Download ARVO 2016 Annual Meeting Abstracts 515 Neuro

ARVO 2016 Annual Meeting Abstracts
515 Neuro-Ophthalmology, Visual Fields, Systemic Disease
Thursday, May 05, 2016 8:00 AM–9:45 AM
Exhibit/Poster Hall Poster Session
Program #/Board # Range: 5977–5996/D0068–D0087
Organizing Section: Eye Movements/Strabismus/Amblyopia/NeuroOphthalmology
Program Number: 5977 Poster Board Number: D0068
Presentation Time: 8:00 AM–9:45 AM
Development and Reliability of the Tangent Corner Test (TCT)
Visual Field Evaluation
Chris A. Johnson1, Michael Wall2, Lars Frisén3, Tana Wagschal4.
1
Ophthalmology, Univ of Iowa, Iowa CIty, IA; 2Ophthalmology and
Neurology, Univ of Iowa, Iowa CIty, IA; 3Clinical Neuroscience and
Rehabilitation, Univ of Gothenburg, Gothenburg, Sweden; 4Visual
FIeld Reading Center, Univ of Iowa, Iowa CIty, IA.
Purpose: To develop a procedure for measuring the extent of the
visual field along the horizontal meridian to screen
for vigabatrin (Sabril) toxicity that could cause peripheral visual field
constriction, and to assess the test-retest
reliability.
Methods: The Tangent Corner Test (TCT) measures the temporal
extent of the visual field along the horizontal
meridian. The patient sits 1 meter from the corner of a strip of paper
mounted at eye level along the two intersecting walls (left figure),
with large markings for each 5 degree eccentricity out to 110 degrees
and small markings for each degree. The right figure demonstrates
the visual field measurement process. A mirror at the center is used
as a fixation stimulus, and allows the examiner to view the patient’s
eye and head alignment during the testing of each eye separately. A
translucent eye patch occluded the non-tested eye. Illumination of
the TCT was between 10 and 30 candelas per meter squared, and a
tennis ball painted black and mounted on a dowel rod was used as
a high contrast peripheral vision stimulus. Five measurements were
obtained, with the stimulus moving from the periphery at a rate of
2-3 degrees per second. A total of 76 patients with complex partial
seizures were tested on three separate occasions, as part of a clinical
trial sponsored by Lundbeck.
Results: The average location where the peripheral stimulus was
detected was beyond 90 degrees, with average
values between 94 and 96 degrees. The standard deviation of the
five measures was about 2 degrees, and the coefficient of variation
(COV) was approximately 2%. The results below present average
results for the right and left eyes for the three testing sessions. Results
from two other visual field tests (Humphrey Field Analyzer 30-2
SITA. Standard or SITA Fast, and threshold determinations at every
ten degrees from 40 to 70 degrees eccentricity along the nasal and
temporal horizontal meridian) were more variable than the TCT in
this population. (Right eye 95-96 deg, SD= 1.6-1.9 deg, COV= 1.80 2.15%, Left eye 94-95 deg, SD= 1.8-1.9 deg, COV = 1.8 - 2.0%).
Conclusions: The Tangent Corner Test (TCT) provides a reliable
determination of horizontal visual field extent, even in
patients with complex partial seizures who may have cognitive
impairment and attention deficits.
Schematic representation of the Tangent Corner Test (TCT) in the left
panel, and a demonstration of the test procedure in the right panel.
Commercial Relationships: Chris A. Johnson, Lundbeck (F),
Lundbeck (C); Michael Wall, Lundbeck (F), Lundbeck (C);
Lars Frisén, Lundbeck (C); Tana Wagschal, Lundbeck (F)
Support: Visual FIeld Reading Center support from Lundbeck
Clinical Trial: NCT01278173
Program Number: 5978 Poster Board Number: D0069
Presentation Time: 8:00 AM–9:45 AM
Development of a novel device to measure visual fields in infants
and toddlers
PremNandhini Satgunam1, 2, Sourav Datta1, Karthik Reddy2,
Dhruv Joshi2. 1Prof.Brien Holden Eye Research Center, L V Prasad
Eye Institute, Hyderabad, India; 2Srujana Center for Innovation, L V
Prasad Eye Institute, Hyderabad, India.
Purpose: Measuring visual fields is particularly important for
children with brain lesions who are known to have field defects.
Performing perimetry in pediatric population however is challenging.
Only few research instruments (e.g. double arc perimeter) can be used
to test infants and toddlers but these are not commercially available.
We developed a device to measure visual fields for this cohort.
Methods: A hemispherical dome (24 steel meridians separated by
15°, covering the entire 360°), embedded with LEDs and covered in
black cloth was fabricated. The arrangement of the LEDs permitted
measuring the fields at 10° radial intervals. Upper, left and right
visual fields were measurable up to 80°. The lower visual field
measured only up to 50°, as it provided the entrance to place the
child in supine position under the dome. An infrared camera mounted
on top of the dome provided live video to monitor the child’s eye
movement. A computer program controlled the LED stimulus
presentation. 4 infants with normal milestones (NM) and 18 infants
and toddlers with neurological or ocular disorders and associated
developmental delays (DD) were enrolled with parent’s consent.
Results: Average age of the DD group was 11.8 ± 7 months and that
of the NM group was 7 ± 2.7 months. No gross visual field defects
(hemianopia/quadrantanopia) were detected for any group. The
median (± interquartile range) reaction time of the eye/head turn to
the hemi/quadrant stimuli was 331 ± 226 ms for NM and 596 ± 741
ms for the DD group. Full visual field mapping was attempted in both
groups. Out of 24 meridians, on average (± standard deviation) 8 ±
5 meridians and 9± 8 meridians were measured in the NM and DD
groups respectively. The average field extent of the DD group was
35°, 52°, 46°, and 36° in the superior, left, right and inferior fields.
Conclusions: The newly developed device shows promise to map
visual field isopters particularly for the DD group (Fig.1). The
measured visual field extent in the tested DD and NM groups were
comparable. The reaction time, while largely variable showed the
DD group to be slower than the NM group by a factor of 1.8 (no
statistical testing done). The longer duration may reflect processing
delays in afferent, efferent or both systems. Studies with larger
samples will be needed for better understanding the reaction time
differences.
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ARVO 2016 Annual Meeting Abstracts
detection threshold were correlated in the left eye only (p=0.034).
We found no significant correlations between detection thresholds
and either occipital pole thickness, volume or surface area for the
right eye. However a significant correlation between the lower nasal
visual field in the left eye and right occipital volume (p=0.009) was
observed. Occipital pole volumes and surface areas were also much
greater in the right compared to the left cortex (p<0.0001).
Conclusions: VF quadrant deficits do not relate to ppRNFL
distribution in albinism. In contrast we found correlations between
the lower nasal visual field and right occipital cortical gray matter
volume. Occipital pole differences between the two cortices may
underlie VF deficits differences observed between the right and left
eyes in albinism. These results suggest that visual field deficits are
likely to be cortical in origin.
Commercial Relationships: Viral Sheth, None; Irene Gottlob,
None; Zhanhan Tu, None; Sarim Mohammad; Rebecca McLean,
None; Frank A. Proudlock, None
Support: MR/J004189/1 and MR/N004566/1
Fig. 1: Binocular visual field isopter of a 9 months old with
Developmental Delay (DD).
