The 41st meeting of the Upper Midwest Neuro-Ophthalmology Group (UMNOG) took place on July 26, 2019 in Chicago, Illinois. It was hosted by Nicholas Volpe, MD and the Department of Ophthalmology of the Northwestern University School of Medicine. John Chen, MD, PhD, Mayo Clinic, Rochester, Minnesota, and Collin McClelland, MD, University of Minnesota, Minnesota organised the meeting along with co-chairs Nicholas Volpe, MD, Susan Ksiazek, MD, and treasurer, Peter MacIntosh, MD. Around 75 people attended including ophthalmologists, neurologists, neurosurgeons, and neuroradiologists in academic or community practice. This year marked a record attendance of physicians in their fellowship or residency training and medical students. There were 29 podium presentations, the highest so far for UMNOG meetings.
The first presentation was by Marilyn Kay, MD, University of Wisconsin, who described a 49-year-old man with progressive vision loss for several years with findings of bilateral optic neuropathy. He also had progressive peripheral sensory changes that were initially attributed to carpal tunnel disease and lower back disc disease. A genetic consultation resulted in a diagnosis of autosomal recessive biotinidase deficiency. Dr. Kay reminded us that it was just recently that biotin level testing was added to the newborn screening protocol; by 2015 all states in the US had incorporated the test. While biotin deficiency usually presents in childhood, rarely symptom onset can occur in adulthood and therefore biotin deficiency should be considered in the differential of optic neuropathy with associated systemic features in those not screened.
Aaron M. Fairbanks, MD, Mayo Clinic, presented a population-based study on the incidence and visual outcomes of cerebral venous sinus thrombosis (CVST). He reported an incidence of 1.47/100,000 per year (95% confidence intervals: 1.03–1.91), similar to values from the international literature. About half of the women were taking hormonal treatment. All except two patients had good visual outcomes with a median visual acuity of 20/20. One patient required surgical intervention. While audience members articulated that they had often seen poor visual outcomes in CVST, the discussion was that when studied in a population-based manner, the visual outcomes were overall good. It was acknowledged that poor outcomes can occur with CVST, but academic centres may have referral bias towards more severe disease.
Anthony Brune, DO, University of Michigan, presented a research study on the complications of ventriculo-peritoneal shunting for idiopathic intracranial hypertension in 32 patients, the largest retrospective series to date studying this question. He reported an 18.7% failure rate, which included obstruction, over-drainage and infection; this was lower than values previously published. He did, however, report 38 false alarms among the patients, mentioning that the placement of a shunt led to increased medical encounters because of the fear of complications.
Megan Bill, DO, Indiana University, described three patients with pre-retinal haemorrhages in the setting of raised intracranial pressure from different causes without intracranial haemorrhage. She hypothesised that the intracranial hypertension must be getting transmitted to the optic nerve sheath and retinal veins causing capillary rupture. None of the three patients suffered from permanent visual loss.
John Chen, MD, PhD, Mayo Clinic, presented a study on the population-based incidence of optic neuritis in the era of aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) antibodies. He reported an incidence of 3.7 per 100,000 individuals. Multiple sclerosis and idiopathic optic neuritis remained the most common aetiologies. MOG-IgG disease accounted for 5% of optic neuritis cases, and AQP4-IgG seropositive neuromyelitis optica spectrum disorder (NMOSD) accounted for 3%. All patients with NMOSD and majority of the patients with MOG-IgG disease had recurrent optic neuritis, but the patients with MOG-IgG associated optic neuritis had better visual outcomes; thus confirming what non-population-based studies have shown.
J. Brett Farmer, MD, University of Kentucky, discussed two cases of craniopharyngiomas presenting in young patients with obstructive hydrocephalus and papilloedema with one patient ending up with light perception vision, reminding the audience of the various ophthalmic manifestations of these tumours.
