Karl C. Golnik

An ice test for the diagnosis of myasthenia gravis

OBJECTIVE To determine whether ice application to a ptotic eyelid can differentiate myasthenic from nonmyasthenic ptosis. DESIGN Prospective, multicenter, nonrandomized, comparative trial. PARTICIPANTS Twenty patients with myasthenia gravis (MG) and ptosis were evaluated in the neuro-ophthalmology service. CONTROL SUBJECTS: Twenty patients with nonmyasthenic ptosis evaluated in the same locale. METHODS Palpebral fissures were measured before and immediately after a 2-minute application of ice to the ptotic eyelid. MAIN OUTCOME MEASURES The difference in palpebral fissures in millimeters before and after ice application. Two or more millimeters of improvement after ice application was considered a positive ice test result. RESULTS A positive ice test result was noted in 16 of the 20 (80%) patients with MG and in none of the 20 patients without MG (P < 0.001). Of the 4 patients with MG and complete ptosis, 3 had a negative ice test result. CONCLUSIONS The ice test is a simple, short, specific, and relatively sensitive test for the diagnosis of myasthenic ptosis. The sensitivity of the ice test in patients with complete ptosis decreases considerably.

Ophthalmic Manifestations of Rochalimaea Species

Rochalimaea henselae and R. quintana belong to the order Rickettsiales and are thought to be responsible for trench fever, bacillary angiomatosis, and cat scratch disease. We recently examined four patients with intraocular inflammation of unknown origin. Each patient had either unilateral or bilateral moderate loss of visual acuity ranging from 20/25 to counting fingers. Bilateral intraocular inflammation manifested by anterior and posterior segment cells, retinal lesions, macular exudate, and optic nerve head swelling was present to varying degrees. The R. henselae to R. quintana antibody titers were greater than or equal to 1:256 in each case. Marked improvement in vision occurred after treatment with either oral ciprofloxacin hydrochloride and prednisone or doxycycline hyclate. Rochalimaea species should be considered in the differential diagnosis of intraocular inflammation and inflammatory optic neuropathy. Appropriate treatment may result in marked improvement in visual acuity.

Sleep apnea and intracranial hypertension in men

PURPOSE To investigate sleep apnea as an associated finding in idiopathic intracranial hypertension (IIH) in men. DESIGN Multicenter, retrospective, noncomparative interventional case series. METHODS Retrospective review of all men with the diagnosis of IIH seen within the last 5 years at three tertiary care academic ophthalmologic institutions. Cases with sleep apnea (SA) and IIH were identified and reviewed. RESULTS Thirty-two cases of IIH in men were reviewed. Six cases with SA met the modified Dandy criteria for the diagnosis of IIH. Of these six patients, one received acetazolamide alone, four received acetazolamide and continuous positive airway pressure (CPAP), and one was treated with CPAP alone. All patients had preserved central acuity (20/20 or better in both eyes), enlarged blind spots, and optic disc edema in both eyes. Five patients had normal visual fields after treatment, and one patient had residual visual field loss. Three patients had normal optic nerve examinations, with resolution of the optic disc edema at last follow-up. After resolution of the optic disc edema, these three patients were maintained on CPAP but discontinued acetazolamide. Two patients had persistent but improved papilledema and are under continued treatment with acetazolamide and CPAP. One patient had optic disc pallor in both eyes and is stable. CONCLUSIONS SA was a common finding in men meeting the modified Dandy criteria for IIH in adults. Treatment of sleep apnea with nocturnal oxygenation may improve the signs and symptoms of IIH in affected men.

Folate-Responsive Optic Neuropathy

We examined six patients who presented with bilateral, progressive visual loss. Each patient had a bilateral decrease in visual acuity, poor color vision, and central or cecocentral scotomas. Optic nerve head appearance varied from normal to diffusely pale. All patients consumed tobacco, ethanol, or both. Each patient was folate deficient and had normal vitamin B12 levels. Patients were treated with oral folic acid, 1 mg per day. Visual acuity improved bilaterally in all patients (mean: five lines in 2 months). Folate deficiency should be considered in any patient with progressive bilateral optic neuropathy of unknown etiology. Treatment with folic acid can result in significant improvement in visual function.