Commercial Relationships: PremNandhini Satgunam, 4341/
CHE/2015 (patent pending) (P); Sourav Datta, 4341/CHE/2015
(patent pending) (P); Karthik Reddy, 4341/CHE/2015 (patent
pending) (P); Dhruv Joshi, 4341/CHE/2015 (patent pending) (P)
Program Number: 5979 Poster Board Number: D0070
Presentation Time: 8:00 AM–9:45 AM
Comparison of retinal and cortical structures with visual fields in
albinism
Viral Sheth2, Irene Gottlob2, Zhanhan Tu2, Sarim Mohammad1, 2,
Rebecca McLean2, Frank A. Proudlock2. 1Ophthalmology, The
University of Leicester, Leicester, United Kingdom; 2Ulverscroft Eye
Unit, Ophthalmology, The Univrsity of Leicester, Leicester, United
Kingdom.
Purpose: We have previously reported visual field (VF) deficits in
albinism which were more severe in the upper nasal visual field and
also in the left eye. We now investigate whether these VF deficits are
related to retinal or cortical abnormalities, comparing peripapillary
retinal nerve fiber layer (ppRNFL) distributions) and occipital pole
grey matter thickness, volume and surface area measurements to
these VF deficits.
Methods: VF testing and optic nerve optical coherence tomography
(OCT) was completed on 71 eyes with albinism. In all participants
monocular light spot detection threshold were assessed using
automated white on white perimetry with a SITA 24-2 algorithm to
compare the detection threshold for up to 24° around the fixation
point. Detection thresholds were analysed in separate quadrants
(upper nasal, lower nasal, upper temporal and lower temporal) of the
VF. The optic nerve OCT B scan images required manual motion
correction due to nystagmus using Image J. ppRNFL thickness was
analysed on the realigned volumes using Copernicus OCT software
comparing corresponding visual field quadrants and ppRNFL sectors.
Magnetic resonance imaging (MRI) data collected on 18 participants
was segmented using Freesurfer software to calculate occipital pole
gray matter thickness, volume and surface area.
Results: We found no significant correlations between detection
thresholds in four visual field quadrants and corresponding ppRNFL
segments. The ppRNFL adjacent to the macula and central foveal
Program Number: 5980 Poster Board Number: D0071
Presentation Time: 8:00 AM–9:45 AM
Retinal Development in Albinism: Evidence for Residual
Plasticity in Early Childhood?
Helena Lee1, Ravi Purohit2, Viral Sheth2, Gail Maconachie2,
Anastasia Pilat2, Rebecca McLean2, Frank A. Proudlock2,
Irene Gottlob2. 1Ophthalmology, Pediatrics, University of
Southampton, Southampton, United Kingdom; 2University of
Leicester, Leicester, United Kingdom.
Purpose: To characterize postnatal retinal development in infants and
young children with albinism using hand-held spectral domain optical
coherence tomography (HH-SDOCT)
Methods: We obtained 181 mixed cross-sectional and longitudinal
optical coherence tomograms from 44 children with a diagnosis of
albinism and compared them with 297 tomograms obtained from
148 race-matched controls. The mean postnatal age at the time of
examination was 37.8 months (range 0.9 - 83.6) for the albinism
group and 37.7 months (range 0 - 83.3) for the control group.
Retinal layer segmentation was performed manually using ImageJ.
Fractional polynomial modelling was used to analyse the differences
in development of retinal layers with age between the albinism and
control groups.
Results: Normal retinal development is nonlinear, continues until
adolescence and involves migration of the inner retinal layers (IRLs)
away from the central fovea, migration of the cone photoreceptors
into the central fovea and elongation of the outer retinal layers
(ORLs) over time. In comparison to controls, IRL migration from
the fovea is delayed and arrests prematurely in albinism, resulting
in a significantly thicker central macular thickness (p<0.0001).
In contrast, the parafoveal (1000µm from the central fovea) and
perifoveal (2000µm from the central fovea) retinal thicknesses
were significantly decreased in albinism in comparison to controls
(p<0.0001). There is evidence of ongoing foveal ORL elongation in
albinism, although reduced in amplitude in comparison to controls
as a result of significant reductions in the degree of elongation of
the photoreceptor inner (IS) and outer segments (OS) (p<0.0001).
Interestingly, the parafoveal OS measurements were significantly
increased in albinism in comparison to controls (p<0.0001). The
retinal pigment epithelium (RPE) increases in thickness more slowly
resulting in a uniform decrease in RPE thickness across all measured
retinal locations in albinism (p< 0.0001).
Conclusions: We have demonstrated that retinal development is not
arrested in children with albinism, but is ongoing albeit at a reduced
rate and magnitude in comparison to controls. Potentially treatment
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ARVO 2016 Annual Meeting Abstracts
during this crucial period of residual plasticity may promote normal
retinal development and optimise visual function.
Commercial Relationships: Helena Lee, None; Ravi Purohit,
None; Viral Sheth, None; Gail Maconachie, None; Anastasia Pilat,
None; Rebecca McLean, None; Frank A. Proudlock, None;
Irene Gottlob, None
Support: Fight for Sight Award 2015; Medical Research Council,
London, UK (grant number: MR/J004189/1), Ulverscroft Foundation,
Leicester, UK, The National Eye Research Centre and Nystagmus
Network UK.
Program Number: 5981 Poster Board Number: D0072
Presentation Time: 8:00 AM–9:45 AM
Simultaneous PERG and VEP Obtained at High Stimulation
Rates using Deconvolution
Jorge Bohorquez1, Juan Lopez2, Jonathon Toft-NIelsen1,
Mario Valderrama3, Juan C. Bohorquez2, Fredy Segura2,
Vittorio Porciatti4, Ozdamar Ozcan1. 1Biomedical Engineering,
University of Miami, Coral Gables, FL; 2Electrical Engineering, Los
Andes University, Bogota, Colombia; 3Biomedical Engineering, Los
Andes University, Bogota, Colombia; 4Bascom Palmer Eye Institute,
Miami, FL.
Purpose: To investigate the Visual Evoked Potentials (VEPs)
adaptation at high rates.
Methods: Monocular Pattern Electroretinograms (PERGs) and
Visual Evoked Potentials (VEPs) were simultaneously acquired at
stimulation frequencies: 2, 10, 15, 20 and 25 reversals per second
(rps) in 7 normal subjects (18-35yo). Two stimulation paradigms
were used: constant rate and slightly-jittered sequences at same rates.
Constant rates generated steady state responses (SSR) that were
analyzed in frequency domain. Jittered rates generated quasi-SSR that
were deconvolved to retrieve transient responses. The spectra of the
raw recordings was analyzed to assess the influence of the stimulation
on the background neural activity. In a second experiment, 32s long
recordings consisting of 8s at 2rps followed by 8s at 25rps and return
to 8s at 2rps were conducted to study the temporal dynamics of the
neural activity. Time-frequency analysis was performed with the
Morlet wavelet transform.
