Ophthalmic presentation of giant cell arteritis in African-Americans.

PurposeTo determine the differences in the presentation of ophthalmic giant cell arteritis between African-Americans and Caucasians.MethodsThis was a multicenter retrospective case series comparing African-American patients with ophthalmic GCA to a previously published Caucasian cohort. Neuro-ophthalmic centers across the United States were contacted to provide data on African-American patients with biopsy-proven ophthalmic giant cell arteritis. The differences between African-American and Caucasian patients with respect to multiple variables, including age, sex, systemic and ophthalmic signs and symptoms, ocular ischemic lesions, and laboratory results were studied.ResultsThe Caucasian cohort was slightly older (mean=76.1 years) than the African-American cohort (mean=72.6 years, P=0.03), and there was no difference in sex distribution between the two cohorts. Headache, neck pain, and anemia were more frequent, while jaw claudication was less frequent in African-Americans (P<0.01, <0.001, 0.02, and 0.03 respectively). Acute vision loss was the most common presentation of giant cell arteritis in both groups, though it was less common in African-Americans (78 vs 98% of Caucasians, P<0.001). Eye pain was more common in African-Americans (28 vs 8% of Caucasians, P<0.01).ConclusionsThe presenting features of ophthalmic giant cell arteritis in African-Americans and Caucasians are not markedly different, although a few significant differences exist, including higher rates of headache, neck pain, anemia, and eye pain, and lower rates of jaw claudication and acute vision loss in African-Americans. Persons presenting with suspicious signs and symptoms should undergo evaluation for giant cell arteritis regardless of race.

Non-arteritic anterior ischaemic optic neuropathy and the treatment of erectile dysfunction.

AIM: To determine the association between Viagra (sildenafil) and Cialis (tadalafil) and non-arteritic anterior ischaemic optic neuropathy (NAION). METHODS: A retrospective matched case-control study was conducted. 38 cases of NAION in males were identified from an academic ophthalmology practice in Birmingham, Alabama, and matched (on age) to 38 controls without a history of NAION. Self reported information regarding past and current use of Viagra and/or Cialis was obtained via a telephone questionnaire from interviewers who were not blind to case status. RESULTS: Overall, males with NAION were no more likely to report a history of Viagra or Cialis use compared to similarly aged controls (odd ratio (OR) 1.75, 95% confidence interval (CI) 0.48 to 6.30 and OR 1.82, 95% CI 0.21 to 15.39). However, for those with a history of myocardial infarction, a statistically significant association was observed (OR 10.7, 95% CI 1.3 to 95.8). A similar association was observed for those with a history of hypertension though it lacked statistical significance (OR 6.9, 95% CI 0.8 to 63.6). CONCLUSIONS: For men with a history of myocardial infarction or hypertension the use of Viagra or Cialis may increase the risk of NAION. Physicians prescribing these medications to patients with these conditions should warn them about the potential risk of NAION.

Disk edema and cranial MRI optic nerve enhancement: how long is too long?

A 43-year-old woman presented with painful visual loss and optic disk edema in the right eye (OD) diagnosed as optic neuritis. Initial non-gadolinium-enhanced fat suppressed cranial magnetic resonance imaging (MRI) was normal. Three months later, the disk edema persisted and a gadolinium-enhanced MRI scan of the brain and orbits with fat suppression showed enhancement of the optic nerve OD, most consistent with an optic nerve sheath meningioma. The diagnostic difference between optic neuritis and optic nerve sheath meningioma is discussed.

Catastrophic antiphospholipid antibody syndrome manifesting as an orbital ischemic syndrome.

Painful bilateral ophthalmoparesis, marked proptosis, increased intraocular pressure, and blindness developed in a 29-year-old woman with protein C deficiency and catastrophic antiphospholipid syndrome. Magnetic resonance imaging of the orbits showed bilateral proptosis, globe tenting, and tethering of the optic nerves consistent with an orbital ischemic syndrome. Despite aggressive therapy for antiphospholipid syndrome, the patient died. The autopsy showed necrosis of orbital tissues. This is the first report of orbital ischemic syndrome from protein C deficiency and antiphospholipid syndrome.

Atypical features prompting neuroimaging in acute optic neuropathy in adults.

BACKGROUND: Acute optic neuropathy due to an intracranial lesion may masquerade as optic neuritis or nonarteritic anterior ischemic optic neuropathy (NAION). We reviewed the records of patients who presented with acute unilateral optic neuropathy that was initially diagnosed as optic neuritis or NAION but who ultimately proved to have an underlying structural lesion. METHODS: Retrospective observational case series. We reviewed the records of patients with the initial diagnosis of optic neuritis or NAION in whom the diagnosis was changed to an intracranial etiology at four tertiary care neuro-ophthalmology centres between 1995 and 1998. RESULTS: Eight cases were identified in which atypical features prompted further investigation, including neuroimaging, leading to the diagnosis of an intracranial etiology for the optic neuropathy. Five patients were discovered to have neoplasms (a tuberculum sellae meningioma in two cases, an optic nerve sheath meningioma in two cases and a metastatic lesion in one case), and three patients had intracranial sarcoidosis. Atypical features for optic neuritis included a progressive course, absence of pain, optic atrophy at presentation, lack of significant visual improvement and age over 40 years. For NAION, the atypical features included progressive course, optic atrophy on presentation, absence of vasculopathic risk factors and preceding transient visual loss. INTERPRETATION: Clinicians should be aware that patients with intracranial lesions may present with acute optic neuropathy mimicking optic neuritis or NAION and that certain atypical features should warrant consideration for neuroimaging.

