Headaches due to giant cell arteritis following herpes zoster ophthalmicus in an elderly patient.

Herpes zoster ophthalmicus (HZO) with post-herpetic neuralgia (PHN) and giant cell arteritis (GCA) are two diseases more commonly seen in the elderly population. Each has potentially serious and preventable visual complications by differing mechanisms. Treatments for the two diseases differ. Antiviral medications are used in HZO and high-dose corticosteroids in GCA. These two entities could potentially coexist in the same patient, leading to a complicated diagnostic scenario where a potentially treatable disease could be overlooked. Here, we report a patient who was suffering from PHN following zoster ophthalmicus who developed GCA within a time frame suggesting a potential pathogenic association with the reactivation of latent varicella zoster virus (VZV). This association could be either direct with viral vessel infiltration leading to the arteritis or by an indirect dysimmune route. A pathophysiological association with VZV leading to the development of GCA is proposed.

Calcific retinal embolism as an indicator of severe unrecognised cardiovascular disease.

OBJECTIVE: To describe the association between calcific retinal embolism (CRE) and cardiac valve stenosis. DESIGN AND SETTING: Retrospective chart review of patients with clinical criteria for CRE. PATIENTS: 24 patients with CRE who underwent two dimensional echocardiography between 1976 and 1998. RESULTS: Nine patients (38%) had calcific valve stenosis, which was haemodynamically severe in five patients (four aortic and one mitral), four of whom had no cardiac symptoms. Six patients underwent surgical intervention (aortic valve replacement in three patients, mitral and aortic valve replacement in one patient, removal of calcific cardiac pseudotumour in one patient, and carotid endarterectomy in one patient). CONCLUSIONS: CRE may be the presenting feature of otherwise asymptomatic, clinically important underlying cardiovascular disease and, in particular, haemodynamically severe calcific valve stenosis.

Recurrence of Susac syndrome (retinocochleocerebral vasculopathy) after remission of 18 years.

Susac syndrome (retinocochleocerebral vasculopathy) is a syndrome of unknown pathogenesis. The triad of multifocal encephalopathy, visual loss, and hearing loss is caused by microangiopathy of the brain, retina, and cochlea. The illness tends to be monophasic, and to our knowledge, recurrence after years of remission has not been reported. We describe a 51-year-old woman with symptoms, signs, and brain magnetic resonance imaging findings consistent with recurrence of Susac syndrome 18 years after remission. Clinicians should be aware of the possibility of late recurrence of Susac syndrome when evaluating patients with a distant history of the syndrome who present with complaints referable to the brain, retina, and cochlea.

Ophthalmic manifestations of lightning strike.

Every year in the USA, 100-150 people die and 1000-1500 others are injured by lightning strikes. Ophthalmic and neurologic injuries from lightning strike are common. The most common permanent ocular sequela is cataract, but many areas of the eye can be affected. Prompt evaluation by an ophthalmologist is imperative for maximizing outcomes. Incidence and mechanisms of lightning strike injury are summarized, with special emphasis on the treatment of ocular injuries.

Association of high-dose intravenous methylprednisolone with reversal of blindness from lightning in two patients.

OBJECTIVE: To report possibly beneficial effects of treatment with high-dose corticosteroids given intravenously to two patients with loss of vision after lightning strikes. DESIGN: Case reports. PARTICIPANTS: Two patients who suffered the effects of a lightning strike. INTERVENTION: High-dose intravenous methylprednisolone (NASCIS-2 Protocol). MAIN OUTCOME MEASURES: Vision recovery, pupil responses, and optic nerve appearance. RESULTS: One patient had unilateral ophthalmoscopically visible abnormality with light perception vision and a relative afferent defect in that eye; vision recovered to 20/25. The other patient had no light perception, nonreactive pupils, and normal fundus examinations in both eyes; vision recovered bilaterally to normal (20/20). CONCLUSIONS: High-dose intravenous corticosteroid treatment in these patients may have had a role in their visual recovery.

Retinocochleocerebral vasculopathy.

