Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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August 15, 2024
Neuroborreliosis
- Background:
- Lyme disease is an infectious disease caused by a spirochete of the genus Borrelia, transmitted by an infected tick bite. The initial sign of infection is a flu-like illness and an enlarging skin lesion called erythema migrans. Additionally, cardiac complications (arrhythmia) and Lyme arthritis can be present. The neurologic complications of Lyme disease are named neuroborreliosis, and the central nervous system is affected in 10–15% of cases. The most common neurologic complications are cranial neuritis, meningitis, and radiculoneuritis.
- Clinical Presentation:
- The initial stage includes flu-like illness and skin lesions. Patients can present with peripheral neuropathy, facial nerve palsy, myelopathy, encephalitis, or meningitis.
- Our patient lived in a rural area with dogs, but there was no history of tick bites. Lumbar puncture showed elevated protein values (182 mg/dL) on CSF. Serologic tests for Borrelia Burgdorferi antibodies in the CSF and peripheral blood were positive for IgM and negative for IgG. The serodiagnosis of acute Borrelia infection was confirmed with a Western Blot assay, which was positive for IgM.
- Key Diagnostic Features:
- The imaging findings are variable and include periventricular white matter lesions hyperintense on FLAIR and T2WI, and also spinal cord myelitis, which may enhance, meningeal enhancement, cauda equina enhancement, and cranial nerve enhancement (the most frequently affected is VII, followed by V and III).
- A 2-step serologic testing is recommended for the diagnosis (peripheral blood and/or CSF), first with an enzyme-linked immunosorbent assay (ELISA), and, if positive, a confirmatory Western Blot.
- Differential Diagnosis:
- Bilateral facial nerve palsy is a rare condition and is frequently associated with an underlying pathology such as: Guillain Barré syndrome (shows conus medullaris and cauda equina nerve root enhancement; CN VII is the most common cranial nerve affected), sarcoidosis (dura-arachnoid thickening, cranial nerve and leptomeningeal enhancement, diffuse or focal, and pituitary stalk/hypothalamus thickening), leukemia (meningeal dural-based or pial tumor are > intraparenchymal lesions; frequently hyperdense on CT, T2/FLAIR iso- to hypointense, and with restricted diffusion on DWI), and vasculitis (T2/FLAIR lesions with arterial narrowing on DSA and smooth, homogeneous, concentric arterial wall thickening in VWI).
- Treatment:
- Most cases of Lyme disease can be treated successfully with a few weeks of antibiotics (doxycycline and amoxicillin, cefuroxime axetil, or phenoxymethylpenicillin).
- This patient was immediately started on targeted antibiotic therapy with progressive resolution of the neurologic deficits. This reinforces the importance of high clinical suspicion and prompt etiologic investigation, through laboratory tests and MRI, which in this case lead to adequate treatment and prevention of long-term sequelae.
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