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Borrelia rhombencephalomyelopathy
T Kuntzer1, J Bogousslavsky, J Miklossy
1Department of Neurology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
Abstract:
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.
Insights
Borrelia burgdorferi infection can cause long-term central nervous system damage, including brain-stem dysfunction and myelitis. Penicillin therapy normalized cerebrospinal fluid abnormalities in patients with neuroborreliosis.
Area of Science:
- Neurology
- Infectious Diseases
- Pathology
Background:
- Borrelia burgdorferi infection, the causative agent of Lyme disease, can lead to neurological complications.
- Chronic or undiagnosed infections may present with complex central nervous system (CNS) involvement.
Observation:
- Three patients with long-standing, undiagnosed Borrelia burgdorferi infection exhibited biphasic CNS involvement.
- Initial symptoms included acute brain-stem dysfunction, followed by progressive myelitis or relapsing-remitting rhombencephalitis.
Findings:
- Cerebrospinal fluid analysis revealed elevated total IgM levels, which normalized post-penicillin treatment.
- Neuropathology showed microgliosis, meningovascular inflammation, and ischemic infarcts in the myelencephalon.
- Spirochetes were identified in the leptomeninges and around periventricular vessels, associated with obliterative inflammatory vasculopathy.
Implications:
- This study provides pathological evidence linking Borrelia burgdorferi infection to cerebrovascular disease.
- Neuroborreliosis shares pathogenetic mechanisms with neurosyphilis, highlighting the importance of considering spirochetal infections in CNS vasculopathies.