[Anterior spinal artery infarct: amyotrophic lateral sclerosis-like]

Psychiatric disorders accompanying infection with human immunodeficiency virus are frequent and varied. They canthe medullary infarct is a rare injury, with a difficult diagnosis. Usually, it is due to an aortic disease or to iatrgenic cause. We relate the observation of a patient presenting an anterior spinal infarct simulating an amyotrophic lateral sclerosis. Through this observation, we discuss the particular semiology of this medullar pathology. A 15-year-old girl, who developed sudden tetraparesis, tetrapyramidal syndrome and amyotrophy of the hands. The medullary MRI showed an extended hypersignal from C3 to C6 [compatible with an infarct in the anterior spinal artery territory]. The etiologic investigations were unremarkable. The clinical presentation of our patient and MRI results confirm the diagnosis of medullar infarct but absence of any objective sensory disorder suggest an ALS-like syndrome. The anterior horn cell region is in the most distally perfused part of the anterior spinal artery territory and is vulnerable to hypoperfusion. It can explain the clinical picture. The diagnosis of spinal cord infact must be suspected in case of atypical ALS syndrome with sudden onset

[Neurosyphilis presenting as cerebellar stroke]

Neurosyphilis complicates 5-10% of untreated syphilis. Syphilitic cerebral ischemic stroke occurs in young individuals with variable frequency. Vertebrobasilar territory ischemia is rare. Syphilitic cerebellar infarct is exceptional. We report the case of 58 year-old-man, chronic smoker, who presented an acute cerebellar syndrome. Computed tomography scan of the brain showed a left cerebellar infarct. The serology tests of syphilis were positive in blood and cerebrospinal fluid. HIV serology was negative. The patient was treated successfully by penicillin G with good clinical course. Syphilitic cerebral ischemic stroke occurs usually in the Carotid territory. Our patient present a cerebellar infarct with positive syphilitic serology in blood and cerebrospinal fluid. The characteristic of our patient is the fast and complete recovery under treatment with penicillin G, and the rarity of case of syphilitic cerebellar ischemic vascular accident reported in the literature. One case of cerebellar stroke was reported. Syphilitic cerebellar ischemic stroke is exceptional. Systematic tests of syphilitic serology should always be performed when a young person has one or several cerebrovascular accident

[Homocystinuria discovered by repetition of cerebrovascular thrombosis]

Homocystinuria is genetic autosomal recessive disease. The responsible gene of this disease is found in chromosome 21. Its prevalence in France is estimated at one case/ 60000 persons. The neurological manifestations of this disease are rare and diverse. Venous and cerebrovascular accidents are frequent among children who suffer from homocystinuria. These manifestations reveal sometimes the existence of the disease. We report a case of homocystinuria discovered through repeated ischemic cerebrovascular accidents from witch a 22 year-old patient suffers. The patient suffered at the age of 17 and 22 from five neurological episodes of ischemic cerebrovascular accidents associated with epileptic crises and right lower limb phlebitis. The association of venous and arterial thrombotic accidents evocates homocystinuria, particularly. Our clinical case highlights the importance of examining systematically every child suffering from a first cerebrovascular accident for homocystinuria, especially if it is associated with a marfanoid aspect

[Metabolic myopathy]

The muscle, as in any living cell, many chemical reactions such as synthesis of biological macromolecules, active transport of molecules across cell membranes, production of force and movement, require a continual input of energy. The muscle function involves the integrity of metabolic pathways that ensure the synthesis of adenosine triphosphate [ATP], especially during the transition from resting state to that of a maximum contraction, it also regenerated from either exogenous oxidative fuels [fatty acids, carbohydrates], or from the body reserves of [glycogen]. The metabolic dysfunctions, it means the enzymic deficiencies lead to a metabolic myopathy. The main causes of ATP deficiency and its consequences on the muscle cells function, relate to deficiencies in beta-oxidation of fatty acids, those of glycolysis and the mitochondrial respiratory chain

[Reversible post traumatic cortical blindness]

