Long-term efficacy of fractionated conformal radiotherapy for the management of primary optic nerve sheath meningioma.

BACKGROUND/AIM: Fractionated conformal radiotherapy (FCRT) is now used to treat vision-threatening optic nerve sheath meningioma (ONSM), but long-term efficacy and safety data are lacking; the purpose of this study was to assess these key data. METHODS: This is a retrospective chart review with prospective follow-up of adult patients treated with FCRT for primary ONSM at four academic medical centres between 1995 and 2007 with ≥10 years of follow-up after treatment. RESULTS: 16 patients were identified with a mean post-treatment follow-up of 14.6 years (range: 10.5-20.7 years). The mean age at symptom onset was 47.6 years (range: 36-60 years). FCRT was performed at a mean of 2.3 years after symptom onset (range: 0.2-14.0 years). At last follow-up, visual acuity had improved or stabilised in 14 of the 16 (88%) patients, and 11 (69%) had retained or achieved ≥20/40. The mean deviation on automated perimetry remained stable (-14.5 dB pretreatment vs -12.2 dB at last follow-up; p=0.68, n=10). Two (11%) patients had persistent pain, proptosis or diplopia, compared with six (38%) pretreatment (p=0.11). Two (13%) patients developed radiation retinopathy more than 6 months after completion of therapy, one (50%) of whom had worse visual acuity compared with pretreatment. No patient developed tumour involvement or radiation damage in the fellow eye. CONCLUSION: FCRT stabilises or improves visual function in patients with primary ONSM and is associated with a low risk of significant ocular sequelae. This treatment should be considered instead of surgery in patients with primary ONSM who require intervention due to loss of visual sensory and/or ocular motor function.

Risk of Acute Ischemic Stroke in Patients With Monocular Vision Loss of Vascular Etiology.

BACKGROUND: To evaluate the risk of concurrent acute ischemic stroke and monocular vision loss (MVL) of vascular etiology. DESIGN: Retrospective, cross-sectional study. SUBJECTS: Patients aged 18 or older diagnosed with MVL of suspected or confirmed vascular etiology who had no other neurologic deficits and who received brain MRI within 7 days of onset of visual symptoms were included. METHODS: A medical record review was performed from 2013 to 2016 at Yale New Haven Hospital. Patients were included if vision loss was unilateral and due to transient monocular vision loss (TMVL), central retinal artery occlusion (CRAO), or branch retinal artery occlusion (BRAO). Any patients with neurologic deficits other than vision loss were excluded. Other exclusion criteria were positive visual phenomena, nonvascular intraocular pathology, and intracranial pathology other than ischemic stroke. MAIN OUTCOME MEASURES: The presence or absence of acute stroke on diffusion-weighted imaging (DWI) on brain MRI. RESULTS: A total of 641 records were reviewed, with 293 patients found to have MVL. After excluding those with focal neurologic deficits, there were 41 patients who met the inclusion criteria and received a brain MRI. Eight of the 41 subjects (19.5%) were found to have findings on brain MRI positive for acute cortical strokes. The proportion of lesion positive MRI was 1/23 (4.3%) in TMVL subjects, 4/12 (33.3%) in CRAO subjects, and 2/5 (40%) in BRAO subjects. Brain computed tomography (CT) scans were not able to identify the majority of acute stroke lesions in this study. CONCLUSIONS: Patients with MVL of vascular etiology such as TMVL, CRAO, or BRAO may have up to 19.5% risk of concurrent ischemic stroke, even when there are no other neurologic deficits. These strokes were detected acutely with brain MRI using DWI but were missed on CT.

Visual prognosis in compressive optic neuropathy secondary to sphenoid sinus mucocele: A systematic review.

