Jumping Jack Flash.

A 38-year-old woman presented with blurred vision and "jumping" of the right eye for 7 months. Magnetic resonance imaging of the head was normal. Intermittent intorsion of the right eye was noted on examination, consistent with superior oblique myokymia. She was initially treated with carbamazepine but stopped after becoming light-headed. The diagnosis and treatment of superior oblique myokymia are discussed.

Perioperative posterior ischemic optic neuropathy: review of the literature.

Posterior ischemic optic neuropathy (PION) is an uncommon cause of perioperative visual loss. Perioperative PION has been most frequently reported after spinal surgery and radical neck dissection. The visual loss typically presents immediately after recovery from anesthesia, although it may be delayed by several days. Visual loss is often bilateral and profound with count fingers vision or worse. The examination findings are consistent with an optic neuropathy; however the funduscopic examination is initially normal. The cause is unknown, although patient-specific susceptibility to perioperative hemodynamic derangements is likely. No treatment has proven to be effective. The prognosis for visual recovery is generally poor.

The silent sinus syndrome: maxillary sinus atelectasis with enophthalmos and hypoglobus.

PURPOSE OF REVIEW: The silent sinus syndrome is a rare clinical entity of spontaneous enophthalmos and hypoglobus caused by an alteration of the normal orbital architecture and function from maxillary sinus collapse in the setting of chronic sinusitis. RECENT FINDINGS: The maxillary sinus collapse appears to result from the development of negative sinus pressure from an acquired obstruction of the maxillary sinus outflow. Patients most often present with symptoms relating to enophthalmos, although few report any symptoms of sinus disease. SUMMARY: Characteristic radiographic features of the maxillary sinus including opacification and collapse of the antral walls with inward bowing of the orbital floor are necessary for diagnosis.

Unexplained visual loss.

Dominant optic atrophy is the most common heredodegenerative optic neuropathy. Typically, patients present with slowly progressive, bilaterally decreased central visual acuity. Subtle central or cecocentral visual field defect and normal peripheral isopters are demonstrated with perimetry. A defect in blue-yellow discrimination (tritan error axis) is the most common type of dyschromatopsia, however protan and deutan axes may be superimposed. A characteristic optic disk appearance includes temporal disk pallor with excavation. An autosomal dominant inheritance pattern can often be elicited from the family history.

Horner syndrome.

Horner syndrome refers to the constellation of signs resulting from the interruption of sympathetic innervation to the eye and ocular adnexae. Classically, the clinical findings include a triad of ipsilateral blepharoptosis, pupillary miosis, and facial anhidrosis. The history, additional clinical examination features, and pharmacologic testing may help localize the lesion and suggest an etiology. An appropriate evaluation of Horner syndrome and a timely elucidation of the etiology may allow for a potentially life-saving intervention.

Foggy visual field defect.

A 32-year-old female migraneur developed a right incongruous homonymous hemianopia after taking the antiepileptic agent, topiramate. The visual field defect partially resolved when the medication was discontinued. The differential diagnosis of the homonymous hemianopia is discussed.

Posterior ischemic optic neuropathy after hemodialysis.

PURPOSE: To report a case of visual loss from posterior ischemic optic neuropathy (PION) after hemodialysis. DESIGN: Observational case report. METHODS: Neuro-ophthalmic examination, neuro-imaging including computed tomography (CT) scan, magnetic resonance imaging (MRI) of the head and orbits, and magnetic resonance angiography (MRA) of the neck and cerebral vasculature, as well as electrophysiologic testing including electroretinogram (ERG) and visually evoked response (VER) were performed. RESULTS: Acute onset of painless bilateral no light perception vision with absent pupillary response to light and normal funduscopic examination occurred shortly after completion of hemodialysis. Computed tomography scan and MRA results were normal. Magnetic resonance imaging scan showed small vessel ischemic white matter changes. Electroretinogram results were normal and the VER was unrecordable. CONCLUSIONS: Visual loss after hemodialysis is a rare complication and is associated with anemia and hypotensive events. The visual loss is usually a result of anterior ischemic optic neuropathy. We were unable to find another instance in the literature of visual loss after hemodialysis resulting from PION.

