[Cerebral salt wasting syndrome in bacterial meningitis]. / Syndrome de perte de sel d'origine cérébrale avec hyponatrémie au cours d'une méningite bactérienne.

Subarachnoid hemorrhage is the most common cause of cerebral salt wasting syndrome. There are few reports of this condition in infectious meningitis. We describe a patient with hyponatremia and bacterial meningitis. Hyponatremia rapidly improved after administration of sodium chloride. The purpose of this report is to alert clinicians to the fact that hyponatremic patients with central nervous system disease do not necessarily have a syndrome of inappropriate secretion of antidiuretic hormone (SIADH), but may have cerebral salt wasting syndrome. By contrast with SIADH, the treatment requires saline administration.

[Conjugal progressive systemic sclerosis]. / Sclérodermie conjugale.

INTRODUCTION: Familial occurrence of progressive systemic sclerosis is unusual. The occurrence of conjugal scleroderma is exceptional. EXEGESIS: We report here a case of systemic sclerosis in a wife and husband who both developed the onset of illness within a 10-year period. Solvent exposure was noted. CONCLUSION: The etiology of systemic sclerosis remains unknown. Environmental factors may play role in its pathogenesis.

[Sjögren's syndrome with autonomic failure and epilepsy]. / Syndrome de Sjögren avec épilepsie et dysautonomie.

INTRODUCTION: Primary Sjogren syndrome is considered as the most frequent connective tissue disease. Neurological complications may affect the peripheral nervous system and to lesser extent the central nervous system. Autonomic system nervous dysfunction and epilepsy have been rarely reported. EXEGESIS: We present on case of Sjogren's syndrome with epilepsy and autonomic nervous system dysfunction. The epilepsia respond to valproate. CONCLUSION: Autoimmune investigations for Sjogren's syndrome should be initiated in any patient presenting with unexplained neurologic manifestations.

[Association of hereditary hemochromatosis and pernicious anaemia]. / Hémochromatose génétique et anémie de Biermer.

INTRODUCTION: Hereditary hemochromatosis is inherited as an autosomal recessive trait. It is characterized by increased absorption of dietary iron. The association between pernicious anaemia and hereditary hemochromatosis has never been described. EXEGESIS: We report a case of paradoxical association of hereditary hemochromatosis and pernicious anaemia. CONCLUSION: It seems that pernicious anaemia may prevent manifestations of hemochromatosis. We suppose that this protective role is due to atrophic body gastritis with iron malabsorption.

[Mycobacterium marinum nodular lymphangitis]. / Lymphangite nodulaire due à Mycobacterium marinum.

BACKGROUND: Mycobacterium marinum has been recognized for some forty years. It generally occurs after trauma in a patient who manipulates tropical fish living in an aquarium. CASE REPORT: We report the case of an exotic fish seller who developed M. marinum nodular lymphangitis after being bitten by one of the fish. DISCUSSION: Nodular lesions occurring after trauma in subjects living in France who are in contact with exotic fish suggest the diagnosis of M. marinum. Nodular lymphangitis is seen in about 50% of the cases. Prolonged antibiotic therapy is required. Several antibiotics are effective including minocycline used in our case for 2 months after complete healing of the wound. Surgery is exceptionally required.

[Neurosarcoidosis. Review of the literature]. / Les neurosarcoïdoses. Revue de la littérature.

Sarcoidosis is a systemic granulomatous disease of unknown etiology. The frequency of neurologic manifestations varies from 5 to 15% of cases. Systemic manifestations of sarcoidosis are often present and the diagnosis is still based on histopathological studies. In nearly half of the patients no systemic manifestations are found and the diagnosis of sarcoidosis may be difficult. Main neurologic involvements are cranial and peripheral nerve lesions. Other manifestations include aseptic meningitis, hydrocephalus, parenchymatous granulomata of the central nervous system, hypothalamic dysfunction and myopathies. There is more than one manifestation of neurosarcoidosis in 30% of the patients. Lumbar puncture is useful to rule out other diseases but CSF changes are not specific, neither elevated serum angiotensin converting enzyme levels. Computed tomography is helpful, but magnetic resonance imaging is the best diagnostic tool. The granulomatous nature of the disease can only be confirmed by tissue sampling and brain biopsies are sometimes required. There have been no controlled trials of treatment for neurosarcoidosis but corticosteroids are the main stay of therapy. The prognosis is often good and 55% of patients reported have had a complete recovery. In case of failure, immunosuppressive and radiation therapy can be used. Surgery is indicated to establish tissue diagnosis, in the presence of increased intracranial pressure, or to remove brain tumor.