Sphenoid sinus mucoceles (SSMs) are rare, benign lesions that can expand, often presenting with ocular symptoms-decreased vision, diplopia, visual field defects, proptosis, and external ophthalmoplegia. Reported cases are few, visual compromise varies, and factors affecting visual prognosis are poorly characterized. We investigate whether prompt surgical intervention (within 2 weeks of visual symptom onset) affects best-corrected visual acuity (BCVA) regained in patients with vision loss secondary to compressive SSM. We present a retrospective review of three cases and published literature to date. Our primary outcome was BCVA regained after surgical intervention; secondary outcomes included change in visual field defect and ophthalmological symptoms other than vision loss. Our three cases of SSM varied in onset, ranging from several hours to several months with patients aged from 13 to 80 years. All patients had severe vision loss to light perception (LP) or worse. Rapid neuro-imaging and urgent surgical intervention improved vision to count fingers at best. Of the two patients who underwent prompt decompression, one improved from no LP to LP and the other did not recover any vision. The patient who had visual loss for 3 months before intervention improved from LP to 20/400. Findings from our literature search, which yielded 12 cases of urgent intervention, supported the variability in visual prognosis despite prompt surgical intervention. SSMs are rare, pathologically benign lesions which can expand to cause ocular involvement. Prompt diagnosis and surgical decompression are recommended, but visual recovery may be limited even with urgent intervention.

Genetic Anticipation in Familial Neuromyelitis Optica: Case and Literature Review.

OBJECTIVES: To describe genetic anticipation in a mother and daughter with antiaquaporin 4 (AQP4) antibody-positive neuromyelitisoptica (NMO). METHODS: Retrospective case review. RESULTS: A woman with onset of transverse myelitis at age 38 was found to have a positive AQP4 antibody during work-up of recurrent symptoms. Subsequently, she developed intermittent episodes of monocular vision loss with optic nerve involvement that were treated with intravenous methylprednisolone and chronic rituximab. Eighteen years after initial presentation, her 78-year-old mother, with a history of recurrent urinary tract infections, also developed monocular vision loss and her anti-AQP4 antibody was positive. Previous reports of genetic anticipation in familial NMO are identified and discussed. CONCLUSIONS: These cases highlight genetic anticipation in familial NMO. Disease onset can occur with a chronological age difference of as much as 40 years between parent and child. Patients with NMO should be counseled regarding the possibility of subsequent disease onset in family members, particularly parents, with significant differences in calendar or chronological year of onset.

Antiphospholipid Antibody Syndrome: Raised Intracranial Pressure Without Cerebral Venous Sinus Thrombosis.

Antiphospholipid antibody syndrome (APS) has been reported to cause elevated intracranial pressure, but usually this is due to cerebral venous sinus thrombosis (CVST). We present a 36-year old man with APS with elevated intracranial pressure with neuro-ophthalmic, renal and hematological involvement without identifiable CVST.

Topiramate-induced palinopsia: a case series and review of the literature.

BACKGROUND: To report palinopsia as a possible side effect of topiramate. METHODS: Case series and review of the literature. RESULTS: Nine patients in our series, and 4 previously reported patients, who developed palinopsia while on topiramate, are reviewed. All patients were women, and comorbidities included migraine, idiopathic intracranial hypertension, and bulimia nervosa. Palinopsia resolved in 8 patients after stopping or decreasing the dose of topiramate. The lowest dose of topiramate causing palinopsia was 25 mg twice a day. More than half of our patients reported exacerbation of visual disturbance in early morning or late evening. CONCLUSIONS: Topiramate-induced palinopsia may be underdiagnosed because physicians do not inquire about such visual symptoms.

Ocular myasthenia gravis.

PURPOSE OF REVIEW: To review ocular myasthenia gravis (OMG), a localized form of myasthenia gravis clinically involving only the extraocular, levator palpebrae superioris, and orbicularis oculi muscles. RECENT FINDINGS: Ocular manifestations can masquerade as a variety of ocular motility disorders, including central nervous system disorders and peripheral cranial nerve palsies. While sparing the pupils, the diagnosis and management can be challenging. SUMMARY: Because several diagnostic and treatment options are available for OMG, clinicians must decide the sequence and combination based on the level of disease activity and patient disability.

