ABSTRACT
The 42nd meeting of the Upper Midwest Neuro-Ophthalmology Group (UMNOG) took place on 24 July 2020 in an inaugural virtual format due to COVID-19 precautions. Eighty-seven people attended virtually, including 25 trainees, which marked the highest UMNOG meeting attendance on record. We present a synopsis of the meeting presentations.
KEYWORDS: Proceedings, Midwest, neuro-ophthalmology, UMNOG
The 42nd meeting of the Upper Midwest Neuro-Ophthalmology Group (UMNOG) took place on 24 July 2020 in an inaugural virtual format due to COVID19 precautions (Figure 1). John Chen, MD, PhD, Mayo Clinic, Rochester, Minnesota and Collin McClelland, MD, University of Minnesota, Minnesota co-organised the meeting. Eighty-seven people attended virtually, including 25 trainees, which marked the highest UMNOG meeting attendance on record. There were 22 podium presentations and six flea circus cases to end the meeting.
Figure 1.
A screen shot displaying a group of attendees of the 42nd Meeting of the Upper Midwest Neuro-Ophthalmology Group held virtually for the first time on July 24, 2020
The first presentation was delivered by Randy Kardon MD, PhD, University of Iowa who described a 57-year-old male Veteran with a history of long-standing unilateral caecocentral scotoma attributable to damage of the retinal nerve fibre layer (RNFL) from a military enemy laser. The case highlighted the utilisation of optical coherence tomography (OCT) and segmentation analysis to identify which layers of the retina were damaged and to what extent. Dr. Kardon reminded the audience that laser injuries can occur to the cornea, retina, and optic nerve from a variety of sources (domestic laser pointers or military weapons) and the beam may be invisible to the human eye.
Yibing Zhang, University of Michigan, presented a case series that assessed visual outcomes in surgically treated anterior visual pathway meningiomas. Visual outcomes were better in patients with pre-operative binocular visual dysfunction compared with pre-operative monocular visual dysfunction. Among bilateral cases, visual improvement occurred in 73% and there were no instances of vision loss in the pre-operatively better-sighted eye. However, only a minority of this subgroup (27%) gained sufficient vision to improve activities of daily living. Surprisingly, factors that did not preclude a favourable visual outcome included the presence of optic disc pallor, large tumour size, and pre-operative optic canal involvement on neuro-imaging.
Ivy Dreizin, MD, University of Wisconsin, described a challenging case of a middle-aged woman who presented with progressive vision loss in the right eye followed by the left eye within 3 months. Her magnetic resonance imaging (MRI) revealed a vague area of abnormality at the optic chiasm and adjacent right optic tract and one focal unidentified bright object (UBO). Serum anti-aquaporin 4 and anti-myelin oligodendrocyte glycoprotein (MOG) antibody testing were normal. She had a normal cerebrospinal fluid (CSF) including no oligoclonal bands. Her vision failed to improve with intravenous steroids. She later developed numbness starting in her heels and ascending to the abdomen. Cervical MRI was normal but the thoracic MRI showed a short lesion at T2 with cord expansion. She was diagnosed with an atypical demyelinating disorder. After group discussion, most agreed that treatment with rituximab was indicated to cover for seronegative neuro-myelitis optica (NMO) spectrum disease.
Francisco R. Sanchez Moreno, MD, Mayo Clinic, presented a case series of five male elderly patients with transient bilateral central scotoma on awakening. Neuro-ophthalmic evaluation of these patients was unrevealing (optic nerve, neuroimaging, visual fields). Four of the five (80%) had macular drusen and multifocal electroretinogram (ERG) showed blunted central responses in the two patients that were tested. A dark adaptation curve was obtained on one patient and was also abnormal. This appears to be a different entity than the previously described transient monocular vision loss upon awakening by Bouchard et al. because the symptomology and demographics were different. The aetiology for this phenomenon remains unclear; however, the unrevealing neurologic work-up and lack of progression to permanent vision loss indicated this is a benign diagnosis and based upon the concomitant high rate of subtle macular disease may represent an autoregulatory failure resulting in a supply/demand mismatch of macular photoreceptors during low-light conditions.
Doug Snyder MD, Saint Louis University, described a case of a 58-year-old man who presented with bilateral blurred vision and dyschromatopsia without associated pain on eye movements after taking hydroxychloroquine for a suspected COVID-related infection. His ocular exam was unremarkable and his OCT macula was normal except he had ganglion cell layer loss in both eyes. COVID 19 polymerase chain reaction and antibodies were negative. His MRI was negative and he did not respond to intravenous steroids. Leber’s hereditary optic neuropathy point mutation testing was negative and nutritional optic neuropathy-associated vitamin levels were normal. Hydroxychloroquine associated optic neuropathy was felt by the author to be the likely culprit based upon the exclusion of alternative potential aetiologies and considering the temporal association.
