin outfoldings, respectively. Both encode active phosphatases. It is striking to note that mutations in MTMR13 also cause peripheral neuropathy with myelin outfoldings, although it belongs to a pseudophosphatase subgroup, since its closest homologue is MTMR5/Sbf1. This is the first human disease caused by mutation in a pseudophosphatase, emphasizing the important function of these putatively inactive enzymes. MTMR13 may be important for the development of both the peripheral nerves and the trabeculum meshwork, which permits the outflow of the aqueous humor. Both of these tissues have the same embryonic origin.

Adolescent↗
PubMed2003

[A new case report of spinal cord compression secondary to beta-thalassemia].

Extramedullary hematopoiesis is an unusual cause of spinal cord compression. We report the case of a 39-year-old man who presented symptoms of progressive dorsal spinal cord compression. Hemogramm showed erythrocytosis with microcytosis. Hemoglobin electrophoresis revealed heterozygous beta-thalassemia. Diagnosis of extramedullary hematopoiesis was based on magnetic resonance imaging findings. Radiological and therapeutic options in this rare condition are briefly discussed.

Adult↗
PubMed2003

[Cerebral tuberculomas].

We report eight cases of brain tuberculoma. The clinical presentation was polymorphous: partial epilepsy (n=4), headache (n=3), hemiplegia (n=1), meningitis (n=1), cerebellar syndrome (n=1). Six patients also had pulmonary tuberculosis, one had tuberculosis of the genital organs, and one had HIV co-infection. The brain CT scan and MRI were highly contributive to diagnosis and follow-up. Despite good compliance with an anti-tuberculosis regimen for at least 14 months, the course was favorable in only six patients. Adjunction of corticosteroids led to radiological improvement. Assessment of cell immunity demonstrated a diminished immunomodulator ratio. Cerebral tuberculoma should be searched for in patients with unexplained neurological manifestations and several intracerebral lesions, particularly if pulmonary or visceral tuberculosis and/or immunodepression is part of the clinical picture.

Adrenal Cortex Hormones↗
PubMed1999

[Neurological manifestations indicative of brucellosis].

Eight patients presented neurological signs secondary to Brucella infection. The clinical presentation was a meningoencephalitis in three cases, a meningoencephalomyelitis in one case, an epiduritis with spinal cord compression in one case, an acute polyradiculoneuritis in two cases and a chronic polyradiculoneuritis in one case. Acoustic nerve was impaired in seven cases. Cerebrospinal fluid (CSF) analysis revealed a lymphocytic meningitis and a high protein concentration in all cases. The agglutination test titers were elevated in the serum and in the CSF of seven patients (> or = 1/80) and two patients respectively. Brucella melitensis culture was disclosed in the blood of one patient and in the CSF of two patients. Three patients were treated by the association cycline and rifampicin whereas a tritherapy including cycline, rifampicin and TMP-SMZ was used in the other cases. Outcome was favorable in seven cases. This study outlines the polymorphism of neurological manifestations due to brucellosis, even in familial cases and this diagnostic must be especially done in Middle East and South Mediterranean countries.

Acute Disease↗
PubMed1999

[Polyneuropathy due to glue-sniffing intoxication. Report of a case].

We report a case of a 24 years old female who presented a glue sniffing neuropathy. Symptoms began with progressive weakness which initially involves the lower extremities. Neurological examination noted a decreased Achille reflexes. Nerve conduction studies showed a slowing nerve conduction velocities, a delayed F waves and distal latencies and diffuse nerve conduction blocks. The disease continued to worsen beyond two weeks after stopping intoxication with persistent evidence of ongoing demyelination. 40 days later we have noted an improvement in nerve conduction velocities and in conduction blocks, neuropathy is the most frequent glue sniffing complications, there is a clinic and electric improvement of the polyneuropathy after stopping intoxication.

Adult↗
PubMed1998

Therapeutic strategies against epilepsy in Mediterranean countries: a report from an international collaborative survey.

