How Lingering Fusional Adaptation Influences the Bielschowsky Head Tilt Test in Superior Oblique Paresis.

BACKGROUND: The lack of a positive Bielschowsky head tilt test (BHTT) is commonly seen as an indicator that superior oblique paresis (SOP) is not present. This study investigated the influence of fusion on the BHTT in unilateral SOP. PATIENTS/METHODS AND MATERIAL: We analyzed vertical fusional vergence using our eye-tracking haploscope and the value of BHTT difference (BHTTD) in 11 patients who were diagnosed with congenital unilateral SOP and able to fuse. RESULTS: Patients used one of three different mechanisms of vertical vergence to achieve fusion. The three fusional mechanisms were associated with a significantly different BHTTD (p < 0.05). Seven of the eleven patients used vertical recti-mediated fusion and had a mean BHTTD ± SD of 21.7 ± 6.3 prism diopters (PD). Three of these patients whom we measured after a patch test for at least 30 min showed a decreased BHTTD (12.7 ± 3.8 PD). Three of the eleven patients used a mixed (oblique/rectus) fusional mechanism and had a mean BHTTD ± SD of 9.3 ± 8.6 PD. Of these patients, the one whom we measured after patching showed an increase of 11 PD in BHTTD. The remaining patient used oblique muscle-mediated fusion and had a BHTTD of only 3 PD that increased to 21 PD after patching. One explanation for this BHTT behavior in the latter patient involves lingering vergence adaptation of the "paretic" superior oblique muscle (SOM) and contralateral inferior oblique muscle, which makes these muscles more effective when activated, as is the case on ipsilateral head tilt (part of the ocular counter-roll mechanism), lessening the expected increase in hyperdeviation. Similarly, in our patients with mixed fusion, the vergence-adapted "paretic" SOM and contralateral superior rectus muscle are activated on ipsilateral and contralateral tilt, respectively, lessening the hyperdeviation in both directions. In the other seven patients, however, the vergence-adapted ipsilateral inferior rectus muscle and contralateral superior rectus muscle are activated on contralateral tilt, accentuating the BHTTD. CONCLUSION: Depending upon the specific muscles used for vertical fusion, the BHTTD is decreased or increased. The presence of a large BHTTD points to lingering or persisting fusional tonus involving the vertical rectus muscles. The lack of a positive BHTT does not rule out the diagnosis of SOP, but rather may be caused by lingering or persevering fusional tonus involving the oblique muscles. Performing the BHTT after a patch test for a minimum of 30 minutes may be necessary to reveal the BHTTD, supporting the diagnosis of SOP.

Disconjugacy of Eye Movements during Attempted Fixation: A Sufficient Marker for Amblyopia?

BACKGROUND: To investigate whether detection of disconjugacy of eye movements during attempted fixation, or interocular position instability, may serve as a single sensitive test for amblyopia. PATIENTS/METHODS AND MATERIAL: Binocular eye movements were recorded at 500 Hz using the EyeLink 1000 eye tracker (SR Research Ltd., Kanata, Ontario, Canada) and analyzed using EyeLink software and Matlab (MathWorks, Natick, MA, USA). Eight subjects (four amblyopes, one successfully treated amblyope, and three non-amblyopes: 7 - 44 years) were asked to fixate on a stationary cross subtending 0.5° at 57 cm. Interocular position instability was quantified by calculating the minimum area bivariate contour ellipse (BCEA) encompassing 68% of the difference between right and left eye position points during 20-second viewing epochs. For statistical analysis, BCEA values, as well as visual acuity and stereoacuity, were normalized by base-10 logarithm transformation. RESULTS: The amblyopic subjects with persistent vision loss (one anisometropic, two strabismic, one deprivation; uncorrected visual acuity range 20/60 - 20/300, corrected stereoacuity range nil-400 arcsec) showed significantly higher interocular position instability (larger 68% BCEAs) than the non-amblyopic subjects (uncorrected visual acuity range 20/20 - 20/800, corrected stereoacuities of 20 arcsec) and the successfully treated strabismic amblyope (to the 20/20 level of visual acuity and 70 arcsec of stereoacuity) during binocular viewing trials; p < 0.01. Interocular position stability was strongly correlated with stereoacuity (in that better stereoacuity was associated with lower 68% BCEAs; r = 0.95), but not with visual acuity (r = 0.20). CONCLUSION: Interocular position instability appears to differentiate amblyopic from non-amblyopic subjects and appears to improve after successful treatment. Interocular position instability may therefore prove to be a single sensitive test for the presence of amblyopia. As a difference measure, it is inherently less susceptible to head motion and calibration error, as well as to conjugate eye motion, and as such is expected to be somewhat immune to latent nystagmus. Interocular position instability may also be useful to guide treatment, especially in preverbal children, and to assess the efficacy of novel treatments. Further research is required to establish optimal interocular position instability thresholds and to determine how specific this measure is to amblyopia.

