[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

[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

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

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

Inherited factor Vll deficiency [about one case]

Factor VII or proconvertine is a vitamin K dependent clotting factor which intervenes in the exgonic road of blood coagulation. Factor VII defect is an inherited disease with autosomic recessive transmission. It's a rare pathology, the incidence is estimated at 1/500000 of the general population. We report here a case of a 55 year old woman hospitalized for an anemia which occurred after dental extraction followed by hemorrhage. The paraclinical investigations found an isolated enlargement of Quick's time with a normal active cephalin time. The different clotting factors rates were measured and a striking factor VII defect was noted. By this observation, we wish to emphasize the clinical, biological and therapeutical particularities of this exceptional affection