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Purpose: To determine the ocular and systemic safety of using topical Lambda-Cyhalothrin (LCL) in a canine model of infantile nystagmus syndrome (INS). The rationale for this proposal is based on a case study of a patient whose INS improved after inadvertent ocular exposure to a pyrethroid pesticide containing LCL.Methods: After in-vitro safety testing and IUCAC approval, we studied increasing concentrations of topical LCL drops (0.002% to 0.07%) in canines with a purposely bred defect in the RPE65 gene resulting in both retinal degeneration and INS. We collected data on ocular and systemic effects and performed eye-movement recordings (EMR).Results: At the 0.07% concentration dose of LCL, there was minimal, reversible, conjunctival hyperemia. There was no other ocular or systemic toxicity. At the 0.06% dose, there was a visible decrease in the INS and EMR showed a 153%-240% increase in the nystagmus acuity function and a 30%-70% decrease in amplitude across gaze. There was also a 40%-60% decrease in intraocular pressure while on the drop in both eyes.Conclusion: This animal study suggests this new pharmacological agent has potential for topical treatment of both INS and diseases with raised intraocular pressure. Further, this new treatment approach confirms the importance of extraocular muscle proprioception in ocular motor diseases and their treatment. [ABSTRACT FROM AUTHOR]

Medicine has a six-fold role to play in war, 1) selection of suitable military personnel, 2) to protect against disease, 3) to give medical attention and treatment to those who are ill and injured, 4) to assist in the rehabilitation of the disabled, 5) research to improve etiology and treatments of disease, and 6) the unit surgeon serves as a special staff officer to the military commander at all levels of a battalion and above, along with the attorney and chaplain, and supplies medical input into all plans whether it be training or combat operations. This article focuses on the realization by soldiers, since antiquity, the importance of the visual system in battle, how this was measured, and how modern military visual requirements have evolved along with the science of ophthalmology. Necessity and natural selection were the driving forces for recruitment and assignment in ancient and medieval armies. Since the advent of mechanized warfare, more soldiers can perform more tasks, more uniformly, radically changing the way soldiers are selected. Modern military duty eligibility requirements and assignments are now the result of special tests and documentation procedures resulting from a mixture of medical knowledge, science, and potential legal consequences. Nowhere is this more evident in the stringent visual system requirements for military service. [ABSTRACT FROM AUTHOR]

Purpose: To test the hypothesis that eye muscle surgery in treatment of patients with acquired downbeat nystagmus results in improvement measures of visual and ocular motor function.Methods: This is a prospective, interventional case series analysis of clinical and electrophyisological data before and after eye muscle surgery in 17 patients with acquired downbeat nystagmus who did not respond to medical treatments. Outcome measures included: 1) routine demography and clinical characteristics, 2) subjective oscillopsia (SO), 3) binocular best-corrected visual acuity in the null position (BVA), 3) primary position strabismic deviation (SD), 5) anomalous head posture (AHP), 6) contrast sensitivity function (CS), and 7) nystagmus slow phase velocity (SPV). All patients were followed at least 12 months. Parametric and non-parametric statistical analysis of outcome measure data above pre- and post-treatment were perfomed using standard software on grouped data using computerized software.Results: Patients' age ranged from 5 to 85 years (average 27 years). About 59% were male. Follow up ranged from 1-10 years (average 2.0 years). Around 70% had an associated central nervous systemic diagnosis, 100% had an AHP, oscillopsia and decreased CS, 53% had other eye disease, and 59% had strabismus. There were no complications from surgery. There were signficant post-treatment improvements in mean/median group BVA, SO, SD, AHP, CS, and SPV.Conclusion: This study supports the hypothesis that eye muscle surgery as treatments for patients with acquired downbeat nystagmus can result in improvements in multiple aspects of ocular motor and visual functions. [ABSTRACT FROM AUTHOR]

The most common forms of nystagmus in infancy and childhood are Infantile Nystagmus Syndrome (INS), Fusion Maldevelopment Nystagmus Syndrome (FMNS), and Spasmus Nutans Syndrome (SNS). This review will sumamrize the clinical and eye movement recording characteristics of these disorders, thus reinforcing the importance of eye movement technology in both the discoveries underlying pathophysiology and the guidance of clinical interventions. [ABSTRACT FROM AUTHOR]

Purpose To test the hypothesis that the topical carbonic anhydrase inhibitor brinzolamide (Azopt) has beneficial effects versus placebo on measures of nystagmus and visual acuity in adult subjects with infantile nystagmus syndrome (INS). Design Prospective, cross-over, double masked clinical trial. Methods Setting: Single centre. Study population: Five subjects ≥18 years old with typical INS and bestbinocular visual acuity in their primary position null zone ETDRS 55 letters to 85 letters (20/200 to 20/50) and had no previous treatment for nystagmus. Intervention: In a randomised order, each subject received one drop of Azopt or placebo in both eyes three times a day separated by a washout period of at least a week followed by Azopt or placebo in both eyes three times a day; thus each subject got the drug and placebo, each acting as his or her own control. Outcome measures: The nystagmus acuity function and INS waveforms obtained from eye movement recordings, binocular optotype visual acuity, using the ETDRS protocol analysed individually and as a group before and after Azopt and placebo. Results Versus placebo and baseline measures, topical Azopt significantly improved; INS waveform characteristics in the primary position null zone, group mean values of the nystagmus acuity function across gaze ( p<0.01) and group mean ETDRS binocular letter visual acuity ( p<0.05). There was a predictable decrease in intraocular pressure (IOP) without any systemic or ocular adverse events. Conclusions Although a prospective large-scale clinical trial is needed to prove effectiveness, an eye-drop-based therapy for INS may emerge as a viable addition to optical, surgical, behavioural and systemic drug therapies for INS. [ABSTRACT FROM AUTHOR]