Scanning laser polarimetry of the retinal nerve fiber layer in central retinal artery occlusion.

PURPOSE: To report the results of scanning laser polarimetry (NFA/GDx; Laser Diagnostic Technologies, San Diego, CA). DESIGN: Prospective, consecutive observational case series. PARTICIPANTS: Ten consecutive patients with central retinal artery occlusion (CRAO). METHODS: Neuro-ophthalmic examination and scanning laser polarimetry of the retinal nerve fiber layer (RNFL) of 10 patients with CRAO. MAIN OUTCOME MEASURES: Duration of visual loss, visual acuity, funduscopy, and scanning laser polarimetry of the RNFL in 10 eyes of 10 patients with CRAO. RESULTS: The duration of visual loss before examination and scanning laser polarimetry ranged from 1 to 7 days. Visual acuity was counting fingers at 1 foot or worse in all 10 eyes with CRAO, and funduscopy revealed pallid retinal edema accompanied by a cherry red spot in all affected eyes. Funduscopy of the fellow eyes in all but one patient, who had anterior ischemic optic neuropathy and was subsequently diagnosed with giant cell arteritis, revealed no acute changes. Scanning laser polarimetry of all eyes with CRAO revealed diffuse attenuation of the retardation of the RNFL. Scanning laser polarimetry of the fellow eye revealed a normal bimodal distribution in the eight patients in whom it could be measured. In one patient who was examined 1 day after the onset of visual loss, repeat nerve fiber analysis 6 weeks later revealed further depression of the RNFL compared with the initial scan. Repeat analysis of the RNFL in four other patients showed persistence of the diffuse depression noted during their initial examinations. CONCLUSIONS: Scanning laser polarimetry in CRAO reveals diffuse depression of the retardation of the RNFL, which occurs acutely after the onset of visual loss. To our knowledge these patients represent the first reports of scanning laser polarimetry of the RNFL in acute CRAO.

Recovery of visual function in patients with biopsy-proven giant cell arteritis.

OBJECTIVE: To assess the visual function of patients with giant cell arteritis (GCA) who had visual loss from either anterior ischemic optic neuropathy (AION) or central retinal artery occlusion and had a subsequent improvement in visual acuity after treatment with corticosteroids. DESIGN: Retrospective, observational case series. PARTICIPANTS: Thirty-two consecutive patients with biopsy-proven GCA treated at one institution between January 1992 and December 1997. INTERVENTION: Treatment with intravenous methylprednisolone 250 mg every 6 hours for 3 days, followed by oral prednisone 1 mg/kg daily for at least 4 weeks duration. MAIN OUTCOME MEASURES: The number of patients with an improvement in visual acuity after treatment with intravenous methylprednisolone; neuro-ophthalmic evaluation, including visual acuity, funduscopy, and visual field examination of these patients. RESULTS: Improvement in visual acuity occurred in 5 of 39 eyes (13%) with visual loss from biopsy-proven GCA, and all 5 patients had AION. Despite the improvement of visual acuity in these 5 patients, perimetry revealed marked constriction of the visual field in each affected eye. CONCLUSIONS: The prognosis for visual improvement in GCA is poor. Although an improvement in visual acuity occurred in 5 of our patients, marked constriction of the visual field was present in all of them.

Optic disc structure and shock-induced anterior ischemic optic neuropathy.