A collaborative survey was performed to compare prescribing strategies for the treatment of epilepsy in Mediterranean countries, based on analysis of 500 questionnaires compiled by physicians in 14 different countries. For partial seizures, carbamazepine was the drug of choice in most countries, whereas the second choice of drug differed widely. For primarily generalized tonic-clonic seizures, valproic acid was usually preferred, but other drugs used widely in some countries included phenobarbital, phenytoin and carbamazepine. Lamotrigine was the most popular second-line drug for primarily generalized tonic-clonic seizures in the European countries. In patients where the initial drug failed, switching to an alternative monotherapy was usually the preferred strategy, but advocates of early use of combination therapy exceeded 30% in the respondents of seven countries. Most respondents, in all countries except Turkey, did not prescribe drugs to prevent recurrence of febrile seizures; however, intermittent prophylaxis with a benzodiazepine was advocated by a considerable number of physicians, and continuous prophylaxis was prescribed by a significant minority of respondents in France, Syria and Tunisia. New drugs were rarely used as first-line treatment due to high cost and inadequate experience. Overall, this survey indicates that there is a wide variability in therapeutic practices between and within countries. This information may be useful for the implementation of national educational activities and for the design of pragmatic trials aimed at comparing different therapeutic strategies.

Adult↗
PubMed1998

[Unverricht-Lündborg disease: clinical and electrophysiologic study of 19 Maghreb families].

We describe clinical, electrophysiological and genetic features in 44 patients with Unverricht-Lündborg disease from 19 families living in North African countries (Tunisia, Algeria and Morocco). The mean age of patients was 25.3 years; mean age was at onset 11.3 years. The disease began more frequently with seizures (91 per cent) or myoclonus (80 p. 100) than ataxia (16 p. 100). Subsequently myoclonus and generalized seizures were present in all patients, cerebellar signs were absent in four cases. EEG findings included normal background activity (90 p. 100), spontaneous fast generalized spikes (93 p. 100) and photosensitivity (70 p. 100). Antiepileptic polytherapy (clonazepam and/or phenobarbital and/or valporic acid) was used in 84 per cent of cases. Antiepileptic drugs were more effective in controlling epileptic seizures (less than one seizure/month in 60 p. 100) than myocloni which persisted daily in 64 p. 100 of cases. Mean duration of the disease was 13.5 years. One patient died of status epilepticus. Consanguinity was noted in 17 families (first degree in 15 families). Linkage to chromosome 21q 22.3 was confirmed in 11 families. We noted an inter and intrafamilial variability of clinical signs and disease course.

Adolescent↗
PubMed1997

[Familial multiple sclerosis: study of 2 cases of a northern African family].

We report two cases of multiple sclerosis (MS) beginning in a mother and her daughter at 40 years of age. The diagnosis of MS was certain for both patients (Poser et al., 1983). Clinical features, evolution and response to treatment are comparable in both cases. Cerebral and medullar cord MRI and/or CT Scan showed characteristic lesions of demyelination on periventricular white matter, corpus callosum and brainstem.

Adult↗
PubMed1995

[Spinal cord compression in bone fluorosis. Apropos of 4 cases].

The authors report four cases of spinal cord compression (three at cervical level and one at dorsal level) due to vertebral osteosclerosis secondary to chronic fluoride intoxication. Roentgenograms showed typical diffuse densification of vertebral bodies, calcifications of bony insertions of many ligaments, discs and interosseous membranes. Urinary fluoride was markedly increased in two cases. In the other two cases the bone biopsy was suggestive of skeletal fluorosis. Spinal computed tomography showed severe cord compression due to posterior osteophytes. Good improvement was observed after surgical decompression in one case. Fluorosis was described as a consequence of endemic exposure to high fluoride content in soil and natural ground water in North Africa. Fluorotic myelopathy was due to bone excrescences mainly affecting the spine.

Bone Diseases↗
PubMed1995

[Claude syndrome caused by mesencephalic infarction: 2 cases].

Paramedian midbrain infarcts limited to the oculomotor nerve fibers are uncommon. We studied 2 cases where the clinical syndrome included a third cranial nerve palsy and a contralateral cerebellar ataxia. The CT scan disclosed a paramedian midbrain tegmental infarct, so that it is possible to term our two cases Claude's syndrome. The oculomotor nerve fascicular palsies were complete in one case and limited to the extraocular muscles in the second case. The selective involvement of oculomotor function suggests intraaxial fascicular organisation of the third cranial nerve in the brainstem.

Cerebellar Ataxia↗