A Teenage Boy With a Radiation-Induced High-Grade Astrocytoma.

ABSTRACT: A 12-year-old boy developed acute headache and vomiting. MRI brain showed a partially cystic suprasellar mass. He underwent cyst fenestration, but the cyst regrew, so he underwent transcranial subtotal resection of the mass. The pathologic diagnosis was adamantinomatous craniopharyngioma. Residual tumor was treated with proton beam radiation therapy, and panhypopituitarism was treated with hormone replacement therapy, including growth hormone. Serial brain MRI scans over several years showed no evidence of tumor recurrence. But at four years after radiation, surveillance MRI showed a new focus of nonenhancing FLAIR hyperintensity in the left basal ganglia attributed to gliosis caused by radiotherapy. Seven months later, he developed progressive right hemiparesis, expressive aphasia, and blurred vision, prompting reevaluation. MRI brain showed new enhancing and T2/FLAIR hyperintense lesions in the midbrain, basal ganglia, thalamus, anterior temporal lobe, and optic tract. The abnormal regions showed low diffusivity and relatively high regional blood flow. Stereotactic biopsy disclosed a WHO Grade 4 astrocytoma, likely radiation-induced. A germline ataxia telangiectasia mutation was found in the tumor tissue. The risk of radiation-induced pediatric brain malignancies is low but may have been increased by the mutation.

Planet of the apex.

A 63-year-old man with diabetes presented with unilateral ptosis and an exotropia. A diagnosis of isolated diabetic III nerve palsy was made. Subsequent neuro-ophthalmologic evaluation showed multiple cranial nerves involvement consistent with a diagnosis of orbital apex syndrome. Review of past medical history was significant for a previous nasopharyngeal carcinoma, and biopsy of the involved site was consistent with tumor recurrence. This case highlights the importance of correct medical history taking and anatomo-clinical correlation in neuro-ophthalmology.

Clinical course of asymptomatic patients with papilledema from idiopathic intracranial hypertension.

OBJECTIVE: Idiopathic intracranial hypertension (IIH) is defined as elevated intracranial pressure (ICP) with normal cerebrospinal fluid content in the absence of an identifiable cause. Patients often experience symptoms related to elevated ICP (e.g., headache); however, a subgroup of patients with elevated ICP may have optic disc edema without any associated symptoms. There are limited data about this subgroup in the literature. Our aim in this study was to characterize the initial clinical findings and visual outcomes over the follow-up period in this group of asymptomatic patients. METHODS: We performed a retrospective review of all patients who were referred to the neuro-ophthalmology service at Baylor College of Medicine for evaluation of papilledema between January 2012 and June 2020. Medical records of 139 consecutive patients with papilledema were reviewed. Patients were included in the analysis if they met the criteria for the diagnosis of IIH, had bilateral optic disc edema, and did not have any symptoms of elevated ICP. RESULTS: Of the 139 charts reviewed, 5 patients met the inclusion criteria. All 5 patients were female with a mean age of 25.2 years (range, 13-48 years). The mean body mass index was 36.3 kg/m2 (range, 31.5-40 kg/m2), and the mean follow-up period was 3 years (range, 12 months-5 years). CONCLUSION: Our results demonstrate that the disease course for patients who present with asymptomatic IIH can be variable, yet still visually significant. Despite the absence of symptoms, patients can progress to symptomatic disease or experience persistent optic disc swelling or pallor even with the use of medication to lower ICP. To our knowledge, this is the first retrospective study characterizing the clinical course of papilledema from IIH in asymptomatic individuals.

An eye for an eye, a tooth for a tooth.

A 77-year-old man presented with the complaint of a painless decrease in vision in both eyes for 1 year. He underwent bilateral cataract surgery without improvement. Neuro-imaging and genetic testing for mitochondrial disease was negative. Examination was consistent with a diagnosis of toxic-nutritional optic neuropathy. On further questioning the patient admitted chronic ingestion of mouthwash 3 times daily in the past 18 months after completion of dental work. After discontinuation of mouthwash ingestion, visual acuity and fields improved. This case shows how chronic ingestion of mouthwash can lead to a bilateral toxic optic neuropathy, even in the absence of other risk factors.

A high-pressure situation.