Cortical blindness [C.B] is a specific form of cerebral blindness associated with a lesion of the geniculo-striated optical ways. The interest of our case is to emphasise on the physiopathological mechanisms associated with this affection and its traumatic origin. This is a 42 years old patient, a victim of a traffic accident with multiple pelvic, forearm fractures and cranio-facial traumatism with exception to the orbits. The initial examination showed a loss of consciousness [Glascow Coma Scale 10] without any associated neurological deficit. The cerebral TDM documented bilateral diffuse occipital hypodensity with mass effect on median structures, without associated bone fractures. 72 hours later in the intensive care unit, she regained consciousness and increased intracranial pressure syndrome associated with complete bilateral blindness without any other localized sign was found. The evolution was satisfying as a result of early introduction of an anticoagulant treatment with a complete vision recovery. The etiology of the cerebral blindness is various. However, the post traumatic origin is rare. In our case, this association is original and opens new ways of research focusing its physiopathology in order to establish an early treatment and to ameliarate the visual prognosis

[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

[Cerebrovascular accidents and pregnancy]

Although cerebrovascular accidents [CVA] during pregnancy and post-partum period constitute a major cause of maternal death, yet it is still not well known. It affect pregnant women essentially in the 2nd and 3rd trimesters and in the post-partum period. We reported here certain clinical, aetiological and therapeutical aspects of CVA in cases of pregnancy and post-partum period. It concerns 8 pregnant or post-partum cases [1.2%] presented with CVA hospitalized in the neurology department [B] between 1998 and 2004. The average age was 33,7 years. Risk factor were represented by oral contraception's [6 cases], multiparity [3 cases] and arterial hypertension [2 cases]. Carotid region affection was found in 75% of cases. The dominent aetiological factor was secondary cerebral thrombophlebitis in one case and one to deficient in protein S in the other. CVA risk does not seem higher than that found in general population. The treatment of CVA during pregnancy and in post-partum period is the same recommended in non pregnant CVA cases

syndrome of Tolosa Hunt

The Syndrome of Tolosa Hunt [STH] is a curiosity whose pathogenesis is completely unknown. It is a diagnosis of elimination, obliging to review the list of the painful ophthamloplegias. We report five original observations by their mode of presentation. Our study relates five patients hospitalized for assessment unilateral of orbital and retro-orbital pains, with an attack of the occulomotricity, enroled between 1993 and 2004. The average age of our patients was 43,6 years. The cerebral scanner was performed in all cases and MRI in two cases only. The attack of the cranial nerves observed in our series was in four cases for the common oculomotor nerve, three cases for the external oculomotor nerve, three cases for the optic nerve, the attack of the ophthalmic branch VI was found in two cases. The advent of the scanner and the MRI simplified discussion of the differential diagnosis. The corticotherapy has a spectacular effect on the favorable evolution of the disease

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

Etat de mal epileptique generalise non convulsif [a propos d'un cas]

The authors report one case of noncovulsive generalized status epilepticus [NGS] who has been followed since 7 years. This case presents two particularities * The duration of the NGS is long with several episodes;* Usual treatment wasn't efficient. However, Acetazolamide proves to be better after one year of following up: in fact there has been improvement of consciousnes and EEG

Les syndromes syrincomyeliques: analyse clinique et resultats therapeutiques de 18 cas

The authors report 18 cases of syringomyelia syndromes at the department of neurology in Rabat between February 1978 and April 1986 these cases results from the following reasons 12 syringomyelias; 4 syringomyelias syndromes whose origin is tumoral; 1 syringomyelia syndrome wich is post traumatic; 1 syringomyelia syndrome which is post tuberculosis arachnoiditis. We have observed a 17 years old patient who suffers from a syringomyelia and EHELERS-DANLOS syndrome particulary with the dislocation of Cl C2

propos d'un cas de bilharziose nerologique

We report the first case of a radiculomedullar lesion observed in our service. It was caused by the Shistosoma Haematubium. A review of the literature showed us that it was an exceptional localization while the disease in extremely frequent. Because of this large frequency, in an endemic country, every non justified neurological lesion should lead one to think of the diagnosis

polyradiculonevrites[Analyse d'une serie homogene de 33 observations]

Throught the study of 33 Polyneuropathy cases, the authors have found that this disease is frequent in our country, especially in the aspect of the Guillain Barr‚ Syndrome - The evolution was usually good. Death occured in 12% of the cases but the motor after-effects were frequent