Characteristics of morphologic macular abnormalities in neuroimmunology practice.

BACKGROUND: Morphologic macular abnormalities (MMAs) are frequently seen on macular optical coherence tomography (OCT) imaging in neuroimmunology practice, yet studies pragmatically assessing prevalence and risk factors of MMAs to date are limited. OBJECTIVE: To describe the characteristics of MMAs in a neuroimmunology-based academic practice. METHODS: Cross-sectional study of 1450 patients (2900 eyes) who underwent spectral-domain macular OCT between June 2010 and June 2012. The association between MMAs and demographic variables was analyzed using mixed-effects logistic regression. Odds ratios (ORs) were calculated per 5-year age increments. RESULTS: MMAs were observed in 338/2872 eyes (11.7%) of 232/1445 participants (16.1%). The most common abnormalities identified, included drusen (6.0%), epiretinal membrane (ERM; 5.5%), and microcystoid macular pathology (MMP; 1.9%). Overall, patients with MMAs were older (OR: 1.79, p = 5 × 10-5) and more likely to be males (OR: 2.45, p = 0.014). In particular, advancing age was associated with higher risk of drusen and ERM (OR: 1.80 and 4.26, p = 2 × 10-5 and 7 × 10-3, respectively). MMP prevalence declined with age (OR: 0.73, p = 0.015) and was associated with African-American ethnicity (OR: 15.0, p = 5 × 10-5). CONCLUSION: Unexpected or incidental MMAs are common in patients assessed with OCT in neuroimmunology practice, emphasizing the importance of comprehensive OCT image review for risk stratification and appropriate ophthalmology referral.

Scatter Photocoagulation Does Not Reduce Macular Edema or Treatment Burden in Patients with Retinal Vein Occlusion: The RELATE Trial.

PURPOSE: To determine whether scatter and grid laser photocoagulation (laser) adds benefit to ranibizumab injections in patients with macular edema from retinal vein occlusion (RVO) and to compare 0.5-mg with 2.0-mg ranibizumab. DESIGN: Randomized, double-masked, controlled clinical trial. PARTICIPANTS: Thirty-nine patients with central RVO (CRVO) and 42 with branch RVO (BRVO). METHODS: Subjects were randomized to 0.5 mg or 2.0 mg ranibizumab every 4 weeks for 24 weeks and re-randomized to pro re nata ranibizumab plus laser or ranibizumab alone. MAIN OUTCOME MEASURES: Mean change from baseline best-corrected visual acuity (BCVA) at week 24 for BCVA at weeks 48, 96, and 144 for second randomization. RESULTS: Mean improvement from baseline BCVA at week 24 was 15.5 and 15.8 letters in the 0.5-mg and 2.0-mg CRVO groups, and 12.1 and 14.6 letters in the 0.5-mg and 2.0-mg BRVO groups. For CRVO, but not BRVO, there was significantly greater reduction from baseline mean central subfield thickness (CST) in the 2.0-mg versus 0.5-mg group (396.1 vs. 253.5 µm; P = 0.03). For the second randomization in CRVO patients, there was no significant difference from week 24 BCVA in the ranibizumab plus laser versus the ranibizumab only groups at week 48 (-3.3 vs. 0.0 letters), week 96 (+0.69 vs. -1.6 letters), or week 144 (+0.4 vs. -6.7 letters), and a significant increase from week 24 mean CST at week 48 (+94.7 vs. +15.2 µm; P = 0.05) but not weeks 96 or 144. For BRVO, there was a significant reduction from week 24 mean BCVA in ranibizumab plus laser versus ranibizumab at week 48 (-7.5 vs. +2.8; P < 0.01) and week 96 (-2.0 vs. +4.8; P < 0.03), but not week 144, and there were no differences in mean CST change from week 24 at weeks 48, 96, or 144. Laser failed to increase edema resolution or to reduce the ranibizumab injections between weeks 24 and 144. CONCLUSIONS: In patients with macular edema resulting from RVO, there was no short-term clinically significant benefit from monthly injections of 2.0-mg versus 0.5-mg ranibizumab injections and no long-term benefit in BCVA, resolution of edema, or number of ranibizumab injections obtained by addition of laser treatment to ranibizumab.

