Stacy L. Pineles

Isolated Third, Fourth and Sixth Cranial Nerve Palsies From Presumed Microvascular Versus Other Causes: A Prospective Study

PURPOSE To estimate the proportion of patients presenting with isolated third, fourth, or sixth cranial nerve palsy of presumed microvascular origin versus other causes. DESIGN Prospective, multicenter, observational case series. PARTICIPANTS A total of 109 patients aged 50 years or older with acute isolated ocular motor nerve palsy. TESTING Magnetic resonance imaging (MRI) of the brain. MAIN OUTCOME MEASURES Causes of acute isolated ocular motor nerve palsy (presumed microvascular or other) as determined with early MRI and clinical assessment. RESULTS Among 109 patients enrolled in the study, 22 had cranial nerve III palsy, 25 had cranial nerve IV palsy, and 62 had cranial nerve VI palsy. A cause other than presumed microvascular ischemia was identified in 18 patients (16.5%; 95% confidence interval, 10.7-24.6). The presence of 1 or more vasculopathic risk factors (diabetes, hypertension, hypercholesterolemia, coronary artery disease, myocardial infarction, stroke, and smoking) was significantly associated with a presumed microvascular cause (P = 0.003, Fisher exact test). Vasculopathic risk factors were also present in 61% of patients (11/18) with other causes. In the group of patients who had vasculopathic risk factors only, with no other significant medical condition, 10% of patients (8/80) were found to have other causes, including midbrain infarction, neoplasms, inflammation, pituitary apoplexy, and giant cell arteritis (GCA). By excluding patients with third cranial nerve palsies and those with GCA, the incidence of other causes for isolated fourth and sixth cranial nerve palsies was 4.7% (3/64). CONCLUSIONS In our series of patients with acute isolated ocular motor nerve palsies, a substantial proportion of patients had other causes, including neoplasm, GCA, and brain stem infarction. Brain MRI and laboratory workup have a role in the initial evaluation of older patients with isolated acute ocular motor nerve palsies regardless of whether vascular risk factors are present.

Long-term results of the surgical management of intermittent exotropia

PURPOSE To examine long-term surgical success rates (>10 years) for patients with intermittent exotropia and the risk factors for failure of surgery in these patients. METHODS An attempt was made to contact all patients who underwent surgical treatment for intermittent exotropia between the years of 1970 to 1998 with a minimum postoperative follow-up of 10 years. Each patient underwent a detailed sensory and motor examination, including measurements of near and distance stereoacuity, cover testing, and ocular rotations. Patients were classified as achieving an excellent, fair, or poor outcome on the basis of motor and sensory outcomes. Risk factor analysis was performed to evaluate associations with a poor outcome and reoperations. RESULTS Of 197 patients identified, 50 were reevaluated. When combined motor/sensory criteria for surgical success were used, we found that 38% of patients achieved an excellent outcome, whereas 34% and 28% achieved a fair or poor outcome, respectively. When only the motor criteria were used, we found that 64% had an excellent outcome, whereas the remaining patients achieved either a fair (18%) or a poor (18%) outcome. During the follow-up period, 60% of patients required at least one reoperation. Multivariate risk factor analysis determined that anisometropia (p = 0.03) was associated with a poor outcome, whereas postoperative undercorrection (p = 0.04) and lateral incomitance (p = 0.06) were associated with reoperations. CONCLUSIONS Long-term surgical results in intermittent exotropia are less encouraging when sensory status is added to the evaluation. Patients with anisometropia, lateral incomitance, and immediate postoperative undercorrection are at increased risk for poor outcomes and to require reoperations.