We report 10 patients with retinocochleocerebral vasculopathy and review the clinical and diagnostic considerations in previously reported patients with this uncommonly recognized disease. The clinical manifestations include acute and subacute multifocal and diffuse encephalopathic symptoms, hearing loss, and visual loss attributable to microangiopathy affecting the arterioles of the brain, retina, and cochlea. Diagnosis is facilitated by demonstration of retinal arteriolar occlusions without uveitis or keratoconjunctivitis, mid- to low-frequency unilateral or bilateral sensorineural hearing loss, and numerous small foci of increased signal in the white and gray matter on T2 weighted brain magnetic resonance imaging. Because many conditions may produce any combination of strokelike cerebral symptoms, encephalopathy, hearing loss, and visual loss, the differential diagnosis for retinocochleocerebral vasculopathy includes connective tissue disease, demyelinating disease, procoagulant state, infection, neoplasm, and more routine mechanisms of cerebral and retinal ischemia. Brain biopsy specimens demonstrate only minimal nonspecific periarteriolar chronic inflammatory cell infiltration with or without microinfarcts. The demonstration of subclinical arteriolar microangiopathy in muscle biopsy specimens, documented in 3 of our patients may assist in making the diagnosis. The clinical course appears to be monophasic. In addition to corticosteroids, treatment options include immunosuppressant agents (cyclophosphamide or azathioprine) aspirin, calcium channel blockers (nimodipine), intravenous immunoglobulin, and plasmapheresis. The etiology of the disease is unknown, but histopathologic and laboratory evidence suggests that an immune-mediated mechanism may be involved.

Giant cell vasculitis is a T cell-dependent disease.

BACKGROUND: Giant cell arteritis (GCA) is a systemic vasculitis that preferentially targets medium-sized and large arteries. The etiopathogenesis of the syndrome is not known, and because of the paucity of information concerning the mechanisms of blood vessel wall damage, treatment options are limited. Clues to pathogenic events in this arteritis may derive from understanding the function of tissue-infiltrating cells. Arterial injury in GCA is associated with the formation of granulomas that are composed of T cells, activated macrophages, and multinucleated giant cells. To examine the role of T cells, we implanted inflamed temporal arteries from patients with GCA into severe combined immunodeficiency (SCID) mice and studied whether the vascular lesions were T cell-dependent. MATERIALS AND METHODS: Temporal artery specimens from patients with GCA were engrafted into SCID mice. The histomorphologic appearance of fresh arteries and grafts retrieved from the mice was compared by two-color immunohistochemistry, and the functional profile of tissue-infiltrating cells was analyzed by semiquantifying cytokine transcription with a polymerase chain reaction (PCR)-based assay system. The repertoire of tissue-infiltrating T cells was assessed for the presence of dominant T cell populations by using T cell receptor beta-chain-specific PCR followed by sequencing. To investigate the role of T cells in the activation of tissue-infiltrating macrophages, T cells were depleted from the arterial grafts by treating the mice with T cell-specific antibodies and the production of monokines was monitored. To demonstrate the disease relevance of T cells expanding in the implants, T cells were isolated from tissue segments and adoptively transferred into mice implanted with syngeneic arteries. The in situ production of lymphokines was then determined. RESULTS: The inflammatory infiltrate penetrating all layers of the arterial wall persisted in the xenotransplants, indicating that the inflammatory foci represent independent functional units. Similar quantities of T cell- and macrophage-derived cytokines were detected in fresh and engrafted tissue. However, the diversity of tissue-infiltrating T cells decreased following implantation. T cells with identical T cell receptors were expanded in different mice that had been engrafted with tissue fragments from the same patient, indicating that T cell survival in the arterial wall was a nonrandom process. To confirm the disease relevance of these T cells, T cell depletion and reconstitution experiments were performed. Antibody-mediated elimination of T cells from the xenotransplants resulted in the attenuation of the production of the monokines, IL-1 beta and IL-6. Adoptive transfer of syngeneic tissue-derived T cells, but not of peripheral blood T cells, into engrafted SCID mice enhanced the transcription of IL-2 and IFN-gamma in the implanted arteries. CONCLUSIONS: The vascular lesions of GCA are maintained in human artery-mouse chimeras, indicating that all cellular and noncellular components necessary for the disease are present in the temporal artery. Activation of tissue-infiltrating T cells and macrophages depends upon an infrequent subpopulation of lesional T cells that have a survival advantage in the xenotransplants. The selective proliferation of these T cells in the arteries suggests that there is recognition of a locally expressed antigen. Therefore, these T cells should be candidate targets for the development of novel therapeutic strategies in GCA.

