Phosphorylated Neurofilament Heavy Chain Correlations to Visual Function, Optical Coherence Tomography, and Treatment

Objective. To correlate visual and neurologic clinical scores and treatment of optic neuritis and multiple sclerosis (MS) patients with assays of serum phosphorylated neurofilament heavy chain (pNF-H) and optical coherence tomography (OCT) measurements of axonal loss. Design/Methods. The Optic Neuritis Treatment Trial (ONTT) randomized 457 patients with acute optic neuritis to intravenous methylprednisolone (IVMP) followed by oral prednisone, oral prednisone or placebo treatment arms. We quantified serum pNF-H levels in 175 ONTT patients 5 years after study entry. We performed OCT measurements of macular volume and the retinal nerve fiber layer (RNFL) in a subset of 51 patients at year 15. Results. Elevated pNF-H levels at year 5 correlated to poorer visual function at study entry. Lower 15 year macular volumes and RNFL thickness correlated better with follow-up than with baseline visual function measures. With IVMP treatment, 15 year RNFL differences of the fellow eye (FE) minus the affected eye (SE) RNFLFEmSE correlated with five-year pNF-H levels. PNF-H was reduced by half with IVMP relative to placebo or by 40% relative to prednisone. Conclusions/Relevance. Acute optic neuritis patients who have more severe visual loss during initial presentation have a higher incidence of axonal loss that was slightly suppressed with IVMP treatment

A Shot in the Dark

History & ExamA 74 year-old man with a chief complaint of difficulty with night time driving for several years as wellas difficulty going from a lighted room to a dark room. PMH of high cholesterol, BPH, hypothyroidism,GERD, glottic squamous cell cancer without recurrence, and a prior history of alcoholism. POHx:cataract extraction right eye and AMD. Medications includeMultivitamin, Aspirin, Synthroid, Tamsulosin, Omeprazole, Crestor. Drinks wine intermittently andstopped drinking after possible DT's. He owns a bar. His initial exam VA: 20/25 OU, normal IHCP,CFVF: OD constricted, OS normal. Pupils with no APD but were sluggish. IOP's 13 mm HgOU. Motility showed some slight decreased abduction OU: 5ET primary, 10ET left, 5ET right gazes.SLE: PCIOL OD, NS cataract OS. Optic nerves had 0.7 cups with difficulty in appreciating any pallor.He had a few RPE macular changes OU. The HVF appeared reliable with severe constriction OD>OS(MD -29 and -17 respectively). RNFL OCT showed a mean thickness of 69 and 61 micronsrespectively. Testing was later done

Neuro-ophthalmic disease and optical coherence tomography: glaucoma look-alikes

The use of optical coherence tomography (OCT)-measured retinal nerve fiber layer (RNFL) thickness in neuro-ophthalmic disease has grown since its first use in glaucoma and retinal diseases. OCT-measured RNFL in nonglaucomatous optic neuropathies shows thinning, which may mimic those seen in glaucoma. This article aims to provide insight regarding the use of OCT in nonglaucomatous optic neuropathies and sheds light on common patterns of RNFL loss in different nonglaucomatous optic neuropathies. RNFL thinning is most likely to occur in the temporal peripapillary quadrant than in other quadrants in nonglaucomatous optic neuropathies. The pattern of RNFL thinning in ischemic optic neuropathy and optic nerve head drusen is more likely to mimic the pattern found in glaucoma due to the superior and inferior quadrant predilection. OCT-measured RNFL thickness in Alzheimer's disease reveals thinning superiorly and inferiorly, whereas superior and temporal thinning is seen in Parkinson's disease. The thinning observed in neurodegenerative diseases is believed to be multifactorial including causes such as axonal degeneration and retrograde degeneration. However, more studies are needed to further study these changes. OCT is a valuable tool in evaluating the peripapillary RNFL in both glaucomatous and nonglaucomatous optic neuropathies. This technology may be used for both research and clinical purposes to assess disease progression in optic neuropathies and diseases that affect the central nervous system. OCT-measured RNFL thickness remains complimentary to the clinical examination skills in the evaluation of nonglaucomatous optic neuropathies

Visual loss due to intracranial hypotension in a patient with optic nerve head drusen

