Hello everyone,
I would appreciate your thoughts on this complex case. (Sorry for the long email.)
76 years-old man presents in June 2025 for 3rd opinion of progressive painless insidious vision loss of both eyes which started ~ 2020. He notices peripheral>central blurred vision subjectively. No prior episodes of transient vision loss or pain with eye movements. No head/orbital trauma. Had a tick bite with rash ~ 10 years ago, treated, Lyme was negative. COVID and COVID Does not recall a significant event prior to vision loss. There have been no clinical neurological symptoms. Initial available exam in late 2022, revealed bilateral optic neuropathy (diffuse RNFL and GCL thinning, dyschromatopsia).
Reportedly had high myopia before cataract surgery OU.
BCVA 20/70 OD, 20/80 OS, Ishihara 1/11 OD, 3/11 OS. No RAPD.
OCT RNFL: severe diffuse RNFL and GCL thinning OU.
24-2 VF:
Extensive serologic work up over the past two years have been negative.
He has undergone lumbar puncture x2, which revealed elevated CSF protein (119 and 91) otherwise normal.
CT C/A/P negative for occult malignancy. Has a 4.6 cm abdominal aortic aneurysm.
(2023-2025) Work up summarized below:
Neuroimaging:
- 12/1/2023 MRI thoracic spine with without contrast: Normal spinal cord signal, morphology and postcontrast. Capacious spinal canal. No herniated disc or significant disc bulging. Mild/moderate facet arthropathy. Mild osteoarthritis of costovertebral costotransverse joints.
- 12/1/2023 MRI C-spine with and without contrast: 1. Normal spinal cord signal and morphology. Normal postcontrast imaging. 2. Generally moderate diffuse spondylosis with facet arthropathy. 3. Anterior spinal cord flattening secondary to spondylitic ridging at C3-4, C4-5 and C5-6 without altered cord signal. 4. No herniated disks. 5. Normal paravertebral soft tissues.
- 10/6/2023 MRI brain and orbits with and without contrast: 1. Optic nerve and chiasm atrophy. 2. Otherwise, normal orbital contents. 3. Moderate cerebral atrophy. 4. Mild chronic microangiopathy. 5. No acute intracranial process.
Pertinent labs/tests:
- 5/2023: Negative/WNL folic acid, B12, MMA, platelets, copper, syphilis, Lyme, B1
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10/2023 Lumbar puncture (per chart review, I don't have the results): opening pressure 12 (prone), negative OCB but elevated IgG synthesis rate (12.6) and IgG index (0.63). CSF protein elevated to 119. Normal glucose. Only 1 WBC. Gram stain, culture and fungal culture negative. Mayo autoimmune/paraneoplastic panel - negative in CSF (Mayo ENC2).
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12/2023: "Formal neuropsych testing showed low-average scores, suggested cortical asymmetry more pronounced to right hemisphere." MMSE 28/30.
- 12/8/2023 EMG/NCS: 1. mild sensorimotor peripheral neuropathy, demyelinating and axonal in nature. 2. there is no evidence of active denervation or myopathy.
- 12/8/2023 VEP: Abnormal, delayed N75, P100, and N145 latencies with both left and right eye stimulation.
- 3/2024: Negative/WNL AQP4 (CBA in serum), MOG-IgG (CBA in serum), paraneoplastic/autoimmune panel (Recoverin ab negative, no CRMP5 or GFAP), arsenic, Mercury, lead, HIV, lyme, bartonella, Quantiferon, ANA, ANCA, ACE, dsDNA, RF, SPEP, HgbA1C, ESR, CRP, SSA, SSB
- 12/2024 Lumbar puncture: RBC 19, WBC 2 (57% lymph), protein 91, glu normal,
He was started on Brimonidine by his previous neuro-ophthalmologist for neuro protection, and I advised to continue.
Already on ASA.
Has sleep apnea, not wearing CPAP, advised to get re-evaluated for mask.
I referred to glaucoma for evaluation of normal tension glaucoma (although GCL loss is way out of proportion).
I would really appreciate your thoughts and recommendations whether there is something else I could check or repeat.
He was also interested in clinical trials for optic neuropathies.
Thank you,
Negar
Negar Moheb, M.D.
Co-Director, Neuro-Ophthalmology Section
Assistant Professor of Neurology
Lehigh Valley Fleming Neuroscience Institute
1250 S Cedar Crest Blvd, Suite 405
Allentown, PA 18103
T 610-402-8420 | F 610-402-1689
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