Meari Taguchi MD, Henry Ford Hospital, presented the case of a 72 year-old man with no light perception vision in both eyes with normal pupillary reflexes. He had a significant history of poorly controlled type II diabetes mellitus with proliferative diabetic retinopathy status post pan-retinal photocoagulation. During his admission to the hospital for diabetic ketoacidosis, he had daily episodes of head deviation, unresponsiveness to commands and upper arm stiffness. Electroencephalogram testing revealed the diagnosis of cortical blindness secondary to bilateral occipital status epilepticus. His vision returned to baseline after appropriate treatment with multiple anti-antiepileptic medications.
Judy Chen, MD, PhD, University of Wisconsin, described a 67-year-old white Caucasian female whose past medical history was significant for recurrent sinusitis that presented with acute decreased vision in the left eye. Exam showed a visual acuity of 20/30 and count fingers only in the right and left eyes, respectively, with complete colour vision loss and a relative afferent pupillary defect in the left eye. On fundus examination, she had 360-degree optic disc oedema in the left eye and mild superior optic disc oedema in the right eye. Infectious and inflammatory workup were negative except acute phase reactants (erythrocyte sedimentation rate and C-reactive protein) were markedly elevated. A maxillofacial computed tomography (CT) scan noted complete opacification of the right frontal and maxillary sinuses and near-complete opacification of the right anterior ethmoid air cells. A CT angiogram/CT venogram of the head showed no aneurysm or venous sinus thrombosis. Brain and orbital MRI showed contrast enhancement of the optic nerve sheath bilaterally. The final diagnosis was biopsy-proven giant cell arthritis presenting as bilateral optic peri-neuritis.
John J. Chen, MD, PhD, Mayo Clinic, presented a retrospective study of 115 patients with one or more episodes of MOG-associated optic neuritis (MOG-ON). The average time to recovery of patients treated with intravenous methylprednisolone (IVMP) within 2 days of onset of vision loss was 1.9 days compared with 12.1 days in optic neuritis attacks treated later than 2 days. Those treated within 2 days had less severe visual acuity loss at the time of treatment and better final visual acuity after IVMP compared with those treated later than 2 days. Dr. Chen reported that this data supports the notion that early IVMP leads to faster recovery and better outcomes in MOG-ON. Furthermore, pain often precedes vision loss (on average by 4.2 days) suggesting there may be a therapeutic early window for corticosteroid treatment that reduces the risk of permanent deficits. Providers may in the future consider empirically treating patients with a history of recurrent MOG-ON with a short course of corticosteroids at the onset of eye pain.
John Riley Wilkinson, MD, University of Kentucky, presented a retrospective chart review to assess the utility of enhanced depth imaging OCT (EDI-OCT) in the diagnosis of optic disc drusen (ODD). All eyes in the ODD group who had abnormal fundus autofluorescence (FAF) findings of drusen also demonstrated structures consistent with the ODD on EDI-OCT using criteria from the ODD consortium (hyper-reflective outer margin with signal-poor core). This study showed that EDI-OCT is an effective way to identify optic disc drusen that is not clinically apparent and is useful as an adjunct to FAF.
Sangeeta Khanna, MD, Saint Louis University, discussed a retrospective study that assessed the correlation between double Maddox rod testing, fundus photography, and OCT methods of measuring torsion. Two different methods were used to evaluate the disc fovea angle (DFA) as a marker of objective torsion: OCT DFA and fundus photo DFA which had a high correlation with each other. Skew torsion can be reliably detected by large inter-ocular differences >6 degrees with the DFA in the hypertropic eye trending positive and in the hypotropic eye more negative. Excyclotorsion is hard to accurately assess due to large variation in non-strabismic individuals. Automated undilated OCT-DFA can be used as an accurate tool for measuring objective fundus torsion compared with the dilated fundus exam.
Susan Carleton Benes, MD, Neuro-Ophthalmology and Orbit: Colorado, described a visit to Ethiopia to teach neuro-ophthalmology at local ophthalmology residency programmes and to train a local oculoplastic surgeon to perform optic nerve sheath fenestrations. One of the surgical challenges was poor exposure because the learning surgeon did not possess an appropriate retractor.