The purpose of this report is to summarize an understanding of the ocular motor system in patients with albinism. Other than the association of vertical eccentric gaze null positions and asymmetric, (a) periodic alternating nystagmus in a large percentage of patients, the ocular motor system in human albinism does not contain unique pathology, rather has "typical" types of infantile ocular oscillations and binocular disorders. Both the ocular motor and afferent visual system are affected to varying degrees in patients with albinism, thus, combined treatment of both systems will maximize visual function. [ABSTRACT FROM AUTHOR]

We have observed dramatic effects of tactile tongue stimulation on nystagmus eye movements in patients with acquired blindness, and we report these results. Six adult subjects (3 subjects with light perception or worse vision and 3 normal subjects) were included in this study. Causes of blindness included traumatic explosion, anterior ischemic optic neuropathy, and central retinal artery occlusion. Duration of blindness was 15, 3 and 1.5 years, respectively. A video eye tracking system (Eyelink 1000) was used to record eye movements. The eye movement recording (EMR) was repeated four times in a span of 20 min. Two of the EMRs were performed without tongue stimulation and two with tongue stimulation in randomized order. A tongue stimulus was applied to the surface of the tongue using a Brainport device that produces an electrical tactile stimulus. The nystagmus waveform characteristics and frequency were analyzed. We found that all blind subjects showed continuous jerk nystagmus with slow and quick phases, mainly in horizontal plane in their primary eye positions. The recorded nystagmus waveforms were jerk with linear velocity slow phases. When the tongue stimulus was applied, the frequency of nystagmus was significantly reduced by 47, 40, and 11%, and relative amplitude was reduced by 43, 45, and 6% for three blind subjects, respectively. In conclusion, we think our results that tongue stimulation influences nystagmus eye movements support a link between non-visual sensory input and ocular motor activity. [ABSTRACT FROM AUTHOR]

The study of the clinical and electrophysiological effects of eye muscle surgery on patients with infantile nystagmus has broadened our knowledge of the disease and its interventions. Prospective, comparative, interventional case series. Twenty-four patients with a vertical head posture because of electrophysiologically diagnosed infantile nystagmus syndrome. The ages ranged from 2.5 to 38 years and follow up averaged 14.0 months. Thirteen patients with a chin-down posture had a bilateral superior rectus recession, inferior oblique myectomy and a horizontal rectus recession or tenotomy. Those 11 with a chin-up posture had a bilateral superior oblique tenectomy, inferior rectus recession and a horizontal rectus recession or tenotomy. Outcome measures included: demography, eye/systemic conditions and preoperative and postoperative; binocular, best optically corrected, null zone acuity, head posture, null zone foveation time and nystagmus waveform changes. Associated conditions were strabismus in 66%, ametropia in 96%, amblyopia in 46% and optic nerve, foveal dysplasia or albinism in 54%. Null zone acuity increased at least 0.1 logMAR in 20 patients ( P < 0.05 group mean change). Patients had significant ( P < 0.05) improvements in degrees of head posture, average foveation time in milliseconds and infantile nystagmus syndrome waveform improvements. This study illustrates a successful surgical approach to treatment and provides expectations of ocular motor and visual results after vertical head posture surgery because of an eccentric gaze null in patients with infantile nystagmus syndrome. [ABSTRACT FROM AUTHOR]

Purpose: To discuss the common characteristics of infants and children with nystagmus and review treatments and their evidence. Methods: The NEI-sponsored classification of eye movement abnormalities and strabismus and a PubMed search for papers from 1995 through 2005 on interventions for Infantile Nystagmus Syndrome (INS) are reviewed. Results: The review of treatments revealed 18 papers on surgical or pharmacological interventions and six papers describing other interventions. There is only one randomized controlled trial, with all the other studies being uncontrolled trials/case series. Conclusions: Previous science and studies on interventions for nystagmus in infancy and childhood have laid an important foundation for future work in this field, which should include a randomized clinical trial. [ABSTRACT FROM AUTHOR]

Involuntary occular oscillations in infancy and childhood can be understood, diagnosed, classified and treated as a result of modern electrophysiology and neuroanatomical knowledge. [ABSTRACT FROM AUTHOR]

Abstract: The only Confederate president, Jefferson Davis, led a long and eventful life. He was a Mississippi planter, a husband, a father, West Point graduate, war hero, congressman, senator, secretary of war, and finally President of the Confederate States of America. In many ways he was a study of contrast with his northern counterpart, Abraham Lincoln. Davis was personally courageous and a rich, educated, southern aristocrat who did not deeply understand the political process or have the refined personal skills necessary to work well with others. Prior to his Presidency he served with distinction in two wars, but as a result of his confederate activity and pro-slavery philosophy he is one of the least discussed famous Americans. Davis''s health was a constant problem and he suffered an almost fatal attack of “malaria” in 1836. In the winter of 1857–1858 he again was seriously ill and by the end of February 1858 a chronic, relapsing, ocular inflammatory condition began. Using historical evidence from multiple sources, this paper will propose a diagnosis of the Confederate President''s ocular condition and consider how this could have influenced his military and political decisions. [Copyright &y& Elsevier]