Results: The maximum amplitudes for steady state PERG (SSPERG) and steady-state VEPs (SS-VEP) were obtained at 15 and
10rps, respectively. Spectra of SSR raw recordings showed enhanced
spectral peaks at stimulation frequencies and its harmonics. High rate
steady state VEPs (>2rps), on the other hand, produced enhanced
background Electroencephalogram (EEG) power at alpha-band
(~10Hz) compared to low rate (2rps) responses; this effect was not
observed on the PERG spectra. The time-frequency analysis on the
multi-rate recordings verified the increase of the EEG alpha power
(~25%), synchronized with the onset of the high-rate stimulation and
a return to 2rps baseline.
Transient VEPs obtained by deconvolution at 15rps and higher were
of much higher amplitudes compared to corresponding SS-VEPs.
Synthetic SS-VEPs computed using deconvolved transient responses
successfully predicted the acquired steady-state responses.
Conclusions: Rate characteristics of transient and SS-PERGs were
consistent with previous studies. Deconvolved transient VEPs,
however, revealed no amplitude reduction at high rates as expected
from analysis of SS-VEPs. Superposition models for the generation
of evoked responses predict the spectra enhancement at stimulation
rates and harmonics well but not the rate-dependent alpha power
enhancement. This increase may help to explain the amplitude of the
high-rate transient VEP. Results are relevant for better understanding
of the VEP generators.
Commercial Relationships: Jorge Bohorquez, None; Juan Lopez,
None; Jonathon Toft-NIelsen, None; Mario Valderrama, None;
Juan C. Bohorquez, None; Fredy Segura, None; Vittorio Porciatti,
None; Ozdamar Ozcan, None
Program Number: 5982 Poster Board Number: D0073
Presentation Time: 8:00 AM–9:45 AM
The Utility of MRI Neuroimaging in Acute Isolated
Mononeuropathies of Cranial Nerves III, IV, or VI
Fiona Seager. Ophthalmology, Georgetown Univerisity Hospital/
Washington Hospital Center, The Plains, VA.
Purpose: The debate over the need for MRI neuroimaging in the
initial workup of acute oculomotor mononeuropathies is ongoing.
While most cases are due to microvascular ischemia, studies have
demonstrated that 1-15% are due to intracranial neoplasm, stroke,
aneurysm, inflammation, and infection. This study seeks to analyze
the etiology of acute isolated mononeuropathies of cranial nerves III,
IV, and VI based on neuroimaging to help determine the benefit of
early MRI neuroimaging.
Methods: This study is a retrospective chart review. Patients were
screened using ICD-9 codes for diplopia and cranial nerve II, IV, &
VI palsies in the billing database at MedStar Washington Hospital
Center from April 2000 to April 2015. Patients selected were 50 years
of age and older, presented within 1 month of onset of symptoms, and
obtained CT/MRI/MRA brain scans at time of presentation or up to
6 months following. Exclusion criteria: history of strabismus, orbital
disease, head trauma, prior neurosurgical intervention, prior lumbar
puncture, neurological symptoms indicating additional neurologic
dysfunction, and artifact affecting radiology read. Data collected
included age, race, gender, duration of symptoms, type of diplopia,
cranial nerve affected, cause of mononeuropathy (if known), history
of trauma, MRI finding(s), vascular risk factors, and findings of other
results, if known, to include erythrocyte sedimentation rate (ESR),
C-reactive protein (CRP), lumbar puncture (LP), acetylcholine
receptor antibody, and temporal artery (TA) biopsy.
Results: Of the 81 patients recruited, 30 qualified for selection.
Patient demographics: 16 (55%) female, 17 (59%) African
American, 3 (10%) Hispanic, 5 (17%) Asian, 3 (10%) declined
to identify, 1 (3%) Caucasian. All patients had 1 or more vascular
risk factors. Palsies identified: 3rd 6 (21%), 4th 9 (31%), 6th 7
(24%), combination 7 (24%). Patients with symptoms of headache
on presenation: 11 (38%). Palsy etiology: 13 (45%) microvascular
ischemia, 5 (17%) neoplasm, 3 (10%) cerebrovascular accident, 2
(7%) infection, 1 (3%) inflammation, 5 (17%) undetermined.
Conclusions: While most oculomotor palsies were due to
microvascular ischemia, a substantial 37% were due to other
potentially serious and treatable conditions warranting early MRI
neuroimaging.
Commercial Relationships: Fiona Seager, None
Program Number: 5983 Poster Board Number: D0074
Presentation Time: 8:00 AM–9:45 AM
Genetic screening using next generation sequencing in an atypical
presentation of episodic ataxia with dominant inheritance
Gail Maconachie1, Mervyn G. Thomas1, Cris Constantinescu2,
Viral Sheth1, Rebecca McLean1, Irene Gottlob1. 1University of
Leicester, Leicester, United Kingdom; 2University of Nottingham,
Nottingham, United Kingdom.
Purpose: Episodic ataxia (EA) is typically characterised by
intermittent periods of ataxia and abnormal visual problems
of variable expression. We aim to characterise the phenotypic
spectrum and explore the genetic basis, using next generation
sequencing (NGS), of familial EA which presented with episodic
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ARVO 2016 Annual Meeting Abstracts
diplopia, ataxia and environmental triggers similar to those
reported by other EA types.
Methods: Three affected brothers, with onset within the third decade,
their affected mother, the unaffected father and 4 offspring were
recruited. All affected subjects and their offspring underwent a full
ophthalmology and neurological assessment including eye movement
recordings (EMR) and MRI. Genetic analysis was undertaken using
NGS, screening for candidate loci known to be associated with EA.
Affected subjects were prescribed a trial of both acetazolamide and
4-aminopyridine to help with symptoms.
Results: The primary presentation of all 3 brothers was vertical
diplopia of variable severity and duration, which reversed
throughout the day. The mother reported diplopia when she
was younger although resolved during pregnancy. All affected
subjects and offspring were noted to have abnormal vertical
saccades, smooth pursuit and fixation. Two subjects had endpoint
nystagmus and one subject reported migraines. All MRI scans
were normal. The eldest brother had dysarthria and ataxia prior
to starting treatment. NGS excluded all previously reported EA
genes including KCNA1, CACNA1A, CACNB4 and SLC1A3
genes suggesting a potential novel gene within this family.
Acetazolamide did not subjectively improve symptoms, however,
4-aminopyridine was found to subjectively reduce the frequency
of symptoms in the most severely affected subjects.
Conclusions: We report a dominant inheritance of an atypical
episodic ataxia which may harbour a potential novel gene. We show
this EA type responds well to 4-aminopyridine which suggests that
this could be an inherited channelopathy. This finding may help
narrow the selection for possible causative gene mutations.
Commercial Relationships: Gail Maconachie;
Mervyn G. Thomas, None; Cris Constantinescu, None;
Viral Sheth, None; Rebecca McLean, None; Irene Gottlob, None
Support: Ulverscroft Foundation
Program Number: 5984 Poster Board Number: D0075
Presentation Time: 8:00 AM–9:45 AM
Choroidal thickness changes in patients with hemodynamically
significant carotid stenosis
Serena Telani1, Ilaria Percivale1, Laura Landi1, Simone Mambrini2,
Domenico Palombo2, Antonio Uccelli3, Carlo E. Traverso1,
Michele M. Iester1. 1Eye Clinic of Genoa, Genova, Italy;
2
Cardiovascular surgery clinic of Genoa, Genoa, Italy; 3Neurologic
department of Genoa, Genoa, Italy.