Treatment of optic nerve sheath meningioma with three-dimensional conformal radiation

PURPOSE To report a patient recovering vision after three-dimensional conformal radiotherapy for optic nerve sheath meningioma. METHODS Radiotherapy was delivered by a three-dimensional conformal technique in 28-180-cGy fractions. RESULTS Visual acuity improved from 20/200 to 20/30, and the visual field defect resolved. CONCLUSION Visual loss from optic nerve sheath meningioma can be reversed by three-dimensional conformal radiotherapy.

Isolated Progressive Visual Loss after Coiling of Paraclinoid Aneurysms

BACKGROUND AND PURPOSE: The proximity of the paraclinoid segment of the internal carotid artery to the visual pathways may result in visual deficits when patients present with aneurysms in this segment. Although surgical clip ligation of these aneurysms has been the standard of care for decades, the advent of coil embolization has permitted endovascular therapy in those aneurysms with favorable dome-to-neck ratios. Although immediate nonprogressive visual loss after coil embolization of paraclinoid aneurysms has been well described, isolated progressive visual loss immediately or shortly following coil embolization, to our knowledge, has not. We have identified 8 patients who experienced progressive loss of vision, unassociated with any other neurologic deficits, developing immediately or shortly after apparently uncomplicated coil embolization of a paraclinoid aneurysm. MATERIALS AND METHODS: This study is a retrospective case series of 8 patients seen at 4 separate academic institutions. Inpatient and outpatient records were examined to determine patient demographics, previous ocular and medical history, and ophthalmic status before endovascular embolization. In addition, details of the primary endovascular therapy and subsequent surgical and nonsurgical interventions were recorded. Follow-up data, including most recent best-corrected visual acuity, postoperative course, and duration of follow-up were documented. RESULTS: Eight patients developed progressive visual loss in 1 or both eyes immediately or shortly after apparently uncomplicated coiling of a paraclinoid aneurysm. MR imaging findings suggested that the visual loss was most likely caused by perianeurysmal inflammation related to the coils used to embolize the aneurysm, enlargement or persistence of the aneurysm despite coiling, or a combination of these mechanisms. Most patients experienced improvement in vision, 2 apparently related to treatment with systemic corticosteroids. CONCLUSION: Patients in whom endovascular treatment of a paraclinoid aneurysm is contemplated should be warned about the potential for both isolated nonprogressive and progressive visual loss in 1 or both eyes. Patients in whom progressive visual loss occurs may benefit from treatment with systemic corticosteroids.

Pneumosinus dilatans: A sign of intracranial meningioma

BACKGROUND Pneumosinus dilatans affecting the sphenoid and posterior ethmoid sinuses has been reported in association with spheno-orbital meningiomas and is believed by some authors be a sign of an adjacent meningioma. METHODS We report the case of a 57-year-old man who developed progressive neurologic signs and symptoms consistent with a frontal lobe lesion. Neuroimaging studies revealed a large partially cystic mass at the base of the anterior cranial fossa that appeared to be invading the left frontal lobe and that was associated with pneumosinus dilatans of the adjacent left frontal sinus. RESULTS Although the appearance of the mass by neuroimaging was thought to be most consistent with a malignant glioma, the lesion was found at craniotomy to be a benign meningothelial meningioma. CONCLUSIONS Many previous cases of progressive optic neuropathy associated with pneumosinus dilatans affecting the sphenoid and posterior ethmoid sinuses have been found to be caused by adjacent optic nerve sheath meningiomas. This case provides further evidence that pneumosinus dilatans is a sign of intracranial meningioma.

Clinical characteristics in 53 patients with cat scratch optic neuropathy.