Stability of intraocular pressure after retinal reattachment surgery during electroconvulsive therapy for intractable self-injury in a 12-year-old autistic boy.

We present a 12-year-old autistic boy who underwent electroconvulsive therapy (ECT) for intractable self-injury toward his head and eyes in the context of acute bilateral retinal detachment and reparative surgery. The patient received 3 ECTs before retinal reattachment surgery, and resumed ECT 2 weeks postoperatively. Bilateral intraocular pressures were monitored before and after the first 7 ECTs and intermittently after ECT for 10 months of maintenance ECT. There was no evidence of sustained intraocular pressure elevation or instability. This report supports the safety of ECT for repetitive self-injury in youth before and after emergent ophthalmologic surgery for trauma-related injury.

MRI shows a region-specific pattern of atrophy in spinocerebellar ataxia type 2.

In this study, we used manual delineation of high-resolution magnetic resonance imaging (MRI) to determine the spatial and temporal characteristics of the cerebellar atrophy in spinocerebellar ataxia type 2 (SCA2). Ten subjects with SCA2 were compared to ten controls. The volume of the pons, the total cerebellum, and the individual cerebellar lobules were calculated via manual delineation of structural MRI. SCA2 showed substantial global atrophy of the cerebellum. Furthermore, the degeneration was lobule specific, selectively affecting the anterior lobe, VI, Crus I, Crus II, VIII, uvula, corpus medullare, and pons, while sparing VIIB, tonsil/paraflocculus, flocculus, declive, tuber/folium, pyramis, and nodulus. The temporal characteristics differed in each cerebellar subregion: (1) duration of disease: Crus I, VIIB, VIII, uvula, corpus medullare, pons, and the total cerebellar volume correlated with the duration of disease; (2) age: VI, Crus II, and flocculus correlated with age in control subjects; and (3) clinical scores: VI, Crus I, VIIB, VIII, corpus medullare, pons, and the total cerebellar volume correlated with clinical scores in SCA2. No correlations were found with the age of onset. Our extrapolated volumes at the onset of symptoms suggest that neurodegeneration may be present even during the presymptomatic stages of disease. The spatial and temporal characteristics of the cerebellar degeneration in SCA2 are region specific. Furthermore, our findings suggest the presence of presymptomatic atrophy and a possible developmental component to the mechanisms of pathogenesis underlying SCA2. Our findings further suggest that volumetric analysis may aid in the development of a non-invasive, quantitative biomarker.

Principal component analysis of cerebellar shape on MRI separates SCA types 2 and 6 into two archetypal modes of degeneration.

Although "cerebellar ataxia" is often used in reference to a disease process, presumably there are different underlying pathogenetic mechanisms for different subtypes. Indeed, spinocerebellar ataxia (SCA) types 2 and 6 demonstrate complementary phenotypes, thus predicting a different anatomic pattern of degeneration. Here, we show that an unsupervised classification method, based on principal component analysis (PCA) of cerebellar shape characteristics, can be used to separate SCA2 and SCA6 into two classes, which may represent disease-specific archetypes. Patients with SCA2 (n=11) and SCA6 (n=7) were compared against controls (n=15) using PCA to classify cerebellar anatomic shape characteristics. Within the first three principal components, SCA2 and SCA6 differed from controls and from each other. In a secondary analysis, we studied five additional subjects and found that these patients were consistent with the previously defined archetypal clusters of clinical and anatomical characteristics. Secondary analysis of five subjects with related diagnoses showed that disease groups that were clinically and pathophysiologically similar also shared similar anatomic characteristics. Specifically, Archetype #1 consisted of SCA3 (n=1) and SCA2, suggesting that cerebellar syndromes accompanied by atrophy of the pons may be associated with a characteristic pattern of cerebellar neurodegeneration. In comparison, Archetype #2 was comprised of disease groups with pure cerebellar atrophy (episodic ataxia type 2 (n=1), idiopathic late-onset cerebellar ataxias (n=3), and SCA6). This suggests that cerebellar shape analysis could aid in discriminating between different pathologies. Our findings further suggest that magnetic resonance imaging is a promising imaging biomarker that could aid in the diagnosis and therapeutic management in patients with cerebellar syndromes.

