Correction: Management of nystagmus in children: a review of the literature and current practice in UK specialist services.

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Management of nystagmus in children: a review of the literature and current practice in UK specialist services.

Nystagmus is an eye movement disorder characterised by abnormal, involuntary rhythmic oscillations of one or both eyes, initiated by a slow phase. It is not uncommon in the UK and regularly seen in paediatric ophthalmology and adult general/strabismus clinics. In some cases, it occurs in isolation, and in others, it occurs as part of a multisystem disorder, severe visual impairment or neurological disorder. Similarly, in some cases, visual acuity can be normal and in others can be severely degraded. Furthermore, the impact on vision goes well beyond static acuity alone, is rarely measured and may vary on a minute-to-minute, day-to-day or month-to-month basis. For these reasons, management of children with nystagmus in the UK is varied, and patients report hugely different experiences and investigations. In this review, we hope to shine a light on the current management of children with nystagmus across five specialist centres in the UK in order to present, for the first time, a consensus on investigation and clinical management.

An audit of the outcome of amblyopia treatment: a retrospective analysis of 322 children.

BACKGROUND/AIMS: Little is known about the effectiveness of occlusion therapy in hospital settings. A retrospective analysis was conducted to assess modalities, outcome and hospital costs of children treated for amblyopia with patching in a UK clinic. METHODS: Notes of 322 children with amblyopia discharged after occlusion treatment were selected consecutively and reviewed. Data collated included age at presentation, amblyopia type, visual acuity (VA; before/after occlusion and at discharge), number of prescribed hours of occlusion, duration of patching treatment, number of glasses prescribed and number of visits attended or failed to attend. Hospital treatment costs were estimated. RESULTS: Mixed amblyopes were prescribed the longest amount of patching (mean 2815 h over 23 months) followed by strabismic (1984 h) and anisometropic (1238 h) amblyopes. 319 amblyopes received glasses and five atropine treatment. The percentage of patients reaching VA of 6/12 was best in the anisometropic and strabismic groups (>75%) and worse in mixed amblyopia (64%). Average hospital costs were estimated at pound1365. CONCLUSION: Although the mean duration of treatment was long, involving many hospital visits, the visual outcome was variable, unsatisfactory (<6/9) and more expensive than necessary. As compliance has been identified as a major problem methods to improve amblyopia treatment are needed, possibly by using educational/motivational intervention.

Eye movement involvement in Parry-Romberg Syndrome: a clinicopathologic case report.

We report the case of a 38-year-old woman who developed a progressive bilateral disease in which the eye motility disorder-diplopia-is the outstanding feature over a period of 12 years. The muscle biopsy of the medial rectus muscle did not show any trace of striated muscle. To the best of our knowledge, this is the first pathological report in an affected extraocular muscle of a patient with Parry-Romberg syndrome (PRS). Previous rare reports of diplopia in PRS have been attributed to enophthalmos, progressive atrophy of the orbit, ocular motor nerve dysfunction, or mechanical restrictions.

Reduction of congenital nystagmus in a patient after smoking cannabis.

INTRODUCTION: Smoking cannabis has been described to reduce acquired pendular nystagmus in MS, but its effect on congenital nystagmus is not known. PURPOSE: To report the effect of smoking cannabis in a case of congenital nystagmus. METHODS: A 19-year-old male with congenital horizontal nystagmus presented to the clinic after smoking 10 mg of cannabis. He claimed that the main reason for smoking cannabis was to improve his vision. At the next clinic appointment, he had not smoked cannabis for 3weeks. Full ophthalmologic examination and eye movement recordings were performed at each visit. RESULTS: Visual acuity improved by 3 logMar lines in the left eye and by 2 logMar lines in the right eye after smoking cannabis. The nystagmus intensities were reduced by 30% in primary position and 44%, 11%, 10% and 40% at 20-degree eccentricity to the right, left, elevation and depression, respectively, after smoking cannabis. CONCLUSION: Cannabis may be beneficial in the treatment of congenital idiopathic nystagmus (CIN). Further research to clarify the safety and efficacy of cannabis in patients with CIN, administered for example by capsules or spray, would be important.

