[A report of 9 cases of neurosarcoidosis]. / Neurosarcoïdose. Etude de neuf cas.

INTRODUCTION: Neurological involvement in sarcoidosis is rare and highly variable. To date, no consensus was reached about the diagnosis approach. We report a case series of 9 patients with neuosarcoidosis, with favorable outcome under therapy. MATERIALS AND METHODS: We examined a case series of 9 patients with neurosarcoidosis. Clinical, radiological, therapeutic features and outcome were studied. RESULTS: Six of the nine patients were females. Patients' age ranged from 31 to 70 years. Initial neurological symptoms lead to the diagnosis of systemic sarcoidosis in all patients. Central nervous system involvement was found in 77 percent with cranial nerve involvement in 55 percent. Twenty-three percent of patients presented with peripheral neuropathy and 33 percent with meningitis. The diagnosis was definite in 2 patients, probable in one and possible in six others. All patients were given steroid therapy. Total remission was obtained in three and partial remission in three. Three patients remained stable and one died. CONCLUSION: Histological signs are not constant in neurosarcoidosis. The lack of these signs should lead the physician to search for latent extraneurological symptoms which are suggestive of the diagnosis. Nervous biopsy can thus be avoided.

[Idiopathic hypereosinophilic syndrome presenting with oculomotor paresis]. / Paralysie oculomotrice révélant un syndrome hyperéosinophilique essentiel.

A 42-year-old patient with a known depressive syndrome developed diplopia. The neurological examination revealed a peripheral neurogenic syndrome with incoordination of the left arm. Biological and radiological findings were in agreement with the diagnosis of idiopathic hypereosinophilic syndrome. Treatment with corticosteroids was effective.

[Cerebral thrombophlebitis revealing hyperthyroidism: two cases report and literature review]. / Thrombophlébite cérébrale révélant une hyperthyroïdie: à propos de deux cas et revue de la littérature.

INTRODUCTION: Two cases of hyperthyroidism revealed by a cerebral venous thrombosis are reported. EXEGESIS: Clinical symptomatology consisted of intra-cranial hypertension and convulsive crisis associated to thyrotoxicosis. Evolution was favorable with etiologic and symptomatic therapy. CONCLUSION: The cerebral venous thrombosis risk on hyperthyroidism is discussed.

[Neuro-Behcet's syndrome and thrombosis of Rosenthal's basilar vein: a report of twelve cases]. / Thrombose de la veine basilaire de Rosenthal et angio-Behçet cérébral. Etude de 12 cas.

INTRODUCTION: Deep cerebral vein thrombosis is a specific clinical pattern of neuroBehçet. The clinical features of ischemic stroke by Rosenthal or internal cephalic vein thrombosis predominate. METHOD: A retrospective analysis of twelve cases of Rosenthal vein thrombosis revealing Behçet disease were analyzed. These cases accounted for one-quarter of the NeuroBehçet patients treated in our unit. RESULTS: The typical pattern of Rosenthal vein thrombosis clinical was present in all patients who developed a diencephalo-mesencephalic syndrome. CONCLUSION: The clinical presentation is highly suggestive of diagnosis confirmed by imaging. Outcome is favorable with corticosteroids and anticoagulation.

[Cavernous sarcoid granuloma of the skull base: a case report]. / Granulome sarcoïdien de la loge sphéno-caverneuse.

A case of cavernous sarcoid granuloma, revealed by a slit sphenoidal syndrome is reported. Brain MRI showed a cavernous pseudo-tumoral lesion. The diagnosis of sarcoidosis was confirmed by the epithelioid and giant-cell granuloma without caseous necrosis found at hepatic biopsy. Outcome was favorable with corticosteroid therapy alone.

[Sneddon's syndrome: 4 cases and a review of the literature]. / Le syndrome de Sneddon: 4 cas et revue de la littérature.

Sneddon syndrome is an association of livedo racemosa and cerebrovascular ischemic events generally occurring in young adults. This is an uncommon chronic progressive arterio-occlusive disorder of unknown cause involving small and medium sized vessels. We report four cases. One case was disclosed by cerebral hemorrhage. One pathogenic hypothesis suggests the involvement of an idiopathic progressive inflammatory arteriopathy or secondary thrombotic disorder comparable with antiphospholipid syndrome.

[Neurological manifestations of xeroderma pigmentosum]. / Les manifestations neurologiques du xeroderma pigmentosum.

Xeroderma pigmentosum is a genodermatosis with neurological manifestations in approximately 18p. 100 of cases. Polymorphous and variably associated signs are observed, progressing with the clinical course. The etiology of the neurological breach remains unknown. We report two siblings who had xeroderma pigmentosum with intellectual deficiency, a pyramidal, cerebellar and cordonal syndrome, ophthalmoplegia, and axonal peripheral neuropathy. We discuss the epidemiological, clinical and electrophysiological aspects of the neurological breach in xeroderma pigmentosum.

[Brainstem tuberculomas: two cases]. / Tuberculomes du tronc cérébral.

We report two cases of brainstem tuberculomas with favorable outcome after medical treatment alone. Tuberculomas should be suspected in all cases presenting space-occupying lesions of the brainstem.

[Neurologic complications of varicella in adults]. / Les complications neurologiques de la varicelle chez l'adulte.

Three patients aged 32, 30 and 36 years, had chicken pox then developed acute cerebellar ataxia (for two) and acute polyradiculoneuritis. Cerebrospinal fluid (CSF) protein content was increased and varicella virus serology was positive in both blood and CSF. All three patients improved with aciclovir.

[Bilateral optic neuritis and ponto-mesencephalic involvement shown by MRI in Miller-Fisher syndrome]. / Névrite optique bilatérale et atteinte ponto-mésencéphalique à l'IRM au cours d'un syndrome de Miller-Fisher.

We report a case of Miller-Fisher syndrome with ophthalmoplegia, ataxia and areflexia. The main characteristics of this case were bilateral optic neuropathy and ponto-mesencephalic abnormalities on MRI. This case confirms the possible central neurologic localisation in this rare syndrome.

[Association of amyotrophic lateral sclerosis with scleroderma. Study of 2 cases]. / Association sclérose latérale amyotrophique et sclérodermie. Etude de 2 cas.

We report a clinical association of diffuse scleroderma and amyotrophic lateral sclerosis (ALS) in two patients. Scleroderma was diagnosed on skin, digestive, osteoarticular, pulmonary lesions and inflammatory syndrome. ALS was suspected on the association of diffuse amyotrophy, fasciculations, pyramidal tract involvement and electrophysiological data. Chronic medulla ischemia and or immune abnormalities are proposed as potential pathological mechanisms for ALS but fortuitous association can not be excluded.

Linkage analysis of families with severe childhood autosomal recessive muscular dystrophy in Morocco indicates genetic homogeneity of the disease in north Africa.

It has been previously shown in Tunisian and Algerian families that the locus for SCARMD maps to the proximal part of 13q, and in Algerian families that the disease is associated with deficiency of the 50 kDa dystrophin associated glycoprotein (50DAG). We have tested this linkage in six families from Morocco where this disease is also prevalent. In one family the 50DAG was tested and found to be negative in a muscle biopsy. Our results showed similar linkage in this country, with statistical tests indicating genetic homogeneity between the three Maghreb countries.