OBJECTIVE To describe the clinical manifestations and to identify risk factors associated with visual outcome in a large cohort of patients with cat scratch optic neuropathy (CSON). DESIGN Multicenter, retrospective chart review. PARTICIPANTS Fifty-three patients (62 eyes) with serologically positive CSON from 5 academic neuro-ophthalmology services evaluated over an 11-year period. METHODS Institutional review board/ethics committee approval was obtained. Data from medical record charts were collected to detail the clinical manifestations and to analyze visual outcome metrics. Generalized estimating equations and logistic regression analysis were used in the statistical analysis. Six patients (9 eyes) were excluded from visual outcome statistical analysis because of a lack of follow-up. MAIN OUTCOME MEASURES Demographic information, symptoms at presentation, clinical characteristics, length of follow-up, treatment used, and visual acuity (at presentation and final follow-up). RESULTS Mean patient age was 27.8 years (range, 8-65 years). Mean follow-up time was 170.8 days (range, 1-1482 days). Simultaneous bilateral involvement occurred in 9 (17%) of 53 patients. Visual acuity on presentation ranged from 20/20 to counting fingers (mean, 20/160). Sixty-eight percent of eyes retained a visual acuity of 20/40 or better at final follow-up (defined as favorable visual outcome). Sixty-seven percent of patients endorsed a history of cat or kitten scratch. Neuroretinitis (macular star) developed in 28 eyes (45%). Only 5 patients had significant visual complications (branch retinal artery occlusion, macular hole, and corneal decompensation). Neither patient age nor any other factor except good initial visual acuity and absence of systemic symptoms was associated with a favorable visual outcome. There was no association between visual acuity at final follow-up and systemic antibiotic or steroid use. CONCLUSIONS Patients with CSON have a good overall visual prognosis. Good visual acuity at presentation was associated with a favorable visual outcome. The absence of a macular star does not exclude the possibility of CSON. FINANCIAL DISCLOSURE(S) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Incidence of ocular misalignment and diplopia after uneventful cataract surgery

PURPOSE To evaluate the incidence of ocular misalignment and diplopia after uneventful cataract surgery. SETTING An outpatient private practice eye institute. METHODS One hundred thirty-eight patients referred to 1 cataract surgeon were prospectively evaluated. Orthoptic evaluations were performed within 1 month before and then 1 day, 1 week, and 1 month after cataract surgery. Anesthesia was by retrobulbar injection, and cataract extraction was done by phacoemulsification. RESULTS Cataract surgery was performed in 118 patients. Preoperatively, 16 patients had ocular misalignment; 10 were phoric, 4 were intermittently tropic, and 2 were tropic. Follow-up evaluation was obtained for 101 patients (86%) at 1 day, 91 (77%) at 1 week, and 88 (75%) at 1 month. A change in ocular alignment occurred in 22 of 101 patients (22%) at 1 day, 9 of 91 (10%) at 1 week, and 6 of 88 (7%) at 1 month. Only 1 patient who had a change in alignment at 1 month was symptomatic. CONCLUSIONS A persistent change in ocular alignment after uneventful cataract surgery occurred in 7% of patients. However, symptomatic diplopia was uncommon (1 in 118; 0.85%) in this relatively small series.

Ocular Vascular Thrombotic Events: Central Retinal Vein and Central Retinal Artery Occlusions

We prospectively assessed associations of thrombophilia— hypofibrinolysis with central retinal vein occlusion (CRVO) (40 patients) and central retinal artery occlusion (CRAO) (9 patients). We used polymerase chain reaction measures for thrombophilia (factor V Leiden, prothrombin, C677T MTHFR, platelet glycoprotein PlA1/A2) and hypofibrinolysis (plasminogen activator inhibitor-1 4G4G). Serologic thrombophilia measures included protein C, protein S (total and free) and antithrombin III, homocysteine, lupus anticoagulant, anticardiolipin antibodies IgG-IgM, and factors VIII and XI. Serologic hypofibrinolysis measures included Lp(a) and plasminogen activator inhibitor activity. For comparison with 40 CRVO and 9 CRAO patients, 80 and 45 race—gender matched controls were studied. The factor V mutation was more common in CRVO (3/40, 8%) than controls (0/79, 0%), P = .036, as was high (>150%) factor VIII (12/40, 30%) versus (4/77, 5%), P = .0002. Low antithrombin III (<80%) was more common in CRVO (5/39, 13%) than in controls (2/73, 3%), P = .049. Homocysteine was high (≥13.5 µmol/L) in 5/39 (13%) CRVO patients versus 2/78 controls (3%), P = .04. Three of 9 CRAO patients (33%) had low (<73%) protein C versus 2/37 controls (5%), P = .044. Two of 9 CRAO patients (22%) had high (≥13.5 µmol/L) homocysteine versus 0/42 controls (0%), P =. 028. Four of 9 CRAO patients had the lupus anticoagulant (44%) versus 4/33 (12%) controls (P = .050). CRVO is associated with familial thrombophilia (factor V Leiden, factor VIII, low antithrombin III, homocysteinemia), and CRAO is associated with familial and acquired thrombophilia (low protein C, homocysteinemia, lupus anticoagulant), providing avenues for thromboprophylaxis, and triggering family screening.