Glucocorticoid-mediated repression of cytokine gene transcription in human arteritis-SCID chimeras.

Giant cell arteritis (GCA) is a vasculitic syndrome that preferentially affects medium and large-sized arteries. Glucocorticoid therapy resolves clinical symptoms within hours to days, but therapy has to be continued over several years to prevent disease relapses. It is not known whether and how glucocorticoids affect the function of the inflammatory infiltrate or why the disease persists subclinically despite chronic treatment. GCA is self-sustained in temporal arteries engrafted into SCID mice, providing a model in which the mechanisms of action and limitations of glucocorticoid therapy can be examined in vivo. Administration of dexamethasone to temporal artery-SCID chimeras for 1 wk induced a partial suppression of T cell and macrophage function as indicated by the reduced tissue concentrations of IL-2, IL-1beta, and IL-6 mRNA, and by the diminished expression of inducible NO synthase. In contrast, synthesis of IFN-gamma mRNA was only slightly decreased, and expression of TGF-beta1 was unaffected. These findings correlated with activation of the IkappaBalpha gene and blockade of the nuclear translocation of NFkappaB in the xenotransplanted tissue. Dose-response experiments suggested that steroid doses currently used in clinical medicine are suboptimal in repressing NFkappaB-mediated cytokine production in the inflammatory lesions. Chronic steroid therapy was able to deplete the T cell products IL-2 and IFN-gamma, whereas the activation of tissue-infiltrating macrophages was only partially affected. IL-1beta transcription was abrogated; in contrast, TGF-beta1 mRNA synthesis was steroid resistant. The persistence of TGF-beta1-transcribing macrophages, despite paralysis of T cell function, may provide an explanation for the chronicity of the disease, and may identify a novel therapeutic target in this inflammatory vasculopathy.

Systemic amyloidosis with temporal artery involvement mimicking temporal arteritis.

A 77-year-old man presented with jaw claudication, arthralgias and myalgias, weight loss, marked fatigue, and thickened temporal arteries. No vasculitis was seen on the temporal artery biopsy specimen, but amyloidosis was suspected and confirmed with Congo red staining. Subsequent bone marrow biopsy revealed multiple myeloma. Although the patient initially was thought to have temporal arteritis, the results of temporal artery biopsy directed further investigations that led to the diagnosis of systemic amyloidosis. Systemic amyloidosis should be considered in the differential diagnosis when patients, especially men, present with clinical findings suggestive of temporal arteritis but without evidence of vasculitis in temporal artery biopsy specimens.

Spontaneous recovery rate in traumatic sixth-nerve palsy.

PURPOSE: To estimate the spontaneous recovery rate of isolated traumatic sixth-nerve palsy. METHOD: A retrospective chart review over a 24-year period at a single institution, excluding patients who were first seen more than 6 weeks after injury, to reduce bias toward nonrecovery. RESULTS: The Kaplan-Meier survival estimate of spontaneous recovery at 6 months was 27% (95% confidence interval [CI], 5% to 44%) in unilateral traumatic sixth-nerve palsy and 12% (95% CI, 0% to 33%) in bilateral traumatic sixth-nerve palsy. CONCLUSION: Spontaneous recovery from isolated traumatic sixth-nerve palsy may be lower than previously reported. A prospective study is needed to provide a more accurate estimate of recovery rate.

Asthma caused by topical application of ketorolac.