Node way out.

A 47-year-old woman without a history of melanoma experienced visual hallucinations, abnormal visual field, and an ERG suggestive of melanoma-associated retinopathy (MAR). A lymph node biopsy showed malignant melanoma. Appropriate evaluation, management, and treatment of MAR is discussed.

Optic nerve enhancement on orbital magnetic resonance imaging in Leber's hereditary optic neuropathy.

An 18-year-old man with Leber's hereditary optic neuropathy and bilateral visual loss had optic nerve enhancement on T1-weighted orbital fat-suppressed magnetic resonance imaging. To our knowledge, this is the first reported case of optic nerve enhancement on orbital magnetic resonance imaging in Leber's hereditary optic neuropathy.

Cat-scratch disease presenting as neuroretinitis and peripheral facial palsy.

A 40-year-old woman with Cat-scratch disease sought treatment for neuroretinitis OD and right peripheral facial nerve palsy. To our knowledge, this is the first case of an adult with a peripheral facial nerve palsy from Cat-scratch disease and the first case of a patient with both neuroretinitis and peripheral facial nerve palsy.

A bad eye and a sore lip.

A 48-year-old woman developed painful visual loss in the left eye, meningismus, and painful oral ulcers. Magnetic resonance imaging of the brain with gadolinium demonstrated enhancement of the left optic nerve. Lumbar puncture showed a lymphocytic pleocytosis, and a biopsy specimen of one of the oral ulcerations was consistent with Behçet's disease. Epidemiologic factors and diagnostic criteria for Behçet's disease are discussed.

Other intracranial mass lesions.

This article outlines the various signs and symptoms associated with intracranial gliomas, meningiomas, and hypertensive hemorrhages. It emphasizes the neurologic and neuro-ophthalmologic presentations of these intracranial mass lesions. Important pathways that subserve horizontal eye movements are examined and the ocular motility disorders associated with lesions of these fibers are outlined.

Childhood sarcoidosis.

A 10-year-old black girl exhibited a bilateral lower motor neuron facial palsy and bilateral hearing loss. Gadolinium-enhanced magnetic resonance imaging of the brain showed enhancement of the cranial nerve VII-VIII complex bilaterally. Disc elevation developed in both eyes, and the patient exhibited facial swelling. Examination of a right parotid biopsy specimen demonstrated noncaseating granulomas, consistent with the diagnosis of sarcoidosis.

Hemianopic anosognosia.

In a prospective study of 32 consecutive patients with homonymous visual field defects due to ischemic infarcts we found hemianopic anosognosia (HAN), defined as the unawareness of visual loss in the homonymous hemifield (or hemiquadrant), in 20 patients (62%). HAN, although occurring predominantly in right-side lesions in 16 of 26 patients (62%) was also present in four of six patients (or 67%) with left-side lesions. This group of patients has been presented in a prior report on positive spontaneous visual phenomena. HAN was associated with somatic anosognosia in nine patients and hemineglect in 17 patients. Dissociation between somatic and hemianopic anosognosia, as well as between hemineglect and HAN, was present in several patients, indicating that these phenomena may be independent of each other. Eight patients had pure homonymous hemianopia; that is, hemianopia without cognitive, motor, or somatosensory deficits. Four of these patients (Group A) had awareness of the visual deficit, whereas three patients (Group B) had HAN. Patients in these two groups had similar anatomic lesions. Patients with phosphenes, photopsias, or visual hallucinations were usually aware of their visual field loss. We suggest that HAN is most often related to failure of discovery of the deficits, occasionally to severe visual hemineglect, sometimes to generalized cognitive impairment, or to a combination of these factors. We further conclude (1) there is no specific cortical area for conscious visual perception; (2) visual awareness is processed by a distributed network including multiple visual cortices, parietal and frontal lobes, the pulvinar, and lateral geniculate bodies (lesions localized at various nodes or centers in the network may produce similar phenomena); and (3) both hemispheres are involved in visual processing and conscious awareness.

The Pulfrich effect and its relationship to retinal illumination.

The Pulfrich stereo-illusion effect occurs in patients with monocular anterior visual system disease when viewing a pendulum. It also can be induced by placing a monocular neutral-density filter in a normal subject. We studied 10 normal subjects to determine if the Pulfrich effect could be induced by monocular illumination in addition to monocular neutral-density filtration. Eighteen of 20 eyes observed the Pulfrich effect with monocular illumination, but the rotation of the ellipse was opposite in direction to that of the neutral-density filter. Our data suggests that the Pulfrich effect results from intereye luminance disparity.

Positive spontaneous visual phenomena limited to the hemianopic field in lesions of central visual pathways.

We prospectively studied 32 patients with ischemic infarction of the retrochiasmal visual pathways. Positive spontaneous visual phenomena (PSVP) in the blind hemifield were present in 13 patients (41%). The PSVP were subdivided into phosphenes, photopsias, visual hallucinations, palinopsia, and agitated delirium with hemianopia. PSVP were never associated with auditory or other sensory positive phenomena, except in patients with agitated delirium. Patients with photopsias, phosphenes, palinopsia, and visual hallucinations had similar lesions in MRI/CT, suggesting no anatomic area unique for these four phenomena. However, there was a significant difference in the severity of associated neurologic deficits between hemianopic patients with and without PSVP. Larger lesions destroying anteriorly located visual association areas precluded the development of PSVP, which may be related to release from inhibitory input of visual regions bordering the damaged area. Patients with the syndrome of agitated delirium and hemianopia had specific lesions involving the mesial aspect of the occipital lobe, the parahippocampal gyrus, and hippocampus.