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Neurosyphilis is the clinical consequence of Treponema pallidum nervous system infection in any stage of the disease. It can reveal parenchymal metabolism changes in single-photon emission computed tomography (SPECT) images and cause optic nerve involvement, which may be unilateral or bilateral and manifest as perineuritis, papilloedema or as anterior/ retrobulbar optic neuritis. The last one usually leads to rapid visual failure.

A 58-year-old woman reported recurrent chest pain and dyspnea on efforts for the last 2 months and a history of bilateral subacute reduction in visual acuity, with a 30-days evolution to amaurosis 20 years before, with no clear etiology. In the neurological exam, we found bilateral amaurosis, absence of photomotor reflex and multidirectional nystagmus. Fundoscopy revealed pallor of both optical discs. Montreal Cognitive Assessment was 16/30 (8 points lost due to amaurosis, 3 points to calculations and 2 points to late evocation). Upon investigation, ascending aorta ectasia and moderate aortic insufficiency were identified. Serum VDRL (1/256) and FTA Abs IgM and IgG were positive. Cerebrospinal fluid revealed 16 cells/mm³ (92% mononuclear), protein levels of 63 mg/dL and positive VDRL (1/16). Magnetic resonance imaging of the brain and orbits showed atrophy of the optic nerves and optic chiasm and mild biparietal atrophy. SPECT revealed posterior temporoparietal and anterior temporal lobes hypometabolism. She was then diagnosed with tertiary syphilis and treated with crystalline penicillin 4.000.000 U 4/4h for 14 days, without improvement of the symptoms.