Purpose: To determine whether there were changes in choroidal
thickness in patients with hemodynamically significant carotid
stenosis and to evaluate if there was a choroidal thickness variation
after endoarterectomy surgery.
Methods: Prospective, longitudinal study. All the enrolled patients
were affected by unilateral hemodynamically significative (>70%)
carotid stenosis. The patients underwent complete ophthalmologic
examination, IOP measurements, imaging using Enhanced Depth
Imaging (EDI)-OCT Heidelberg, blood pressure and heart rate
evaluation at baseline (before endoarterectomy) and then the same
exams were performed 1 week, 1 month and 3 months from surgery.
Choroidal thickness was measured at three predetermined points:
subfoveal and 2 mm nasally and temporally from the fovea.
Results: 15 consecutive patients were recruited. A significant
(p=0.007) difference (57. 75 + 12.4 um (mean + SD)) was found
for choroidal thickness between ispilateral to carotid stenosis eye
and controlateral one in the same patient. No significant difference
was found in visual function, IOP, blood pressure and heart rate.
A significant (p<0.05) difference was found in choroidal thickness
before and after endoarterectomy surgery (170.25 + 92.24 um and
231 + 161.90 um, respectively), in particular at subfoveal point.
Conclusions: Our data show that an hemodynamically significant
stenosis is responsible for a reduction of choroidal thickness in the
ipsilateral eye, which appears to improve after endoarterectomy surgery.
Choroidal thickness seems to be related to carotid blood flow.
Commercial Relationships: Serena Telani, None;
Ilaria Percivale, None; Laura Landi, None; Simone Mambrini;
Domenico Palombo, None; Antonio Uccelli, None;
Carlo E. Traverso, None; Michele M. Iester, None
Program Number: 5985 Poster Board Number: D0076
Presentation Time: 8:00 AM–9:45 AM
Meningiomas of the anterior visual pathways. Advancement in
diagnosis, treatment, and follow-up
Steven A. Newman. Ophthalmology, University of Virginia,
Charlottesville, VA.
Purpose: The anterior visual pathways may be affected by
meningiomas originating within the optic nerve sheath, in the area of
the optic canal, anterior clinoid, or tuberculum. Advances in imaging
(CT and MRI) now permits diagnosis, often without need for biopsy.
Improvements in quantitation of psychophysics (automated perimetry
static perimetry), anatomic assessment (OCT assessment of nerve
fiber layer thickness and ganglion cell segmentation analysis) have
dramatically improved our ability to make a diagnosis and assess
the level of damage. Surgical approach can still be effective in
decompressing pressure on the optic nerve and chiasm. If the optic
nerve sheath is involved, surgery has been generally replaced by
fractionated radiation therapy.
Methods: A retrospective review of 31 patients with optic nerve and
chiasmal involvement of meningiomas with follow-up of up to 34
years was reviewed.
Results: While most patients were identified with progressive visual
loss, some lesions were picked up fortuitously when imaging was
done for other reasons. Visual acuity was variable ranging from 20/20
or better to NLP. The advent of quantitative retinal and optic nerve
assessment has shown variable results. Long-term compression can
lead to significant optic atrophy and corresponding nerve fiber layer
thinning, but compression at the orbital apex can mask previous
damage due to swelling of the optic nerve fibers (“green disease”).
More recent introduction of ganglion cell analysis by segmentation
algorithm may allow distinction of preceding and irreversible
damage. Natural history of meningiomas affecting the anterior visual
pathways is quite variable with some stable over decades while others
progress over months to years. Surgical decompression and, perhaps
more importantly, fractionated radiation therapy may play a role in
improving and prolonging visual function.
Conclusions: Meningiomas are a common cause of optic nerve
dysfunction. Judicious use of fractionated radiation therapy and
surgical decompression may be helpful in improving and protecting
visual function. The advent of OCT is an important addition,
particularly with ganglion cell analysis for detecting the severity of
damage. In spite of substantial damage, psychophysical improvement
may still occur with treatment.
Commercial Relationships: Steven A. Newman, None
Program Number: 5986 Poster Board Number: D0077
Presentation Time: 8:00 AM–9:45 AM
Ophthalmic Features of Multiple System Atrophy
Maria D. Garcia, Jose Pulido, John J. Chen. Mayo Clinic,
Rochester, MN.
Purpose: Few studies have analyzed the ocular features of Multiple
System Atrophy (MSA), a rapidly progressive neurodegenerative
These abstracts are licensed under a Creative Commons Attribution-NonCommercial-No Derivatives 4.0 International License. Go to http://iovs.arvojournals.org/
to access the versions of record.
ARVO 2016 Annual Meeting Abstracts
disorder characterized by parkinsonism, autonomic dysfunction, and/
or ataxia. Therefore, we conducted a retrospective, case series to gain
a better understanding of the ocular manifestations of MSA.
Methods: A search for patients seen at Mayo Clinic Rochester who
had a mention of MSA in the chart and had an eye examination
between January 1, 2005 and December 31, 2014 yielded 285 cases.
Of the 285 cases, we identified 43 cases of true MSA (35 with a
diagnosis of MSA and 8 with features of MSA overlapping with
another neurodegenerative disorder). Each of these 43 patients was
further reviewed for ocular abnormalities related to MSA.
Results: Binocular diplopia from MSA was observed in 8 out of
43 patients (18.6%). There were 3 cases due to skew deviation, 1
of possible skew deviation, 2 of divergence insufficiency, 1 of both
skew deviation and divergence insufficiency, and 1 of an unspecified
esotropia. In addition, monocular diplopia was reported in 6 patients
(14%), predominantly due to dry eyes. One patient had monocular
diplopia from trichiasis due to ocular cicatricial pemphigoid. 10
out of 43 (23.3%) reported oculomotor abnormalities other than
misalignment of the eyes, which included hypometric saccades
(N=4), poor smooth pursuit (N=6), downbeat nystagmus (N=2),
upgaze palsy (N=1) and dysfunctional vestibulo-ocular reflex (N=1).
One of the patients with poor saccades also had concordant bilateral
optic atrophy.
Conclusions: Our study confirms that oculomotor abnormalities
and diplopia are the most common ocular findings in patients with
MSA. Diplopia was most commonly caused by skew deviation and
divergence insufficiency. Interestingly, there was a single case of
bilateral optic atrophy, which has rarely been reported in MSA. There
was also a case of ocular cicatricial pemphigoid among our MSA
cohort, which is interesting because pemphigoid has been associated
with other neurologic conditions, including Parkinson’s disease, but
has not been reported in MSA in the past.