PURPOSE: To describe a patient who developed unilateral shock-induced anterior ischemic optic neuropathy (SIAION) after gastrointestinal hemorrhage followed by presumed idiopathic nonarteritic anterior ischemic optic neuropathy (NAION) in the fellow eye. DESIGN: Retrospective, observational case report and literature review. METHODS: The case history of an 80-year-old man who developed SIAION, followed by NAION in the fellow eye, was reviewed. All previously reported cases of SIAION were reviewed. MAIN OUTCOME MEASURES: Neuro-ophthalmic examination, including visual acuity, funduscopy, and automated perimetry. RESULTS: An 80-year-old man, with a history of gastrointestinal bleeding from a duodenal ulcer, was hospitalized and received four units of packed red blood cells after he was found to be severely anemic (hemoglobin 6.7 g/dl). Three days later he complained of loss of vision of the right eye. Neuro-ophthalmic examination 2 weeks later disclosed a visual acuity of counting fingers at 6 inches in the right eye and 20/40 in the left eye, with a right relative afferent pupillary defect and a superior altitudinal visual field defect. Funduscopy revealed optic disc edema with a temporal parapapillary hemorrhage in the right eye and a small optic disc, with no cup, in the left eye. A diagnosis of SIAION secondary to anemia was made. Six weeks later he developed a new inferior altitudinal visual field defect in the left eye and diffuse optic disc swelling. He had no signs or symptoms of giant cell arteritis or polymyalgia rheumatica, his hemoglobin at this time was 11.9 g/dl, and the Westergren erythrocyte sedimentation rate was 6 mm/hour. CONCLUSIONS: Our patient developed optic disc swelling of the right eye after an episode of gastrointestinal hemorrhage (SIAION). The disc swelling in the left eye occurred 8 weeks later, when his hemoglobin had increased to 11.9 g/dl. The timing of the ischemic optic neuropathies suggests that the acute anemia led to involvement of the first but not the second eye. The configuration of the optic disc may have predisposed not only to the second event (NAION) but also to the first episode (SIAION).

Nonarteritic anterior ischemic optic neuropathy.

Nonarteritic anterior ischemic optic neuropathy refers to an idiopathic ischemic process of the anterior portion of the optic nerve. The typical presentation is sudden and painless visual loss with examination features of an optic neuropathy. Among the various associated risk factors are optic disc morphology, advanced age, systemic arterial hypertension, diabetes mellitus, and nocturnal hypotension. Currently, there is no proven effective long-term treatment for this disorder.

Is normal tension glaucoma actually an unrecognized hereditary optic neuropathy? New evidence from genetic analysis.

Normal tension glaucoma and dominant optic atrophy share many overlapping clinical features, and differentiating between these two diseases is often difficult. The gene responsible for dominant optic atrophy is the OPA1 gene located on chromosome 3. This gene encodes for a protein product that is involved in mitochondrial metabolic function. Recent genetic linkage analysis of patients with normal tension glaucoma has shown an association with polymorphisms of the OPA1 gene. This association suggests that normal tension glaucoma may actually be a hereditary optic neuropathy with a pathophysiology based in mitochondrial dysfunction.

Acute demyelinating optic neuritis.

Acute demyelinating optic neuritis associated with multiple sclerosis (MS) is the most common cause of inflammation of the optic nerve. The Optic Neuritis Treatment Trial (ONTT) has provided important clinical data on the use of corticosteroids, and demonstrated that patients with characteristic inflammatory lesions within the brain on magnetic resonance imaging had a greater chance of developing clinically definite MS (CDMS). The current approach to patients with optic neuritis has been modified by the results of the Controlled High-Risk Subjects Avonex Multiple Sclerosis Prevention Study (CHAMPS). Patients with an initial clinical episode of demyelination (optic neuritis, incomplete transverse myelitis, or brain-stem/cerebellar syndrome) and at least two characteristic demyelinating lesions within the brain were randomized to receive interferon beta-1a or placebo after initial treatment with intravenous corticosteroids. At the 3-year point patients treated with interferon beta-1a showed a 50% less risk of CDMS. The results of this study have set the standard for patients with a first bout of demyelinating optic neuritis.

Metastatic synovial sarcoma to the skull base and orbit.

PURPOSE: To report a case of metastatic synovial sarcoma to the parasellar area and orbit. DESIGN: Interventional case report. METHODS: A 43-year-old woman with a history of a right forearm synovial sarcoma treated with excision, radiation, and chemotherapy 16 months previously presented with blurred vision of the right eye, pain with eye movement, epistaxis, and a right superior division cranial nerve III palsy. Magnetic resonance imaging showed a right parasellar and right orbital apex mass. RESULTS: Pathologic examination of the parasellar biopsy revealed metastatic synovial sarcoma. At 6-month follow-up, the neuro-ophthalmic examination was unchanged, and repeat magnetic resonance imaging showed no recurrence of the tumor. CONCLUSIONS: Metastatic synovial sarcoma should be added to the differential diagnosis of parasellar and orbital tumors.