Is intravitreal bevacizumab an effective treatment option for nonarteritic anterior ischemic optic neuropathy?

Nonarteritic anterior ischemic optic neuropathy (NAION) causes sudden profound loss of vision with no known cause or cure. Various treatment modalities, both surgical and pharmacologic, have been tried without success. The purpose of our retrospective study was to evaluate the effect of intravitreal bevacizumab (Avastin) as a treatment option for NAION. We evaluated demographics of 5 patients and compared visual acuity and automated visual fields prior to and following intravitreal bevacizumab injection. Visual acuity at presentation was 20/20 in 4 of 5 patients and 20/150 in 1. Visual acuity improved to 20/40 in the patient who presented with decreased acuity and decreased slowly in 3 patients and rapidly in 1. All patients presented with variable visual field defects: 1 improved slightly, 3 progressed, and 1 remained stable. One patient subsequently developed NAION in the fellow eye. These results are consistent with the natural course of the disease, and bevacizumab did not appear to have a dramatic effect on the clinical outcome in this small series of patients with NAION.

Ocular motor dysfunction in Parry-Romberg syndrome: four cases.

The authors describe four patients with Parry-Romberg syndrome (PRS) who had abnormal eye movements, ptosis, and facial hemiatrophy. There were delays in diagnosis in all cases, often requiring multiple imaging studies, prior to diagnosis of PRS. These cases demonstrate the variable ophthalmic manifestations of PRS, which can lead to difficulty in diagnosis. Case 1 was a 23-year-old woman with lateral rectus paresis who presented with horizontal diplopia and developed ptosis and facial hemiatrophy. Case 2 was a 28-year-old woman with medial rectus weakness adjusted to diplopia by head turn and gaze preference. Case 3 was a 68-year-old woman who presented with dry eye symptoms and was noted to prefer right gaze and have left hemifacial atrophy. Case 4 was a 68-year-old woman who presented with ptosis, enophthalmos, and restriction of up gaze. The ocular motor defect in all of these cases is best explained by muscle fibrosis rather than nerve paresis.

Long-term response to fractionated radiotherapy of presumed optic nerve sheath meningioma.

BACKGROUND/AIMS To review the long-term results of treatment of optic nerve sheath meningiomas (ONSMs) with conformal radiotherapy. METHODS Eleven patients with presumed ONSM were treated with fractionated conformal radiotherapy using 45-54 Gy in 25-30 fractions and followed for more than 5 years. Affected eye visual acuity in logMAR notation, colour vision, threshold perimetry, mean deviation (dB) and imaging were studied before and after treatment. ANALYSIS Included adverse effects of treatment and the frequency of affected eye maintained or improved vision. RESULTS There were nine women and two men with a mean age of 45.3. Vision or field loss was the most common presentation. All had abnormal MR imaging. The follow-up period after radiotherapy was 61-156 months (mean 89.6). Visual acuity was unchanged or improved in 10 patients (91%). The average difference between pretreatment and final logMAR visual acuity was 0.08, while the average difference in visual-field mean deviation was -4.63 dB. The radiographic appearance of the tumours was stable in size in nine patients and decreased in two. No major side effects from radiation were seen. CONCLUSIONS Vision, MRI and complication outcomes are favourable for using fractionated conformal radiotherapy for ONSM.

Recurrent vision loss after endoscopic sinus surgery managed with palmar pressure.

A 41-year-old woman underwent endoscopic sinus surgery and 24 hours later, she developed acute orbital emphysema with marked vision loss. CT showed disruption of the inferior aspect of the medial wall of the orbit with fat herniation. She was immediately treated with the application of bedside palmar pressure to the globe with complete return of her vision without the need for a needle aspiration or orbital decompression. Weeks later, the patient experienced 2 more episodes of transient monocular visual loss that were again successfully treated with the same palmar pressure. Bedside digital decompression may be a potentially helpful method of restoring vision in cases of orbital emphysema with acute vision loss. We advise attempting this simple bedside procedure before committing the patient to a more invasive needle aspiration or surgical decompressive procedure.