The purpose of this report is to summarize clinical and electrophysiological effects of extraocular muscle surgery in patients with INS. Our hypothesis is that surgery on the extraocular muscles of patients with INS changes their nystagmus resulting in improved vision and visual functions. All patients had all four virgin horizontal recti operated on, either for strabismus alone, nystagmus alone, for a head posture due to an eccentric null zone alone or for a head posture due to an eccentric null zone plus strabismus. All patients have been followed for at least 12 months. Subjective outcome measures include the pre- and post-operative binocular best optically corrected null zone acuity (NZA) in 75 patients and gaze dependent acuity (GDA) in 12 patients. Objective outcome measure included null zone width (NZW) in 75 patients. The results are summarized as follows: NZA increased .1 LogMar or greater in 75% with those patients ≤8 years significantly better. Subjective GDA and NZW measured from eye movement recordings showed persistent, significant increases. This report adds to the evidence that surgery on the extraocular muscles in patients with INS has independent neurological and visual results, from simply reposition the head, eye(s) or visual axis. [ABSTRACT FROM AUTHOR]

Objective: To describe the clinical and electrophysiological characterization of four family members from three generations who have X-linked infantile periodic alternating nystagmus (XIPAN). Methods: Complete clinical ophthalmological evaluation, pedigree analysis, electroretinograms (ERG), eye movement recordings (EMR), color vision, and fundus photography were performed on all subjects. Results: Three males in two generations and one female were examined. Clinical examinations showed a jerk/pendular nystagmus with a latent component, strabismus, and a significant refractive error in the three affected males, while the female had only myopic astigmatism. ERG, color contrast, and fundus examinations were normal in all four family members. All four family members showed EMR abnormalities with infantile jerk/dual jerk and pendular nystagmus waveforms. The female had nystagmus present on EMR only and all patients showed (a)periodicity to their nystagmus. Conclusions: In this family with no other congenital visual sensory system disease, affected males had obvious periodic alternating nystagmus, strabismus, and refractive errors, while the female had clinically ‘silent’ periodic nystagmus that is probably a marker for the carrier state. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this report is to characterize the clinical and electrophysiological effects of extraocular muscle surgery in 15 patients with oculo-cutaneous albinism (OCA) and infantile nystagmus syndrome (INS). Our hypothesis is that surgery on the extraocular muscles of patients with OCA and INS changes their nystagmus and their visual function. Design: Interventional, prospective, cohort, noncomparative case series. Methods: All 15 patients had surgery on all four virgin horizontal recti; three for strabismus alone, three for nystagmus alone, five for an eccentric gaze null zone alone, and four for an eccentric gaze null zone plus strabismus. All patients have been followed for at least six months. All 15 patients had the subjective outcome measure of pre- and postoperative binocular best optically corrected acuity (BBOCA). Objective outcome measures included anomalous head posture (AHP) in nine patients, eye movement recording measures of expanded nystagmus acuity function (NAFX) in 10 patients, null zone position (NUZP) and null zone width (NUZW) in 10 patients, and foveation time (FOV) in nine patients. Results: The results are summarized as follows; BBOCA increased 0.1 LogMar or greater in 14 of 15 patients. In those operated on for an AHP with or without associated strabismus the AHP improved significantly (P < .01 for all). The NAFX, NUZP, NUZW, and FOV measured from eye movement recordings showed persistent, significant increases in all patients (P < .01 for all). Conclusions: This report adds to the evidence that surgery on the extraocular muscles in patients with INS has independent neurologic and visual results. [Copyright &y& Elsevier]

: ObjectiveWe wished to determine the effectiveness of horizontal rectus tenotomy in changing the nystagmus of patients with congenital nystagmus and, secondarily, how their visual function changed.: DesignThis was a prospective, noncomparative, interventional case series.: ParticipantsTen adult patients with varied associated sensory defects and oculographic subtypes of congenital nystagmus (including asymmetric periodic or aperiodic alternating nystagmus) and no nystagmus treatment options.: MethodsBy using standard surgical techniques, simple tenotomy of all four horizontal recti with reattachment at the original insertion was accomplished. Search-coil eye movement recordings and clinical examinations were performed before and 1, 6, 24, and 52 weeks after surgery.: Main outcome measuresThe primary outcome measure was the expanded nystagmus acuity function, obtained in “masked” fashion directly from ocular motility recordings. Secondary outcomes included breadth of null zones, preoperative and postoperative masked measures of visual acuity (Early Treatment Diabetic Retinopathy Study [ETDRS] chart), and the National Eye Institute Visual Function Questionnaire (NEI-VFQ-25).: ResultsAt 1 year after tenotomy and under binocular conditions, 9 of 10 patients had persistent, significant postoperative increases in the expanded nystagmus acuity function of their fixing (preferred) eye; 1 remained high, and 1 was not tested under the same conditions. Average foveation times increased in all 9 fixing (preferred) eyes. Binocular visual acuity measured with the ETDRS chart increased in 5 patients and was unaffected in five, whereas the NEI-VFQ-25 showed an improvement in vision-specific mental health in 9 patients. There were no adverse events. Tenotomy also radically changed the periodicity of one patient''s asymmetric periodic or aperiodic alternating nystagmus.: ConclusionsIn 9 of 10 adult patients with clinical and oculographic variations in their congenital nystagmus, tenotomy resulted in significant improvements in a nystagmus measure and subjective visual functions. [Copyright &y& Elsevier]

Congenital nystagmus (CN) is an aperiodic oscillatory eye movement disorder of unknown etiology. We examined the effect of horizontal rectus tenotomy with simple re-attachment on the dimensionality of the dynamical mechanism underlying CN. The correlation dimensions (CDs) were calculated from eight patients who had tenotomy surgery. We found no significant differences in the CDs that could be associated with the surgery. The change in dimensionality was less than 5% on average. The results suggest that the tenotomy has no effect, or only a quite small effect, on the underlying mechanism of the CN beats. [Copyright &y& Elsevier]