Widefield Fundus Fluorescein Angiography Features of Uveitis Associated with Juvenile Idiopathic Arthritis.

PURPOSE: To evaluate the wide-field fundus fluorescein angiography (WFA) characteristics of uveitis associated with juvenile idiopathic arthritis (JIA-uveitis). METHODS: Retrospective review of records. WFA with Spectralis (Heidelberg) of JIA-uveitis patients were analyzed using the scoring system by Angiography Scoring for Uveitis Nomenclature. RESULTS: Thirty-seven eyes of 20 patients were studied. A total score of at least 1 was noted in 27 eyes (72.97%). WFA features included optic disc hyperfluorescence (51.35%), macular leakage (27.03%), retinal vascular staining/leakage at posterior pole (27.03%) and peripheral retina (64.86%), capillary leakage at the posterior pole (37.84%), and peripheral retina (59.46%). A decision to change the management plan was made in 8 of 9 patients with bilateral quiet anterior chambers after WFA results. CONCLUSION: More than 70% of JIA-uveitis eyes showed some WFA-evidence of posterior segment inflammation, which changed the course of therapy for a major proportion of patients with no clinically active anterior chamber inflammation.

Retinal and optic nerve head pathology in Susac's syndrome.

PURPOSE: This article describes the first retinal histopathologic findings in a patient with Susac's syndrome (SS). DESIGN: Observational case report. PARTICIPANT: A 51-year-old white woman diagnosed with SS. METHODS: Eyes from a 51-year-old white woman diagnosed with SS were obtained at autopsy. One retina was dissected and processed for adenosine diphosphatase (ADPase) flat embedding. Selected areas were processed further for transmission electron microscopy. MAIN OUTCOME MEASURES: Histopathologic examination using ADPase flat-embedding technique. RESULTS: There were vaso-occlusive changes in the retinal periphery resulting in small areas of capillary dropout. Cross-sections demonstrated serous filled spaces between the retinal blood vessels and the internal limiting membrane. Lumens adjacent to these spaces appeared compressed and sometimes closed, but without thrombosis. Decreased ADPase activity in some peripheral blood vessels suggested endothelial cell dysfunction and vaso-occlusion. In the optic nerve head, numerous corpora amylacea were observed in the vicinity of capillaries with thickened walls and narrow lumens. Transmission electron microscopy demonstrated thickened and amorphous vascular basal lamina and open endothelial cell junctions in some retinal blood vessels. CONCLUSIONS: The serous deposits with compression of retinal vessel lumens observed histologically probably represent the so-called string of pearls described clinically in SS. Chronic extension of these serous deposits along the vessel wall possibly are the cause of retinal arterial wall plaques as described by Gass and other investigators. In the optic nerve head, corpora amylacea are probably a result of microinfarcts resulting from optic nerve head capillary angiopathy. Accumulation of amorphous material in the basal lamina, loss of viable endothelial cells, and capillary dropout suggest that SS may be an endotheliopathy.

Contact focusing multimodal microprobes for ultraprecise laser tissue surgery.

Focusing of multimodal beams by chains of dielectric microspheres assembled directly inside the cores of hollow waveguides is studied by using numerical ray tracing. The device designs are optimized for laser surgery in contact mode with strongly absorbing tissue. By analyzing a broad range of parameters it is demonstrated that chains formed by three or five spheres with a refractive index of 1.65-1.75 provide a two-fold improvement in spatial resolution over single spheres at the cost of 0.2-0.4 attenuation in peak intensity of the central focused beam. Potential applications include ultra precise laser ablation or coagulation in the eye and brain, cellular surgery, and the coupling of light into photonic nanostructures.

RAPIDLY PROGRESSIVE NEOVASCULAR GLAUCOMA FROM CYTOMEGALOVIRUS RETINITIS IN A NON-HUMAN IMMUNODEFICIENCY VIRUS PATIENT.

