A case of CNS Lyme disease presenting with multiple cranial neuropathies and mimicking a B cell lymphoma
Neurology April 18, 2017 vol. 88 no. 16 Supplement P1.312
The goal of this case is to describe CNS Lyme disease with multiple cranial neuropathies as well as to show how it can mimic a B cell lymphoma.
A 57-year-old male presented with a prodrome of malaise and fevers followed by an intractable right occipital (base of skull) and temporal headache, nuchal (large ligament at back of neck) and radicular (pain radiating from spine often ending in an extremity) pain, and diplopia (double vision). On exam, he had a right third nerve palsy with partial pupil involvement.
With initial concern for aneurysm, an MRA was unrevealing. A peripheral Lyme serology returned positive, and it was decided to pursue contrasted MRI and LP to investigate infectious and inflammatory etiologies.
MRI brain and orbits with contrast revealed enhancement of the left and right CN III, right CN V, and right CN VII. LP showed lymphocytic pleocytosis (WBC 930) and elevated protein (92). HSV and VZV returned negative. CSF Lyme antibodies were positive with a CSF/serum ratio for IgG of 24.8 and for IgA of 1.3. IgM was greater than assay in both the serum and CSF. Cytology and flow cytometry were notable for lambda CD5+ B cell clonality. MRI of the cervical and thoracic spine and CT of the chest, abdomen, and pelvis were unremarkable. While he received empiric ceftriaxone, acyclovir, and one day of high-dose steroids after the LP, he continued on ceftriaxone for 28 days, and his headache and third nerve palsy completely resolved.
This case highlights how Lyme disease should be considered in the differential diagnosis for multiple cranial neuropathies. Further, as prior case reports of CNS Lyme patients have shown polyclonal and monoclonal lymphocyte expansion, this case demonstrates the diagnostic uncertainty regarding whether flow cytometry reflects a reactive process to infection or a new lymphoma. Treatment with antibiotics and clinical improvement helped to clarify the etiology in this case.