Congenital nystagmus (CN) is an aperiodic oscillatory eye movement disorder. Horizontal rectus tenotomy with simple re-attachment has been proposed as a therapy for CN. This therapy might affect vision and/or eye movements. Another paper deals with improvements in visual acuity. This and the companion paper examine changes in eye movements. In this study, we examined the effect of tenotomy on nystagmus waveforms using wavelet spectral analysis. No common effect was found across the patients on the wavelet spectra of the CN beat, suggesting that tenotomy surgery has no effect, or only a quite small effect, on the waveform structure of CN. [Copyright &y& Elsevier]

Objective: To report the clinical, electrophysiologic, and radiographic analysis of four patients presenting with nystagmus and diagnosed with achiasma or hypochiasma and summarize all I I patients reported with this syndrome. Methods: Each patient underwent complete ophthalmologic examination, fundus photography, ocular motor recordings (OMR), visual evoked potentials (VEP), visual field testing (VFT), and magnetic resonance imaging (MRI) of the optic pathways. Results: Reduced vision, strabismus, optic nerve dysplasia, congenital nystagmus (CN), and see-saw nystagmus were present in all patients. One of these four patients had congenital hydrocephalus and three of the four patients had amblyopia. Monocular VFT was full in the older patients tested. OMR showed dysconjugate vertical, multiplanar ocular oscillations with CN slow phases, welldeveloped foveation periods, and occasional spontaneous uniocular saccades. VEPs showed crossed asymmetry and increased latency consistent with uncrossed optic pathways and optic nerve dysplasia. Specific MRI studies showed no chiasm in three patients and a hypoplastic chiasm in one. Conclusions: These patients further elucidate the spectrum of human achiasma/hypochiasma. Careful intraocular and ocular motor evaluation found optic nerve anomalies and seesaw nystagmus in all patients. Patients with congenital optic nerve hypoplasia/dysplasia with or without other midline neurologic defects should be carefully evaluated for the clinical presence of see-saw nystagmus (SSN). The occurrence of the conditions CN, SSN, and optic nerve anomaly strongly suggests achiasma/hypochiasma. Electrophysiologic and radiographic evaluations should be considered in these patients. [ABSTRACT FROM AUTHOR]

A case of a 7-year-old male with X-linked hyper-IgM syndrome who developed cerebellar cortical atrophy and optic nerve atrophy following a brief febrile illness is discussed. After an episode of gastroenteritis, he experienced cognitive deterioration, progressive ataxia, dysarthria, cerebellar eye signs, and bilateral optic nerve atrophy that stabilized after seven months. Although we believe that he had direct neural infection, extensive microbiologic, immunologic, and radiologic investigations failed to identify a definite etiology to explain his neurologic abnormalities. This is, to our knowledge, the first reported neuro-ophthalmic disease associated with the hyper-IgM syndrome. [ABSTRACT FROM AUTHOR]

Deals with a study which characterized adults who required surgical correction of their strabismic deviation. Importance of strabismus diagnosis and treatment; Methodology of the study; Discussion of post-operative results.

Presents a study which investigated the application of adjustable horizontal rectus surgery for treatment of distance-near dysjugate ocular deviations. Characteristics of the patients; Methods used; Findings and discussion.

Purpose: To compare contrast sensitivity using a commercially available technique between patients with infantile nystagmus syndrome (INS) and controls. Design: Prospective, comparative case series. Methods: The functional acuity contrast test (FACT) was used binocularly in patients with INS and aged-matched controls with best acuity under normal lighting using a progression of high-quality sine wave grating size changes with increasing spatial frequencies. Results: Nine INS patients aged 5 to 11 years and 25 controls were tested. The INS patients’ best binocular acuity ranged from 20/125 to 20/30, and that of the controls was 20/25 or better. When compared with age-matched controls, INS patients displayed deficiencies in contrast sensitivity syndrome using the FACT test. Conclusions: The FACT or a similar testing system may have potential as a commercially available outcome measure in clinical trials in patients with INS. [Copyright &y& Elsevier]

Purpose: We report a new method of measuring visual function in patients with INS by demonstrating the effects of eccentric gaze and forced time restriction on optotype recognition. Design: Cross-sectional, comparative case-series. Methods: No-time-restricted acuity (NTRA) and time-restricted acuity (TRA) for 19 patients and 18 controls were measured at multiple horizontal gaze angles. Results: INS patients showed a significant lower visual acuity than controls (P < 0.0001). The TRA were significantly decreased in most gaze positions relative to NTRA in INS patients (P = 0.03) while there were no difference among controls. About half of the INS patients showed that their best visual acuity was gaze dependent, matching the preferred head posture in both NRTA and TRA paradigms. Conclusions: Gaze-dependent visual acuity tested with and without time restriction may be a useful measure of visual function in INS patients and could be used in interventional clinical trials. [Copyright &y& Elsevier]

