ABSTRACT
The 43rd meeting of the Upper Midwest Neuro-Ophthalmology Group (UMNOG) took place on July 23, 2021. For the second sequential year, the meeting was held virtually due to the COVID-19 pandemic. The meeting was held in the honour of the late Ivy Dreizin MD. Ninety people attended virtually marking the highest UMNOG meeting attendance on record. There were 23 podium presentations interspersed with numerous personal testimonials recognising Dr Dreizin and her immense contributions to the UMNOG community.
KEYWORDS: Proceedings, Midwest, neuro-ophthalmology, UMNOG
The 43rd meeting of the Upper Midwest Neuro-Ophthalmology Group (UMNOG) took place on July 23, 2021. For the second sequential year, the meeting was held virtually due to the COVID-19 pandemic. The meeting was held in the honour of the late Ivy Dreizin MD, who was a longtime champion of UMNOG, a highly respected neuro-ophthalmologist, and a friend to many. John Chen MD PhD, Mayo Clinic, Rochester, Minnesota, and Collin McClelland MD, University of Minnesota, Minnesota co-organised the meeting. Ninety people attended virtually marking the highest UMNOG meeting attendance on record. There were 23 podium presentations interspersed with numerous personal testimonials recognising Dr Dreizin and her immense contributions to the UMNOG community.
The first presentation was delivered by Michael Lee MD, University of Minnesota, who described an association between morning glory disc anomaly (MGDA) and ipsilateral enlargement of the pre-chiasmatic optic nerve and chiasm. Four patients with MGDA were identified; none had other neurological diagnoses including neurofibromatosis type 1. Neuroimaging showed enlargement without enhancement of the ipsilateral chiasm in all unilateral cases; in two cases, the ipsilateral pre-chiasmatic optic nerve was also expanded. A literature review revealed five other cases with similar findings. It remains unclear whether these radiographic findings represent inactive, low-grade gliomas, or an alternative benign process.
Jonathan Trobe MD, University of Michigan, presented a retrospective investigation showing the correlation of macular sparing and homonymous paracentral scotomas with lesion location in posterior cerebral artery (PCA) ischaemic strokes. A cohort of 94 patients whose magnetic resonance imaging (MRI) showed exclusive PCA territory infarcts were studied. The study found that lesions sparing the posterior 50% of the visual cortex often produced macular sparing of the central 15 degrees of the visual field with no significant difference in the visual field spared when comparing pathology sparing 25% versus 50% of the posterior visual cortex. Homonymous paracentral scotomas confined to the central 15 degrees of the visual field were associated with lesions isolated to the posterior 50% (or less) of the visual cortex. This study supports that the central 15 degrees of the visual field is mapped onto at least 50% of posterior visual cortex.
John Mitchell MD, Marshfield Clinic in Wisconsin, described an interesting case of a 40-year-old white male who presented to the hospital after having a severe “thunder-clap” headache following intercourse. The patient demonstrated clinical findings of a one-and-a-half syndrome attributable to a brainstem stroke. The anatomy of this finding was reviewed.
Padmaja Sudhakar MD, University of Kentucky, presented a young woman with a recently diagnosed suprasellar lesion producing mass effect on the optic chiasm and suprasellar portions of the internal carotid artery. She had a normal neuro-ophthalmologic evaluation including full automated perimetry, however, given the proximity of the suprasellar mass to the optic chiasm, she underwent a trans-sphenoidal resection. During the operation, there was unexpected drainage of polymicrobial pus and debris with cultures eventually growing methicillin-resistant Staphylococcus epidermidis and Cutibacterium acnes. She was started on the appropriate antibiotics and follow up MRI showed post-operative changes and decreased previously infected tissue within the sella. Pituitary abscesses account for less than 1% of all pituitary lesions and the authors remind us of the importance of considering this diagnosis in the differential of sellar lesions.
