Longitudinal Transverse Myelitis with Locked in Syndrome Revealing a Systemic Lupus Erythematosus

Abstract

The neuropsychiatric manifestations of lupus are very heterogeneous and are a source of significant morbidity and mortality. Transverse myelitis is a rare but serious complication of systemic lupus erythematosus, classically described as an acute attack with a poor functional prognosis. It most often complicates the course of a previously diagnosed lupus and corresponds clinically to a complete medullary syndrome associating a symmetrical bilateral sensitivo motor deficit and sphincter disorders. We report a rare case of a young woman who was admitted to intensive care for a rapidly evolving flaccid tetraparesis with impaired consciousness. The explorations concluded that there was lupus with an inaugural neurological disorder such as longitudinal transverse myelitis associated with a secondary locked-in syndrome and a pontine ischemic stroke. Our observation has several particularities: the inaugural character and the longitudinal form of lupus myelitis as well as the serious association at the outset with a locked-in syndrome secondary to a cerebrovascular accident.