Yibing Zhang, BA, University of Michigan, presented a study on the visual outcomes after surgical resection of anterior visual pathway meningiomas. She found that nearly three-fourths of the patients had improvement in vision following the surgery, while about 15% were stable and 15% worsened. While none of the pre-operative ophthalmic or imaging features were predictive, post-operative reduction in intracranial optic nerve or chiasm displacement were found to be modest predictors of favourable outcome, thus providing some guidance to surgeons and ophthalmologists caring for these patients.
Padmaja Sudhakar, MD, University of Kentucky, presented a complex case of a 54-year-old patient with features of progressively worsening multiple cranial neuropathy, weight loss and chronic cough. Extensive work-up revealed an upper lobe lung cavitary lesion with hypermetabolic mediastinal lymph nodes. Finally, disseminated mycobacterium avium-intracellulare was diagnosed based on the biopsy from the lesion and treatment was started, but the patient expired soon after. This rare disease can be challenging to diagnose and Dr. Sudhakar reflected that delay in the diagnosis, empiric steroid treatment started elsewhere, and delay in starting the antibiotics likely contributed to the poor outcome.
Sophia Chung, MD, presented two patients with visual complaints initially thought to be multiple sclerosis, but later diagnosed as myelin oligodendrocyte glycoprotein (MOG-IgG) associated disorder. The audience was reminded of features which should alert the clinician to a diagnosis of MOG-IgG associated disease such as recurrence of inflammation with discontinuation of steroids, and perineural and long segment involvement of the optic nerve.
Sasha Mansukhani, MBBS, Mayo Clinic, presented a case of vigorous pupillary unrest associated with pain and alternating anisocoria in a 24-year-old patient with a background history of postural orthostatic tachycardia syndrome (POTS). While eye drops (brimonidine, phenylephrine) acting on the sympathetic system did not abolish the unrest, tropicamide eye drops acting on the parasympathetic system (PNS) did, thus localising the fluctuations to the PNS. It remains unclear whether her history of POTS and oral use of fluoxetine were contributory to the prominent pupillary unrest. There remains more to be understood about pupillary oscillations.
Nicholas Volpe, MD, Northwestern University, studied the role of automated pupillometry (AP) as an adjunct to clinical examination in patients with acute vision loss with the help of a survey, responses to clinical vignettes and a clinical study. He summarized the results of an electronic survey reporting that clinicians were “neutral” to “somewhat likely” to use AP. The clinicians trusted AP over the swinging flash light test (SFT), and in the clinical study, 50% of relative afferent pupillary defects detected by AP were not detected on SFT. Despite the superiority of AP to SFT done by technicians, ophthalmologists rated AP as only “neutral” or “somewhat useful”. He concluded that though a useful tool, there are many barriers to widespread AP adoption, including a low perceived value.
Deena Tajfirouz, MD, Mayo Clinic, presented a patient with idiopathic Horner’s syndrome who was prescribed apraclonidine eye drops for symptomatic relief of his ptosis. When seen a year later, he had developed tachyphylaxis to apraclonidine with loss of the mydriatic effect and little effect on the ptosis that had been evident at initial presentation. After a drug holiday for three months, a repeat trial showed a return of the super-sensitivity.
Edward Cohn, MD, MBA, MPH, a neuro-ophthalmologist from Royal Oak, Michigan discussed a potpourri of three interesting cases. A 49-year-old patient with gradually progressive vision loss and right optic nerve pallor was found on neuroimaging to have a compressive optic neuropathy from a right anterior communicating artery aneurysm. The second patient was a 33-year-old male presenting with headaches and diplopia, whose ocular examination was indicative of dorsal midbrain syndrome. He was found to have a pineal region mass on magnetic resonance imaging. The third was an impressive video of volitional nystagmus.
Tatiana Deveney, MD, University of Michigan, described a 67-year-old female who presented with episodic transient right-sided vision loss with associated headaches. She had been treated in 1992 with whole brain radiation for a presumed left temporal fossa meningioma. Neuroimaging performed for her current symptoms revealed new oedema in the posterior left occipital lobe and occlusion of the left transverse sinus on cerebral angiography. Electroencephalography showed left cerebral hemisphere slowing. While cortical venous sinus thrombosis and focal encephalitis were on the differential, she was eventually thought to have stroke-like migraine attacks after radiation therapy (SMART) syndrome, a rare condition with only about 100 cases reported in the literature.
