Meningoencephalitis due to Borrelia miyamotoi
Abstract & Commentary
By Dean L. Winslow, MD, FACP, FIDSA, Chairman, Department of Medicine, Santa Clara Valley, Medical Center; Clinical Professor, Stanford University School of Medicine, is Associate Editor for Infectious Disease Alert.
Dr. Winslow is a consultant for Siemens Diagnostic
Synopsis: An 80 year old woman from New Jersey with a past history of non-Hodgkin’s lymphoma presented with progressive confusion over 4 months. Spirochetes were detected in CSF by light microscopy and the presence of B. miyamotoi was confirmed by PCR. The patient initially received ceftriaxone and subsequently completed a 30-day course of IV penicillin G and recovered.
Source: Gugliotta JL, et al. Meningoencephalitis from Borrelia miyamotoi in an immunocompromised host. New Eng Jrl Med 2013;368:240-5.
An 80-year-old woman with a past history of non-Hodgkin’s lymphoma (follicular type, stage IIA) developed progressive confusion, unsteady gait, difficulty hearing and weight loss over a 4 month period. (She had initial treatment of her lymphoma with CAVP-rituximab in 2005 followed by rituximab every 6 months until several months prior to onset of her symptoms in 2012.) She lived on a farm in New Jersey and spent time outside but reported no known tick exposures, although she developed erythema migrans in 2007 and received a 2 week course of doxycycline with complete resolution of her symptoms at that time. MRI of the brain performed in early 2012 4 months after onset of neurologic symptoms was unremarkable. Lumbar puncture showed 65 WBC/mm3 (23% pmn, 70% lymphocytes, 6% monocytes, and 1% uncharacterized cells), protein > 300 mg/dL and glucose 33 mg/dL. Giemsa stain of CSF revealed spirochetes. The patient experienced chills, fever and mild hypotension 9 hours after receiving her initial dose of cetriaxone, consistent with a Jarisch-Herxheimer reaction. PCNG 24 million units IV daily was initiated and her mental status improved dramatically over the initial 5 days of treatment. After approximately 4 weeks of antimicrobial treatment the CSF WBC had decreased to 21 cells/mm3, protein had decreased to 168 mg/dL, and glucose had increased to 41 mg/dL. Organisms were no longer seen.
Spirochetes seen in the initial specimen of CSF failed to grow in culture. EIA of CSF for B. burgdorferi antigen was negative and antibodies to B. burgdorferi were negative on both serum and CSF. Immunoblot analyses were also negative. Real-time PCR showed amplification of a Borrelia species but primers to amplify OspA gene were negative, ruling out B. burgdorferi. Sequencing and phylogenetic analysis of 16S rRNA and flagellin genes were consistent with B. miyamotoi-like spirochetes.
The genus borrelia can be divided into two taxonomic groups: basically Lyme disease and relapsing fever. Of the relapsing fever group, B. recurrentis (louse-borne), B. hermsii (Ornithodos ticks), B. lonestari (Amblyomma ticks), B. theileri (Boophilus ticks), and B. miyamotoi (transmitted by Ixodes ticks in North America and Haemophysalis ticks in Japan) have been identified. Rare human cases of B. miyamotoi infections have been described in Russia (mainly in patients presenting with nonspecific fever), but it is possible that cases are under-reported since this organism is commonly present in Ixodid ticks world-wide. Studies in residents of New England demonstrate antibody reactivity to this organism may be present in up to 3% of individuals. It is likely that B. miyamotoi may be a cause of “idiopathic meningoencephalitis” in both immunocompetent and immunocompromised patients. A recurrent theme of the 37 years I have spent since medical school is “hold on to your hat—here’s another new disease!”