Uncertainty with a Twist of Lyme (video)

The patient was admitted and started on Ceftriaxone for possible CNS Lyme disease. ID consultation suggested CNS Lyme to be unlikely; antibiotics were stopped. The patient insisted that she had "chronic Lyme disease". MRI orbits demonstrated diffuse enlargement and hyperenhancement of bilateral cranial nerves 3, 5, 7/8 and right cranial nerves 9 /10; mild abnormal enhancement of the superior and inferior colliculus, bilateral optic canals, and right optic nerve. Repeat lumbar puncture again showed elevated WBC count and protein; flow cytometry was negative. CT chest/abdomen/pelvis was negative for occult malignancy. A PET scan showed increased uptake (likely physiologic) in the right breast, with subsequent normal breast MRI. IVIG was initiated for possible autoimmune/inflammatory process. The patient's visual acuity worsened to 20/150 OD and hand motion OS. Leptomeningeal biopsy was recommended, however neurosurgery felt the risks of a biopsy outweighed the benefits and declined. IV methylprednisolone was then initiated (1g /day x 5 days). Subsequent ophthalmologic exam demonstrated improved visual acuity (20/40-2) OD and stable vision OS. PLEX with oral steroid taper was initiated; subsequent exam demonstrated improved visual acuities of 20/25-3 OD and 20/400 eccentrically OS (niph); the bilateral optic disc swelling had resolved. The etiology of her leptomeningeal disease remained unclear. The optic nerve sheath was considered for potential biopsy target and the patient was referred to oculoplastics, however repeat MRI orbits demonstrated resolution of the previously seen abnormal enhancement of the right optic nerve. We again advocated for biopsy of an involved lumbosacral nerve root. Neurosurgery acquiesced and carried out a biopsy of the leptomeninges (L5 rootlet). Pathology demonstrated metastatic carcinoma consistent with invasive lobular carcinoma of breast origin (ER/PR positive; HER2Neu negative). The patient was started on high dose systemic methotrexate x 5 cycles and subsequently letrozole/palbociclib for treatment of metastatic leptomeningeal breast cancer.