Purpose Methods Results Conclusion To assess the long‐term stability of clinical measures of convergence (near point of convergence [NPC] and positive fusional vergence [PFV]) in participants enrolled in the Convergence Insufficiency Treatment Trial‐Attention and Reading Trial (CITT‐ART) who received 16 weeks of office‐based vergence/accommodative therapy.A total of 310 children, 9–14 years old, with symptomatic convergence insufficiency were enrolled in CITT‐ART. Some 270 completed both their 16‐week primary outcome visit followed by a 1‐year follow‐up visit. Of those 270, 181 (67%) were randomised to the vergence/accommodative therapy. Of the 181 in the vergence/accommodative group, 121 (67%) reported not receiving any additional treatment after the 16‐week primary outcome visit. The mean change in NPC, PFV and percentages of children classified by the predetermined success criteria of convergence (normal NPC [<6 cm] and/or improved by ≥4 cm; normal PFV [passing Sheard's criterion and base‐out break >15Δ] and/or improved by ≥10Δ) were compared at the 16‐week primary outcome visit and 1 year later.Of the 121 who returned for their 1‐year follow‐up visit, there was no significant change in mean adjusted NPC (reduction of −0.2 cm; 95% CI: −1.0 to 0.5 cm) at 1 year. There was a statistically significant decrease in mean‐adjusted PFV (−4.7∆; 95% CI: −6.5 to −2.8Δ) at 1 year. There were similar percentages of participants classified as ‘normal’ (p = 0.30), ‘normal and/or improved’ (p > 0.50) and ‘normal and improved’ (p > 0.14) based on NPC and PFV at the 1‐year visit compared with the 16‐week primary outcome visit.The improvements in NPC and PFV following 16 weeks of vergence/accommodative therapy (with no reported additional treatment thereafter) in children with symptomatic convergence insufficiency persisted 1‐year post‐treatment. [ABSTRACT FROM AUTHOR]

We report a case of bilateral bifid insertion of superior rectus muscles, in a patient with oculocutaneous albinism as an incidental intraoperative finding during eye muscle surgery. The muscle was successfully operated on and the patient did well postoperatively. To our knowledge, this is the first report of this anomaly. [ABSTRACT FROM AUTHOR]

Visual electrodiagnostics and eye movement recording are important additional clinical tools in evaluation, diagnosing and management of ophthalmic and neurological disorders. Due to their objectiveness and non-invasiveness they can play an important role in pediatric ophthalmology. The WSPOS (World Society of Pediatric Ophthalmology and Strabismus) consensus statement gives insight into basic principles and highlights the clinical application of both visual electrodiagnostic tests and eye movement recording. [ABSTRACT FROM AUTHOR]

Purpose: We report a female adult with congenital nystagmus who responded with improved visual function and oculographic parameters after taking the anorexic diet drug Tenuate Dospan (diethylproprionate; Watson Laboratories, Inc., Corona, California).Methods: Observational case report. Clinical ophthalmic examination and ocular motility recordings were performed before and after administration of the drug Tenuate Dospan.Results: The binocular visual acuity of the patient improved from 20/70 to 20/50, her exotropic deviation decreased from 12 to 4 prism diopters, her stereopsis increased from none to 200 seconds/arc, and her ocular motility recordings showed increased foveation periods and a broadened null zone.Conclusion: For unexplained reasons, the anorexic stimulant Tenuate Dospan "paradoxically" improved the nystagmus and binocular function in this patient with congenital nystagmus. This observation may be the basis for investigation of a new pharmacological treatment approach to patients with congenital nystagmus or strabismus. [ABSTRACT FROM AUTHOR]

PURPOSE: We report a female adult with congenital nystagmus who responded with improved visual function and oculographic parameters after taking the anorexic diet drug Tenuate Dospan (diethylproprionate; Watson Laboratories, Inc., Corona, California).METHODS: Observational case report. Clinical ophthalmic examination and ocular motility recordings were performed before and after administration of the drug Tenuate Dospan.RESULTS: The binocular visual acuity of the patient improved from 20/70 to 20/50, her exotropic deviation decreased from 12 to 4 prism diopters, her stereopsis increased from none to 200 seconds/arc, and her ocular motility recordings showed increased foveation periods and a broadened null zone.CONCLUSION: For unexplained reasons, the anorexic stimulant Tenuate Dospan “paradoxically” improved the nystagmus and binocular function in this patient with congenital nystagmus. This observation may be the basis for investigation of a new pharmacological treatment approach to patients with congenital nystagmus or strabismus. [Copyright &y& Elsevier]

Purpose: To survey paediatric eye care providers to identify current patterns of prescribing for hyperopia. Methods: Paediatric eye care providers were invited, via email, to participate in a survey to evaluate current age‐based refractive error prescribing practices. Questions were designed to determine which factors may influence the survey participant's prescribing pattern (e.g., patient's age, magnitude of hyperopia, patient's symptoms, heterophoria and stereopsis) and if the providers were to prescribe, how much hyperopic correction would they prescribe (e.g., full or partial prescription). The response distributions by profession (optometry and ophthalmology) were compared using the Kolmogorov–Smirnov cumulative distribution function test. Results: Responses were submitted by 738 participants regarding how they prescribe for their hyperopic patients. Most providers within each profession considered similar clinical factors when prescribing. The percentages of optometrists and ophthalmologists who reported considering the factor often differed significantly. Factors considered similarly by both optometrists and ophthalmologists were the presence of symptoms (98.0%, p = 0.14), presence of astigmatism and/or anisometropia (97.5%, p = 0.06) and the possibility of teasing (8.3%, p = 0.49). A wide range of prescribing was observed within each profession, with some providers reporting that they would prescribe for low levels of hyperopia while others reported that they would never prescribe. When prescribing for bilateral hyperopia in children with age‐normal visual acuity and no manifest deviation or symptoms, the threshold for prescribing decreased with age for both professions, with ophthalmologists typically prescribing 1.5–2 D less than optometrists. The threshold for prescribing also decreased for both optometrists and ophthalmologists when children had associated clinical factors (e.g., esophoria or reduced near visual function). Optometrists and ophthalmologists most commonly prescribed based on cycloplegic refraction, although optometrists most commonly prescribed based on both the manifest and cycloplegic refraction for children ≥7 years. Conclusion: Prescribing patterns for paediatric hyperopia vary significantly among eye care providers. [ABSTRACT FROM AUTHOR]