[An unusual form of neurosarcoidosis isolated in association with a tumoral lesion, chronic meningitis, hydrocephalus and hypothalamo-hypophyseal involvement]. / Une forme exceptionnelle de neurosarcoïdose isolée associant lésion tumorale, méningite chronique, hydrocéphalie et atteinte hypothalamo-hypophysaire.

A 30-year-old woman developed progressive left sided hemiparesis with intracranial hypertension signs. CT scan and MRI showed a large temporo parietal cystic mass with marked surrounding edema. Surgical excision was performed, and histological analysis revealed an inflammatory granuloma. No disease elsewhere was found and all classical causes of granulomas such as tuberculosis, toxoplasma, fungus infections, inflammatory diseases, lymphomas and cancers were excluded. No treatment was administered and she remained neurologically stable for two years. Afterwards, she developed chronic meningo-encephalitis, hypothalamic-pituitary dysfunction and hydrocephalus requiring decompression. Sarcoidosis was suspected, a steroid therapy was initiated, she gradually improved and a ventricular biopsy confirmed this diagnosis. Nervous system lesions complicate the course of sarcoidosis in 5 to 15% of patients and most commonly involve the cranial and peripheral nerves. CNS involvement is typically meningeal with a predilection for the hypothalamic region. Intracranial mass lesions are rare and their occurrence in the absence of disease elsewhere is still more unusual. Three presentations have been described: an isolated intra parenchymatous mass, multiples nodules, and subdural plaques, that can be mistaken for meningiomas, gliomas or metastases. When systemic manifestations of sarcoidosis are absent, the diagnosis is difficult, and Gd-enhanced MRI is now considered the diagnostic method of choice. However brain biopsy is sometimes necessary. Corticosteroids are the mainstay of therapy. Immunosuppressive agents are also used and brain irradiation has been tried in some refractory cases. Surgical approach may be indicated to establish tissue diagnosis, to perform decompression and to remove brain tumors.

[Cerebral panthrombophlebitis in a paucisymptomatic patient]. / Panthrombophlébite cérébrale chez un patient paucisymptomatique.

In most cases, extensive cerebral venous thrombosis present themselves with a severe clinical outcome and poor prognosis. We present the case of a 59-years-old patient with a slight rather unrevealing symptoms but suffering from a cerebral thrombosis impacting on both superficial and deep venous system. The etiologic assessment revealed activated protein C resistance. Clinical evolution under systemic anticoagulation was prompt, with complete repermeabilization of the various venous structures.

[Occupational exposure to cadmium and renal cancer. Apropos of a case]. / Exposition professionnelle au cadmium et cancer du rein. A propos d'un cas.

INTRODUCTION: Cadmium is a carcinogenic substance and bronchial and prostatic cancer may be readily identified as professional diseases. It is also a nephrotoxic substance but renal carcinoma are rarely imputed to this kind of intoxication. CASE REPORT: We report the observation of a 59-year-old woman with a long unprotected professional exposure to cadmium. She developed an epidermoid carcinoma of the kidney and later of the lung. Levels of cadmium in blood samples were abnormally high, confirming the existence of a chronic impregnation. CONCLUSION: We have judged cadmium to be responsible for her renal disease: the fact of a long unprotected professional exposure, the scarcity of renal epidermoid carcinoma, the well established nephrotoxicity of this substance and the association with other cancers, appear to us as convincing evidence, and the kidney cancer of our patient has been considered a professional disease.

Creutzfeldt-Jakob disease with long duration and panencephalopathic lesions: molecular analysis of one case.