Commercial Relationships: Maria D. Garcia, None; Jose Pulido,
None; John J. Chen, None
Program Number: 5987 Poster Board Number: D0078
Presentation Time: 8:00 AM–9:45 AM
Nonvascular Retinal Imaging Markers of Preclinical Alzheimer’s
Disease
Cláudia Y. Santos1, 4, Lenworth N. Johnson6, 4, Jessica L. Alber2, 4,
Brian Fernandez5, Yen Y. Lim3, Peter J. Snyder2, 4. 1Interdisciplinary
Neuroscience Program, University of Rhode Island, Providence, RI;
2
Department of Neurology, Warren Alpert Medical School of Brown
University, Providence, RI; 3The Florey Institute of Neuroscience
and Mental Health, The University of Melbourne, Melbourne, VIC,
Australia; 4Lifespan-Rhode Island Hospital, Clinical Research
Center, Providence, RI; 5Heidelberg Engineering, Inc, Franklin,
MA; 6Department of Neuro-Ophthalmology, Warren Alpert Medical
School of Brown University, Providence, RI.
Purpose: Successful treatment of Alzheimer’s disease (AD) requires
early detection. It is known that individuals with symptomatic
AD have beta-amyloid (Aβ) plaques in the brain and concomitant
accumulation of Aβ plaques in the retina.1,2 In the preclinical stage
of AD there is accumulation of neocortical Aβ plaques. The aim
of this study was to characterize early structural retinal changes in
preclinical AD.
Methods: Sixty-three adults aged 55-75 with a self-reported first
degree family member with AD and subjective memory complaint
underwent 18F-florbetapir PET to quantify the degree of neocortical
Aβ accumulation. Neocortical PET standardized uptake value ratio
(SUVr) were summed and normalized to the whole cerebellum.
Participants underwent spectral-domain optical coherence
tomography (OCT) including blue laser autofluorescence (BAF)
imaging (Heidelberg SPECTRALIS system) to identify retinal Aβ
plaque inclusion bodies and measure retinal layer thickness.
Results: The values of PET SUVr, the surface area of Aβ plaque
inclusion bodies, and the thickness of the inner plexiform layer (IPL)
are provided in the Figures 1 and 2. There was a moderate correlation
(r= 0.46, p=0.02) between PET neocortical amyloid burden and the
surface area of inclusion bodies (Figure 1), and moderate correlation
(r= 0.41, p=0.03) between the surface area of inclusion bodies and
an increase in the thickness of the IPL – but not for other retinal
neuronal layers (Figure 2).
Conclusions: The positive correlation between the surface area
of inclusion bodies in the retina and amyloid aggregation in the
neocortex suggests that BAF might allow for visualization of
the fibrillary form of Aβ in preclinical AD subjects. The IPL is a
cholinergic rich layer in the vertebrate retina3, and early cholinergic
changes in preclinical disease appear to co-occur with the onset of
amyloid deposit in the major cholinergic centers of the brain. 4-6 OCT
imaging of these early neuropathic changes in the retina may provide
biomarkers that are both indicative of disease burden and progression
and also reflective of time-specific changes in the neocortex.
Aβ+ CN older adult: example of inclusion bodies around vessel
walls, and in peripheral regions of the retina.
Commercial Relationships: Cláudia Y. Santos, None;
Lenworth N. Johnson, None; Jessica L. Alber, None;
Brian Fernandez, None; Yen Y. Lim, None; Peter J. Snyder, None
Support: Supported by unrestricted research grant from Pfizer Inc. to
Dr. Peter J.Snyder.
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to access the versions of record.
ARVO 2016 Annual Meeting Abstracts
Program Number: 5988 Poster Board Number: D0079
Presentation Time: 8:00 AM–9:45 AM
Disorders of Higher Visual Processing in Patients after Stroke
Christian Lueck1, 2, Brendan Tonson-Older2, 3, Ted Maddess3,
Jason Bell4. 1Neurology, The Canberra Hospital, Canberra, ACT,
Australia; 2Medical School, Australian National University,
Canberra, ACT, Australia; 3John Curtin School of Medical Research,
Australian National University, Canberra, ACT, Australia; 4School of
Psychology, University of Western Australia, Crawley, WA, Australia.
Purpose: Damage to specific areas outside the primary visual cortex
can cause selective deficits of higher visual processing. Patients with
stroke may have undetected abnormalities of higher visual processing
because these are not routinely assessed. If present, these could
impact significantly on recovery and residual function. This pilot
study aimed to determine the extent of these abnormalities in patients
who had recovered from stroke.
Methods: 26 patients (65.5 ± 13.9 years; 12 female) who had
recovered from stroke were compared with 29 age-matched
controls (67.6 ± 13.1 years; 17 female). Ophthalmological causes
of visual loss were excluded. Higher visual function was assessed
using Ishihara charts and Farnsworth-Munsell D15 (FMD15),
kinematography, random dot testing (depth), stereofly, line bisection,
and the Cambridge facial memory test (CFMT).
Results: Looking at the entire group, stepwise regression showed
that random dot and CFMT were the most significant predictors
of stroke (F1,77 = 5.08, p = 0.027). At a false positive rate of
10%, random dot classified 38.4% (± 17.6% SE) of patients and
CFMT classified 28.0% (± 9.1% SE). Principal component analysis
revealed two independent factors which accounted for 29.9% and
21.3% of the variance, respectively. Variables which contributed
significantly to the first factor were random dot, FMD15, CFMT and
kinematography. Line bisection, Ishihara and CFMT contributed to
the second factor. The receiver operator characteristic yielded an area
under the curve of 0.75 ± 0.071 (mean ± SE).
Conclusions: Many patients with stroke had undetected
abnormalities of higher visual processing. As a group, this was most
obvious in terms of random dot testing for depth perception. These
findings have potential relevance to the process of rehabilitation and
to residual post-stroke function. Further investigation in the form of a
larger trial is warranted.
Commercial Relationships: Christian Lueck, None;
Brendan Tonson-Older, None; Ted Maddess, nuCoria Pty Ltd
(F), nuCoria Pty Ltd (P), EyeCo Pty Ltd, (I), nuCoria Pty Ltd (I);
Jason Bell, None
Program Number: 5989 Poster Board Number: D0080
Presentation Time: 8:00 AM–9:45 AM
Examining retinal structure and function in brain injury patients
with homonymous hemianopia
Jakaria Mostafa, Suzanne Wickum, Laura J. Frishman, Jason Porter.
College of Optometry, University of Houston, Houston, TX.
Purpose: Recent studies report structural damage to retinal ganglion
cells and their axons following an acquired cortical lesion, possibly
due to retrograde degeneration. Building on these works, we sought
to quantify (1) the extent to which abnormalities exist in inner retinal
structure and function in brain injury patients with homonymous
hemianopias (HHs) and (2) how retinal alterations relate to functional
abnormalities of the central visual pathway.
Methods: Volume scans of the macula and optic nerve head were
acquired with spectral domain optical coherence tomography in 24
eyes of 12 HH patients (5 stroke, 7 traumatic brain injury [TBI];
time post-injury = 2.5 ± 2.8 years, range: 6 months – 9 years).
Peripapillary retinal nerve fiber layer thickness (RNFLT) and macular
ganglion cell-inner plexiform layer thickness (GCIPLT) were
quantified globally and in sectors, and compared with instrumentbased normative data. Photopic negative response (PhNR) amplitudes
were measured using the full-field flash electroretinogram and
compared to normative data. Mean deviation (MD) was quantified
via 30-2 standard automated perimetry. Structural and functional
measures were related on global and local scales.