Deena Tajfirouz MD, Mayo Clinic, presented a case series of four post solid-organ transplant patients in their 60s on maintenance immunosuppression with calcineurin inhibitors who presented with a progressively fatal neurologic disease with progressive optic neuropathy and eventually myelopathy. An extensive evaluation including cerebrospinal fluid (CSF) studies, serologic studies for known infectious and inflammatory disorders, whole body fluorodeoxyglucose computed tomography positron emission tomography, and biopsies of the optic nerves were unrevealing. Despite treatment with high dose steroids, plasma exchange, intravenous immunoglobulin (IVIG), rituximab, mycophenolate, and discontinuation of tacrolimus or cyclosporin, the patients continued to worsen. The cause of their fatally progressive neurologic disease was thought to be due to calcineurin inhibitor neuro-toxicity with similar findings on MRI.
Muhammad Asad Fraz MD, Henry Ford Hospital, presented a case of a 70-year-old Vietnamese female with multiple vascular risk factors who presented with gradually worsening left eye vision associated with left temporal and peri-orbital headaches for a few months. Examination was notable for a left eye dense central scotoma, absent colour vision, a relative afferent pupillary defect, and optic disc pallor. Fundus examination of the right eye showed peri-papillary cotton wool spots and haemorrhages with a normal disc. Fluorescein angiography was negative for a choroidal filling defect, or retinal vascular occlusion/delay, though it did show leakage from retinal vessels in the same areas as haemorrhages and cotton wool a spots in right eye, suspicious for retinal vasculitis. MRI showed localised bifrontal pachymeningeal enhancement. Serological studies were notable for p-anti-neutrophil cytoplasmic antibody and myeloperoxidase positivity favouring a clinical diagnosis of atypical microscopic polyangiitis with isolated retinal vasculitis in the right eye and optic nerve ischaemia in the left eye.
John Chen MD PhD, Mayo Clinic, presented a multi-centre retrospective study evaluating the utilisation of optical coherence tomography (OCT) in distinguishing between acute optic neuritis (ON) in myelin oligodendrocyte glycoprotein (MOG) associated disorder (MOGAD) from multiple sclerosis (MS). They evaluated 64 patients with MOGAD and 50 patients with MS and found that OCT-derived peripapillary retinal nerve fibre layer (pRNFL) thickness in acute ON can help differentiate these two entities. The authors reminded us of the importance of this finding since MOG antibody testing can be slow or inaccessible. OCT can be further used to differentiate cases of low MOG antibody titre results, as false positives are known to occur. Furthermore, they noted that pRNFL thickening is a sensitive marker for confirming acute ON in MOGAD but less so in MS.
Adrienne Jarocki BS, University of Michigan, presented two cases of arachnoid cysts that leaked after head trauma to form hygromas causing increased intracranial pressure and papilloedema. One patient required a cystoperitoneal shunt and the other was managed with acetazolamide. Their literature search identified four previously reported cases.
Nathaniel Zaroban BA, University of Nebraska College of Medicine and Nebraska Medicine Truhlsen Eye Institute, shared a case series analysis of retinal electrodiagnostic testing in visual snow (VS). Eight patients with the diagnosis of visual snow who did not have other ocular pathologies were studied. They concluded that increased electroretinogram (ERG) b-wave amplitudes in VS subjects in response to dark-adapted stimuli may implicate retinal hyper-responsiveness as a contributing factor in VS pathophysiology. It was noted that larger ERG studies of a greater number of VS subjects are warranted to further validate and characterise retinal dysfunction in VS.
Judy Chen MD, University of Wisconsin, presented three interesting cases of painless visual loss occurring across three generations of women in one African-American family attributable to Leber’s hereditary optic neuropathy (LHON). Each case had a unique presentation. A 10-year-old girl presented with severe acute painless bilateral vision loss and was found to harbour anti-retinal antibodies concerning for cancer-associated retinopathy/optic neuropathy. She was treated with IVIG therapy before the diagnosis of LHON was made. The 10-year-old’s mother presented at age 42 with chronic progressive vision loss over a few months. Six months later, the 10-year-old’s 60-year-old grandmother presented with progressive painless bilateral vision loss. Discussion was held regarding the interesting disparity in ages of presentation of vision loss and the unusual occurrence among females in this case. The non-specific nature of anti-retinal antibodies was also discussed.
