A 14-year-old girl presents with headache, visual loss to counting fingers vision, and severe bilateral disc edema with macular star figure OU. The outside ophthalmologist makes the diagnosis of “bilateral neuroretinitis” due to cat scratch disease and sends the Bartonella titer. There is an incomitant esotropia and the secondary diagnosis of “sensory esotropia” is made. The cat scratch titer returns 2 weeks later but is completely negative. The patient is referred to the neuro-ophthalmology service where an MRI reveals a large intracranial tumor producing papilledema OU with significant leakage of exudate into the macula bilaterally.\nBilateral optic disc edema with a macular star figure (ODEMS) is NOT by itself diagnostic for infectious neuroretinitis. The ophthalmoscopic findings are not pathognemonic and papilledema can mimic the fundus appearance of ODEMS. It is my recommendation to consider neuroimaging for all bilateral optic disc edema cases.
A 25-year-old woman presents with chronic and unresolved loss of vision OD that occurred acutely two months prior to presentation. There is a right RAPD, a central scotoma OD on visual field testing, and a normal optic disc. Computed tomography (CT) scan of the head in the emergency room was normal. A diagnosis of chronic optic neuritis” is made.\nChronic “optic neuritis” is a real diagnosis but the clinician should be careful to insure that the patient has a confirmed diagnosis of multiple sclerosis and no alternative etiology for the optic neuropathy. Typical optic neuritis resolves (20/40 or better vision) in over 90% of cases and lack of improvement should be a big “red flag” that the diagnosis is incorrect. In my experience patients with chronic”“optic neuritis” due to demyelinating disease often have a systemic MS course that mirrors the course of their optic neuropathy (i.e., primary or secondary progressive rather than relapsing-remitting course). One should also be cautious of using the term “optic neuritis” when one truly means optic neuropathy. Misusing or mixing terms (e.g., papilledema) or creating neologisms (e.g., “ischemic optic neuritis”) also is potentially misleading to your colleagues. The bottom line is that typical optic neuritis usually gets better over time and patients with an atypical course (e.g., “chronic optic neuritis”) should have evaluation for alternative etiologies for their optic neuropathy including neuroimaging. There are potentially dangerous conditions that could mimic the presentation of acute painful optic neuropathy and suggest a diagnosis of demyelinating optic neuritis (e.g., pituitary apoplexy or ophthalmic artery aneurysm). In addition there are subsets of variants of demyelinating optic neuropathy that may not have as favorable a rate of recovery and therefore might suggest alternative diagnoses (e.g., neuromyelitis optica). Finally, some patients with the mitochondrial disorder, Leber hereditary optic neuropathy (bilateral acute visual loss with cecocentral scotomas in a young male) can be associated with MS like illness. Although rare, I would accept the diagnosis of “chronic optic neuritis” as a diagnosis of exclusion if a high quality MR scan of the head and orbit with gadolinium has excluded a compressive lesion, the patient has clear diagnosis of prior multiple sclerosis, and alternative MS mimics have been considered and adequately excluded. I typically will inform the treating neurologist of the findings and my concerns and if there is any history of transverse myelitis or if the optic neuropathy is bilateral then I would consider evaluation for neuromyelitis optica (NMO) including NMO antibody testing. I would also consider ordering the mitochondrial genetic testing for Leber hereditary optic neuropathy if the patient has bilateral central or cecocentral scotomas especially in a young man.