[Landau-kleffner syndrome]

Landau-Kleffner syndrome is a rare disorder caraterised by acquired aphasia of children, paroxysmal electroencephalographic disturbance and in 75% of cases epileptic seizures and cognitives disturbances. We report a 6 year old daughter who presented epileptic seizures and six months later regression langage and a behaviour of deaf. This patient had a global aphasia. The understanding was affected because of the cortical deafness. The electroencephalogramme showed discharges in the temporal bilateral regions. The cerebral scan and the potential evoked auditory were normal. The Landau-Kleffner syndrome's diagnosis was confirmed. The patient was treated by valproic acid and corticosteroids with good evolution. The stoopage of treatment has brought more seizure and the worsening aphasia. The authors discussed the pathogenic hypothesis, the semiology and the treatment of the Landau-Kleffner syndrome

[Epidemiological and clinical aspects of neurosyphilis in Morocco]

Neurosyphilis accounts for 56%-70% of all visceral syphilis and is a complication in 5%-10% of cases of untreated syphilis. The aim of this study was to evaluate the epidemiological aspects and clinical presentations of neurosyphilis in Morocco through a series of 201 patients attending the Centre for Neurological Services at the university hospital in Rabat between 1986 and 1997. The mean age of the patients was 41.26 [SD 9.23] years [range: 17-70 years]; the majority [91%] were male. The incidence of neurosyphilis in Morocco is high. From 31 cases per year in 1985, it has fallen since 1990 to reach 10 cases in 1997. Among the different clinical presentations recorded, chronic meningoencepahalitis was the commonest, followed by meningovasculitis, tabes dorsalis and optic atrophy

Chickenpox at a Moroccan adult

Chickenpox is a benign viral affection. But, that can have complications especially in late onset of the disease. We report a case who presented witha myelitis two days after having developed typical skin eruption of varicella, with fever. The clinical signs decreased after Aciclovir and corticosteroids treatment. We also discuss the mechanism and the treatment of this varicella myelitis especially in the late onset

Adrenoleukodystrophy

The adenoleukodystrophy is recessive X linked hereditary affection related to the X peroxysomal origin to a deficiency in acyl-coenzyme A-synthetase. We report a case of a patient aged 9 years born after a consanguinous mariage, with no similar case in the family, who presented at the age of 8 with a cerebellar syndrome and decreasing hearing acuity. The neurological exam inationfound a cerebellar and an extrapyramidal syndrome and a deficiency of the pelvic girdle. The paraclinical investigations were in favor of myelinising affection the adrenoleukodystrophy. The patient was treated by hydrocortisone [15 mg morning, 5 mg evening]. Adrenoleukodystrophy which is a rare affection found in 1 out 400 birthis, related to the X chromosome. The gene was localised on the chromosome Xp28. It is near to factor VIII, the glucose 6 phosphate deshydrogenase, the myopathv of Emmery-dreyfus and the genes of green and red pigments. The gene was identified by Aubourg. Actually the genetic counceling is importantt in that affection, and that is thanks to the prenatal diagnosis possible by the very long chain fatty acid dosage in the chorionic villi where the culture of the amniotic cells at 9 weeks of amenorrhea permitting the diagnosis in 100% of case

syndromes neurologiques paraneoplasiques [mise au point]

The authentic paraneoplastic neurological syndromes are represented by the subacute encephalomyeloneuritis, the subacute cerebellar degeneration, opsoclonus myoclonus, Lambert Eaton syndrome, and paraneoplastic retinopathy. In about 50% of the cases, these syndromes are revelators of cancer, which can be found few years later. All the malignant diseases seem to be able to give a rise to paraneoplastic syndromes. However, their frequency is more elevated in lung cancer, especially small cell lung cancer. These clinical manifestations have attracted more interest recently, since the discovery of anti-tumoral antibodies directed against neural targets, suggesting the existence of dysfunctionning of the immune system in the pathogeny of these syndromes