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.

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.

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.

Tempo(ral) was the heart of the matter.

A 71-year-old woman was admitted with fever, headache, and weight loss associated with elevated inflammatory markers. She developed acute bilateral ophthalmoplegia and asymmetrical ptosis, rapidly followed by anterior ischemic optic neuropathy. Although the first temporal artery biopsy was negative, contralateral temporal artery biopsy revealed features consistent with giant cell arteritis. Even while under steroid therapy, she died a few days later from myocardial infarction. Acute bilateral complete ophthalmoplegia is a rare presentation of a limited number of possible diseases. Among these, giant cell arteritis should be suspected in the appropriate clinical scenario.

Retrospective, Multicenter Comparison of the Clinical Presentation of Patients Presenting With Diplopia From Giant Cell Arteritis vs Other Causes.

BACKGROUND: Although giant cell arteritis (GCA) is a well-known cause of transient and permanent vision loss, diplopia as a presenting symptom of this condition is uncommon. We compared symptoms and signs of patients presenting with diplopia from GCA to those from other causes. METHODS: This was a multicenter, retrospective study comparing the clinical characteristics of patients presenting with diplopia from GCA with age-matched controls. Demographic information, review of symptoms, ophthalmic examination, and laboratory data of biopsy-proven patients with GCA were compared with those of age-matched controls presenting with diplopia. RESULTS: A total of 27 patients presented with diplopia from GCA, 19 with constant diplopia, and 8 with transient diplopia. All patients with constant diplopia from GCA were matched with 67 control subjects who had diplopia from other etiologies. Patients with GCA were more likely to describe other accompanying visual symptoms (58% vs 25%, P = 0.008), a greater number of systemic GCA symptoms (3.5, GCA vs 0.6, controls, P < 0.001) such as headache (94% [17/18] vs 39% [23/67]; P < 0.001), jaw claudication (80% [12/15] vs 0% [0/36]; P < 0.001), and scalp tenderness (44% [7/16] vs 7% [3/43]; P < 0.001). Ocular ischemic lesions (26% vs 1%, P < 0.001) were also common in patients with diplopia from GCA. Inflammatory markers were elevated significantly in patients with GCA vs controls (erythrocyte sedimentation rate: 91% [10/11] vs 12% [3/25], P < 0.001; C-reactive protein: 89% [8/9] vs 11% [2/19], P < 0.001). CONCLUSIONS: GCA is a rare but serious cause of diplopia among older adults and must be differentiated from other more common benign etiologies. Our study suggests that most patients with diplopia from GCA have concerning systemic symptoms and/or elevated inflammatory markers that should trigger further work-up. Moreover, careful ophthalmoscopic examination should be performed to look for presence of ocular ischemic lesions in older patients presenting with acute diplopia.

Thick corneas, large pupils, and a giant problem.

An 87-year-old woman presented 1 month after uneventful cataract surgery with ipsilateral corneal edema. She was diagnosed with pseudophakic bullous keratopathy and scheduled for endothelial transplantation. A few days later, however, she presented with bilateral corneal edema, dilated pupils, and further reduction of visual acuity. Neuro-ophthalmic evaluation disclosed a bilateral ocular ischemic syndrome causing complete visual loss. Temporal artery biopsy was consistent with giant cell arteritis. Corneal decompensation should be considered as a rare presentation of giant cell arteritis, a diagnosis that ophthalmologists should suspect in any case of unilateral or bilateral ocular ischemic syndrome.

The QuantiFERON-TB Gold In-Tube Assay in Neuro-Ophthalmology.

BACKGROUND: Although QuantiFERON-TB Gold In-Tube (QFT-GIT) testing is regularly used to detect infection with Mycobacterium tuberculosis, its utility in a patient population with a low risk for tuberculosis (TB) has been questioned. The following is a cohort study analyzing the efficacy of QFT-GIT testing as a method for detection of active TB disease in low-risk individuals in a neuro-ophthalmologic setting. METHODS: Ninety-nine patients from 2 neuro-ophthalmology centers were identified as having undergone QFT-GIT testing between January 2012 and February 2016. Patients were divided into groups of negative, indeterminate, and positive QFT-GIT results. Records of patients with positive QFT-GIT results were reviewed for development of latent or active TB, as determined by clinical, bacteriologic, and/or radiographic evidence. RESULTS: Of the 99 cases reviewed, 18 patients had positive QFT-GIT tests. Of these 18 cases, 12 had documentation of chest radiographs or computed tomography which showed no evidence for either active TB or pulmonary latent TB infection (LTBI). Four had chest imaging which was indicative of possible LTBI. None of these 18 patients had symptoms of active TB and none developed active TB within the follow-up period. CONCLUSIONS: Based on our results, we conclude that routine testing with QFT-GIT in a low-risk cohort did not diagnose active TB infection. We do not recommend routine QFT-GIT testing for TB low-risk individuals, as discerned through patient and exposure history, ocular examination, and clinical judgment, in neuro-ophthalmology practice.

