Optic Perineuritis in Behçet Disease.

Background: Optic perineuritis (OPN), an uncommon optic neuropathy, has previously not been described in patients with Behçet disease (BD). We conducted this study to describe the clinical features, response to treatment, and outcome of OPN due to BD, with particular emphasis on those features that might distinguish this from the idiopathic variety.

Methods: This is a retrospective, case series review of all patients with a diagnosis of OPN seen in a hospital-based neurology department from 2008 to 2014 who also met the international criteria for the diagnosis of BD.

Transient ocular motor nerve palsies associated with presumed cranial nerve schwannomas.

Background: Cranial nerve schwannomas are radiologically characterized by nodular cranial nerve enhancement on magnetic resonance imaging (MRI). Schwannomas typically present with gradually progressive symptoms, but isolated reports have suggested that schwannomas may cause fluctuating symptoms as well.

Methods: This is a report of ten cases of presumed cranial nerve schwannoma that presented with transient or recurring ocular motor nerve deficits.

Non-compressive disorders of the chiasm.

Chiasmal dysfunction produces a characteristic clinical picture, regardless of the mechanism. In most cases a compressive lesion is the cause. In occasional cases, however, no such extrinsic mass is found and other possible etiologies must be explored.

A weed by any other name.

A 39-year-old white man presented with intractable headaches and papilledema. The initial workup, with normal MRI and MRV but elevated cerebrospinal fluid protein raised concerns about the putative diagnosis of idiopathic intracranial hypertension, and his condition remained refractory to maximum medical treatment. Angiography revealed cerebral venous sinus stenosis, thought to represent chronic thrombosis.

Bilateral sequential nonarteritic anterior ischemic optic neuropathy: a comparison of visual outcomes in fellow eyes using quantitative analysis of goldmann visual fields.

Objective: To better define the concordance of visual loss in patients with nonarteritic anterior ischemic optic neuropathy (NAION).

Methods: The medical records of 86 patients with bilateral sequential NAION were reviewed retrospectively, and visual function was assessed using visual acuity, Goldmann visual fields, color vision, and relative afferent papillary defect. A quantitative total visual field score and score per quadrant were analyzed for each eye using the numerical Goldmann visual field scoring method.

Neuroretinitis: review of the literature and new observations.

Neuroretinitis (NR) is an inflammatory disorder characterized by optic disc edema and subsequent formation of a macular star figure. The underlying pathophysiology involves increased permeability of disc vasculature, but the etiology is not fully defined. In some cases, NR is probably due to an infectious process involving the disc; in others, a postviral or autoimmune mechanism is more likely.

Vasculopathic cranial ocular motor neuropathy following sudden emotional stress.

We describe three patients who experienced onset of a microvascular ocular motor nerve palsy in the setting of sudden emotional stress. Such emotional states are accompanied by a marked increase in sympathetic tone in some individuals. Mechanisms by which these autonomic changes might produce an ischemic cranial nerve palsy include intra-cranial vasoconstriction and transient systemic hypotension due to alterations in cardiac function.

Photo essay. Isolated fascicular third nerve palsy.

A 72-year-old hypertensive woman developed acute diplopia and right upper lid ptosis. Examination showed a partial right third nerve palsy with complete ptosis, moderately impaired adduction and supraduction, and sparing of infraduction and the pupil. There were no other neurologic deficits.

Recurrent idiopathic neuroretinitis: natural history and effect of treatment.

Background: Previous reports have emphasized the self-limited nature of idiopathic neuroretinitis. There is less information about a subgroup of patients who suffer recurrent episodes with worse visual outcome. We sought to better characterize the clinical features of recurrent idiopathic neuroretinitis including the effects of immunosuppressive treatment.

Multiple sclerosis on steroids.

A 57-year-old man developed acute bilateral vision loss clinically consistent with bilateral optic neuritis. Within 1 month of diagnosis, he developed progressive and severe neurologic dysfunction, and repeat MRI demonstrated enhancement of the optic chiasm and optic tracts, as well as a large enhancing lesion within the right parieto-occipital lobe. Stereotactic-guided brain biopsy demonstrated demyelination consistent with multiple sclerosis.

Optic perineuritis secondary to Wegener's granulomatosis.

Background: Optic perineuritis (OPN) is an inflammatory condition involving the optic nerve sheath because of a variety of causes. We describe three patients in whom OPN was secondary to Wegener's granulomatosis (WG) and compare the clinical findings in these cases with those of idiopathic OPN.

