Borrelia rhombencephalomyelopathy
T. Kuntzer, J. Bogousslavsky, J. Miklossy, A. J. Steck, R. Janzer and F. Regli
Department of Neurology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
Three patients, in whom the diagnosis of Borrelia burgdorferi infection was
unknown for several years, developed a biphasic involvement of the central
nervous system: an acute brain-stem dysfunction was followed up, in two
patients, by a progressive, disabling myelitis and, in one patient, by
further relapsing-remitting episodes of severe multifocal
rhombencephalitis. The most consistent cerebrospinal fluid abnormalities in
the analysis of sequential specimens were elevated total IgM levels that
normalized after penicillin therapy. The neuropathologic findings in one
patient showed microgliosis and meningovascular involvement of the central
nervous system, resulting in two ischemic infarcts in the myelencephalon.
Few spirochetes were localized in the leptomeninges and around subependymal
vessels of the fourth ventricle. The vascular element consisted of an
obliterative inflammatory vasculopathy in the medullary parenchyma. This
study (1) provides pathologic evidence that a vascular disease induced by B
burgdorferi is a pathogenetic mechanism for cerebrovascular diseases, and
(2) emphasizes the similarities between neuroborreliosis and neurosyphilis.