Results: Global or sectoral GCIPLT, RNFLT and PhNR amplitude
were reduced in 79%, 71% and 50% of eyes, respectively. Global
measures of inner retinal (GCIPLT) and axonal (RNFLT) structure
were thinner in eyes in which more time elapsed since injury
(R2=0.42 and 0.62, respectively). The significant linear relationships
(P<.05) found across eyes between MD and (1) global GCIPLT
(R2=0.29), (2) global RNFLT (R2=0.17) and (3) PhNR amplitude
(R2=0.19) support that eyes with more extensive field loss tend to
have more abnormalities in inner retinal structure and function.
GCIPLT measures were abnormal with significantly greater
frequency in retinal sectors corresponding to the side of hemianopic
field loss versus those corresponding to the side with spared vision
(P<.05, chi-squared test).
Conclusions: Preferential damage of inner retinal structure in
locations corresponding to the side of hemianopic field loss and the
higher prevalence of inner retinal structural damage in long-standing
brain injury patients are suggestive of retrograde degeneration. A
better understanding of changes in inner retinal structure and function
may provide insights on the pathology involved in visual impairment
following brain injury.
Commercial Relationships: Jakaria Mostafa, None;
Suzanne Wickum, None; Laura J. Frishman, None; Jason Porter,
None
Support: NIH Grant P30 EY007551, Fight for Sight Summer
Student Fellowship, University of Houston College of Optometry
Program Number: 5990 Poster Board Number: D0081
Presentation Time: 8:00 AM–9:45 AM
Abnormal Eye Movement Behavior during Reading in
Parkinson’s Disease
Caroline Yu, Ali Shariati, Timothy Lee, Yaping J. Liao. Stanford
School of Medicine, Stanford, CA.
Purpose: Vision difficulties have been well described in patients
with Parkinson’s Disease (PD), but the etiologies underlying PD
patients’ difficulties in performing functional tasks like reading
are not well understood. In this study, we performed clinical and
infrared oculography studies of reading in PD in order to better
delineate the causes.
Methods: We performed a prospective cross-sectional study of
23 treated patients with PD and 25 controls with no known visual
symptoms. We assessed subjective visual disability using the
National Eye Institute Visual Function Questionnaire (VFQ-25)
(n=16), measured reading speed using a rapid number naming
task called the King-Devick test (n=23), and further analyzed
ocular motor behavior during reading in PD patients with good
visual acuity using 500-Hz infrared oculography (RED500,
SensoMotoric Instruments) (n=9). Stimuli included fixation,
number-reading, and word-reading tasks. Statistical analysis was
performed using the Mann-Whitney test (Prism).
Results: PD patients reported decreased visual function with a mean
VFQ-25 score of 81±3.6 (n=16), and 56% had moderate or extreme
difficulties with near activities. Using the King-Devick (KD) test,
we found that PD patients were significantly slower readers than
age-matched controls (PD: 68.3±6.5s, ctrl: 48.8±2.4s, p = 0.009),
which correlated with subjective decrease in near activity on VFQ-25
(r2=0.49). On infrared oculography, PD patients had significantly
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ARVO 2016 Annual Meeting Abstracts
slower number reading speed (PD: 100.2 ±12.1 numbers per minute
(npm), ctrl: 145.0±20.6 npm, p=0.05) and word reading speed (PD:
135.3±15.1 words per minute (wpm), ctrl: 230.2±21.0 wpm, p=0.01).
The slower reading was attributable to more frequent (PD: 6.6±0.3
saccades/line, ctrl: 4.9±0.1 saccades/line, p=0.08) and smaller
saccades, more regressions (PD: 9.1%, ctrl: 5.3%, p=0.05), and
longer fixations (PD: 266±13 ms/line, ctrl: 233±14 ms/line, p=0.05).
Conclusions: The majority of treated PD patients reported visual
disability, especially with reading and near work which correlated
with worse performance on a rapid number reading task. Using
infrared oculography to study reading behavior, we found that PD
patients exhibited abnormalities in saccades, fixations, and reading
behavior, which were attributable to ocular motor abnormality,
impaired ocular motor planning, and cognitive dysfunction.
Commercial Relationships: Caroline Yu, None;
Ali Shariati; Timothy Lee, None; Yaping J. Liao, None
Program Number: 5991 Poster Board Number: D0082
Presentation Time: 8:00 AM–9:45 AM
Patterns of visual field changes in thyroid eye disease
Catherine J. Choi1, Susel Oropesa2, Alison B. Callahan2,
Lora R. Glass1, Livia Teo3, Michael Kazim2, Suzanne K. Freitag1.
1
Ophthalmic Plastic Surgery, Massachusetts Eye and Ear Infirmary,
Boston, MA; 2Ophthalmology, Edward S. Harkness Eye Institute
of the New York Hospital Presbyterian Medical Center, New York,
NY; 3Ophthalmic Plastic Surgery, Singapore National Eye Centre,
Singapore, Singapore.
Purpose: To provide a systematic description of patterns of visual
field changes in dysthyroid optic neuropathy (DON).
Methods: A retrospective, non-comparative review of patients age 18
or older at the time of diagnosis of DON, with documented 24-2 or
30-2 Humphrey Visual Field (HVF) testing between April 1991 and
July 2015 at the Massachusetts Eye and Ear Infirmary and Edward
S. Harkness Eye Institute of Columbia University was conducted
with IRB approval. 100 visual fields in 68 patients between the
ages of 35 and 87 with fixation loss <20% and false positives and
negatives <33% were included. In an abnormal VF, at least three
consecutive points on pattern deviation plot depressed by 5 dB
level or more were required. Abnormal VFs were then classified
as one of the 17 mutually exclusive pre-specified patterns from the
Ocular Hypertension Treatment Study (OHTS) (altitudinal, arcuate,
nasal step, paracentral, partial arcuate, temporal wedge, central,
hemianopia, inferior depression, partial hemianopia, partial peripheral
rim, peripheral rim, quadrant, superior depression, total loss, vertical
step, and widespread) or “other”.
Results: The five most common patterns were other (27%), partial
arcuate (26%), partial peripheral rim (10%), arcuate (8%) and
altitudinal (7%). Average mean deviations for the five most common
patterns were: other -4.82 dB +/-5.68, partial arcuate -6.32 dB+/3.41, partial peripheral rim -5.67 dB +/- 1.78, arcuate -13.5 dB
+/- 11.8, and altitudinal -11.5 dB +/- 6.16. Further sub-classification
showed a predominance of inferior VF defects, ranging from 40% to
93% of each category (Table 1, Figure 1). Of the 78 VFs in these five
categories combined, 53 (68%) were inferior VF defects.
Conclusions: This study was the first application of systematic
classification of VF in DON using a validated method. While
the OHTS VF categories are geared towards classification of
glaucomatous patterns, the predominance of a spectrum of
inferior VF defects in DON was demonstrated. This is thought
to be consistent with the anatomy of the orbital apex where the
position of the optic nerve within the lesser wing of the sphenoid
in relation to the annulus of Zinn places the superior aspect of the
optic nerve closest to the enlarged extraocular muscles. The high
proportion of VF falling under the “other” category, however,
does demonstrate the need for a more specific and tailored VF
classification system for DON.