Sayyada Hyder, BA, University of Kentucky, presented a case of isolated downbeat nystagmus and its potential association with anti-glutamic acid decarboxylase (GAD) antibody positivity. A paraneoplastic antibody panel was positive for anti-GAD65 antibody however no other features of anti-GAD-associated disease were present. Since the literature has shown that anti-GAD antibodies can decrease the production of gamma-aminobutyric acid and induce Purkinje cells dysfunction leading to vertical nystagmus, anti-GAD antibodies were considered the main culprit for this patient’s presentation despite the lack of other symptoms. No malignancy was found on metastatic work-up. Close monitoring for the ensuing 4 years demonstrated no progression despite a lack of immunotherapy (patient declined).
Jonah Yousif, BS, University of Michigan Kellogg Eye Centre, presented a retrospective study regarding the clinical and imaging features of 75 patients with dorsal midbrain syndrome that included patients with at least one neuro-ophthalmic sign of dorsal midbrain dysfunction. The most common presenting neuro-ophthalmic signs included reduced up-gaze in 93%, horizontal misalignment in 49% and light-near dissociation in 37%. Three or more features of DMS were found in 84% and only 4% exhibited a single finding-reduced up-gaze.
Neena Cherayil, MD, Northwestern University, described a 67-year-old male who was referred for evaluation of blurry vision and intermittent diplopia. He also noted concurrent enlargement of a testicular nodule. On examination, he was found to have bilateral disc oedema and diffuse ophthalmoplegia. Brain and orbital MRI showed diffuse enhancement of the extraocular muscles and thickening of the pachymeninges with infiltration of the right cavernous sinus. Orchiectomy was performed and pathology showed ill-defined necrotising and non-necrotising granulomas. A falx stereotactic intracranial biopsy was performed, which showed a low-grade B-cell proliferation with plasmacytes indicating lymphoma. Finally, a biopsy of skin lesions that appeared simultaneously with the onset of visual symptoms revealed findings of necrobiotic xanthogranuloma – a rare non-Langerhans cell histiocytosis. The patient was placed on rituximab with some improvement of vision.
Collin McClelland, MD, University of Minnesota, presented a 58-year-old male who developed gradually worsening binocular diplopia for 9 months with measurements consistent with a left 4th nerve palsy. The patient had a history of two endoscopic resections of a pituitary adenoma 2 and 2.5 years prior to presentation to neuro-ophthalmology. Maxillofacial CT disclosed opacification of the right maxillary sinus with a downward displacement of the floor of the right orbit, which led to the diagnosis of chronic maxillary atelectasis causing a right hypotropia that was mimicking a left 4th nerve palsy. A discussion of performing an orbital floor reconstruction and/or strabismus surgery after an antrostomy ensued between the participants.
Eileen Feng, BA, University of Michigan, reviewed documentation by emergency medicine physicians and consulting neurologists in the evaluation of diplopia patients. The most common deficiency in ED physicians was the lack of strabismus testing in 51% of the cases followed by a lack of history and strabismus exam in 35% of the cases. In contrast, 52.4% of the consulting neurologists only completed a history and ocular versions exam, while 31% completed a history, ocular versions, and ocular alignment by cover test in primary position without evaluation of eccentric gaze positions. Diagnostic mis-steps accounted for total charges of 116,248.00 USD attributable to unindicated or incorrect studies.
Justin J. Grassmeyer, PhD, University of Nebraska, presented a case of radiation-induced bilateral oculomotor nerve palsy 20 years after radiation treatment. MRI brain showed post-surgical changes related to resection of her frontal lobe astrocytoma and coronal/axial views with the enhancement of both oculomotor nerves. Based on the presentation of chronic, progressive bilateral oculomotor palsy, persistent-isolated oculomotor nerve enhancement on MRI, an otherwise negative work-up, and no response to steroids, a diagnosis of radiation-induced cranial neuropathy was determined.
Jorge Kattah, MD, OSF Illinois Neurological institute, presented a case to illustrate an early ocular motor sign of thiamine deficiency. Dr Kattah presented a video of a patient’s bilateral gaze-holding failure resulting in horizontal gaze paretic nystagmus from thiamine deficiency, which resolved after thiamine replacement. He concluded by proposing that horizontal gaze paretic nystagmus may be a common pre-encephalopathy symptom in thiamine deficiency, which is important to recognise early since these cases can be treated expeditiously to avoid irreversible complications.
Ashwini Kini, MD, University of Kentucky, described a 19-year-old male referred to the neuromuscular clinic for evaluation of peripheral neuropathy and the inability to walk since childhood. Neuro-ophthalmic examination revealed diffuse limitations of eye movements consistent with chronic progressive external ophthalmoplegia. Visual acuity was mildly diminished and OCT showed thinning of the RNFL. Visually evoked potentials were delayed further indicating bilateral optic neuropathy. In addition, ERG showed mild cone dysfunction. Genetic testing was positive for a polymerase gamma (POLG) mutation rendering a final diagnosis of a mitochondrial myopathy secondary to Alpers syndrome. Unfortunately, the patient succumbed at age 27 after going into status epilepticus.