PURPOSE: To describe a case of neovascular glaucoma from cytomegalovirus (CMV) retinitis in a human immunodeficiency virus-negative patient with immunosuppression after stem-cell transplant for multiple myeloma. METHODS: Retrospective case report. RESULTS: A 71-year-old man on monthly infusion of daratumumab for multiple myeloma after stem-cell transplant presenting with a 2-week history of floaters, photophobia, and blurry vision was found to have polymerase chain reaction-confirmed CMV retinitis associated with diffuse occlusive vasculitis. The patient was human immunodeficiency virus negative with a CD4 count of 450/mm3. Despite immediate aggressive treatment, the patient developed neovascular glaucoma with poor visual outcome. CONCLUSION: Cytomegalovirus retinitis in human immunodeficiency virus-negative patients is becoming more prevalent with increasing use of systemic immunosuppression therapy for various reasons. Patients with non-human immunodeficiency virus related CMV retinitis can have severe ischemia atypical of the classic CMV retinitis and should be followed closely for neovascularization.

Antagonism of vascular endothelial growth factor for macular edema caused by retinal vein occlusions: two-year outcomes.

PURPOSE: To determine the long-term effects of intraocular antagonism of vascular endothelial growth factor (VEGF) in patients with macular edema caused by retinal vein occlusions (RVOs). DESIGN: Prospective randomized trial. PARTICIPANTS: Twenty patients with macular edema caused by branch RVOs (BRVOs) and 20 patients with central RVOs (CRVOs). METHODS: After the month 3 primary end point, patients were seen every 2 months and received injections of an anti-VEGF agent as needed for recurrent edema. MAIN OUTCOME MEASURES: Mean change from baseline best-corrected visual acuity (BCVA) at month 24 with assessment of other parameters of visual function and center subfield thickness (foveal thickness [FTH]). RESULTS: For 17 patients with BRVO who completed 2 years of follow-up, the mean improvement from baseline in BCVA at month 24 was 17.8 letters compared with 15.6 letters at month 3. Improvement by at least 6, 3, or 2 lines occurred in 18%, 59%, and 76% of patients, respectively. The Snellen equivalent BCVA at month 24 was 20/40 or better in 10 patients. With an average of 2 injections of ranibizumab during year 2, the mean FTH at month 24 was 245.8 µm compared with 217.1 µm at month 3 and 481.5 µm at baseline. For 14 patients with CRVO who completed 2 years of follow-up, the mean improvement in BCVA at month 24 was 8.5 letters compared with 12.0 letters at month 3. Improvement by at least 6, 3, or 2 lines occurred in 14%, 21%, and 43% of patients, respectively. The Snellen equivalent BCVA at month 24 was 20/40 or better in 4 patients. With an average of 3.5 injections of ranibizumab in year 2, mean FTH at month 24 was 338 µm compared with 278 µm at month 3 and 533 µm at baseline. Duration of RVO >1 year at study entry and nonperfusion of perifoveal capillaries for 360 degrees correlated with reduced visual outcomes. CONCLUSIONS: Antagonism of VEGF provides substantial long-term benefit to patients with macular edema caused by RVO, but frequent injections are required in some patients with BRVO and most patients with CRVO.

Ranibizumab for macular edema due to retinal vein occlusions: implication of VEGF as a critical stimulator.

Macular edema is a major cause of vision loss in patients with central retinal vein occlusion (CRVO) or branch retinal vein occlusion (BRVO). It is not clear how much of the edema is due to hydrodynamic changes from the obstruction and how much is due to chemical mediators. Patients with macular edema due to CRVO (n = 20) or BRVO (n = 20) were randomized to receive three monthly injections of 0.3 or 0.5 mg of ranibizumab. At the primary endpoint, month 3, the median improvement in letters read at 4 m was 17 in the 0.3-mg group and 14 in the 0.5-mg group for CRVO, and 10 and 18, respectively for the BRVO group. Optical coherence tomography (OCT) showed that compared to injections of 0.3 mg, injections of 0.5 mg of ranibizumab tended to cause more rapid reductions of central retinal thickening that lasted longer between injections, but in 3 months, excess central retinal thickening which is a quantitative assessment of the macular edema, was reduced by approximately 90% in all four treatment groups. There was no correlation between the amount of improvement and duration of disease or patient age at baseline, but there was some correlation between the aqueous vascular endothelial growth factor (VEGF) level at baseline and amount of improvement. These data indicate that excess production of VEGF in the retinas of patients with CRVO or BRVO is a major contributor to macular edema and suggest that additional studies investigating the efficacy of intraocular injections of ranibizumab are needed.

The effect of acute superior oblique palsy on torsional optokinetic nystagmus in monkeys.