Don't drink in the valley.

A 39-year-old man presented with chronic headaches and intermittent blurred vision with previous neuroimaging and blood work that was reportedly normal. He had papilledema and further questioning elicited a history of extensive alcohol use, unexplained weight loss, and night sweats. Magnetic resonance imaging of the brain demonstrated communicating hydrocephalus and leptomeningeal enhancement. The patient underwent ventriculoperitoneal shunt placement and leptomeningeal biopsy, which was initially unrevealing. Cerebrospinal fluid eventually yielded positive titers for coccidioides, a diagnosis that was confirmed by biopsy culture results.

Preserved Visual Acuity in Anterior Ischemic Optic Neuropathy Secondary to Giant Cell (temporal) Arteritis.

OBJECTIVE: To evaluate the prevalence and clinical profile of patients with biopsy-proven arteritic anterior ischemic optic neuropathy presenting with preserved visual acuity of 20/40 or better and those with an initial poor visual acuity of 20/50 or worse through a retrospective chart review. RESULTS: Nine of 37 patients with arteritic anterior ischemic optic neuropathy presented with a preserved visual acuity of 20/40 or better in the affected eye. All patients with preserved visual acuity had initial visual field defects that spared the central field. All 37 patients immediately received high-dose corticosteroid therapy. Visual acuity worsened by > 2 lines in one of nine patients (11%) with preserved visual acuity, with a corresponding progression of visual field constriction. CONCLUSION: Although preserved visual acuity of 20/40 or better has traditionally been associated with the nonarteritic form of anterior ischemic optic neuropathy, giant cell arteritis should still be strongly considered, especially if they have giant cell arteritis systemic symptoms.

Neuro-ophthalmic presentations and treatment of Cryptococcal meningitis-related increased intracranial pressure.

OBJECTIVE: To illustrate three different ophthalmic presentations of cryptococcal meningitis (CM). INTRODUCTION: CM is the most common manifestation of extra-pulmonary cryptococcosis. Intracranial hypertension occurs in up to 75% of patients with CM and is associated with increased mortality. CM can present to the ophthalmologist as vision loss, papilledema, abducens palsy, and/or other cranial neuropathies. PARTICIPANTS AND METHODS: We report three cases, two C. neoformans and one C. gattii, highlighting the various CM presentations. The first was a woman immunosuppressed following kidney transplantation in whom idiopathic intracranial hypertension (IIH) was initially suspected. The second was a man immunocompromised by previously undiagnosed HIV/AIDS who presented with signs and symptoms of increased intracranial pressure. The third case is an immunocompetent man with bilateral disc edema and an incomplete macular star diagnosed with presumed neuroretinitis. Further evaluation revealed positive CSF cryptococcal antigen with culture positive for C. gattii. CONCLUSIONS: Ophthalmologists should be aware that cryptococcosis can mimic more benign etiologies including IIH and neuroretinitis. Additionally, C. gattii, an emerging organism, can infect immunocompetent patients. In contrast to the typical treatment of increased ICP, serial lumbar punctures are recommended while acetazolamide and surgical CSF shunting may be harmful.

Visual recovery after surgical decompression of an occipital intraventricular cyst.

: A 62-year-old woman presented with a chronic left homonymous visual field defect because of a right occipital cyst. Serial visual field examination documented stable visual fields for 12 months, after which there was worsening of visual fields associated with enlargement of the cyst. Surgical decompression of the occipital cyst resulted in marked improvement of the visual field defect over 9 months. This case demonstrates that surgical decompression of cystic lesions adjacent to posterior visual pathways can result in recovery of chronic visual field loss.

Acute visual loss: just the beginning?

A 47-year-old man presented with sudden visual loss, optic disk edema, retinal ischemia, and limited upgaze in the left eye. Initial MRI revealed thickened, enhancing left optic nerve. Extensive work-up for an inflammatory and infiltrative etiology was positive only for Borrelia burgdorferi IgM by Western blot. Six weeks later the patient had numbness and weakness on his left side. MRI showed enhancing lesions extending from the left optic nerve to the optic chiasm, along the visual pathways bilaterally, mainly on the right side from optic tract to lateral geniculate body and pulvinar. Stereotactic biopsy of the right pulvinar lesion revealed glioblastoma. The tumor progressed rapidly, and the patient died 11 weeks after the onset of first symptoms.