La Revue de médecine interne
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Case Reports
[Rare neurological manifestations of West Nile virus infection in an immunocompetent patient].
Neuro-invasive disease is the most dreaded complication of West Nile virus infection. We report the case of an immunocompetent patient who developed a meningeal syndrome associated with cerebellar ataxia, facial diplegia and hemiparesis caused by West Nile virus infection with a favourable outcome. ⋯ Because of the increasing prevalence of West Nile virus, this infection should be suspected in the event of atypical neurological manifestations even in immunocompetent patients.
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Type I interferonopathies (IP1) are a heterogeneous group of Mendelian diseases characterized by overactivation of the type I interferon (IFN) pathway. They are caused by monogenic (rarely digenic) mutations of proteins involved in this key pathway of innate immunity. IP1 transmission can be dominant, recessive or X-linked and penetrance differs from one IP1 to another. ⋯ Almost all patients display overexpression of the type I IFN pathway detected, for instance, by the evaluation of IFN-stimulated genes expression, referred as "interferon signature". The related morbidity and mortality are high. However, the beneficial effect on certain symptoms of targeted therapies inhibiting type I IFN, such as JAK inhibitors, has led to a promising improvement in the management of these patients.