Case of the Month
Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO
March 2014
Next Case of the Month coming April 1 . . .
Lyme Neuroborreliosis (LNB)
- LNB is a tick-transmitted (Ixodes) multisystem disease caused by the spirochete Borrelia burgdorferi. It is the most common vector-borne infection in the United States and in some regions of Canada, Europe, and Asia. The third most common affected site is the CNS, which is involved in 10–15 % of infected individuals in both Europe and the United States.
- Approximately 85% of European disease presents with Bannwarth syndrome, a painful lymphocytic meningoradiculitis, with or without paresis. Encephalomyelitis is a very rare complication of progressive course of the disease, and MRI is very helpful in assessing the presence of rare tumefactive WM lesions that may mimic a neoplastic process.
- Clinical Presentation: Nonspecific, including headache, fatigue, cognitive slowing, and memory difficulty
- "Lyme disease can do anything" in the CNS. Lymphocytic meningitis, cranial neuropathy (particularly facial palsy ), and radiculoneuritis (motor or sensory or both) constitute the classic triad of LNB.
- Key Diagnostic Features: LNB is primarily a clinical diagnosis. Laboratory testing [positive Lyme serologies with or without positive (CSF) Lyme antibodies] should support the diagnosis. Diagnosis can be confirmed by IgG Western blot (IgM and IgG immunoblots if early disease is suspected; IgG WB alone if late disease is suspected). On MRI, subcortical and periventricular white matter lesions (≥3 mm in diameter), nerve-root or meningeal enhancement can be seen. Cervical cord pathology is unusual. Some lesions show restricted diffusion and low signal on ADC.
- DDx: Include diseases with neuropathy and/or CSF pleocytosis, such as vasculitic neuropathy, cytomegalovirus infection, tuberculosis, and neurosarcoidosis. A tumefactive lesion can sometimes mimic a primary neoplasm. DDx of bilateral facial palsy includes Guillain-Barre syndrome, AIDS, other meningitis causes, and perineural spread of tumor.
- Rx: Doxycycline, amoxicillin, cefuroxime. Late or severe disease requires intravenous ceftriaxone (2 g/day for 30 days). Single-dose doxycycline (200 mg orally) can be used as prophylaxis in selected patients.
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