Purpose: To report the repeatability of a digital head-posture measuring system when used to record anomalous head postures in children. Design: Prospective study and clinical laboratory investigation. Methods: Using a digital head-posture measuring system, we measured 36 different anomalous head postures in 27 children with infantile nystagmus syndrome. Repeatability values and 95% limits of repeatability of measurements were generated for anomalous head postures. Results: Among the 27 children, 3 had 2 head postures (right and left head turns) in 2 different directions; 6 had 2-dimensional head postures that were considered 2 different head postures; and 18 had a 1-dimensional head posture. There were 5 chin-up or chin-down postures, 23 head-turn postures, and 8 head-tilt postures in a total of 36 anomalous head postures. The repeatability value for all anomalous head postures was less than 10 degrees. Ninety-five percent limits of repeatability yielded ranges of less than 10 degrees for all anomalous head postures. Conclusions: The digital head-posture measuring system is a valid and reliable device for measuring 3-dimensional head postures in children with nystagmus. [ABSTRACT FROM AUTHOR]

Purpose: To report the accuracy and repeatability of a new digital head posture measuring system. Design: Prospective study, clinical laboratory investigation. Methods: The digital head posture measuring system consists of a head-mounted motion tracker, a standard personal computer, and customized software to sample and display 3-dimensional (3D) head posture in real-time. Using a mechanical head posture measuring device as a reference, 3D head positions of an artificial head and 12 human subjects were recorded with the digital head posture device. Accuracy of the digital device outputs, relationship between digital outputs and actual head rotations, and repeatability of the tests were analyzed. Results: The digital head posture device showed consistent outcomes when compared to the mechanical one. The digital outputs of 3D rotations are very close to actual artificial head and human head rotations. The correlation coefficients of the linear relationship between the digital outputs and actual head movements were greater than 0.99. Repeatability tests for the artificial head and human subjects for all 3D rotations had 95% limits of agreement angles less than ±6 degrees and ±8 degrees, respectively. Conclusions: The digital head posture device is an acceptable device with high accuracy, repeatability, and validity in measuring head posture in 3 dimensions. [Copyright &y& Elsevier]

Information about the meeting titled "Understanding Congenital Nystagmus: A Research Workshop" that was held in Oxford, England in September 2005 is presented. The meeting was made possible by the dedication of the England Nystagmus Network. International scientist were interested in understanding and discussing the treatments of one of the most common forms of nystagmus in infancy and childhood.

Oculocutaneous albinism (OCA) is a genetically inherited autosomal recessive condition. Individuals with OCA lack melanin and therefore are susceptible to the harmful effects of solar ultraviolet radiation, including extreme sun sensitivity, photophobia and skin cancer. OCA is a grave public health issue in sub-Saharan Africa with a prevalence as high as 1 in 1000 in some tribes. This article considers the characteristics and prevalence of OCA in sub-Saharan African countries. Sun-induced adverse health effects in the skin and eyes of OCA individuals are reviewed. Sun exposure behavior and the use of photoprotection for the skin and eyes are discussed to highlight the major challenges experienced by these at-risk individuals and how these might be best resolved. [ABSTRACT FROM AUTHOR]

Concussion is a common injury in childhood and has the potential for substantial impact on quality of life. Visual issues have been increasingly recognized as a common problem after concussion. Many children initially seek care for concussion with their pediatrician, making it even more important for pediatricians to recognize, evaluate, and refer children with visual issues after concussion. This clinical report is intended to support the recommendations in the companion policy statement on vision and concussion and provides definitions of some of the physiologic aspects of the visual system as they relate to concussion. A description of clinically feasible testing methodologies is provided in more detail to aid the clinician in assessing the visual system in a focused fashion after concussion. This guidance helps direct clinical management, including support for return to school, sports, and other activities, as well as potential referral for subspecialty care for the subset of those with persistent symptoms. [ABSTRACT FROM AUTHOR]

Visual symptoms are common after concussion in children and adolescents, making it essential for clinicians to understand how to screen, identify, and initiate clinical management of visual symptoms in pediatric patients after this common childhood injury. Although most children and adolescents with visual symptoms after concussion will recover on their own by 4 weeks, for a subset who do not have spontaneous recovery, referral to a specialist with experience in comprehensive concussion management (eg, sports medicine, neurology, neuropsychology, physiatry, ophthalmology, otorhinolaryngology) for additional assessment and treatment may be necessary. A vision-specific history and a thorough visual system examination are warranted, including an assessment of visual acuity, ocular alignment in all positions of gaze, smooth pursuit (visual tracking of a moving object), saccades (visual fixation shifting between stationary targets), vestibulo-ocular reflex (maintaining image focus during movement), near point of convergence (focusing with both eyes at near and accommodation (focusing with one eye at near because any of these functions may be disturbed after concussion. These deficits may contribute to difficulty with returning to both play and the learning setting at school, making the identification of these problems early after injury important for the clinician to provide relevant learning accommodations, such as larger font, preprinted notes, and temporary use of audio books. Early identification and appropriate management of visual symptoms, such as convergence insufficiency or accommodative insufficiency, may mitigate the negative effects of concussion on children and adolescents and their quality of life. [ABSTRACT FROM AUTHOR]