Evan Jameyfield MD, University of Illinois at Chicago, presented a case of a 43-year-old male with a history of Sturge-Weber syndrome, right eye blindness secondary to optic nerve compression, and glaucoma who presented with right-sided ptosis sequentially followed by left-sided ptosis. The initial concern was due to expanding haemangioma, which had previously caused similar symptoms. However, the patient then developed left-sided diffuse ophthalmoplegia, slurred speech and dysphagia. Ultimately, the patient was diagnosed with myasthenia gravis and had a treatment refractory clinical course.
In the first case of the afternoon sessions, Collin McClelland MD, University of Minnesota, presented a case of a 15-year-old girl with a medical history of neurofibromatosis type 2 with associated bilateral vestibular and trigeminal schwannomas. She presented for evaluation of recurrent, painless episodes of ocular motor deficits lasting days to months and always localising to partial or complete left ocular motor palsy including: left upper eyelid ptosis and left eye adduction; supraduction; and infraduction deficits. Her most recent occurrence, however, did not resolve, leaving the patient with a persistent moderate angle exotropia with an adduction deficit. Atrophy of the left medial rectus was evident on a recent MRI of the orbits. Careful examination of the course of the left oculomotor nerve was unrevealing for ocular motor nerve enhancement or expansion. The patient had an identical twin sister with a radiographically visible, but asymptomatic, left oculomotor nerve schwannoma. The patient was given a presumptive diagnosis of symptomatic oculomotor nerve schwannoma manifesting initially as an intermittent palsy before becoming constant.
Next, Mangayarkarasi Thandampallayam MB BS, University of Kentucky, presented the case of a 73-year-old man who presented with ophthalmoparesis that progressed to complete ophthalmoplegia and neuromuscular respiratory failure necessitating intubation. His pupillary examination was initially normal, however, the day after presentation both pupils became fixed in mid dilation. Lumbar puncture demonstrated elevated protein without concurrent elevation in white blood cells and the patient was diagnosed with Miller Fisher syndrome. He was treated with IVIG but continued to worsen, becoming quadriplegic. At that time, it was revealed that he had eaten several week-old clam chowder ten hours prior to presentation. Testing for botulism was positive and botulinum anti-toxin was administered. The case reinforced that a high index of suspicion for botulism is indicated for any patient presenting with acute neuromuscular weakness involving multiple cranial nerves including pupillary involvement.
Juno Cho MA, University of Michigan, presented retrospective data on visual field deficits associated with retrochiasmal lesions. Their study of 83 cases assessed the specificity of visual field patterns for anatomic locations. The most specific patterns included homonymous hemianopia with contralateral nerve fibre bundle deficit (optic tract with rostral extension), “pie-in-the-sky” deficits (Meyer’s Loop), and macular sparing (primary visual cortex and posterior optic radiations). These study findings matched well to the classically taught localisation. Conversely, quadrantanopias, homonymous paracentral scotomas, and incongruous homonymous hemianopias did not demonstrate high specificity for any one anatomic location.