BACKGROUND: Ketorolac tromethamine 0.5 percent ophthalmic solution is a widely used nonsteroidal anti-inflammatory drug (NSAID) in ophthalmology. Ketorolac eye drops have not been implicated previously as a cause of NSAID-induced asthma. STUDY DESIGN: A patient with severe asthma after topical application of ketorolac is described. The current ophthalmic indications for topical application of ketorolac and reported hypersensitivity reactions with systemic use of ketorolac are reviewed. RESULTS: A 44-year-old woman with chronic asthma, rhinosinusitis, and nasal polyps inadvertently was given ketorolac to be applied topically. After applying the first dose of ketorolac, an exacerbation of her asthma developed, necessitating hospital admission. CONCLUSIONS: Topical application of ketorolac is safe in the vast majority of ophthalmology patients. However, NSAID eye drops should not be prescribed for patients with aspirin or NSAID allergy or the combination of asthma and nasal polyps unless the patient is known to tolerate aspirin without trouble.

Neurologic complications associated with internal jugular vein cannulation in critically ill patients: a prospective study.

To determine the frequency of neurologic complications after internal jugular cannulation, we performed a prospective study in 66 consecutive critically ill patients. Of these 66 patients, one had Horner's syndrome probably associated with a large carotid sheath hematoma (2%; 95% CI, 1 to 8%). No cerebrovascular complications occurred. The frequency of neurologic complications following internal jugular vein catheterization is low in critical illness.

Intracranial plasma cell granuloma presenting as an optic neuropathy.

A 40-year-old man presented with a left optic neuropathy. Magnetic resonance imaging demonstrated a contrast-enhancing mass along the course of the left trigeminal nerve and in the region of the left cavernous sinus with suprasellar extension. Preoperatively, he had a serum polyclonal gammopathy. Pathologic diagnosis was an intracranial plasma cell granuloma. The patient responded to high-dose steroids with resolution of his optic neuropathy, marked decrease in the size of the mass, and resolution of the serum polyclonal gammopathy. Intracranial plasma cell granuloma is a rare lesion; only 11 cases have been described in the literature. Almost half of the patients presented with vision loss as their chief complaint.

The four-meter confrontation visual field test.

Confrontation visual fields have limited value in testing paracentral vision. We have used a four-meter confrontation test for several years at the Mayo Clinic for screening of the central field. This test can identify paracentral scotomas and macular sparing in a homonymous hemianopia. The optics of this technique parallel those of the two-meter tangent screen examination in which a scotoma is greatly enlarged by doubling the test distance. Although in common usage by some neuro-ophthalmologists, this simple technique is useful as an office screening device for evaluating paracentral vision.

Acquired oculomotor, trochlear, and abducent cranial nerve palsies in pediatric patients.

Between January 1966 and December 1988, 160 pediatric patients (age range, 0 to 17 years) were seen at the Mayo Clinic with an acquired oculomotor (35 patients), trochlear (19 patients), abducent (88 patients), or multiple (18 patients) cranial nerve palsy. The clinical findings in the 160 pediatric patients were compared with the results obtained in other reviews of cranial nerve palsies in the pediatric age group and with the adult Mayo Clinic patients with acquired cranial nerve palsies. Trauma was the most common reason for an acquired cranial nerve palsy in our pediatric group. The percentage of patients with an acquired cranial nerve palsy resulting from trauma was significantly greater in the pediatric group (42.5%) than in adults (15.4%) (P < .01). The difference between the percentage of adults (15.2%) and pediatric patients (16.9%) with a cranial nerve palsy secondary to a neoplasm was not statistically significant (P = .28).

Causes and prognosis in 4,278 cases of paralysis of the oculomotor, trochlear, and abducens cranial nerves.

We collected data from a large series of patients with ocular palsies and compared them with data in previous series from the Mayo Clinic. The largest group of patients among 4,278 cases was that in which the cause was undetermined for a long period of follow-up. The abducens nerve was most commonly affected. The probability of establishing a diagnosis was higher in patients younger than 50 years and among those with associated neurologic findings or multiple ocular palsies. The prognosis for recovery was best in the vascular group but was better than 50% for all groups except those with tumors. Investigation may be tailored to each patient according to clinical findings and probabilities of finding a cause, and judicious clinical judgement should be exercised.