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.

The crossed paralyses. The original brain-stem syndromes of Millard-Gubler, Foville, Weber, and Raymond-Cestan.

In this article, the crossed syndromes of Millard-Gubler (facial palsy and contralateral hemiparesis), Foville (facial palsy, conjugate gaze paralysis, and contralateral hemiparesis), Weber (oculomotor palsy and contralateral hemiparesis), and Raymond-Cestan (internuclear ophthalmoplegia and contralateral hemiparesis) are detailed from the original reports. These and other related syndromes were instrumental in establishing important principles in brain-stem localization: the occurrence of cranial nerve palsies contralateral to hemibody motor or sensory disturbances, the concepts of the medial longitudinal fasciculus and conjugate gaze, and the corticobulbar innervation of the facial nerve nucleus.

Oculomotor synkinesis following a midbrain stroke.

A 33-year-old man developed a complete third nerve palsy in the setting of acute bacterial endocarditis. MRI revealed an ischemic stroke in the cerebral peduncle involving the third nerve fascicle. Subsequently, he was observed to have paradoxic elevation of the eyelid on adduction and downgaze. To the authors' knowledge, this is the first demonstration of oculomotor synkinesis after an acquired, ischemic CNS lesion.

Evidence for preganglionic pupillary involvement in superficial siderosis.

A 36-year-old man presented with spinal myoclonus, ataxia, hearing loss, and unilateral pupillary dilation. MRI demonstrated hemosiderin deposition along the superficial surfaces of the brain, brainstem, cerebellum, and spine. The pupillary changes were localized to the preganglionic oculomotor nerve. In contrast to vasculopathic oculomotor nerve palsies, superficial siderosis may cause selective involvement of the superficially located pupillary fibers.

Persistent positive visual phenomena in migraine.

Ten patients with migraine developed persistent positive visual phenomena lasting months to years. The complaints were similar in their simplicity and involvement of the entire visual field and usually consisted of diffuse small particles such as TV static, snow, lines of ants, dots, and rain. Neurologic and ophthalmologic examinations were normal, and EEGs were normal in eight of eight patients tested. MRI was normal in all patients except one who had nonspecific biparietal white matter lesions and another with a small venous angioma. Treatment of this unusual complication of migraine was unsuccessful.

Idiopathic intracranial hypertension: relation of age and obesity in children.

The relation between obesity and age in children with idiopathic intracranial hypertension (pseudotumor cerebri) has remained uncertain. The authors reviewed the records of 45 consecutive children with newly diagnosed idiopathic intracranial hypertension seen at two medical centers. Forty-three percent of patients aged 3 to 11 years were obese, whereas 81% of those in the 12- to 14-year age group and 91% of those in the 15- to 17-year age group met criteria for obesity (p = 0.01). Younger children with idiopathic intracranial hypertension are less likely to be obese than are older children or adults.

Gangliogliomas involving the optic chiasm.

We report three patients with gangliogliomas involving the optic chiasm via distinct mechanisms. The ganglioglioma in one patient likely originated in the temporal lobe and spread medially to involve the chiasm, and diffuse spinal cord dissemination also occurred. Chiasmal involvement in this manner and dissemination at presentation are unusual for gangliogliomas. The tumor in a second patient was intrinsic to the hypothalmus and chiasm, while in the third patient, it involved both optic tracts, and a cyst compressed the chiasm laterally. Two patients developed severe bilateral visual loss, while the other had a stable bitemporal hemianopsia. Two patients received radiotherapy, but one continued to lose vision. Although gangliogliomas rarely involve chiasm, the mechanisms by which they produce chiasmal visual loss may be diverse, and the long-term visual prognosis is variable.

Remitting sixth nerve palsy in skull base tumors.

OBJECTIVE: Spontaneous recovery of a sixth nerve palsy is thought to rule out a neoplastic origin. We reviewed cases of sixth nerve palsy that improved without treatment but that ultimately proved to be caused by a tumor at the base of the skull. DESIGN: Case series. SETTING: Hospital-based, neuro-ophthalmology referral practice. PATIENTS: Seven patients with an age range from 7 to 61 years had sixth nerve palsy secondary to a slow-growing neoplasm at the skull base. MAIN OUTCOME MEASURES: Return of lateral rectus function and resolution of diplopia without intervention. RESULTS: Seven patients with sixth nerve palsy caused by skull base tumors experienced spontaneous improvement of their deficit. Recovery time ranged from 1 week to 18 months. No patient was diabetic or had evidence of vascular disease. In one patient, the palsy improved once prior to becoming a fixed deficit, and spontaneous improvement occurred on two to five occasions in the other patients. CONCLUSION: Spontaneous recovery of a sixth nerve palsy can occur in the presence of an extramedullary compression by a tumor at the base of the brain. Possible mechanisms for recovery include remyelination, axonal regeneration, relief of transient compression (eg, resorption of hemorrhage), restoration of impaired blood flow, slippage of a nerve previously stretched over the tumor, or immune responses to the tumor.