Methods: This is a retrospective small case series derived from patients with OPN seen in an outpatient neuro-ophthalmology clinic.

Idiopathic chiasmal neuritis: clinical features and prognosis.

Objectives: To describe the clinical features of idiopathic chiasmal neuritis in a large cohort of patients and to report their visual and neurologic outcomes.

Design: A retrospective medical record review of consecutive patients with chiasmal neuritis at a single institution. Patients with clinical or radiographic evidence of inflammation involving the intraorbital optic nerve and patients with a systemic inflammatory or neoplastic disorder were excluded.

Giant cell arteritis: an updated review.

Giant cell arteritis (GCA) is the most common primary vasculitis of adults. The incidence of this disease is practically nil in the population under the age of 50 years, then rises dramatically with each passing decade. The median age of onset of the disease is about 75 years.

Peripheral field loss: something old, something new.

A 37-year-old patient presented with acute visual field loss diagnosed as non-arteritic anterior ischemic optic neuropathy in the setting of optic nerve drusen. Her visual field loss had progressed when compared to the visual field done 2 years previously. Fundus examination showed bilateral optic nerve head drusen and left retinal nerve fiber layer edema consistent with non-arteritic anterior ischemic optic neuropathy.

Acquired ocular motor apraxia after aortic surgery.

Purpose: To describe an unusual form of acquired ocular motor apraxia.

Methods: Case reports with electronic eye movement recordings.

Results: Three patients had surgery to repair aortic root or arch dissections or aneurysms.

Giant cell arteritis with spontaneous remission.

Background: Clinical manifestations of giant cell arteritis (GCA) are variable. Whether signs and symptoms present in an explosive fashion or insidiously, once manifest the course is usually progressive unless treatment is initiated.

Methods: A retrospective review of patients with GCA seen in an outpatient neuro-ophthalmology clinic.

Superior oblique myokymia: efficacy of medical treatment.

Purpose: Superior oblique myokymia (SOM) is an uncommon disorder characterized by episodic monocular oscillopsia. Several medications have been reported to be of benefit for some patients with this condition, but the efficacy of medical treatment has not been well established and little long-term follow-up data are available. The purpose of this study was to better clarify the role of medical therapy in the management of SOM.

Recovery of postoperative visual loss following treatment of severe anaemia.

A 29-year-old pregnant woman noted acute visual loss following emergent Caesarean section complicated by excessive uterine bleeding. Postoperative visual acuity was count fingers in both eyes. Funduscopic changes were consistent with a diagnosis of anaemia-associated ischaemic optic neuropathy and retinopathy.

Optic neuropathy in patients using amiodarone.

Objective: To refine the criteria for the diagnosis of amiodarone-related optic neuropathy by including a broader spectrum of clinical features, thus helping to differentiate this entity from nonarteritic anterior ischemic optic neuropathy coincidentally affecting a patient taking amiodarone.

Methods: A retrospective case review of 22 patients who developed optic neuropathy while taking amiodarone, in whom other systemic causes were excluded.

Results: We identified 3 groups of patients: those in whom a diagnosis of amiodarone-induced optic neuropathy seems probable (n = 14), those in whom an association with amiodarone optic neuropathy is indeterminate (n = 5), and those in whom the occurrence of nonarteritic anterior ischemic optic neuropathy seems to be coincidental (ie, unrelated to amiodarone) (n = 3).

Statin-associated myasthenia gravis: report of 4 cases and review of the literature.

A few recent individual case reports have suggested that a myasthenic syndrome may be associated with statin treatment, but this association is not well described. We report 4 patients who developed symptoms of myasthenia gravis within 2 weeks of starting treatment with a statin drug. In 1 case the drug appears to have exacerbated underlying myasthenic weakness, whereas in the other 3 cases, de novo antibody formation appears to be most likely.

Intraorbital optic nerve signal hyperintensity on magnetic resonance imaging sequences in perioperative hypotensive ischemic optic neuropathy.

A 61-year-old man experienced severe bilateral posterior ischemic optic neuropathy after cardiac bypass surgery. Routine magnetic resonance imaging sequences were normal, but diffusion-weighted and fluid-attenuated inversion recovery (FLAIR) sequences showed abnormal hyperintensity within both intra-orbital optic nerves. This imaging abnormality has not been previously reported in this setting.