To characterize the genotypic and phenotypic spectrum of foveal hypoplasia (FH). Multicenter, observational study. A total of 907 patients with a confirmed molecular diagnosis of albinism, PAX6 , SLC38A8 , FRMD7, AHR, or achromatopsia from 12 centers in 9 countries (n = 523) or extracted from publicly available datasets from previously reported literature (n = 384). Individuals with a confirmed molecular diagnosis and availability of foveal OCT scans were identified from 12 centers or from the literature between January 2011 and March 2021. A genetic diagnosis was confirmed by sequence analysis. Grading of FH was derived from OCT scans. Grade of FH, presence or absence of photoreceptor specialization (PRS+ vs. PRS–), molecular diagnosis, and visual acuity (VA). The most common genetic etiology for typical FH in our cohort was albinism (67.5%), followed by PAX6 (21.8%), SLC38A8 (6.8%), and FRMD7 (3.5%) variants. AHR variants were rare (0.4%). Atypical FH was seen in 67.4% of achromatopsia cases. Atypical FH in achromatopsia had significantly worse VA than typical FH (P < 0.0001). There was a significant difference in the spectrum of FH grades based on the molecular diagnosis (chi-square = 60.4, P < 0.0001). All SLC38A8 cases were PRS– (P = 0.003), whereas all FRMD7 cases were PRS+ (P < 0.0001). Analysis of albinism subtypes revealed a significant difference in the grade of FH (chi-square = 31.4, P < 0.0001) and VA (P = 0.0003) between oculocutaneous albinism (OCA) compared with ocular albinism (OA) and Hermansky–Pudlak syndrome (HPS). Ocular albinism and HPS demonstrated higher grades of FH and worse VA than OCA. There was a significant difference (P < 0.0001) in VA between FRMD7 variants compared with other diagnoses associated with FH. We characterized the phenotypic and genotypic spectrum of FH. Atypical FH is associated with a worse prognosis than all other forms of FH. In typical FH, our data suggest that arrested retinal development occurs earlier in SLC38A8, OA , HPS , and AHR variants and later in FRMD7 variants. The defined time period of foveal developmental arrest for OCA and PAX6 variants seems to demonstrate more variability. Our findings provide mechanistic insight into disorders associated with FH and have significant prognostic and diagnostic value. [ABSTRACT FROM AUTHOR]

Purpose: To determine the effectiveness of office‐based vergence/accommodative therapy for improving accommodative amplitude and accommodative facility in children with symptomatic convergence insufficiency and accommodative dysfunction. Methods: We report changes in accommodative function following therapy among participants in the Convergence Insufficiency Treatment Trial – Attention and Reading Trial with decreased accommodative amplitude (115 participants in vergence/accommodative therapy; 65 in placebo therapy) or decreased accommodative facility (71 participants in vergence/accommodative therapy; 37 in placebo therapy) at baseline. The primary analysis compared mean change in amplitude and facility between the vergence/accommodative and placebo therapy groups using analyses of variance models after 4, 8, 12 and 16 weeks of treatment. The proportions of participants with normal amplitude and facility at each time point were calculated. The average rate of change in amplitude and facility from baseline to week 4, and from weeks 4 to 16, were determined in the vergence/accommodative therapy group. Results: From baseline to 16 weeks, the mean improvement in amplitude was 8.6 dioptres (D) and 5.2 D in the vergence/accommodative and placebo therapy groups, respectively (mean difference = 3.5 D, 95% confidence interval (CI): 1.5 to 5.5 D; p = 0.01). The mean improvement in facility was 13.5 cycles per minute (cpm) and 7.6 cpm in the vergence/accommodative and placebo therapy groups, respectively (mean difference = 5.8 cpm, 95% CI: 3.8 to 7.9 cpm; p < 0.0001). Significantly greater proportions of participants treated with vergence/accommodative therapy achieved a normal amplitude (69% vs. 32%, difference = 37%, 95% CI: 22 to 51%; p < 0.0001) and facility (85% vs. 49%, difference = 36%, 95% CI: 18 to 55%; p < 0.0001) than those who received placebo therapy. In the vergence/accommodative therapy group, amplitude increased at an average rate of 1.5 D per week during the first 4 weeks (p < 0.0001), then slowed to 0.2 D per week (p = 0.002) from weeks 4 to 16. Similarly, facility increased at an average rate of 1.5 cpm per week during the first 4 weeks (p < 0.0001), then slowed to 0.6 cpm per week from weeks 4 to 16 (p < 0.0001). Conclusion: Office‐based vergence/accommodative therapy is effective for improving accommodative function in children with symptomatic convergence insufficiency and coexisting accommodative dysfunction. [ABSTRACT FROM AUTHOR]

: PurposeTo report a case of inadvertent intraocular injection of botulinum toxin A (Botox, BTA) resulting in a retinal tear and bullous retinal detachment. The retinal detachment resolved spontaneously, and the tear was treated with laser demarcation with good visual outcome.: DesignInterventional case report.: MethodsA 36-year-old woman underwent Botox injections for paralytic esotropia and developed ocular perforation. Dilated fundus examination showed a slit-like retinal tear infranasally with a bullous retinal detachment nasally.: ResultsThe retinal detachment spontaneously resolved, and the patient was treated with laser demarcation on the following day. After 2 more days, her vision returned to baseline.: ConclusionsBotox did not appear toxic to human intraocular tissues. Close observation may be indicated in the event of an inadvertent intraocular Botox injection. [Copyright &y& Elsevier]