Next, Ashwini Kini MD, University of Kentucky, presented a case of a 46-year-old man with no prior medical history who presented with binocular horizontal diplopia and right arm weakness and ataxia following an upper respiratory infection. MRI of the brain demonstrated numerous T2 hyperintensities, some enhancing, in the deep and subcortical white matter including the subcortical U-fibres, brainstem, bilateral basal ganglia and left thalamus. Lumbar puncture was unremarkable and he was treated with three days of high dose intravenous steroids for a presumptive diagnosis of acute disseminated encephalomyelitis (ADEM). Despite steroid treatment, he worsened clinically and returned with a new left cranial nerve VI palsy. Further history and laboratory evaluation revealed that patient had previously undiagnosed human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS) with CD4 count of 5. Two additional lumbar punctures failed to show laboratory evidence of John Cunningham (JC) virus. He was started on anti-retroviral therapy but continued to decline, developing quadriparesis and dysphagia with an inability to manage secretions. Brain biopsy was consistent with progressive multifocal leukoencephalopathy (PML) noting JC virus positivity in glial cells. The patient unfortunately succumbed to critical illness. This case highlights that while polymerase chain reaction (PCR) is generally very sensitive for JC virus central nervous system infection, its sensitivity can be altered by AIDS and other immunosuppressed states. Thus, if a high degree of suspicion remains for PML despite negative lumbar puncture, brain biopsy may be necessary to confirm the diagnosis.
Jorge Kattah MD, OSF Illinois Neurological Institute, presented a case of a 57-year-old woman who presented with acute onset of ataxia, opsoclonus, macrosaccadic oscillations, and myoclonus. She had exposure to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) several weeks prior, but had no clinical symptoms suggestive of infection and had two negative nasopharyngeal PCR studies for SARS-CoV-2 prior to admission. On the neurologic examination, she had a non-rhythmic truncal titubation, hyperekplexia, pronounced limb and truncal ataxia, and dysdiadochokinesia. Ocular motor exam was notable for unsteady visual fixation with macrosaccadic oscillations and opsoclonus, including bursts noted primarily during fixation block. MRI brain was read as normal though there was suspicion of a T2 hyperintense lesion in the caudate nucleus. Serum SARS-CoV-2 IgG antibodies were found to be present in high titres. She also had bilateral pulmonary ground glass opacities on computed tomography chest consistent with SARS-CoV-2 pneumonitis. Additional extensive workup including CSF testing was unremarkable. She was given a presumptive diagnosis of post-SARS-CoV-2 opsoclonus-myoclonus ataxia syndrome. She was treated with 5 days of intravenous (IV) methylprednisolone and an oral steroid taper with improvement. Additional treatment with rituximab led to a resolution of symptoms and findings by 5-month follow-up.
Ahmad Halawa MD, University of Michigan, presented a case of a 61-year-old woman who developed congestive orbitopathy and bilateral retinal, optic nerve, and cerebral infarctions after removal of a chipped molar tooth. Three days following the procedure she presented with facial pain, swelling, and blurred vision in both eyes. She was febrile to 39 °C with a neutrophilic (85.6%) leukocytosis of 22,000. Initial ophthalmologic assessment was notable for bilateral proptosis, poor pupillary reactivity bilaterally, and elevated intraocular pressures (IOP) to 39 mmHg in the right eye and 43 mmHg in the left eye. Fundus examination demonstrated inferior branch retinal artery occlusion and distal retinal pallor in the right eye and a normal left fundus examination. Imaging revealed ipsilateral masticator and pterygoid muscle abscesses, as well as thrombosis of the right internal jugular vein and sigmoid sinus, both cavernous sinuses and superior ophthalmic veins, and restricted diffusion of both optic nerves and corona radiata. She was diagnosed with catastrophic Lemierre syndrome. She underwent emergent bilateral canthotomy with cantholysis with improvement in IOP to 25 mmHg and 14 mmHg. Broad spectrum antibiotics were initiated, but despite this she went into septic shock requiring intubation and vasopressors.