A 49-year-old woman presented with isolated aphasia followed by dementia and ataxia with a duration of 4 years. Histopathologically there was panencephalic involvement, status spongiosus, and kuru-type plaques. Molecular analysis showed heterozygosity at codon 129 in the prion protein (PrP) gene, and type 2 protease-resistant PrP. The comparison between this case and those previously reported suggests that the panencephalopathic variant of Creutzfeldt-Jakob disease (CJD) is an aspecific end-stage condition displayed by most if not all CJD variants in individual patients with an unusually prolonged course.

[Prevalence of clinical manifestations of allergic reactions in HIV infection. Cross sectional study of 115 subjects]. / Prévalence des manifestations cliniques évocatrices d'allergie au cours de l'infection par le VIH. Etude transversale de 115 sujets.

A cross sectional survey was set up to study the relation between the prevalence of allergic-type reactions during HIV infection course. For each patient, a standardized interview about recent allergic-type manifestations (RATM), skin prick-tests to six common airborne allergens, IgE serum level were done. Among the 115 included patients, the mean CD4 lymphocyte count (CD4) was 214.7/mm3 (range: 0-1328/mm3). RATM were found in 8.8% of patients with CD4 < 50, in 30% of patients with CD4 between 51 and 200, in 36% of patients with CD4 between 201 and 350 and in 11.5% of patients with CD4 < 350 (p = 0.03). The risk of presenting RATM was 4.8 times (95% confidence interval = 1.7-13.5) higher in patients with CD4 between 51 and 350 than in other patients (p = 0.003). The proportion of positive prick-tests did not significantly vary according to the level of CD4. The increased frequency of RATM in patients with CD4 between 51 and 350/mm3 could be due to an allergic predisposition acquired during the course of HIV infection. The mechanisms explaining the reduced frequency of allergic manifestations when immunodeficiency is profound (CD4 < 50/mm3) remain to be explained.

[Prostacyclin in the treatment of hemolytic-uremic syndrome: apropos of a case]. / Intérêt de la prostacycline dans le traitement du syndrome hémolytique et urémique: à propos d'un cas.

A 52 year-old man was hospitalised for acute renal failure with thrombocytopenia and hemolytic anemia without oliguria. A haemolytic-uremic syndrome was diagnosed and prostacyclin infusion was started. Twenty-four hours later, the renal function improved as well as thrombocytopenia and anemia. Recovery occurred after 11 days of treatment. Haemolytic-uremic syndrome treatment is not well codified: plasmaphoresis, fresh frozen plasma, transfusions showed inconstant efficiency and data about prostacycline treatment are rare and often contradictory. Multicentric studies must be started in order to determine the precise benefit of this treatment.

[Horton's disease disclosed by involvement of the lower limbs]. / Maladie de Horton révélée par une atteinte des membres inférieurs.

We report the case of a 62-year old woman hospitalized for acute ischaemia of the right lower limb, caused by stenosis of the superficial femoral artery associated with thrombosis of the deep femoral artery. Thromboendarterectomy was performed, and histology of the operative specimen showed thickening of the media with clusters of giant cells and fragmentation of the internal elastic lamina, without atheroma. The diagnostic of giant cell arteritis was then considered and confirmed by the presence of headaches, 38 degrees C fever and inflammatory syndrome with ESR at 75 mm in the first hour. On the other hand, biopsy of a temporal artery was negative. Corticosteroid therapy was prescribed and gave excellent clinical, laboratory and arteriographic results. It has now been demonstrated that the arteries of the lower limbs may be involved in Horton's giant cell arteritis, which is often overlooked. This involvement is rare and exceptionally proven at histology. Clinically, the most frequently described form is one of pseudo-arteritis with claudication, but a few cases of gangrene have been reported. Withdrawal of corticosteroid might be a facilitating factor. As in our patient, the arteriographic lesions are often segmental, multifocal and symmetrical, predominant in the superficial femoral artery and the arteries of the legs. The lesions regress under corticosteroid therapy, and surgery can usually be avoided. Anticoagulants are commonly prescribed when the large vessels are involved. When surgery is not indicated the diagnosis can be confirmed by biopsy of the temporal artery, which is positive in the majority of cases.