Acute idiopathic blind spot enlargement syndrome: a review of 27 new cases.

OBJECTIVE: To describe the clinical findings in patients with acute idiopathic blind spot enlargement (AIBSE). METHODS: Medical record review of 27 patients with AIBSE (without sufficient optic nerve head swelling to cause blind spot enlargement) seen in 2 academic neuro-ophthalmology units. RESULTS: All patients were women aged between 19 and 53 years. Twenty-three patients reported positive visual phenomena. Visual acuity was normal in 16 patients. All patients had enlarged blind spots of variable size and density. Dyschromatopsia and afferent pupil defects were prevalent. Ophthalmoscopic features included uveitis, mild optic nerve swelling, granularity of macular pigment, subretinal white dots, and peripapillary pigment disturbances. Twelve of the 13 patients who underwent fluorescein angiography had optic disc staining and 5 had retinal pigment epithelial lesions with late staining. Full-field electroretinogram results were normal in 8 of 9 patients, although focal electroretinogram results were abnormal in 8 of 9 patients. Photopsia always decreased but visual fields did not improve. Six patients experienced recurrence. CONCLUSIONS: The clinical features of AIBSE include photopsia, visual field defects, abnormal findings from fundoscopic and fluorescein angiography, and abnormal results of focal electroretinography. The disease affects the peripapillary retina and may cause an afferent pupillary defect. The striking predilection for the peripapillary retina suggests a local etiologic factor and distinguishes AIBSE from the multiple evanescent white dot syndrome. Unlike patients with multiple evanescent white dot syndrome, recovery of visual field did not occur in patients with AIBSE.

Posterior ischemic optic neuropathy after uncomplicated cataract extraction.

PURPOSE: We report a case of posterior ischemic optic neuropathy after uncomplicated cataract extraction. DESIGN: Observational case report. METHODS: Retrospective description of the natural course of posterior ischemic optic neuropathy in one patient seen at an academic institution. RESULTS: The patient had sudden vision loss after uncomplicated cataract surgery with the features of an optic neuropathy, no acute disk swelling, and delayed optic nerve head perfusion on fluorescein angiography. CONCLUSIONS: Posterior ischemic optic neuropathy rarely occurs after uncomplicated cataract surgery, and should be considered in the setting of postoperative vision loss.

Magnetic resonance imaging of acquired Brown syndrome in a patient with psoriasis.

PURPOSE: To report the occurrence of acquired Brown syndrome and associated magnetic resonance imaging findings in a patient with psoriasis. METHODS: A 42-year-old woman with a history of psoriasis developed pain, double vision, and limited elevation of her left eye in adduction. An orbital magnetic resonance image with gadolinium enhancement was obtained. RESULTS: Orbital magnetic resonance image disclosed abnormal enhancement of the left trochlea/tendon complex. The patient's symptoms resolved with corticosteroid therapy. CONCLUSIONS: Acquired Brown syndrome may be associated with psoriasis. The inflammation of the trochlea/tendon complex that can cause acquired Brown syndrome can be demonstrated on magnetic resonance image.

Neuro-ophthalmologic findings in chordoma and chondrosarcoma of the skull base.

Review of the clinical features of 48 patients with chordoma and 49 patients with low-grade chondrosarcoma of the skull base disclosed overlapping clinical profiles but distinctive features. Both tumors occurred at all ages but chondrosarcoma tended to occur in the third and fourth decades. Twenty-five (52%) of the patients with chordoma and 24 (49%) of the patients with chondrosarcoma had ocular symptoms (diplopia or visual impairment) as the initial manifestation of the disease. Of the 59 patients (both groups) with diplopia, the diplopia was initially intermittent in 25 (42%). Headache and diplopia from an insidious abducens nerve palsy was most common in both groups. Abducens nerve palsy occurred in 22 (46%) of the patients with chordoma and 23 (47%) of the patients with chondrosarcoma. Normal examination results were more common in patients with chordoma, whereas visual loss, facial numbness, and multiple cranial neuropathies were more common in patients with chondrosarcoma. The similarities in the clinical features of these tumors reflect their common origin at the central skull base and the vulnerability of the abducens nerves at that site. The differences reflect the tendency of chordomas to originate from the clivus and chondrosarcomas to originate from the temporal bone.

