A 17-year-old girl presented with acute severe holocranial headache for 3 weeks, and loss of vision for 12 days. Loss of vision was acute, bilateral, and painless, which rapidly progressed to complete loss of light perception within 2-3 days. She also had myalgias, but did not have a fever. Her pupils were sluggishly reactive to light. There was bilateral lateral rectus palsy and signs of meningeal irritation. Slit-lamp examination was normal, while fundus examination revealed signs of papillitis. Her cerebrospinal fluid (CSF) analysis was inflammatory (Cells-35, 95% lymphomononuclear cells, protein-76 mg/ dL, glucose-54 mg/dL, and corresponding blood glucose: 102 mg/dL). CSF India ink staining, cryptococcal antigen, CBNAAT for M. tuberculosis, free living microorganism, Gram stain, and microbial cultures were negative. Magnetic resonance (MR) imaging of brain revealed minimal leptomeningeal enhancement in bilateral parafalcine region, and minimal exudates anterior to the midbrain [Figure 1a-c], while spinal cord imaging was normal. Serology for HIV, VDRL aquaporin-4 antibody, ANA, ANCA, and ACE levels were non-contributory. Based on existing evidences, and high prevalence of tuberculosis in India, anti-tubercular therapy (except ethambutol) with oral corticosteroid was started.
However, on follow-up visit 2 weeks later, the patient had deteriorated with onset of seizures, left facial palsy, extremely severe radicular pains in both lower limbs, urinary retention, and arthritis involving predominantly large joints. Thereafter, she also developed bilateral sensorineural hearing loss. Her serum lactate level was normal. Repeated ophthalmological evaluation did not reveal ocular inflammatory changes. There was no effusion in the symptomatic Joints. CSF analysis was repeated which yielded similar results. Repeated MR neuroimaging showed a marked increase in the radiological features ([Figure 1a-c] represents first neuroimaging, while [Figure 1d-f] represents the second neuroimaging). There was an acute infarct in left corona radiata [Figure 1d], T2-weighted and fluid-attenuated inversion recovery (FLAIR) hyperintensities in bilateral basal ganglia [Figure 1d and e], along with meningeal enhancement in bilateral parafalcine, frontoparietal lobes, bilateral sylvian fissures, basal cisterns, bilateral cerebellar folia, interpeduncular cistern, and along multiple cranial nerves [Figure 2]. There was thin linear post-contrast leptomeningeal enhancement involving entire spinal cord and nodular enhancement in D12-L1 vertebral level [Figure 3]. Most interestingly, she had a rounded M pattern of contrast enhancement in the brainstem [Figures 3]. Having a strong suspicion for Lyme's Neuroborreliosis (LNB), enzyme immunoassay for both IgM and IgG was sent which came out to be positive.
Ceftriaxone and doxycycline were started, and thereafter, the patient showed marked clinical improvement. Antibiotics were continued for 6 weeks in view of extensive neurological involvement. There was reduction in radiological abnormalities as well [Figure 4]. However, optic atrophy had already occurred, and she remained with residual visual deficit.