PURPOSE: To investigate the effects of acquired superior oblique palsy (SOP) and corrective strabismus surgery on torsional optokinetic nystagmus (tOKN) in monkeys. METHODS: The trochlear nerve was severed intracranially in two rhesus monkeys (M1 and M2). For each monkey, more than 4 months after the SOP, the ipsilateral inferior oblique muscle was denervated and extirpated. For M2, 4 months later, the contralateral inferior rectus muscle was recessed by 2 mm. tOKN was elicited during monocular viewing of a rotating stimulus that was rear projected onto a screen 43.5 cm in front of the animal. Angular rotation of the stimulus about the center was 40 deg/s clockwise or counterclockwise. RESULTS: The main findings after trochlear nerve sectioning were (1) the amplitude and peak velocity of torsional quick and slow phases of the paretic eye was less than that in the normal eye for both intorsion and extorsion, and (2) the vertical motion of the paretic eye increased during both torsional slow and quick phases. After corrective inferior oblique surgery, both of these effects were even greater. CONCLUSIONS: Acquired SOP and corrective inferior oblique-weakening surgery create characteristic patterns of change in tOKN that reflect alterations in the dynamic properties of the extraocular muscles involved in eye torsion. tOKN also provides information complementary to that provided by the traditional Bielschowsky head-tilt test and potentially can help distinguish among different causes of vertical ocular misalignment.

Acute superior oblique palsy in monkeys: I. Changes in static eye alignment.

PURPOSE: To investigate immediate and long-term changes in static ocular alignment with acute acquired superior oblique palsy (SOP) in monkeys. METHODS: The trochlear nerve was severed intracranially in two rhesus monkeys. After the surgery, the paretic eye was patched for 6 to 9 days, and then binocular viewing was allowed. Three-axis eye movements (horizontal, vertical, and torsional) were measured with binocular, dual search coils. Eye movements were recorded over a +/-20 degrees horizontal and vertical range of fixations before the lesion and then, beginning the first day after surgery. Changes in alignment with +/-30 degrees head tilt were also studied. RESULTS: The main findings were (1) misalignment (10-12 degrees vertical in adduction, down; 10-12 degrees torsional in abduction, down); (2) changes in vertical deviation (VD) with head tilt (Delta 2-6 degrees with left versus right 30 degrees tilt); and (3) changes in comitance and VD over time. During the early postlesion period, before binocular viewing was allowed, VD decreased and comitance improved. Once binocular viewing was allowed, VD increased and comitance worsened. CONCLUSIONS: Rhesus monkeys with induced SOP show a characteristic pattern of misalignment that helps define the ocular motor signature of acute denervation of the superior oblique muscle. The animals also showed striking changes over time in the amount and comitance of the vertical misalignment that depended on whether viewing was monocular or binocular, suggesting a role for proprioception in adaptation to misalignment with habitual monocular viewing.

Acute superior oblique palsy in monkeys: II. Changes in dynamic properties during vertical saccades.

PURPOSE: To investigate vertical and torsional eye motion during and immediately after vertical saccades with acute acquired superior oblique palsy (SOP) in monkeys. METHODS: The trochlear nerve was severed intracranially in two rhesus monkeys. After surgery, the paretic eye was patched for 6 to 9 days, and then binocular viewing was allowed. Three-axis eye movements (horizontal, vertical, and torsion) were measured with binocular, dual search coils. Eye movements were recorded before surgery and then beginning 2 to 3 days after surgery during 20 degrees vertical saccades over a +/-20 degrees horizontal and vertical range. RESULTS: The main findings were: (1) Saccade amplitude in the paretic eye (PE) was smaller than that of the normal eye (NE), especially for downward saccades with the PE in adduction; (2) vertical drift was backward after upward saccades with the PE in adduction or abduction, onward after downward saccades with the PE in adduction, but backward for downward saccades with the PE in abduction, drift time constants averaged 35 ms; (3) peak dynamic blip intrasaccadic torsion increased (relative extorsion), the most for upward saccades with the PE in abduction; (4) postsaccadic torsional drift increased (relative intorsion), the most for downward saccades with the PE in adduction; and (5) the peak velocity-amplitude relationship in vertical saccades was little affected, but the ratio between the peak velocity of the two eyes was a consistent indicator of the palsy. CONCLUSIONS: Rhesus monkeys with acute acquired SOP show characteristic changes in vertical and torsional movements during and immediately after vertical saccades that help define the ocular motor signature of denervation of the SO muscle. These dynamic changes were largely unrelated to the changes in static alignment over time, suggesting that static and dynamic disturbances in SOP are influenced by separate central mechanisms.