Nystagmus characteristics in congenital stationary night blindness (CSNB).

AIM: To analyse nystagmus characteristics in patients with congenital stationary night blindness (CSNB) for differentiation from other forms of early childhood nystagmus. METHODS: Horizontal and vertical eye movements of 10 patients (6-46 years, mean 17.1 years, median 12.5 years) with CSNB (eight with CSNB1, two with CSNB2) were recorded with the scleral magnetic search coil technique or by electro-oculography. Nystagmus characteristics such as the amplitude, frequency, conjugacy and intermittency were analysed. RESULTS: All patients had continuous, pendular, oblique and mostly dysconjugate nystagmus of high frequency and low amplitude. In seven cases, a large horizontal or vertical jerk nystagmus with increasing, decreasing or constant velocity was superimposed. Jerk nystagmus was mostly intermittent and conjugate. Head nodding was found not to be compensatory. CONCLUSIONS: Eye-movement recordings of CSNB patients disclosed specific nystagmus characteristics, such as an oblique direction, superimposed waveforms and dysconjugate eye movements. These features may help to distinct nystagmus in CSNB from other forms of early infancy nystagmus, such as congenital idiopathic nystagmus, latent nystagmus and spasmus nutans. We found nystagmus in CSNB to be similar to the nystagmus reported in blue-cone monochromatism and rod monochromatism, and in patients with a severe sensory defect. The nystagmus characteristics described should prompt electroretinographic investigation in cases of uncertain diagnosis.

Survey of management of acquired nystagmus in the United Kingdom.

PURPOSE: To determine the current management of acquired nystagmus by ophthalmologists and neurologists. METHODS: Questionnaires were sent to ophthalmologists (850) and neurologists (434) in the United Kingdom. Estimated numbers of patients seen with acquired nystagmus, treatment options used, and the results of treatment of the patients were collected. RESULTS: Response rate was 37% for ophthalmologists and 34% for neurologists. The most common causes of acquired nystagmus were estimated to be multiple sclerosis and stroke. 58% of ophthalmologists and 94.5% of neurologists reported seeing patients with nystagmus. The most commonly used medical treatment was gabapentin and baclofen. Other drugs used were clonazepam, carbamazepine, benzhexol, ondansetrone, buspirone, memantine, and botulinum toxin (n=3). Eleven ophthalmologists and 52 neurologists noted symptomatic improvement with medical treatment. Eleven ophthalmologists and 44 neurologists noted improvement in visual acuity (VA). Occurrence of side effects noted with baclofen and gabapentin treatments were similar. CONCLUSION: A variety of drugs are used to treat acquired nystagmus in the UK. Baclofen and gabapentin are the drugs most commonly used and are reported to cause significant improvement in symptoms and VA. Better knowledge of the action of drugs in nystagmus is needed to establish guidelines and to give patients wider access to treatment.

The effects of gabapentin and memantine in acquired and congenital nystagmus: a retrospective study.

BACKGROUND: Pharmacological treatment has been successful in some forms of acquired neurological nystagmus. However, drugs are not known to be effective in idiopathic infantile nystagmus or nystagmus associated with ocular diseases. METHODS: The authors retrospectively analysed Snellen visual acuity (VA), subjective visual function, and eye movement recordings of 23 patients with nystagmus (13 secondary to multiple sclerosis, three associated with other neurological diseases, two idiopathic infantile, and five with associated ocular diseases) treated with gabapentin or memantine. RESULTS: With gabapentin, 10 of 13 patients with nystagmus secondary to multiple sclerosis (MS) showed some improvement. Memantine improved the VA in all three patients with MS who did not improve on gabapentin. There was no change of nystagmus in other neurological disorders. Patients with congenital nystagmus showed reduction of nystagmus and their VA changes depended on the ocular pathology. CONCLUSION: Gabapentin and memantine may be effective in acquired nystagmus secondary to MS. To the authors' knowledge this is the first series of patients showing that gabapentin is effective in improving nystagmus in congenital nystagmus/nystagmus associated with ocular pathology. Memantine may be useful as an alternative drug in treating patients with nystagmus.

