[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.

Prion encephalopathy with insertion of octapeptide repeats: the number of repeats determines the type of cerebellar deposits.

We studied modifications of the molecular layer of the cerebellum in three patients with octapeptide repeat insertion (OPRI). Two brothers carrying a six-OPRI showed only spongiosis in haematoxylin & eosin preparations (H&E), whereas immunocytochemical examination (ICC) with an antiprion protein (PrP) antibody revealed numerous elongated PrP deposits. The third patient from a family with an eight-OPRI had numerous plaques visible in H&E preparations and had been diagnosed as Gerstmann-Straüssler-Scheinker syndrome. So far, 15 other cases from seven families and three individual cases with OPRI have undergone neuropathological examination. Characteristic PrP deposits were seen in six other cases, two isolated cases with a four- and a seven-OPRI, whereas four cases with a six-OPRI came from three different families. Such deposits have never been reported in other cases of prion encephalopathy, without OPRI. Genuine plaques were observed in five out of the 15 other patients. Interestingly, four had an eight-OPRI and one a nine-OPRI. Cases with OPRI are prone to develop different PrP deposits: those only visible on ICC are not to be confused with genuine plaques visible in H&E preparations. Elongated PrP deposits are present in cases with a four- to seven-OPRI, whereas plaques are present when there is an eight- or a nine-OPRI. All these cases should be termed prion encephalopathy with OPRI.

Familial prion disease with a novel 144-bp insertion in the prion protein gene in a Basque family.

Three members of a Basque family carrying a novel six R2 octapeptide repeat 144-bp insertion in the prion protein gene (PRNP) showed a slowly progressive dementia associated with cerebellar signs, myoclonic jerks, and seizures. Although postmortem examination revealed only focal and minimal spongiform degeneration in one subject with a 4-year course, significant astrogliosis and neuronal loss were associated with pronounced spongiform degeneration in the patient with a duration of symptoms of 10 years. Prion protein (PrP)-immunoreactive patches with a unique morphology were present in the molecular layer of the cerebellum in both subjects. Western blot analysis demonstrated the presence of protease-resistant prion protein (PrPres) with the same characteristics (size and ratio of the three differently glycosylated isoforms) of that found in typical sporadic Creutzfeldt-Jakob disease (CJD129M/M, PrPres type 1). The amount of PrPres correlated with presence and severity of spongiform degeneration in the cerebral cortex. The findings suggest that a relatively low rate of PrPres deposition is the cause of the lack of spongiform degeneration in subjects carrying a 144-bp insertion in PRNP. The presence of PrP-immunoreactive patches with unique morphology in the molecular layer of the cerebellum is a hallmark of certain prion encephalopathies with insertional mutations and is useful in the diagnosis of this subtype of human prion disease.