An Ironclad Case of Vision Loss

Inpatient evaluation revealed a positive FTA and reactive RPR (1:8). ACE was elevated at 83U/L (reference 9-67 U/L). Bacterial and fungal cultures, cryptococcal antigen and toxoplasma IgG/ IgM antibodies were negative. Quantiferon TB gold was indeterminate. The patient was treated empirically with valgancyclovir, vancomycin, cefazolin, dapsone, azithromycin, fluconazole and, to cover neurosyphilis, IV penicillin. Lumbar puncture revealed a mild pleocytosis of 9 WBCs, protein of 40 and glucose of 47. Gram stain, culture, KOH prep, cryptococcal antigen and HSV, CMV, VZV, EBV, JC virus and toxoplasmosis PCR were negative. VDRL and treponema pallidum DNA PCR were negative. Beta-2 microglobulin was elevated at 2.7 (reference 0-2.4mg/L) but cytology and flow cytometry were negative, revealing only an increased number of benign lymphocytic and monoctyoid cells. CSF ACE was normal at 1.4 U/L, there were no oligoclonal bands, AFB culture showed no growth and MTB amplification was negative. The patient refused a second lumbar puncture, but a vitreous biopsy revealed negative gram stain, culture, KOH prep and treponema pallidum assay. There was an improvement on empiric antibiotics and IV methylprednisolone from CF to 20/80 OS, but this reversed with cessation of steroids. As vision continued to worsen, a biopsy of a small portion of the optic chiasm was performed and revealed lymphocytic infiltrates in a perivascular and parenchymal distribution, with massive demyelination and axonal preservation. Biopsy was negative for spirochetes. However, acid fast stain revealed innumerable bacilli which PCR revealed to be Mycobacterium haemophilum. The patient stabilized on a regimen of azithromycin, rifabutin, moxifloxacin and prednisone. However, seven months later, in the setting of partial non-compliance with the antibiotic regimen, he developed new left optic nerve enhancement, even as the chiasmal disease improved. One month later, with further non-compliance, there was new extension into the hypothalamus.