One Eye or Two: A Comparison of Binocular and Monocular Low-Contrast Acuity Testing in Multiple Sclerosis

PURPOSE To determine the magnitudes of binocular summation for low- and high-contrast letter acuity in a multiple sclerosis (MS) cohort, and to characterize the roles that MS disease, age, interocular difference in acuity, and a history of optic neuritis have on binocular summation. The relation between binocular summation and monocular acuities and vision-specific quality of life (QoL) was also examined. DESIGN Cross-sectional observational study. METHODS Low-contrast acuity (2.5% and 1.25% contrast) and high-contrast visual acuity (VA) were assessed binocularly and monocularly in patients and disease-free controls at 3 academic centers. Binocular summation was calculated as the difference between the binocular and better eye scores. QoL was measured using the 25-item National Eye Institute Visual Functioning Questionnaire (NEI VFQ-25) and the 10-item neuro-ophthalmic supplement. The relation of the degree of binocular summation to monocular acuity, clinical history of acute optic neuritis, age, interocular acuity difference, and QoL was determined. RESULTS Binocular summation was demonstrated at all contrast levels, and was greatest at the lowest level (1.25%). Increasing age (P < .0001), greater interocular differences in acuity (P < .0001), and prior history of optic neuritis (P = .015) were associated with lower magnitudes of binocular summation; binocular inhibition was seen in some of these patients. Higher magnitudes of summation for 2.5% low-contrast acuity were associated with better scores for the NEI VFQ-25 (P = .02) and neuro-ophthalmic supplement (P = .03). CONCLUSION Binocular summation of acuity occurs in MS but is reduced by optic neuritis, which may lead to binocular inhibition. Binocular summation and inhibition are important factors in the QoL and visual experience of MS patients, and may explain why some prefer to patch or close 1 eye in the absence of diplopia or ocular misalignment.

Fluorescein angiographic identification of optic disc drusen with and without optic disc edema.

Background The fluorescein angiographic criteria for differentiating optic disc drusen (ODD) from optic disc edema have been unclear. We designed a study to identify distinguishing angiographic features of each and to apply them to cases where both drusen and edema were present. Methods A computer search was performed for cases evaluated in a university academic neuro-ophthalmology consultative practice and coded as ODD; all cases were reviewed, and those with fluorescein angiography were selected for further study. Cases were classified as either buried or surface ODD. Ten cases with papilledema were selected for comparison. Eight cases of coexistent drusen and edema were identified. Autofluorescence, early leakage, early blockage, early and late nodular staining, late peripapillary staining, and late leakage were tabulated. Results Two hundred sixteen cases of ODD were identified; 62 (116 eyes) had adequate fluorescein angiography for study. Twenty-three eyes were classified as surface ODD; 90% demonstrated early nodular staining of the disc, with late nodular staining in 90% and late circumferential peripapillary staining in 22%; autofluorescence was visible in 93% with preinjection photography. Eighty-three eyes were classified as buried ODD; 25% demonstrated early nodular staining, with late nodular staining in 29% and late circumferential peripapillary staining in 80%; autofluorescence was visible in 12% of those with preinjection photography. In 9 eyes, buried ODD were present with superimposed true edema. In these eyes, early dye leakage, late nodular hyperfluorescence, and late leakage were present. Conclusion Early and late fluorescein angiographic features reliably distinguish ODD from edema and may be particularly useful when the conditions coexist.

Presence of Erdheim-Chester Disease and Langerhans Cell Histiocytosis in the Same Patient: A Report of 2 Cases

The histiocytic disorders Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD), can both present with multisystem involvement, with the central nervous system and the bone, skin, neuroendocrine, cardiac, respiratory, and gastrointestinal systems potentially affected. The 2 entities occasionally can be difficult to distinguish. Both rarely affect the orbit and the central nervous system, and although there are rare reports of patients with coexistent LCH and ECD, there are no reported cases of the 2 diseases that involve both the orbital and neuroendocrine systems. We report 2 such cases, and review the literature of cases of LCH and ECD occurring in the same patient. The presentation of LCH and ECD in certain patients suggests a possible abnormality in the common CD34 progenitor cell. The coexistence of the 2 disease states should be suspected in patients with atypical presentations of either disorder.