Why is compliance with occlusion therapy for amblyopia so hard? A qualitative study.

OBJECTIVE: To explore parents' perceptions and experiences of occlusion (patching) therapy for treatment of amblyopia in children. METHODS: Qualitative study involving semistructured interviews with 25 families of a child with amblyopia being treated at a specialist clinic. Interviews were tape recorded and transcribed verbatim. Data analysis was based on the constant comparative method, assisted by qualitative analysis software. RESULTS: Parents of children prescribed patching treatment found themselves obliged to manage the treatment. This involved dilemmas and tensions, with many parents describing children's distress, particularly in the early stages of patching treatment. Parents were highly sensitive to the credibility of the treatment, but were sometimes confused by information given in the clinic or did not see clinic staff as authoritative. There was evidence that parents were likely to abandon or modify treatment if no improvement could be detected or if the child continued to suffer socially or educationally. Parents described a range of strategies for facilitating patching, including explanation; normalisation; rewards; customising the patch; establishing a routine; and enlisting the help of others. Whatever their practices in relation to patching, parents were keen to defend their behaviour as that of a "good parent". CONCLUSIONS: Interventions that aim to improve compliance should take account of the difficulties and tensions experienced by parents, rather than simply treating non-compliance as resulting from information deficits. Practical support that builds on strategies described by parents is likely to be of benefit.

Social and visual function in nystagmus.

AIM: To investigate the influence of nystagmus on visual and social function and determine if parents are able to assess visual and social function in children with nystagmus. METHOD: A postal questionnaire comprising 14 questions related to visual function (VF-14) and questions pertaining to social function were sent to all 1013 members of the Nystagmus Network-a UK based organisation for nystagmus sufferers and their families. Visual and social function scores were compared by regression analysis. RESULTS: 180 adult, 233 parent, and 124 child questionnaires were returned. Idiopathic nystagmus was the most common cause. In adults the mean VF-14 score indicated very low visual function, in the same range as patients assessed in low vision services. Children's visual function scored better than adults, between scores of patients with age related macular disease and corneal grafts. There was a strong correlation between perceived visual and social function for adults (p<0.001) and parental assessment of their children (p<0.001), but not between child self assessment of visual and social function. There was strong correlation between parental and child assessment of visual and social function (p<0.001, p<0.001) CONCLUSION: Questionnaires indicated that nystagmus is associated with very low visual function. There is a strong correlation between visual and social impairment. The authors have shown for the first time in an ophthalmic disease that parents are able to estimate the impact of nystagmus on their child both in terms of visual and social functioning, although they underestimate the impact of nystagmus on emotional aspects of wellbeing.

Asymmetry of the optokinetic nystagmus in lesion of the central nervous system.

BACKGROUND: Unilateral lesions of the central nervous system (CNS) may be associated with a reduction of the optokinetic nystagmus (OKN) slow component in the direction of the lesion. The aim of our study was to assess the role played in these cases by the direct injury of the OKN pathways and/or by a possible associated visual field defect. METHODS AND PATIENTS: Monocular OKN was elicited with black and white stripes moving temporally-to-nasally (TN) or nasally-to-temporally (NT) at velocities of 15, 30, 45 and 60 degrees /s. Patients with cortical or chiasmal lesions associated with visual field defects were investigated. OKN was considered asymmetrical if the gain difference between TN and NT stimulation was not within 2 standard deviations of an age-matched control group (n = 86). RESULTS: We examined 12 patients with cortical lesions and 4 patients with chiasmal lesions. Asymmetric OKN gain was measured in 7 patients with cortical lesions associated with a visual field defect, and in 2 patients with chiasmal compression and bitemporal hemianopia. In 2 patients with isolated occipital lesions, OKN asymmetry was explained by the associated visual field defect. CONCLUSION: The interpretation of OKN asymmetry in patients with CNS lesions should not only consider a direct lesion of the OKN pathways but also a sensory deficit due to a visual field defect.

