Neurological complications of Mediterranean boutonneuse fever. Presentation of a case of acute encephalomeningomyelitis and review of the literature

Abstract Mediterranean spotted fever is an infectious disease due to Rickettsia conorii usually considered as benign; however, 10% of cases may have severe complications. We report a patient with celiac disease who developed encephalomeningomyelitis secondary to Mediterranean spotted fever. Meningoencephalitic involvement occurred during the acute phase, with myelitis appearing early during convalescence, as acute onset paraplegia involving the lumbosacral spinal cord. A magnetic resonance study showed multifocal white matter disturbances, with no lesions in the spinal cord. One month following onset, R. conorii antibodies serum level was 1/640. A cutaneous biopsy performed during the acute phase revealed endothelial hyperplasia, intraluminal thrombosis and lymphocytic perivascular infiltrate. Several immunological disturbances were found (circulating immune complexes, antinuclear antibodies, IgG paraproteinemia). The development of a systemic vasculitis is the major pathogenetic factor in the origin of systemic complications of Mediterranean spotted fever. We review the neurological syndromes reported in association with R. conorii infection. Our case is the second described as acute myelopathy complicating Mediterranean spotted fever.