Posterior ischemic optic neuropathy after minimally invasive prostatectomy.

Two patients developed postoperative ischemic optic neuropathy (ION) after laparoscopic radical prostatectomy. One operation was robotically assisted; the other was performed with the conventional laparoscopic technique. These new minimally invasive techniques offer many advantages, but they require steep supine head-flexed (Trendelenburg) positioning. Until they are mastered by surgeons, operative times may be prolonged beyond those associated with the traditional technique. As a result, ION may occur more frequently.

Choroidal and optic nerve infarction in hepatitis C-associated polyarteritis nodosa.

A 39-year-old man presented with headache, weight loss, bilateral subdural hematomas, pansinusitis, and visual loss. The neuro-ophthalmologic examination disclosed deep choroidal lesions and bilateral optic disc edema. Orchiectomy for testicular torsion showed acute vasculitis consistent with polyarteritis nodosa (PAN). Polymerase chain reaction (PCR) testing revealed hepatitis C. This is the first reported case of PAN due to hepatitis C with early findings of choroidal and optic nerve infarction.

Progression of visual field defects in leber hereditary optic neuropathy: experience of the LHON treatment trial.

PURPOSE: To describe the visual fields of patients with Leber hereditary optic neuropathy (LHON), a maternally inherited disorder characterized by bilateral, often sequential vision loss, before and during progressive visual deterioration. DESIGN: Prospective longitudinal follow-up of serial visual fields in patients enrolled onto an open-label, nonrandomized pilot study of topical brimonidine purite as prophylactic treatment after first eye involvement in LHON. METHODS: Nine molecularly confirmed primary mutation patients with LHON with monocular vision loss for less than six months and normal visual function in the other eye were followed prospectively for up to two years. Visual fields were performed on automated perimetry at baseline and on many follow-up visits. RESULTS: Despite normal visual acuity at baseline in all patients, seven patients had some minimal changes in the central visual field of the second eye. All patients had subsequent deterioration of visual acuity, mean deviation, and foveal sensitivity in their second eye. The earliest pattern of abnormality was typically a cecocentral defect enlarging to become a central defect, often with a superior or inferior predilection. The visual field defects in the two eyes of any given patient were remarkably similar. CONCLUSIONS: LHON may be a bilateral condition at onset more frequently than appreciated. Automated static perimetry of the "normal" eye may reveal subclinical findings that typically worsen rapidly over weeks to months to similar central scotomatous damage. Quantitative automated static perimetry is helpful in elucidating the natural history of LHON and in understanding the underlying pathology and pathophysiology of this disease.

Neuro-ophthalmologic presentations of hemicrania continua.

PURPOSE: We describe a series of patients with hemicrania continua, a rare indomethacin-responsive primary headache syndrome, who presented for evaluation with neuro-ophthalmologic signs and symptoms. DESIGN: Observational case series. METHODS: Nine patients between the ages of 29 and 58 years were seen with various neuro-ophthalmologic findings and a unilateral continuous headache. A detailed history was taken from each patient, followed by a focused ophthalmologic and neurologic examination. The risks and benefits of treatment with indomethacin were discussed. Patients were instructed to call after several days of treatment to report any change in their headache and neuro-ophthalmologic symptoms, in addition to any adverse side effects. RESULTS: All patients responded favorably to indomethacin, with rapid near-complete or complete resolution of headache and autonomic symptoms, and treatment was initiated as early as possible. CONCLUSIONS: Because of its absolute response to indomethacin, recognizing the neuro-ophthalmologic symptoms of hemicrania continua as a component of the headache syndrome is critical for prompt initiation of treatment.