Cyclodeviation in skew deviation.

We examined four patients with dorsolateral pontomedullary lesions and skew deviation, with ocular torsion of varying symmetry. In three patients, the hypotropic eye was excyclodeviated relative to the fellow eye. Observations of these patients, combined with recent evidence, suggest that cyclodeviation is a frequent component of skew deviation and may result from variable involvement of utricular, semicircular canal, and cerebelloocular pathways. Although cyclodeviation is a feature of trochlear-nerve palsies, its presence does not exclude skew deviation and underlying brainstem lesions.

Simultaneous corneal laceration repair, cataract removal, and posterior chamber intraocular lens implantation.

Frequently, patients with lens laceration or traumatic cataract coincident with corneal laceration, or both, must undergo two separate procedures-primary repair of the corneal laceration and secondary lens removal with or without intraocular lens implantation. We performed simultaneous corneal laceration repair, extracapsular cataract extraction, and posterior chamber lens implantation in seven patients with lacerating ocular injuries who met inclusion criteria for this procedure. With average follow-up of 10 1/2 months, all seven patients achieved visual acuity of 20/40 or better with spectacle correction. YAG posterior capsulotomy was the only additional procedure. One patient had macular pigmentation consistent with either traumatic or photic maculopathy. There were no other complications attributable to the surgical procedures. We believe that certain lacerating injuries of the anterior segment are particularly amenable to cataract extraction and lens implantation at the time of primary laceration repair. This approach obviates additional operative and anesthetic risks, while affording more timely visual rehabilitation.

Secondary hemorrhage in traumatic hyphema.

We analyzed the records of 132 patients hospitalized between July 1986 and February 1989 for management of traumatic hyphema. The incidence of secondary hemorrhage was compared between patients treated with or without systemic administration of aminocaproic acid in addition to an otherwise identical protocol. Results among patients who were examined within one day of injury disclosed a 4.8% secondary hemorrhage rate in aminocaproic acid-treated patients (three of 63 patients) compared with a 5.4% rate in the patients not treated with aminocaproic acid (three of 56 patients, P = .31). All six patients sustaining secondary hemorrhage recovered visual acuities of 20/40 or better, with five of six patients achieving 20/20 visual acuities. A separate group of 13 patients who were examined more than one day after injury were found to have a secondary hemorrhage rate of 38.5% (five of 13 patients). Macular injury, not secondary hemorrhage, was most often responsible among those patients suffering permanent visual loss. In this study of a predominantly white population, patients had a relatively low incidence of secondary hemorrhage and did not demonstrate detectable benefit from aminocaproic acid administration. Because of the recognized side effects and cost of treatment, further analysis to determine which patients will benefit from treatment with aminocaproic acid is indicated.

Severe sudden visual loss caused by pseudotumor cerebri and lumboperitoneal shunt failure.

PURPOSE: Severe vistral acuity loss associated with pseudotumor cerebri is usually caused by chronic optic disk edema or a retinal abnormality. METHODS: We treated a women, with known pseudotumor cerebri treated with a lumboperitoneal shunt, who developed acute pallied optic disk swelling and visual acuity of R.E.: no light perception and L.E.: 20/70 in association with lumboperitoneal shunt failure. There were no contributory retinal lesions. RESULTS: The patient underwent optic nerve sheath fenestration and lumboperitoneal shunt revision. Visual acuity improved to 20/20 in both eyes. The papilledema resolved. CONCLUSION: The severe sudden visual loss was attributed to axoplasmic stasis and optic nerve ischemia associated with a sudden rise in intracranial pressure.

Visual loss in children with neurofibromatosis type 1 and optic pathway gliomas: relation to tumor location by magnetic resonance imaging.