Commercial Relationships: Catherine J. Choi, None;
Susel Oropesa, None; Alison B. Callahan, None; Lora R. Glass,
None; Livia Teo, None; Michael Kazim, None; Suzanne K. Freitag,
None
Program Number: 5992 Poster Board Number: D0083
Presentation Time: 8:00 AM–9:45 AM
Retinoic Acid and T3 Induce CCL2 in Human Orbital
Fibroblasts – A Model of Thyroid Eye Disease
Daniel Kasprick, Phillip E. Kish, Fatemeh Rajaii, Alfonso Saera-Vila,
Alon Kahana. Ophthalmology and Visual Sciences, University of
Michigan, Ann Arbor, MI.
Purpose: Thyroid eye disease (TED) is a congestive orbitopathy
characterized by enlargement and fibrosis of orbital muscle and fat,
which causes a compartment syndrome leading to pain, diplopia,
corneal exposure and potentially compressive optic neuropathy.
TED can be associated with both hyper- and hypothyroid states, yet
its pathogenesis remains poorly understood. Based on embryologic
studies of orbital development, we hypothesized that orbital
tissues respond to thyroid hormone (T3) and retinoic acid (RA) by
expressing pro-inflammatory cytokines that lead to orbital targeting.
Methods: Fibroblasts from whole orbital fat obtained from patients
undergoing blepharoplasty were grown to confluence in tissue culture
and treated with T3, RA, or a combination of the two for 6 and 9
hours. Following microarray transcriptome analysis, studies focused
on the cytokine CCL2 (a.k.a MCP1). qRT-PCR was used to quantify
CCL2 expression, normalized to 18S gene expression using the
delta-delta method, comparing treatments with RA, T3, RA+T3, and
DMSO control.
Results: CCL2 expression was induced in all three treatment groups
compared with control. T3 alone induced the lowest level of CCL2
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ARVO 2016 Annual Meeting Abstracts
expression (avg 2.28x, 0.65 SEM), then RA alone (avg 3.6x, 0.79
SEM), and RA+T3 induced the highest CCL2 expression (avg 4.25x,
SEM 1.2). Importantly, the range of CCL2 induction reflected the
heterogeneity expected from this clinical disease process, with one
sample inducing CCL2 expression 10.72 fold.
Conclusions: Our data suggest that on average CCL2 expression
can be induced by a combination of RA and T3, with significant
variability in expression level among patients. The variability may
represent an underlying predisposition of certain individuals to
develop TED, accounting for the observed disease heterogeneity. RA
analogs have been used therapeutically for many years, and CCL2
itself is a potential therapeutic target, suggesting new approaches to
this debilitating and disfiguring disease. Analysis of this pathway as a
driver of TED is ongoing, and additional data will be presented.
Figure 1. Relative expression levels of CCL 2 showing greatest
induction of expression with combined RA+T3 treatment.
Commercial Relationships: Daniel Kasprick; Phillip E. Kish,
None; Fatemeh Rajaii, None; Alfonso Saera-Vila, None;
Alon Kahana, Genentech (F), NIH (F)
Support: NIH Grant 1R01EY022633 and Research to Prevent
Blindness.
Program Number: 5993 Poster Board Number: D0084
Presentation Time: 8:00 AM–9:45 AM
Intravenously Administered Neostigmine Combined with Prism
and Alternate Cover Test as a Diagnostic Test for Myasthenia
Gravis with ocular involvement
Dong Ik Kim, Byung Joo Lee, Seong-Joon Kim. Ophthalmology,
Seoul National University College of Medicine, Seoul, Korea (the
Republic of).
Purpose: To address the validity of intravenous neostigmine
administration combined with prism and alternate cover test (PACT)
measurement as a confirmatory diagnostic method for confusing
cases of myasthenia gravis with ocular involvement.
Methods: Neostigmine was administered intravenously in ten
suspicious myasthenic diplopia patients under electrocardiographic
monitoring. Distance deviation at primary position was evaluated
with PACT at 5, 10, 15, 20 and 30 minutes after intravenous injection
of neostigmine. Margin reflex distance and range of duction were also
evaluated at each time points.
Results: 7 out of 21 patients were diagnosed as myasthenic diplopia
by positive neostigmine test. Among these patients, everyone
had abnormal ocular motility and 5 had ptosis. In participants
who showed positive result, impairment of ocular motility was
relieved invariably, but ptosis was not improved in one patient. The
improvement of ocular motility had been occurred within 5 minutes
in all 7 responders and the pharmacological effect reached peak at 5
to 20 minutes (mean: 13 minutes) after neostigmine administration.
Conclusions: Intravenous neostigmine administration combined
with PACT is a rapid, objective and quantifiable method in hardto-diagnose cases of myasthenia gravis with ocular involvement.
In performing neostigmine test for myasthenia gravis with ocular
involvement, not only the lid position, but also ocular motility should
be evaluated quantitatively to avoid a false negative result.
Commercial Relationships: Dong Ik Kim, None; Byung Joo Lee,
None; Seong-Joon Kim, None
Program Number: 5994 Poster Board Number: D0085
Presentation Time: 8:00 AM–9:45 AM
Pilot study to assess the efficiency and safety of the parabulbar
triamcinolone for the treatment of moderate to severe Graves
Ophthalmopathy assessed through STIR magnetic resonance
imaging
Tomas -. Ortiz basso1, 2, Rodolfo L. Vigo1, Mariano -. Sidelnik3,
Eduardo J. Premoli1, Felisa Shokida1. 1Ophthalmology, Hospital
Italiano de Buenos Aires, Buenos Aires, Argentina; 2Centro
Oftalmologico, Santa Rosa, Argentina; 3diagnostic imaging, Hospital
Italiano de Buenos Aires, Buenos aires, Argentina.
Purpose: The efficiency of the parabulbar triamcinolone for treating
the Thyroid – Associated Ophthalmopathy (TAO) is a controversial
issue. We performed a quasi – experimental prospective study to
assess if the parabulbar triamcinolone is effective and safe so as to
decrease the orbital inflammation in patients presenting moderate to
severe TAO.
Methods: It was carried out on patients who suffered from a
moderate to severe active Thyroid – Associated Ophthalmopathy.
Through orbital STIR magnetic resonance imaging it was assessed
whether the Signal Intensity Ratio (SIR) of the extraocular muscles
decreased after a treatment with parabulbar acetonide triamcinolone.
Furthermore, the Clinical Activity Score (CAS) was analyzed
together with the exophthalmometry, before and after the treatment.
Criteria to exclude patients from the treatment were applied to those
suffering from orbitopathy of other ethiology, who had undergone
surgery during the 6 previous months, those treated with local or
systemic corticosteroids during the 6 previous months. A T – test for
paired data was used for statistical analysis.
Results: There were included 15 patients and 28 orbits for the
analysis. The average age was 42 years (SD 15,47) and 66% (n10)
were women.