An 8-year-old boy presented with acute visual loss in the right eye and nausea, vomiting, and diplopia. Imaging revealed a right orbital apex mass. Biopsy showed Langerhans cell histiocytosis (LCH), and the patient was diagnosed with isolated orbital LCH causing an orbital apex syndrome. A 12-month cytarabine chemotherapy course was begun, during which the patient developed bilateral optic disc edema. He was diagnosed with cytarabine-induced intracranial hypertension, which was successfully treated with acetazolamide. The cytarabine course was completed with complete resolution of the LCH lesion. The ophthalmologic relevance of this rare disorder is discussed.

Prognosticators of Visual Acuity After Indirect Traumatic Optic Neuropathy.

BACKGROUND: The purpose of this study is to determine whether there are radiographic and systemic clinical characteristics that can predict final visual outcomes in patients with indirect traumatic optic neuropathy (iTON). METHODS: This study is a retrospective, multicenter case series of adult patients with iTON treated initially at large, urban, and/or academic trauma centers with follow-up at an affiliated ophthalmology clinic. In addition to detailed cranial computed tomography characteristics, demographics, systemic comorbidities, coinjuries, blood products administered, and intracranial pressure, along with other factors, were gathered. LogMAR visual acuity (VA) at the initial presentation to the hospital and up to 12 months follow-up was collected. RESULTS: Twenty patients met inclusion criteria; 16 (80%) were men with a mean age of 40.9 years (±20.9). Mean initial VA was 1.61 logMAR (∼20/800, ± 0.95), and final VA was 1.31 logMAR (∼20/400, ± 1.06). Three patients (4 eyes) had no light perception (NLP) VA at presentation and remained NLP at final follow-up. Of the predictors analyzed, only the initial VA was found to be a significant predictor of visual outcome. The presence of orbital fractures, intraconal and/or extraconal hemorrhage, as well as systemic comorbidities, were not found to significantly affect visual outcome. CONCLUSIONS: After evaluating multiple factors, initial VA was the only factor associated with visual prognosis in iTON. This knowledge may better enable clinicians to predict visual prognosis and set reasonable expectations with patients and families at the time of injury.

Clinical challenge - scratching the surface of the sheath.

A 63-year-old Caucasian man with hypertension, hyperlipidemia, adrenal insufficiency, and history of metastatic spindle cell melanoma previously treated with ipilimumab, presented with unilateral papillitis in the left eye. The disc edema evolved to neuroretinitis with additional MRI findings of perineuritis. Broad laboratory evaluation confirmed active B. henselae infection, and he confirmed exposure to a newly adopted kitten. His vision improved following a course of antibiotics and oral steroids with resolution of clinical findings. To the best of our knowledge, this is the first reported case of cat-scratch neuroretinitis with MRI findings of optic nerve and optic nerve sheath enhancement.

Natural history of patients with Leber hereditary optic neuropathy-results from the REALITY study.

BACKGROUND/OBJECTIVES: REALITY is an international observational retrospective registry of LHON patients evaluating the visual course and outcome in Leber hereditary optic neuropathy (LHON). SUBJECTS/METHODS: Demographics and visual function data were collected from medical charts of LHON patients with visual loss. The study was conducted in 11 study centres in the United States of America and Europe. The collection period extended from the presymptomatic stage to at least more than one year after onset of vision loss (chronic stage). A Locally Weighted Scatterplot Smoothing (LOWESS) local regression model was used to analyse the evolution of best-corrected visual acuity (BCVA) over time. RESULTS: 44 LHON patients were included; 27 (61%) carried the m.11778G>A ND4 mutation, 8 (18%) carried the m.3460G>A ND1 mutation, and 9 (20%) carried the m.14484T>C ND6 mutation. Fourteen (32%) patients were under 18 years old at onset of vision loss and 5 (11%) were below the age of 12. The average duration of follow-up was 32.5 months after onset of symptoms. At the last observed measure, mean BCVA was 1.46 LogMAR in ND4 patients, 1.52 LogMAR in ND1 patients, and 0.97 LogMAR in ND6 patients. The worst visual outcomes were reported in ND4 patients aged at least 15 years old at onset, with a mean BCVA of 1.55 LogMAR and no tendency for spontaneous recovery. The LOESS modelling curve depicted a severe and permanent deterioration of BCVA. CONCLUSIONS: Amongst LHON patients with the three primary mtDNA mutations, adult patients with the m.11778G>A ND4 mutation had the worst visual outcomes, consistent with prior reports.