Periodic alternating nystagmus consists of involuntary oscillations of the eyes with cyclical changes of nystagmus direction. It can occur during infancy (e.g. idiopathic infantile periodic alternating nystagmus) or later in life. Acquired forms are often associated with cerebellar dysfunction arising due to instability of the optokinetic-vestibular systems. Idiopathic infantile periodic alternating nystagmus can be familial or occur in isolation; however, very little is known about the clinical characteristics, genetic aetiology and neural substrates involved. Five loci (NYS1-5) have been identified for idiopathic infantile nystagmus; three are autosomal (NYS2, NYS3 and NYS4) and two are X-chromosomal (NYS1 and NYS5). We previously identified the FRMD7 gene on chromosome Xq26 (NYS1 locus); mutations of FRMD7 are causative of idiopathic infantile nystagmus influencing neuronal outgrowth and development. It is unclear whether the periodic alternating nystagmus phenotype is linked to NYS1, NYS5 (Xp11.4-p11.3) or a separate locus. From a cohort of 31 X-linked families and 14 singletons (70 patients) with idiopathic infantile nystagmus we identified 10 families and one singleton (21 patients) with periodic alternating nystagmus of which we describe clinical phenotype, genetic aetiology and neural substrates involved. Periodic alternating nystagmus was not detected clinically but only on eye movement recordings. The cycle duration varied from 90 to 280 s. Optokinetic reflex was not detectable horizontally. Mutations of the FRMD7 gene were found in all 10 families and the singleton (including three novel mutations). Periodic alternating nystagmus was predominantly associated with missense mutations within the FERM domain. There was significant sibship clustering of the phenotype although in some families not all affected members had periodic alternating nystagmus. In situ hybridization studies during mid-late human embryonic stages in normal tissue showed restricted FRMD7 expression in neuronal tissue with strong hybridization signals within the afferent arms of the vestibulo-ocular reflex consisting of the otic vesicle, cranial nerve VIII and vestibular ganglia. Similarly within the afferent arm of the optokinetic reflex we showed expression in the developing neural retina and ventricular zone of the optic stalk. Strong FRMD7 expression was seen in rhombomeres 1 to 4, which give rise to the cerebellum and the common integrator site for both these reflexes (vestibular nuclei). Based on the expression and phenotypic data, we hypothesize that periodic alternating nystagmus arises from instability of the optokinetic-vestibular systems. This study shows for the first time that mutations in FRMD7 can cause idiopathic infantile periodic alternating nystagmus and may affect neuronal circuits that have been implicated in acquired forms. [ABSTRACT FROM AUTHOR]

Leber's congenital amaurosis (LCA) is a group of inherited blinding diseases with onset during childhood. One form of the disease, LCA2, is caused by mutations in the retinal pigment epithelium–specific 65-kDa protein gene (RPE65). We investigated the safety of subretinal delivery of a recombinant adeno-associated virus (AAV) carrying RPE65 complementary DNA (cDNA) (ClinicalTrials.gov number, NCT00516477). Three patients with LCA2 had an acceptable local and systemic adverse-event profile after delivery of AAV2.hRPE65v2. Each patient had a modest improvement in measures of retinal function on subjective tests of visual acuity. In one patient, an asymptomatic macular hole developed, and although the occurrence was considered to be an adverse event, the patient had some return of retinal function. Although the follow-up was very short and normal vision was not achieved, this study provides the basis for further gene therapy studies in patients with LCA. N Engl J Med 2008;358:2240-8. [ABSTRACT FROM AUTHOR]

Abstract: We displayed backward/forward motion on a computer monitor in the ground plane. Subjects looked at the center of a moving pattern and eye movements of both eyes were recorded with a search coil system. Involuntary nystagmus including vertical version (VV) and horizontal vergence (HV) was recorded. Dynamics of the nystagmus showed that the slow and quick phase of VV and HV were always associated with each other while the monocular horizontal eye movements composed of HV were either symmetrical or asymmetrical. Peak velocity, amplitudes and frequency of the VV and HV responses were quantified. The results suggest that involuntary HV nystagmus can be induced by simple motion from simulated optic flow in the ground plane and the HV nystagmus helps moving subjects to stabilize their gazes on the object of interest. [Copyright &y& Elsevier]

: PurposeTo assess the test-retest reliability of the electronic Early Treatment Diabetic Retinopathy Study (E-ETDRS) visual acuity algorithm using the computerized Electronic Visual Acuity (EVA) tester in children 7 to <13 years old.: DesignTest-retest reliability study.: MethodsThis multicenter study involved 245 subjects at four clinical sites. As the main outcome measure, visual acuity was measured twice using the E-ETDRS testing protocol on the EVA system, which uses a programmed handheld device to communicate with a personal computer and a 17-inch monitor at a 3-m test distance.: ResultsTest–retest reliability was high (r = .94 for right eyes and 0.96 for left eyes) and for both right and left eyes, 89% of retest scores were within 0.1 logarithm of the minimal angle of resolution (logMAR) (five letters) of the initial test score and 99% of retests were within 0.2 logMAR (10 letters). Reliability was high across the age range of 7 to <13 years. Based on 95% confidence level estimates, a change in visual acuity of 0.2 logMAR (10 letters) from a previous acuity measure is unlikely to result from measurement variability.: ConclusionsThe E-ETDRS protocol using the EVA has high test–retest reliability in children 7 to <13 years of age. Potential advantages include better standardization across multiple sites, the ability to directly capture data electronically with an automatic acuity score calculation, the reduction of potential bias by limiting the tester’s role, and the requirement of only a single testing distance for measurements from 20/800 to 20/12. This computerized testing method should be considered when visual acuity is used as an outcome measure in eye research involving children 7 to <13 years old. [Copyright &y& Elsevier]