Vitamin B12 Optic Neuropathy in Autism

Dietary vitamin B12 deficiency was identified as a cause of partially reversible optic neuropathy in 3 autistic children. All of the affected children presented with gradual visual loss. Examination revealed optic atrophy, and further questioning regarding diet revealed that all 3 children had severe food selectivity and highly stereotyped diets that resulted in an almost total lack of animal products in their diets. Vitamin B12 levels were low in all 3 children. Treatment with intramuscular vitamin B12 and normalization of vitamin B12 levels resulted in improvement of visual functioning in all 3 children. These cases illustrate that food selectivity, a known complication of autism, can result in vitamin deficiency that can cause visual loss and optic atrophy. Physicians must have a high index of suspicion when evaluating children with autism and visual loss to detect this rare cause of optic atrophy.

Visual and Systemic Outcomes in Pediatric Ocular Myasthenia Gravis

PURPOSE To evaluate visual and systemic outcomes in pediatric patients with purely ocular myasthenia gravis (OMG) treated at the Childrens Hospital of Philadelphia. DESIGN Retrospective chart review. METHODS Pediatric patients with OMG seen at a single institution over a 16-year period with a minimum follow-up of 1 year were reviewed. Associations of demographic and clinical characteristics with disease resolution, amblyopia, and development of generalized symptoms of myasthenia gravis were analyzed. RESULTS Thirty-nine patients were identified, with a mean age of 5.4 ± 4.8 years and mean follow-up of 4.8 ± 4.3 years. Fifteen patients were treated with pyridostigmine only, 19 (49%) also received steroids, and 15 (38%) underwent thymectomy. Four patients (10%) received steroid-sparing immunosuppressive therapy. Resolution occurred in 10 patients, and generalized symptoms eventually occurred in 9 patients. Although 10 patients were treated for amblyopia, only 1 had amblyopia at the final visit. There was no correlation between sex or age with amblyopia or development of generalized symptoms. Thymectomy, when performed before the onset of generalized symptoms, showed a trend toward protection from the development of generalized symptoms (P = .07). CONCLUSIONS In our series, 24% of patients had disease resolution and 23% had generalized symptoms. Our larger cohort confirms previous findings that treated and untreated pediatric patients with OMG have a relatively low risk of developing generalized symptoms and that related amblyopia is readily reversible. Although our treatments were more aggressive than those previously reported, our rates of amblyopia and development of generalized symptoms are comparable.

Bilateral Abnormalities of Optic Nerve Size and Eye Shape in Unilateral Amblyopia

PURPOSE To evaluate the optic nerve (ON) size and globe shape in amblyopic eyes using high-resolution magnetic resonance imaging (MRI) and to compare these values with those of the sound fellow eye and of normal control eyes. DESIGN Prospective case-control study. METHODS Thirty-four amblyopic patients and 60 normal control patients were evaluated using surface coil MRI. Retrobulbar ON cross-section, maximum globe cross-section, globe noncircularity (globe major axis/minor axis), axial length (AL), and the ratio of AL to ON (AL/ON) were measured. RESULTS Corrected logarithm of the minimum angle of resolution acuity of all 34 amblyopic eyes averaged 0.43 +/- 0.30. The mean retrobulbar ON cross-section was 9.7 +/- 2.4 mm(2), 9.5 +/- 2.3 mm(2), and 10.7 +/- 2.6 mm(2) for amblyopic, fellow, and normal ONs, respectively. Although amblyopic and fellow ONs had similar cross-sections, both were significantly (P = .02) subnormal. AL/ON was 2.7 +/- 1.2 mm(-1), 2.7 +/- 1.0 mm(-1), and 2.3 +/- 0.5 mm(-1), respectively. Although AL/ON significantly exceeded normal in amblyopic eyes (P = .01), there was no significant difference between amblyopic and fellow eyes. Globe noncircularity of amblyopic eyes (1.17 +/- 0.07; P = .002) and fellow eyes (1.15 +/- 0.04; P < .001) was significantly greater than that of control eyes (1.11 +/- 0.04), but amblyopic and fellow eyes did not differ significantly. CONCLUSIONS Unilateral amblyopia is associated with bilaterally, but also with subclinically hypoplastic ONs, greater than normal AL/ON, and abnormally noncircular globe cross-section. These factors evidently do not determine which of the 2 eyes will become amblyopic. Reduced circularity of amblyopic and fellow eyes may reflect optical causes of amblyopia or bilateral dysregulation of globe shape secondary to amblyopia.

