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Open Access

Lyme Neuroborreliosis: Manifestations of a Rapidly Emerging Zoonosis

P. Hildenbrand, D.E. Craven, R. Jones and P. Nemeskal
American Journal of Neuroradiology June 2009, 30 (6) 1079-1087; DOI: https://doi.org/10.3174/ajnr.A1579
P. Hildenbrand
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D.E. Craven
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R. Jones
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P. Nemeskal
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Article Figures & Data

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  • Fig 1.
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    Fig 1.

    Blacklegged tick (I Scapularis): adult female, adult male, nymph, and larva. Reprinted with permission from the Centers for Disease Control and Prevention.

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    Fig 2.

    A, Summary of reported cases of Lyme disease in the United States. There is 1 dot within the county of residence for each reported case. Note the widespread diversity of cases correlating with the prevalence of infected Ixodes species in different geographic areas. Courtesy of the Centers for Disease Control. B, The beige-shaded areas indicate the geographic distribution of recorded clinical cases of Lyme borreliosis. The colored ellipses indicate the distribution of the various Borrelia subspecies. Fig 2B is reprinted with permission from Nature Publishing Group.108

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    Fig 3.

    Erythema migrans rash with the typical target appearance that is virtually diagnostic of Lyme disease.

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    Fig 4.

    Facial neuritis. A 48-year-old woman with headache and peripheral right facial palsy. Prominent enhancement of the fundal tuft and labyrinthine segment of the seventh cranial nerve on postinfusion axial T1 (A) and coronal spoiled gradient-recalled sequences (B). The patient had CSF pleocytosis, Lyme-positive EIA, and Western blot (IgM and IgG) in serum and CSF. Resolution occurred with intravenous ceftriaxone therapy.

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    Fig 5.

    Evolving cranial neuritis. A 71-year-old woman with headache, malaise, fever, and diplopia. Initial coronal postcontrast T1 MR imaging (A and B) with enhancing bilateral third and fifth cranial nerves. C, Nine days later, she developed left facial palsy with enhancing fundal tuft and labyrinthine and tympanic segments of the seventh cranial nerve. The patient had CSF pleocytosis with positive Lyme-EIA and Western blot in both serum and CSF and CSF Lyme PCR-negative findings. Resolution occurred with intravenous ceftriaxone therapy.

  • Fig 6.
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    Fig 6.

    A 50-year-old woman with a history of tick bite and erythema migrans rash treated with doxycycline, who had recurrent erythema migrans rash with headache, fever, nausea, and nuchal rigidity. The patient had CSF pleocytosis with positive Lyme serum EIA and IgM Western blot and negative Lyme antibodies in the CSF. Gradual symptomatic improvement occurred following intravenous ceftriaxone therapy. There has been stable MR imaging for 5 years. Sagittal (A and B) and axial (C) fluid-attenuated inversion recovery images show arcuate and confluent subcortical white matter involvement and callososeptal interface involvement remarkably similar to that in MS, but without involvement of the periventricular white matter.

  • Fig 7.
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    Fig 7.

    A 74-year-old man with 2-year cognitive decline and memory loss. The patient had Lyme-positive serum EIA and Western blot (IgG and IgM) and CSF pleocytosis with CSF positive Lyme IgM and IgG antibodies. The patient improved with intravenous ceftriaxone therapy. The “dot-dash” callososeptal interface (A) and periventricular distribution of involvement (B) would be routinely ascribed to a demyelinating process.

  • Fig 8.
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    Fig 8.

    A 56-year-old woman with neck, bilateral shoulder, and bilateral arm pain. In 2 weeks, she subsequently developed left facial palsy and positive serum EIA and Western blot (IgM and IgG) and CSF Lyme IgG and IgM antibodies. Complete resolution of symptoms occurred with oral doxycycline. Postcontrast sagittal and axial T1-weighted MR images show diffuse thin uniform cervical spinal cord leptomeningeal enhancement without apparent root or ganglion enhancement.

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    Fig 9.

    A 17-year-old boy with right papilledema and orbital pain and rule out pseudotumor. The patient had positive serum EIA and Western blot (IgM and IgG) and CSF Lyme IgM and IgG antibodies. Lyme PCR in the CSF was negative. Complete resolution of symptoms occurred after intravenous ceftriaxone therapy. Right optic nerve edema on fat-saturated T2-weighted fast spin-echo images (A) and right-greater-than-left optic nerve enhancement on coronal fat-saturated contrast-enhanced T1-weighted images (B). C, Bilateral third cranial nerve enhancement (arrowheads) and bilateral retrobulbar compartment congestion (arrow). Note the generalized extraocular muscle enlargement and enhancement, including the insertions. Additional imaging findings included enhancement of right fifth cranial nerve, optic chiasm, and intracanalicular right seventh nerve, all of which were occult to neurologic examination.

Tables

  • Figures
  • Comparison of clinical differences between American and European LNB*

    Manifestations of Lyme Borreliosis
    LNBAmerican LNBEuropean LNB
    SubspeciesB burgdorferi sensu strictoB garinii > B afzelii
    LNB/LD<10%>35%
    Painful radiculitis<10%>50%
    Aseptic meningitis at presentationMajorityMinority
    Intrathecal antibodiesMinority>50%
    Cranial neuropathyVII, rarely othersVII and others
    Chronic encephalomyeloradiculitis<0.1%Unusual, <3%
    Erythema migransCommon, <5 cm erythematous patch at the site of tick biteUncommon
    Chronic skin manifestationsNever10% Acrodermatitis chronica atrophicans: chronic extensor surface discoloration
    ± Swelling
    ± Associated peripheral neuropathy
    Associated Lyme arthritisCommon, pauciarticular; knees ∼50%, weeks-to-months postinfectionRare
    CarditisHeart block, transient possible perimyocarditisRare
    • Note:—LD indicates Lyme disease; LNB, Lyme neuroborreliosis.

    • * Adapted from Pachner and Steiner,8 Hengge et al,16 and Stanek.22

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American Journal of Neuroradiology: 30 (6)
American Journal of Neuroradiology
Vol. 30, Issue 6
June 2009
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Lyme Neuroborreliosis: Manifestations of a Rapidly Emerging Zoonosis
P. Hildenbrand, D.E. Craven, R. Jones, P. Nemeskal
American Journal of Neuroradiology Jun 2009, 30 (6) 1079-1087; DOI: 10.3174/ajnr.A1579

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Lyme Neuroborreliosis: Manifestations of a Rapidly Emerging Zoonosis
P. Hildenbrand, D.E. Craven, R. Jones, P. Nemeskal
American Journal of Neuroradiology Jun 2009, 30 (6) 1079-1087; DOI: 10.3174/ajnr.A1579
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  • Article
    • Abstract
    • Epidemiology and Clinical Presentation
    • Diagnostic Tests for Lyme Disease
    • LNB
    • Pathology
    • Peripheral Nervous System Manifestations
    • CNS LNB
    • Spinal Cord LNB
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