Diagnostic Challenges of Lyme Neuroborreliosis in Inpatient Neurology: A Case Series

Abstract

Lyme disease is a multisystem disorder transmitted through the Ixodes tick and is most commonly diagnosed in northeastern and mid-Atlantic states, Wisconsin, and Minnesota, though its disease borders are expanding in the setting of climate change. Approximately 10%-15% of untreated Lyme disease cases will develop neurologic manifestations of Lyme neuroborreliosis (LNB). Due to varying presentations, LNB presents diagnostic challenges and is associated with a delay to treatment. We discuss three cases of LNB admitted to our referral center in a traditionally low-incidence state to highlight clinical pearls in LNB diagnosis. Three patients from low-incidence areas with prior diagnostic evaluations presented in August with neurologic manifestations of radiculoneuritis, cranial neuropathies, and/or lymphocytic meningitis. MRI findings included cranial nerve, nerve root, and leptomeningeal enhancement leading to broad differential diagnoses. Lumbar puncture demonstrated lymphocytic pleocytosis (range 85-753 cells/uL) and elevated protein (87-318 mg/dL). Each patient tested positive for Lyme on two-tiered serum testing and was diagnosed with LNB. All three cases were associated with a delay to health care presentation (mean 20 days) and a delay to diagnosis and treatment (mean 54 days) due to under-recognition and ongoing evaluation. With the geographic expansion of Lyme disease, increasing awareness of LNB manifestations and acquiring detailed travel histories in low-incidence areas is crucial to prompt delivery of care. Clinicians should be aware of two-tiered serum diagnostic requirements and use adjunctive studies such as lumbar puncture and MRI to eliminate other diagnoses. Treatment with an appropriate course of antibiotics leads to robust improvement in neurological symptoms.

Keywords: central nervous system bacterial infections; central nervous system infections; infectious disease medicine; neurohospitalist.

Figure 1.

Centers for Disease Control and Prevention (CDC) Lyme disease surveillance maps. Lyme disease increased in incidence and geographically expanded from 2001 (a) to 2021 (b). 1 green dot = 1 case of Lyme disease as reported to the CDC. Blue state = high incidence state.

Figure 2.

MRI brain and cervical spine images pre- and post-antibiotic treatment. Case 1 MRI brain pre- (a) and post-treatment (b) shows improvement in bilateral trigeminal nerve enhancement. Case 1 MRI cervical spine pre- (c) and post-treatment (d) shows resolving leptomeningeal enhancement.

Figure 3.

MRI lumbar spine images. MRI lumbar spine pre- (a & c) and post-contrast (b & d) shows smooth diffuse enhancement of the cauda equina nerve roots in Case 2 and 3.