Functional burden of strabismus: decreased binocular summation and binocular inhibition.

IMPORTANCE Binocular summation (BiS) is defined as the superiority of visual function for binocular over monocular viewing. Binocular summation decreases with age and large interocular differences in visual acuity. To our knowledge, BiS has not heretofore been well studied as a functional measure of binocularity in strabismus. OBJECTIVE To evaluate the effect of strabismus on BiS using a battery of psychophysical tasks that are clinically relevant and easy to use and to determine whether strabismus is associated with binocular inhibition in extreme cases. DESIGN Case-control study. SETTING University-based eye institute. PARTICIPANTS Strabismic patients recruited during 2010 to 2012 from a preoperative clinic and control participants with no history of eye disease other than refractive error. INTERVENTION A battery of psychophysical and electrophysiological tests including Early Treatment Diabetic Retinopathy Study visual acuity, Sloan low-contrast acuity (LCA) (2.5% and 1.25%), Pelli-Robson contrast sensitivity, and sweep visual evoked potential contrast sensitivity. MAIN OUTCOME AND MEASURE Binocular summation was calculated as the ratio between binocular and better-eye individual scores. RESULTS Sixty strabismic and 80 control participants were prospectively examined (age range, 8-60 years). Mean BiS was significantly lower in the strabismic patients than controls for LCA (2.5% and 1.25%, P = .005 and <.001, respectively). For 1.25% LCA, strabismic patients had a mean BiS score less than 1, indicating binocular inhibition (ie, the binocular score was less than that of the better eyes monocular score). There was no significant difference in BiS for contrast thresholds on Early Treatment Diabetic Retinopathy Study visual acuity, Pelli-Robson contrast sensitivity, or sweep visual evoked potential contrast sensitivity. Regression analysis revealed a significant worsening of BiS with strabismus for 2.5% (P = .009) and 1.25% (P = .002) LCA, after accounting for age. CONCLUSIONS AND RELEVANCE Strabismic patients demonstrate subnormal BiS and even binocular inhibition for LCA, suggesting that strabismus impairs visual function more than previously appreciated. This may explain why strabismic patients who are not diplopic close 1 eye in visually demanding situations. This finding clarifies the visual deficits impacting quality of life in strabismic patients and may represent a novel measure by which to evaluate and monitor function in strabismus.

Binocular visual function in patients with Boston type I keratoprostheses.

Purpose: To report the postoperative binocular function of patients with Boston type I keratoprostheses implantation for unilateral visual impairment. Methods: Seventeen patients who underwent implantation of a Boston type I keratoprosthesis and had a best-corrected visual acuity better than 20/50 in the contralateral eye before surgery were evaluated. All subjects prospectively underwent sensory testing of binocular function including Bagolini lenses, Worth-4-dot test, stereoacuity at distance and near, and double Maddox rods. In addition, an assessment of ocular rotations and alignment was performed on each subject. Results: Twenty patients with best-corrected visual acuity better than 20/50 in the contralateral eye at the time of keratoprothesis surgery were identified. Seventeen of the 20 patients underwent binocular visual testing, with 16 of 17 (94%) demonstrating binocular function. Second-degree fusion at near was demonstrated via the Worth-4-dot test in 13 of 17 (76%) patients. Third-degree fusion at near was demonstrated in 7 of 17 (41%) patients. Patients with better postoperative sensory binocular function tended to be of younger age (P = 0.05) and have better postoperative visual acuity (P = 0.006). Five patients were found to have some degree of ocular misalignment. Overall, patients with strabismus had worse binocularity (P = 0.04). Conclusions: Implantation of the Boston type I keratoprosthesis in patients with good preoperative visual acuity in the fellow eye is associated with useful binocular function in greater than 90% of patients.