The average SIR for the inferior rectus was 4.07 (SD 1.4) before
the treatment and 2.67 (SD 0.93) (p 0.0003) after it; in the case of
the superior rectus the average SIR was 3.57 (SD 0.86) and 2.8 (SD
0.44) (p 0.0002) after the treatment; in the case of the external rectus
that value was 3.65 (SD 0.76) before the treatment and 2.8 (SD 0.4)
(p 0,0002) after the treatment; and finally, for the internal rectus the
average SIR was 3.93 (SD 1.1) before the treatment and 3.1 (SD
0.58) (p 0.0026) after the treatment.
No patient presented local or systemic complications after the
treatment.
Conclusions: The treatment with parabulbar triamcinolone is
effective and safe do decrease SIR in patients presenting moderate to
severe thyroid-associated ophthalmopathy.
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ARVO 2016 Annual Meeting Abstracts
Although further studies are necessary to compare the efficiency
and safety of local and systemic corticosteroids applying this
technique, they can be effectively replaced when the risk of systemic
corticosteroids are greater than the benefits.
Commercial Relationships: tomas -. ortiz basso, None;
Rodolfo L. Vigo, None; Mariano -. Sidelnik, None;
Eduardo J. Premoli, None; Felisa Shokida, None
Clinical Trial: http://www.ensaiosclinicos.gov.br/, U1111-1177-1925
Program Number: 5995 Poster Board Number: D0086
Presentation Time: 8:00 AM–9:45 AM
Ophthalmic Manifestations of Giant Axonal Neuropathy
(GAN): Towards Establishing Visual Function / Optic Atrophy
Assessments as a Secondary Outcome Measure in an Ongoing
Intrathecal Gene Transfer Trial
Wadih M. Zein1, Diana X. Bharucha-Goebel2, Payam Mohassel2,
Aileen R. Foley2, Tanya J. Lehky2, Melissa Waite3, Jain S. Mina3,
Brett G. Jeffrey1, Brian P. Brooks1, Carsten Bonnemann2. 1OGVFB,
NEI/NIH, Bethesda, MD; 2NINDS / NIH, Bethesda, MD; 3Clinical
Center / NIH, Bethesda, MD.
Purpose: To describe the ophthalmic manifestations of GAN, a rare
autosomal recessive neurodegenerative disease caused by mutations
in the Gigaxonin gene.
To investigate the potential utility of visual function measures as a
secondary outcome measure for a GAN gene transfer clinical trial
(NCT02362438).
Methods: Seventeen children with GAN underwent comprehensive
ophthalmic evaluation as part of a natural history protocol at
the National Institutes of Health (NCT01568658). Age at initial
ophthalmic exam ranged from 4.5 to 15 years (median 9.0 yrs).
Visual acuity, visual field, color vision, and optical coherence
tomography (OCT) data was analyzed. Correlation with systemic
measures of disease severity such as the Motor Function Measure
(MFM32) were undertaken.
Results: Visual acuity ranged from 20/20 to 20/320 (logMAR 0.0
to 1.2; Mean ± SD 0.4 ± 0.3 logMAR). Visual fields were obtained
in 12 patients and showed a deficit in 8/12 (66%). Color vision
was abnormal in 7/17 patients (41%). Anatomic or functional
evidence of optic atrophy was ascertained in 10/17 patients (59%).
Subtle optic nerve pallor with borderline retinal nerve fiber layer
(RNFL) thickness and normal visual function was noted in 5/17
patients (29%). Normal optic nerve exam was present in two
GAN patients (12%). Optic nerve OCT showed an average RNFL
thickness of 72.3 ± 18.0µm OD and 70.7 ± 17.2µm OS (literature
mean for this age group 97.2 µm, first percentile 75.1 µm). RNFL
thickness correlated well with disease severity measured by MFM32
(Spearman r 0.76, R-square 0.57). Average macular ganglion cell
analysis (GCA) thickness was 63.6 ± 12.0µm OD and 64.5 ± 10.2µm
OS. Older patients had thinner RNFL and GCA measurements.
Other ophthalmic features noted in this cohort included strabismus
(5/17, 29%), nystagmus (10/17, 59%), hypometric saccades, and
lagophthalmous.
Conclusions: Optic atrophy is a common manifestation of GAN.
Optic nerve OCT parameters correlate well with disease severity.
Regular ophthalmic exam and OCT are recommended for GAN
patients. Visual function measures and electrophysiologic / anatomic
methods focused on assessing severity of optic atrophy can
potentially serve as a secondary outcome measure in GAN treatment
trials (e.g. the currently recruiting intrathecal gene transfer study
NCT02362438).
Commercial Relationships: Wadih M. Zein, None;
Diana X. Bharucha-Goebel, None; Payam Mohassel, None;
Aileen R. Foley, None; Tanya J. Lehky, None; Melissa Waite,
None; Jain S. Mina, None; Brett G. Jeffrey; Brian P. Brooks,
None; Carsten Bonnemann, None
Support: This work was supported by the Intramural Research
Program of the National Institutes of Health (NIH).
Program Number: 5996 Poster Board Number: D0087
Presentation Time: 8:00 AM–9:45 AM
Malpractice Litigation in Neuro-Ophthalmology
Irfan Khan2, Ashvini Reddy2, Austin Sim1. 1Department of
Internal Medicine, University of Virginia, Charlottesville, VA;
2
Ophthalmology, Johns Hopkins University School of Medicine,
Baltimore, MD.
Purpose:
To report the diagnoses, causes, and outcomes of medical malpractice
lawsuits in neuro-ophthalmology to inform clinical decision-making
and guide risk management.
Methods:
Retrospective review of malpractice verdicts, rulings, settlements
related to ophthalmology in the United States WestLaw® database
from 1930-2014. Data including subspecialty focus of neuroophthalmology, patient age, patient gender, legal allegation, nature of
injury, verdicts, indemnities, and (when applicable) financial award
were collected and analyzed. To enable meaningful comparison,
monetary awards were adjusted to 2015 United States dollars.
Results:
A total of1086 malpractice cases against ophthalmologists were
identified, and 53 (4.9%) of these had subspecialty focus in neuroophthalmology. Overall, 37.7% of outcomes favored the defendant.
The most common diagnoses leading to litigation were failure to
diagnose an optic nerve or other CNS tumor (27%), complications
related to strabismus surgery (24%), failure or delay in diagnosing
GCA (18%), and failure to diagnose a CVA (14%). A total of 10 suits
(19%) resulted in settlement, with mean adjusted indemnities of
$559,414 (median, $726,812.66; range, $83,312.90 - 1,453,184.32).
14 cases (26%) resulted in plaintiff verdict, with mean adjusted
indemnities of $2,733,518 (median, $7,371,927; range, $110,761.73 –
14,733,366.48). Outcomes favoring the plaintiff were more common
in neuro-ophthalmology than in any other specialty, and monetary
awards for malpractice in neuro-ophthalmology were higher than
awards for all other ophthalmology subspecialties except for retina.
Conclusions: Neuro-ophthalmology represents a small percentage
of the litigation in ophthalmology, but is more likely to be associated
with outcomes favoring the plaintiff than any other subspecialty.
Commercial Relationships: Irfan Khan, None; Ashvini Reddy,
None; Austin Sim, None
These abstracts are licensed under a Creative Commons Attribution-NonCommercial-No Derivatives 4.0 International License. Go to http://iovs.arvojournals.org/
to access the versions of record.