PURPOSE: To examine the potential for visual acuity loss, and its relation to extent and location of optic pathway gliomas in a cohort of children with neurofibromatosis type 1 studied with magnetic resonance imaging. METHODS: We reviewed the neuro-ophthalmologic records and brain/orbital magnetic resonance imaging scans for 43 consecutive pediatric patients with neurofibromatosis type 1 and optic pathway gliomas who were followed at the Children's Hospital of Philadelphia. The presence of visual loss, defined as abnormal visual acuity for age in one or both eyes, was determined. Optic pathway gliomas were classified by tumor extent and location according to involvement of the optic nerves, chiasm, and postchiasmal structures by magnetic resonance imaging. RESULTS: Involvement of the optic tracts and other postchiasmal structures at tumor diagnosis was associated with a significantly higher probability of visual acuity loss (P =.048, chi-square test). Visual loss was noted in 20 of 43 patients (47%) at a median age of 4 years; however, three patients developed visual acuity loss for the first time during adolescence. CONCLUSIONS: In pediatric patients with neurofibromatosis type 1 and optic pathway gliomas, the likelihood of visual loss is dependent on the extent and location of the tumor by magnetic resonance imaging and is particularly associated with involvement of postchiasmal structures. Furthermore, older age during childhood (adolescence) does not preclude the occurrence of visual loss. Close follow-up beyond the early childhood years, particularly for those with postchiasmal tumor, is recommended.

Orbital drainage from cerebral arteriovenous malformations.

OBJECTIVE: To describe the neuro-ophthalmic findings in patients with orbital drainage from cerebral arteriovenous malformations (AVMs). METHODS: We reviewed the records of 100 consecutive adult patients with cerebral AVMs who presented to our institution during a 4-year period. All patients with orbital drainage were identified, and their neuro-ophthalmic evaluations were reviewed. RESULTS: Three patients (3%) were identified with orbital drainage from a cerebral AVM. The first patient presented with typical chiasmal syndrome (reduced visual acuity, bitemporal hemianopia, and optic atrophy). Magnetic resonance imaging demonstrated a large left temporal and parietal lobe AVM with compression of the chiasm between a large pituitary gland and a markedly enlarged carotid artery. The second patient presented with headaches and postural monocular transient visual obscurations. Examination revealed normal visual function with minimal orbital congestion and asymmetrical disc edema, which was worse in the left eye. Magnetic resonance imaging revealed a large right parietal and occipital lobe AVM without mass effect or hemorrhage and an enlarged left superior ophthalmic vein. The third patient had no visual symptoms and a normal neuro-ophthalmic examination; a right parietal lobe AVM was discovered during an examination for the cause of headaches. CONCLUSION: Orbital drainage from cerebral AVMs is rare. Manifestations may include anterior visual pathway compression, dilated conjunctival veins, orbital congestion, and asymmetrical disc swelling.

Use of a portable head mounted perimetry system to assess bedside visual fields.

AIM: This study was designed to test the ability of a portable computer driven, head mounted visual field testing system to perform automated perimetry on patients at their bedside and to compare these results with the "gold standard" for bedside examinations, confrontation visual fields. METHODS: The Kasha visual field system is a portable automated perimeter which utilises a virtual reality headset. 37 neurosurgery patients were examined at their bedside with a central 24 degree suprathreshold testing strategy after confrontation visual field testing. The patterns of visual field defects were categorised and compared with the results of confrontation testing. RESULTS: A total of 42 field examinations were completed on 37 patients, and the average testing time for both eyes was 4.8 minutes with the perimetry system. Each of the 11 fields (100%) classified with defects on confrontation testing was similarly categorised on head mounted perimetry. 26 out of 31 (84%) visual fields were normal on both confrontation and perimetry testing, while five out of the 31 fields (16%) which were full on confrontation had visual field defects identified by head mounted perimetry. CONCLUSION: The head mounted, automated perimetry system proved easily portable and convenient for examining neurosurgical patients at their bedside in the perioperative period. The device demonstrated equal sensitivity to confrontation visual field testing methods in detecting field defects and offers the advantage of standardised, quantifiable testing with graphic results for follow up examinations.

Diagnostic tests in neuro-ophthalmology.

This article reviews the diagnostic testing used in the evaluation of several common neuro-ophthalmologic entities including optic nerve disease, pseudotumor cerebri, anisocoria, ptosis, and ocular motor palsies. Emphasis is placed on these bedside tests that help to establish the diagnosis of these common clinical problems. The utility of the cocaine and Tensilon (edrophonium chloride) tests as well as the role of neuroimaging in these conditions are reviewed.