Collin McClelland, MD, University of Minnesota, presented an 80-year-old woman referred for recurrent right eye amaurosis that she described as ‘looking through a glass of milk’. She had suffered a total of 30 episodes over 10 months. Evaluation for transient ischaemic attack (TIA) and giant cell arteritis had not revealed an aetiology. Slit lamp evaluation showed right eye trace flare and cell, and iris transillumination which led to the diagnosis of uveitis-glaucoma-hyphema (UGH) syndrome due to a tilted acrylic intraocular lens. This was confirmed by ultrasound biomicroscopy. Dr. McClelland reminded the audience that ‘white outs’ are frequent in UGH syndrome and can mimic symptoms of TIA and slit-lamp findings can be subtle.
Emily Eton, MD, University of Michigan, described a three-month-old infant who developed bilateral retrogeniculate blindness from an acute stroke in the domain of the left posterior cerebral artery. The restricted diffusion extended across the midline in the splenium of the corpus callosum, demonstrating cerebral diaschisis and providing an explanation for the complete blindness from a unilateral injury.
Robert Tomsak, MD, PhD, Kresge Eye Institute, presented the features of a few patients with a small disc on optical coherence tomography (OCT) including various visual field defects. He summarised the findings in the literature and concluded that a disc area of less than 1.4 mm2 on OCT could be considered as a small disc. However, there is a range of normal and hypoplastic optic nerves and there is overlap between small “normal” optic nerves and hypoplastic optic nerves.
Walter Jay, MD, Loyola University, presented a tribute to Dr. Bill Hoyt and narrated stories and anecdotes. Dr. Jay shared Bill Hoyt’s love for skiing and origins of the Rocky Mountains ski meeting. Dr. Hoyt disclosed to Dr. Jay that the fellows that he had trained were his biggest accomplishment. The group observed a moment of silence in his memory.
Jorge Kattah, MD, OSF Illinois Neurological Institute, presented his study on the presence of ocular lateropulsion (OL) in a cohort of 147 patients with acute vestibular syndrome. He reported that 10 (5.35%) patients had OL and all patients with OL and lateral medullary syndrome had hypometric, centripetal, corrective saccades. He concluded that although infrequent, OL had importance in localisation and pointed towards a central cause of acute vestibular syndrome.
Megan Tuohy, MD, University of Michigan, described a 59-year-old diagnosed with Graves’ disease and compressive optic neuropathy, who was treated with steroids and orbital radiation. He continued to have progressive visual loss and developed new symptoms of diplopia, urinary retention and dizziness. Neuroimaging demonstrated enhancement of cranial nerves three, seven, and eight and the cauda equina. Repeat fundus examination showing confluent retinal lesions with intraretinal haemorrhages directed further testing, leading to the diagnosis of disseminated varicella zoster virus vasculopathy.
Randy Kardon, MD, PhD, University of Iowa, described a patient referred for progressive esotropia and bilateral abduction deficits with reportedly normal neuroimaging. Unilateral raised intraocular pressure with widened ocular pulse amplitude on Goldmann tonometry was the key finding that pointed towards the diagnosis of carotid cavernous fistula (CCF). Video recording of the optic nerve head on the Spectralis showed the striking finding of an asynchronous arterio-venous pulse. A cerebral angiogram confirmed the diagnosis of CCF. Repeat ophthalmic evaluation showed resolution of the increased pulsation of the applanation mires. This demonstrated the phenomenon of angiography-induced thrombosis of CCF and spared the patient from further intervention of CCF closure which had been planned.
Bahareh Hassanzadeh, MD, OSF Illinois Neurological Institute, presented a 64-year-old man with constant right eye and periorbital pain, and intermittent right eye blurred vision for two years. The retinal arteries of the right eye were mildly tortuous compared with the left. Magnetic resonance imaging of the brain and orbits, and a computed tomography angiogram were unremarkable. Fluorescein angiography, however, showed ischaemic changes in the retina of the right eye. Conventional brain angiography was thus performed which found the culprit a right–sided, slow flow, indirect carotid cavernous fistula.