Look and stare optokinetic nystagmus in healthy subjects and in patients with no measurable binocularity. A prospective study.

BACKGROUND: The aim of our study was to assess the difference between look and stare optokinetic nystagmus (OKN) in healthy subjects and in patients with early onset strabismus and no measurable binocularity. METHODS AND PATIENTS: OKN was elicited monocularly with black and white stripes moving horizontally or vertically at 15, 30, 45 and 60 degrees /s. Subjects were instructed either to follow individual stripes across the screen (look OKN) or to fixate stripes in the centre of the screen (stare OKN). We examined 20 healthy subjects (mean age 29 years; range 21 - 39), and 10 patients with no measurable binocularity (mean age 12.7 years; range 5 - 37). OKN gain was measured with photo-oculography. RESULTS: In both groups and at stimulation with the higher velocities gains for look OKN were significantly better than for stare OKN, and gains with horizontal stimuli were significantly better than with vertical stimuli (p < 0.05). In the group with no measurable binocularity horizontal look and stare OKN answers were significantly better for temporal-to-nasal stimulation than for nasal-to-temporal stimulation (p < 0.05). CONCLUSIONS: The performance of look or stare OKN influences the gain in healthy subjects and in patients with no measurable binocularity. Data about both modes of OKN stimulation are helpful to better interpret OKN responses especially in subjects with poor cooperation.

Age related change of optokinetic nystagmus in healthy subjects: a study from infancy to senescence.

BACKGROUND: Optokinetic nystagmus (OKN) gain is asymmetrical between temporal to nasal (TN) and nasal to temporal (NT) stimulation in infancy and decreases at older ages. The age at which OKN gain becomes symmetrical and decreases is debated. The aim was to investigate OKN over the whole lifespan in a large sample of healthy subjects. METHODS: In a prospective, cross sectional study OKN was tested monocularly using TN and NT small field stimulation. Stimulation velocity was 15 degrees /s and 30 degrees /s for children aged under 1 year (n = 97), and 15 degrees /s, 30 degrees /s, 45 degrees /s, and 60 degrees /s for older subjects (1-9 years, n = 66; 10-89 years, n = 86). Gain was measured using infrared oculography. RESULTS: Significant OKN gain asymmetry in favour of TN versus NT stimulation was found during the first 5 months of life (p<0.05). Only at 11 months of age was OKN symmetrical in 100% of the subjects. The percentage of children with symmetrical OKN decreased with increasing stimulus velocity. OKN gain increased in the second and third years (p<0.05 for 15 degrees /s), remained stable until 50 years of age, and showed a small but significant decrease afterwards for the tested velocities (between 6% and 18%, p<0.05). CONCLUSIONS: Infrared oculography is an accurate method to assess OKN, especially in children. Knowledge about change of OKN in healthy subjects could be helpful to interpret OKN in patients with abnormal binocular vision or lesions of the central nervous system.

Serum uric acid levels in optic neuritis.

Uric acid, an antioxidant, is reduced in multiple sclerosis (MS). Patients with gout have a reduced incidence of MS. Optic neuritis (ON), often the first manifestation of MS, is not known to be associated with reduced uric acid. Patients with recent onset of ON were investigated to determine whether uric acid levels were reduced at presentation. Twenty-one patients with ON were included, 17 females and 4 males. The mean (SD) serum uric acid in the ON female group was 184.4 ( +/-55.1) micromol/L (range, 116-309 micromol/L), whilst in the control group it was 235.2 (+/- 50.2) micromol/L (range, 172-381 micromol/L). The difference was statistically significant (chi2 = 8.93, P = 0.003). In the small male cohort, mean (SD) serum uric acid was 305 (+/- 52.1) micromol/L, whilst in the control group it was 328 (+/- 80.4) micromol/L. These differences were not statistically significant. Reduced antioxidant reserve is possibly an early pathogenic mechanism in inflammatory demyelination, and raises the possibility that low uric acid levels could be an indicator of disease activity. Since optic neuropathies of other causes were not investigated, future research needs to determine whether low uric acid represents a unique feature of optic neuritis or is seen in other optic neuropathies.