Acute superior oblique palsy in monkeys: III. Relationship to Listing's Law.

PURPOSE: To investigate the three-dimensional orientation of the eye and its relationship to Listing's Law in monkeys with acute acquired superior oblique palsy (SOP). METHODS: The trochlear nerve was severed intracranially in two rhesus monkeys. Three-axis eye movements (horizontal, vertical, and torsion) were measured with binocular, dual search coils during fixation of targets in a 40 degrees x 40 degrees grid. Rotation vectors were calculated, and Listing's plane (LP) was determined by a least-squares planar fit of eye torsion as a function of horizontal and vertical position. RESULTS: The main findings were: (1) In the paretic eye, there was an immediate and sustained rotation of the orientation plane by approximately 25 degrees in the temporal direction; (2) the thickness of LP, defined as the torsional standard deviation (SD), increased little (by 0.13 degrees in M1 and 0.08 degrees in M2) after SOP, and (3) the SD of intrasaccadic torsion was slightly greater than that during fixation, but there was no change after SOP. CONCLUSIONS: Acute SOP in rhesus monkeys leads to a temporal rotation of LP. This is consistent with a relatively increased extorsion in down gaze due to a loss of normal intorsion by the superior oblique muscle. The SD of torsion increased by only a small amount, implying that the validity of Listing's Law is not affected much by complete SOP, despite the large change in the orientation of LP.

Quantitative eye movement recordings in a patient with acquired bilateral superior oblique palsy before and after a bilateral Harada-Ito procedure.

PURPOSE: We examined the effects of the Harada-Ito procedure on static and dynamic alignment in an adult with acquired bilateral superior oblique palsy (SOP). METHODS: 3D eye movements were recorded before and six weeks after a bilateral Harada-Ito procedure. Superior oblique muscle (SOM) size and contractility were assessed with orbital imaging. RESULTS: On MRI, the left SOM was smaller than the right. Little contractile thickening was present in down gaze for either eye. Preoperatively, the patient had a hypertropia: 1.9 degrees right hypertropia (at down 20 degrees , left 20 degrees ) and 6.4 degrees left hypertropia (at down 20 degrees , right 20 degrees ). Postoperatively, the vertical tropia in all positions was < 1 degrees . Listing's primary position rotated toward straight ahead for the RE but was unchanged for the LE. Postoperatively, for 40 degrees upward saccades peak dynamic intrasaccadic extorsion decreased by 2.2-3.2 degrees for both eyes and for 40 degrees downward saccades by 2.3-3.6 degrees for the RE but was unchanged for the LE. Saccade conjugacy improved and post-saccadic drift lessened for all vertical saccades. CONCLUSIONS: The Harada-Ito procedure produced striking improvements in static and dynamic alignment in bilateral SOP. Some changes were binocular (decreased post-saccadic drift, improved saccade conjugacy, less dynamic extorsion for upward saccades) but others were much greater in the less paretic eye (torsional gradients from up to down gaze, less dynamic extorsion for downward saccades). Both central adaptive and peripheral mechanical changes explain these findings. Our results also imply that the Harada-Ito procedure has more effect when there is residual function of the SOM.

Improving graph-based OCT segmentation for severe pathology in Retinitis Pigmentosa patients.

Three dimensional segmentation of macular optical coherence tomography (OCT) data of subjects with retinitis pigmentosa (RP) is a challenging problem due to the disappearance of the photoreceptor layers, which causes algorithms developed for segmentation of healthy data to perform poorly on RP patients. In this work, we present enhancements to a previously developed graph-based OCT segmentation pipeline to enable processing of RP data. The algorithm segments eight retinal layers in RP data by relaxing constraints on the thickness and smoothness of each layer learned from healthy data. Following from prior work, a random forest classifier is first trained on the RP data to estimate boundary probabilities, which are used by a graph search algorithm to find the optimal set of nine surfaces that fit the data. Due to the intensity disparity between normal layers of healthy controls and layers in various stages of degeneration in RP patients, an additional intensity normalization step is introduced. Leave-one-out validation on data acquired from nine subjects showed an average overall boundary error of 4.22 µm as compared to 6.02 µm using the original algorithm.