Francisco R. Sanchez Moreno MD, Mayo Clinic, presented a population-based observational retrospective study of patient characteristics, treatment courses, associated medical conditions, and morbidity associated with idiopathic intracranial hypertension (IIH). The median age of IIH diagnosis was 27.3-years-old (range 12 to 49); 88.5% were female and 45 (75%) were obese. The median body mass index at diagnosis was 36.4 kg/m2 (range 19.9 to 58.41). Thirty-one (54%) patients had an increase in weight prior to diagnosis. The median lumbar puncture opening pressure was 318 mmCSF (range 260 to 680). Three (5%) required optic nerve sheath fenestration, three (5%) required ventriculoperitoneal shunting, and 11 (18%) underwent bariatric surgery. In terms of visual outcomes, 17% had a decrease in final visual acuity and/or permanent visual field changes including six patients with visual field changes and six with decreased visual acuity. Coexisting systemic diseases included depression (51%), infertility (15%), sleep apnoea (16%), hypertension (15%), polycystic ovarian syndrome (11%), and diabetes mellitus (10%). The study reinforced that IIH is not a benign condition, with nearly one-fifth of patients suffering permanent decreases in visual acuity and visual field changes.
Next, Casey Judge DO, University of Minnesota, presented a case of a 23-year-old with an ocular history of childhood strabismus who presented for evaluation of optic nerve elevation and concern for papilloedema. Ophthalmologic examination demonstrated bilateral optic disc elevation and blurred optic disc margins with questionable optic disc drusen in the right nerve head. B-scan performed using the Butterfly iQ+ hand-held ultrasound device in the same room confirmed the presence of drusen in the right optic nerve head. Technological advances in recent years, such as readily available in-office portable ultrasonography, have increased affordability and access to diagnostic tools that have the potential to reduce unnecessary imaging and invasive procedures.
Next, George Kung BS, Saint Louis University, presented a retrospective study of 29 paediatric patients with diagnoses of pseudopapilloedema (13 patients) and true papilloedema (16 patients) to characterise relevant MRI findings and presenting symptoms associated with either diagnosis. The clinical symptoms most suggestive of IIH included nausea and vomiting (56.5% in IIH versus 23%), double vision (44% in IIH versus 0%) and blurry vision (44% in IIH versus 0%). The prevalence of headache, pulsatile tinnitus, and transient visual obscurations was similar between both groups. MRI findings most specific for IIH included flattening of the posterior sclera (p = .012), diffusion restriction of the optic nerve head (p = .005), venous sinus narrowing (p = .006), and optic nerve sheath tortuosity (p = .041).
Meari Taguchi MD, Henry Ford Hospital, presented a 68-year-old man with intractable trigeminal neuralgia who sustained injury to the right abducens nerve following balloon rhizotomy of the Gasserian ganglion. There were no known complications during the procedure, however, post-operatively the patient noticed binocular horizontal diplopia attributable to a right abducens palsy. After 10 weeks, the patient experienced gradual resolution of diplopia and near complete resolution of ocular misalignment. This case importantly demonstrated a rare complication of a common procedure.
In the last presentation of the day, Yue Li MD, Henry Ford Hospital, presented a case of an 80-year-old man with a history of Stage IIA non-Hodgkin’s lymphoma in remission who presented with diplopia, fevers/chills, anorexia with weight loss, urinary retention, and vesicular skin lesions. The examination was notable for bilateral abducens palsy. Deep tendon reflexes were absent throughout and he had ataxia of the left lower extremity and a possible ataxic gait. Vesicular skin lesions were noted in multiple body regions including the left arm and right lower back. MRI of the brain was negative for enhancement of either abducens nerve, however, MRI of the spinal cord demonstrated diffuse nerve root enhancement. Lumbar puncture demonstrated lymphocytic pleocytosis of 146/mm3, elevated protein to 163 mg/dL, and positive oligoclonal bands. CSF PCR was positive for herpes simplex virus type 2 (HSV-2) and negative for other infectious aetiologies. Vesicular fluid from the right lower back and left arm vesicular lesions was also positive for HSV-2. He was treated with IV aciclovir 10 mg/kg every 8 hours for 14 days. The case demonstrated a rare severe form disseminated HSV-2 with neurologic manifestations including polyradiculitis and ataxia that responded well to high dose IV aciclovir.
Funding Statement
The author(s) reported there is no funding associated with the work featured in this article.
Declaration of interest statement
No potential conflict of interest was reported by the author(s).