Role of the stimulus size in the generation of optokinetic nystagmus in normals and in patients with retinitis pigmentosa.

BACKGROUND: To find out the smallest stimulus size still able to elicit optokinetic nystagmus (OKN). To discuss the differences in the generation of OKN between normals and patients with tunnel vision. METHODS AND PATIENTS: OKN was elicited monocularly with black and white stripes of 2 degrees moving on a screen temporally-to-nasally or nasally-to-temporally at velocities of 15, 30, 45, and 60 degrees /s. In healthy subjects (n = 10) the size of the stimulus was gradually increased from minimal 8 degrees x 0.5 degrees to maximal 48 degrees x 10 degrees. OKN was elicited in retinitis pigmentosa (RP) patients (n = 2) with visual field reduced to the central 10 degrees. OKN gain was measured using infrared oculography. OKN response was considered as normal if it was within 2 standard deviations of the mean OKN of age-matched control groups (n = 29). RESULTS: In healthy subjects the size of the stimulus necessary to elicit normal OKN gain was smaller at low velocity of 15 degrees /s (16 degrees x 1 degrees ) than at higher velocities of 30, 45, and 60 degrees /s (16 degrees - 24 degrees x 3 degrees ). Normal OKN gain was measured in the RP patients only at the low velocity of 15 degrees /s. CONCLUSIONS: Small stimuli are sufficient in normals to elicit good OKN answers. Therefore, poor OKN gain in RP patients may not be explained by their tunnel vision only. We postulate that in these patients the reduction of gain is due to a multifactorial decrease of their visual perception related to disorders of the visual field, the visual acuity, and the contrast sensitivity.

Torsional optokinetic nystagmus: normal response characteristics.

BACKGROUND/AIMS: Few studies have investigated normal response characteristics of torsional optokinetic nystagmus (tOKN). The authors have investigated the effect of stimulus velocity and central/peripheral stimulation on tOKN. METHODS: Torsional OKN was elicited using a sinusoidal grating rotating at velocities of 3 degrees /s to 1000 degrees /s in clockwise and anticlockwise directions. To investigate the effect of central stimulation, stimulus size was varied from 2.86 degrees to 50.8 degrees. An artificial scotoma placed over a 50.8 degrees stimulus was varied from 2.86 degrees to 43.2 degrees to investigate peripheral stimulation. Eight subjects participated in each experiment and torsional eye movements were recorded using video-oculography. The mean slow phase velocity (MSPV) and gain were calculated. RESULTS: The maximum gain occurred in response to 8 degrees /s stimulation. The MSPV increased up to a stimulus velocity of 200 degrees /s achieving a maximum of 3 degrees /s in both directions. MSPV was linearly correlated with the log of stimulus velocity. The smallest field size, rotating at 40 degrees /s, evoked 10% of the gain elicited by the largest display. When the most peripheral stimulus was used, the gain was maintained at 50% of the gain evoked when the full display was used. CONCLUSIONS: A wide range of stimulus velocities can elicit tOKN and peripheral field stimulation contributes significantly to its response.

Differences in the management of amblyopia between European countries.

BACKGROUND: Amblyopia treatment is not standardised and differences between centres and countries have not been systematically investigated. This survey compares the different patterns of orthoptic treatment of amblyopia in the United Kingdom (UK) and three German speaking countries (GSC). METHODS: Questionnaires were sent to orthoptists in the UK and the GSC asking for their preferred choices of treatment of amblyopia between the ages of 6 months to 10 years. RESULTS: The following significant differences in management of amblyopia were found: (1) the number of hours of occlusion per week was higher in the GSC, p<0.0001, (2) orthoptists in the GSC treat amblyopia up to an older age. Orthoptists in the GSC and in the UK predicted similar treatment outcomes. CONCLUSION: Orthoptists in the GSC usually treat patients more intensively and for longer, while the prediction of visual outcome does not differ significantly between countries. These results highlight the lack of standardisation in the treatment of the various types of amblyopia.