Jonah Yousif, BS, University of Michigan, presented a retrospective study of 88 patients with dorsal midbrain syndrome (DMS) who were evaluated by a neuro-ophthalmologist and had corroborative imaging abnormality. The most common aetiology was a tumour in 41%, followed by stroke in 31%. Diplopia and limitation of upgaze were the most common symptom and sign, seen in 64% and 89%, respectively. This study reported on the largest series of imaging-defined DMS.
Johanna Beebe, MD, Park Nicollet Health Services, described a 47-year-old female with a history of tobacco and alcohol use, who presented with three weeks of bilateral, sequential vision loss. Her vision was 20/400 in both eyes and she had bilateral central scotomas on visual field testing. OCT showed bilateral ganglion cell layer thinning. The workup including neuroimaging and blood tests was unremarkable except for mildly low folate levels. She was thought to have nutritional bilateral optic neuropathy and folate supplementation was started. Over the following three months, she developed neuropathic pain in her feet and imbalance, and then one day reported ‘seeing spiders everywhere’. She was admitted for psychosis and ultimately diagnosed with anti-N-methyl-D-Aspartate receptor encephalitis. Her vision improved with treatment. NMDA receptor encephalitis has been reported to be associated with optic neuritis in the past, often with MOG-IgG co-positivity, but this was a unique case of optic neuropathy without evidence of retrobulbar optic neuritis.
Devon Cohen, MD, Mayo Clinic, presented a retrospective chart review of 795 patients who underwent temporal artery biopsy to study the validity of frozen section pathology results for giant cell arteritis (GCA). She reported a sensitivity and specificity of 88.7% and 97.8%, respectively, for detecting arteritis by frozen section and concluded that a negative frozen section should not be relied on to rule out GCA.
Logan DeHoff, a medical student from the University of Kentucky, described a 49-year-old patient with neurofibromatosis type 1. On examination, he was found to have bilateral Lisch nodules on the iris and bilateral multiple choroidal-pigmented lesions on the fundus. The neuro-ophthalmic findings of NF1 and the differential diagnosis of bilateral diffuse uveal melanocytic proliferation were discussed.
Susan Benes, MD, Heart of the Rockies Regional Medical Centre, described her experiences with nomadic patients in Mongolias’s Gobi desert and patients in Ethiopia when she visited and brought neuro-ophthalmology to those parts. While local young faculty and trainees were eager to learn, there was a lack of neuro-ophthalmology sub-speciality services. She discussed how we can best help our fellow physicians around the world and their patients.
Kevin Chodnicki, MD, Mayo Clinic, presented a population-based study on the risk of stroke around an event of central retinal artery occlusion (CRAO). He reported the annual incidence of CRAO was 2.6 per 100,000. In the 15 days before and after CRAO, there were two ischaemic strokes (2.2%) and one haemorrhagic stroke (1.1%). Dr. Chodnicki’s study confirmed the increased incidence of stroke surrounding an episode of CRAO; however, the risk was less than what has been previously reported by institutional–based and big data studies.
Sangeeta Khanna, MD, St. Louis University, described a 41-year-old female with worsening headaches, polyuria and polydipsia, and a sellar mass on neuroimaging. She developed bitemporal field loss and surgical excision was planned. The surgery was difficult due to fibrosis and the biopsy report was inconclusive. This was followed by a repeat attempt at excision and biopsy with the same result, at which point the patient was referred to neuro-ophthalmology. Careful review of history, imaging and pathology slides resulted in the diagnosis of lymphocytic hypophysitis. The patient was treated with steroids with improvement in symptoms and vision.
This concluded the 41st annual UMNOG meeting. The 42nd annual UMNOG meeting will be held on July 24, 2020. Those interested in attending should contact Dr. John Chen at chen.john@mayo.edu or Dr. Collin McClelland at cmc@umn.edu.
Declaration of interest
The authors report no conflicts of interest.