Salma Dawoud, MD, University of Iowa, described the case of a 32-year-old female who at 37 weeks of pregnancy suffered acute ptosis and diplopia consistent with a left oculomotor nerve palsy. MRI brain without contrast showed a prominence in her left anterior clinoid process and diffuse enlargement of the left cavernous sinus suggestive of a clinoid meningioma. Observation was recommended. MRI with contrast after delivery confirmed findings of a clinoid meningioma. Three-week postpartum, without treatment, her left eye ptosis virtually resolved with a mild residual left inferior oblique deficit and a repeat MRI 3-month postpartum showed a decrease in size of the meningioma. This case demonstrates that meningiomas can worsen during pregnancy and can spontaneously regress after delivery. Dr Dawoud then shared a couple of similar cases from the literature as well as the current diagnostic/treatment algorithm for anterior clinoid meningiomas during pregnancy.
Andre Aung, MD, Henry Ford Hospital, presented a 48-year-old female who was admitted to the hospital for pancreatitis and recurrent nausea/vomiting with gradual blurring of vision in both eyes for 1 week. Examination showed horizontal gaze-evoked nystagmus, partial horizontal gaze palsy, and symmetric segmental temporal disc oedema involving the papillomacular bundle of both eyes. MRI brain disclosed a thin T2-hyperintensity in the medial lemniscus. Vitamin levels disclosed decreased B1, B6, and folate levels. Based on her MRI findings and vitamin testing, she was diagnosed with Wernicke’s encephalopathy. The patient was started on intravenous thiamine and transitioned to indefinite oral thiamine with improvement in vision. This case highlights that thiamine deficiency can present with an optic neuropathy and associated optic disc oedema in addition to more typical presenting features of nystagmus and ophthalmoplegia.
Padmaja Sudhakar, MD, University of Kentucky, presented an 87-year-old male with 2 months of vertigo, imbalance, gait difficulties, frequent falls, dysarthria, and diplopia. MRI brain showed abnormal cerebellar lesions. CSF testing showed 1200 copies of JC virus despite previously being apparently immunocompetent. Workup revealed idiopathic lymphocytopenia. Final diagnosis was determined to be progressive multifocal leukoencephalopathy from idiopathic lymphocytopenia that presented with bilateral cerebellar lesions.
Flea Circus Cases
Randy Kardon, MD, University of Iowa, presented a 59-year-old female who was found to have a right occipital lobe meningioma with a left homonymous scotoma after resection. The patient underwent serial OCTs and was found to have progressive homonymous ganglion cell layer thinning that first started 16 months after surgery, highlighting the timeline in one patient between visual cortex injury and transsynaptic degeneration of the retinal ganglion cells.
Aneesh Neekhra, MD, Illinois Retina Institute of Peoria, Illinois, presented a 65-year-old male with deceased vision and bilateral disc oedema. The patient had negative temporal artery biopsy but was found to have a mild elevation of IgG Rocky Mountain spotted fever at 1:256 with normal IgM. It was unclear if this was an atypical case of Rocky Mountain spotted fever or biopsy negative giant cell arteritis.
Judy Chen, MD, University of Wisconsin– Madison, presented a case of apparent response to everolimus therapy in tuberous sclerosis-associated retinal astrocytic hamartoma.
Neena Cherayil, MD, Northwestern University, presented a 19-year-old male with a congenital right 3rd nerve palsy with chronic ptosis and dilated pupil who intermittently has spontaneous adduction and miosis of the pupil consistent with oculomotor palsy with cyclic spasms.
Jorge C Kattah, MD, University of Illinois, shared the video of a 28-year-old myasthenic patient demonstrating that paradoxical fast eye movements can rarely occur in ocular myasthenics. The patient also developed macrosaccadic oscillations after Tensilon testing that lasted for a week, which is a rarely described phenomenon.
Nicholas Volpe, MD, Northwestern University, presented a case of a 27-year-old female with typical idiopathic intracranial hypertension (IIH) who suffered acute worsening of papilloedema and IIH-related symptoms after months of a previously stable course. Dr. Volpe found that the exacerbation occurred following the use of a wraparound constrictive suit after liposuction, which presumably decreased cerebral venous outflow and contributed to worsening IIH.
This concluded the 42nd annual UMNOG meeting. The 43rd annual UMNOG meeting will be held on 23 July 2021. Those interested in attending should contact: Dr. Collin McClelland at cmc@umn.edu or Dr. John Chen at chen.john@mayo.edu
Declaration of interest statement
The authors report no relevant conflicts of interest.