Oscillopsia following orbitotomy for intracranial tumor resection.

BACKGROUND: Oscillopsia is a visual phenomenon in which an individual perceives that their environment is moving when it is in fact stationary. In this report, we describe two patients with pulsatile oscillopsia following orbitocranial approaches for skull base meningioma resection. CASE DESCRIPTION: Two patients, both 42-year-old women, underwent orbitocranial approaches for resection of a right sphenoid wing (Patient 1) and left cavernous sinus (Patient 2) meningioma. Patient 1 underwent uncomplicated resection and was discharged home without neurologic or visual complaints; she presented 8 days later with pulsatile oscillopsia. This was managed expectantly, and MRA revealed no evidence of vascular pathology. She has not required intervention as of most recent follow-up. Patient 2 developed trochlear and trigeminal nerve palsies following resection and developed pulsatile oscillopsia 4 months postoperatively. After patching and corrective lens application, the patient's symptoms had improved by 26 months postoperatively. CONCLUSION: Oscillopsia is a potential complication following skull base tumor resection about which patients should be aware. Patients may improve with conservative management alone, although the literature describes repair of orbital defects for ocular pulsations in traumatic and with some developmental conditions.

Characteristic Visual Field Defect From Lateral Geniculate Body Stroke.

ABSTRACT: A 58-year-old man presented with a complaint of subjective visual field loss on the right side and hypertensive emergency. Examination revealed a right homonymous hemianopia. Computed tomography imaging revealed an acute stroke of the left lateral geniculate body. A few months later, automated perimetry revealed characteristic visual field changes associated with this lesion. In this report, the anatomy, pathophysiology, clinical findings, and previously reported etiologies of lateral geniculate body lesions are reviewed.

A shotgun wedding.

A 69-year-old woman developed a carotid-cavernous fistula (CCF) after firing a shotgun. Initially, the patient had mild visual symptoms, but later on developed prominent features of CCF including chemosis, proptosis, ophthalmoparesis and conjunctival injection . The fistula was embolized via an intravascular coiling procedure. We are unaware of another patient who developed a CCF due to blunt force from shotgun use.

Presentation and Progression of Papilledema in Cerebral Venous Sinus Thrombosis.

PURPOSE: To determine the natural history and visual outcomes of papilledema in cerebral venous sinus thrombosis (CVST). DESIGN: Retrospective observational case series. METHODS: This multicenter study included 7 tertiary care neuro-ophthalmology clinics. Sixty-five patients with CVST were identified who received serial eye examinations with documented papilledema from 2008-2016. Outcome measures included time from diagnosis to papilledema documentation, papilledema progression, time to papilledema resolution, treatment interventions and final visual outcomes. RESULTS: Papilledema was present on initial presentation in 54% of patients or detected later during the course of the disease in 46% of patients. The average time from CVST diagnosis to papilledema documentation was 29 days with a mean (SD) initial Frisén grade of 2.7 (1.3). In 21.5% of cases, papilledema progressed over an average of 55.6 (56.6) days. Time to papilledema resolution was approximately 6 months. Final visual acuity ranged from 20/20 to light perception, with 40% of patients having residual visual field defects on standard automated perimetry. Frisén grade ≥3 (odds ratio [OR] 10.21, P < .0053) and cases with worsening papilledema (3.5, P < .043) were associated with permanent visual field deficits. CONCLUSIONS: Our study indicates the importance of serial ophthalmic evaluation in all cases of CVST. Follow-up fundoscopy is critical given that a subset of cases can show delayed onset and/or worsening of papilledema with time. Specifically, we recommend an ophthalmic examination at the time of initial diagnosis, with repeat examination within a few weeks and further follow-up depending on the level of papilledema or vision changes.

Transient Neurologic Dysfunction in Migraine.

Transient disturbances in neurologic function are disturbing features of migraine attacks. Aura types include binocular visual, hemi-sensory, language and unilateral motor symptoms. Because of the gradual spreading quality of visual and sensory symptoms, they were thought to arise from the cerebral cortex. Motor symptoms previously included as a type of migraine aura were reclassified as a component of hemiplegic migraine. ICHD-3 criteria of the International Headache Society, added brainstem aura and retinal aura as separate subtypes. The susceptibility to all types of aura is likely to be included by complex and perhaps epigenetic factors.