A 58 year old woman with a history of ONH drusen complained of worsening vision OU and headaches. Initial examination revealed visual acuity was 20/60 OD and 20/30 OS, decreased color plates OD, constricted visual fields to confrontation OU, and no afferent pupillary defect. Optic nerves appeared pallorous with obvious ONH drusen. Three months later the visual acuity was noted to be hand motions OD and 20/200 OS. Confrontation visual at that time revealed hand motions centrally OD and count fingers centrally OS. No color plates were detected OU. Optic nerve exam was unchanged. Further testing with MRI of the brain and orbits revealed meningeal enhancement, transtentorial herniation of the left temporal lobe, cerebellar herniation through the foramen magnum, and chiasmal sagging with venous congestion through the optic canals. These findings, together with the patients headache were consistent with intracranial hypotension. MRV did not show any sinus thromboses. Whole spine MRI and CT myelography failed to show any dural tears. Opening pressure was not checked at the time of myelography, but the CSF consistency was normal. The patient was treated with lumbar epidural blood patching. The patients headache resolved the same day as the procedure. On follow up, the headaches resolved and the vision improved to 20/800 OD and 20/70 OS. Repeat MRI of the brain revealed resolution of the meningeal enhancement, chiasmal sagging, cerebellar herniation, and temporal lobe herniation

A Shot in the Dark

A 74 year-old man with a chief complaint of difficulty with night time driving for several years as well as difficulty going from a lighted room to a dark room. PMH of high cholesterol, BPH, hypothyroidism, GERD, glottic squamous cell cancer without recurrence, and a prior history of alcoholism. POHx: cataract extraction right eye and AMD. Medications include Multivitamin, Aspirin, Synthroid, Tamsulosin, Omeprazole, Crestor. Drinks wine intermittently and stopped drinking after possible DT's. He owns a bar. His initial exam VA: 20/25 OU, normal IHCP, CFVF: OD constricted, OS normal. Pupils with no APD but were sluggish. IOP's 13 mm Hg OU. Motility showed some slight decreased abduction OU: 5ET primary, 10ET left, 5ET right gazes. SLE: PCIOL OD, NS cataract OS. Optic nerves had 0.7 cups with difficulty in appreciating any pallor. He had a few RPE macular changes OU. The HVF appeared reliable with severe constriction OD>OS (MD -29 and -17 respectively). RNFL OCT showed a mean thickness of 69 and 61 microns respectively. Testing was later done

Ganglion cell and retinal nerve fiber layer analysis in a case of PION

"Posterior Ischemic Optic Neuropathy (PION) is a retro bulbar optic nerve ischemia presenting with sudden, painless visual loss with the presence of decreased vision, visual field defect, an afferent pupillary defect, yet the retina and optic nerve appear normal initially. PION can be seen in isolation, as part of giant cell arteritis, or post surgical. A PubMed search does not reveal any cases of OCT in the phases of PION. Ganglion cell OCT can help determine when ganglion cell thinning occurs in ischemic optic neuropathy. A case of PION and OCT is presented.

Childish Nerve

Loss of vision ODA 75-year old male with a sudden loss of vision OD.VA: NLP OD, 20/25 OS with correction; RAPD ODMRIInflammatory compression of optic nerve by Rosenthal fibers and fibrillary cells.Steroids1. Hoyama, Cruz , Colli , Matos , Chahud, Isolated low grade pilocytic astrocytoma of the optic nerve in the elderly: case report, Arq Bras Oftalmol. Vol 71(1):97-100, 2008

Cancer Associated Retinopathy (CAR) Presenting Two Years Prior to Diagnosis of Large Cell Neuroendocrine Carcinoma of Unknown Primary

Cancer associated retinopathy (CAR) is a para-neoplastic disease affecting the retina leading to progressive visual loss. CAR is typically associated with small cell lung cancer and gynecologic malignancies and typically precedes the diagnosis of the malignancy

Childish Nerve (Audio)

Loss of vision ODA 75-year old male with a sudden loss of vision OD.VA: NLP OD, 20/25 OS with correction; RAPD ODMRIInflammatory compression of optic nerve by Rosenthal fibers and fibrillary cells.SteroidsAttache