Increased functional connectivity coupling with supplementary motor area in blepharospasm at rest.

OBJECTIVE: To explore the abnormalities of brain function in blepharospasm (BSP) and to illustrate its neural mechanisms by assuming supplementary motor area (SMA) as the entry point. METHODS: Twenty-five patients with BSP and 23 controls underwent resting-state functional MRI, seed-based functional connectivity (FC), correlation analysis, receiver operating characteristic curve (ROC) analysis, and support vector machine (SVM) were applied to process the data. RESULTS: Patients showed that the left medial prefrontal cortex (MPFC), left lingual gyrus, right cerebellar crus I, and right lingual gyrus/cerebellar crus I had enhanced FC with the left SMA, whereas the right inferior temporal gyrus (ITG) had enhanced FC with the right SMA relative to controls. The FC between the left MPFC and left SMA was positively correlated with symptomatic severity. The ROC analysis verified that the abnormal FCs demonstrated in this study can separate patients and controls at high sensitivity and specificity. SVM analysis exhibited that combined FCs of the left SMA were optimal for distinguishing patients and control group at the accuracy of 89.58%, with sensitivity of 92.00% and specificity of 86.96%. CONCLUSIONS: Several brain networks partake in the neurobiology of BSP. SMA plays a vital role in several brain networks and might be the key pathogenic factor in BSP. SIGNIFICANCE: Providing novel evidence for the engagement of the MPFC in the motor symptoms of BSP, enhancing credibility of the thesis that SMA regulates the neurobiology of BSP, and providing ideas of screening susceptible population of BSP using neuroimaging.

Sex related differences in nonmotor symptoms of patients with idiopathic blepharospasm.

Idiopathic blepharospasm shows a female predominance in prevalence, whether there are sex-related differences in distributions of nonmotor symptoms (NMSs) and predictors of quality of life are unknown. Four hundred and twenty-five patients with idiopathic blepharospasm were consecutively recruited, and underwent assessments including dystonia severity, mood disturbances, sleep disturbances, cognition, ocular symptoms, and quality of life. Frequencies and distributions of NMSs, and predictors of quality of life in female and male patients were investigated. NMSs existed in majority of male (94.0%) and female (95.8%) patients. The frequencies of depression, cognition dysfunction, and poor sleep quality were higher in female patients, while the frequency of excessive daytime sleepiness was higher in male patients. More female (79.5%) patients had multiple NMS domains affected than male (70.1%) patients (p = 0.040). Quality of life was associated with depression, anxiety and motor severity for female patients (adjusted R2 = 0.367, p < 0.001), while associated with depression, excessive daytime sleepiness and motor severity for male patients (adjusted R2 = 0.430, p < 0.001). The highly prevalent coexistence of multiple NMSs found in patients with blepharospasm support that blepharospasm is a network disorder. The sex-related differences in the pattern of NMSs and predictors of quality of life may aid the development of tailored management of blepharospasm.

DNAJC6 mutation causing cranial-onset dystonia with tremor dominant levodopa non-responsive parkinsonism: A novel phenotype.

DNAJC6 mutation causes two types of phenotypes: slowly progressive parkinsonism with levodopa response and rapidly progressive parkinsonism with additional manifestations like intellectual disability, epilepsy etc. We report a new phenotype wherein an adolescent girl developed blepharospasm followed by jaw opening, lingual and cervical dystonia followed by tremors of limbs (rest and action) with rigidity, bradykinesia. The dystonia-parkinsonism phenotype has not been described. She had novel homozygous missense mutation in DNAJC6 gene.

Long-term stable efficacy of botulinum toxin A in facial movement disorders with no need for increasing dose.

ABSTRACT: Botulinum toxin A is considered an effective treatment for involuntary facial movements. We examined whether treatment efficacy maintained or changed over time with two products, Botox and Dysport, in patients with hemifacial spasm, facial synkinesis and benign essential blepharospasm.We retrospectively investigated 87 consecutive patients (51 women, 36 men) who had undergone treatment for ≥6 years. Long-term effects, as well as side effects of Botox or Dysport local injections were evaluated. The first three treatments were considered the titration period and not taken into account when testing for dose changes.Mean treatment duration was 10 years (range 6-11, SD 1.0), 2441 treatments were administered, 1162 with Botox and 1279 with Dysport, the two brands were interchanged as needed. Good to full improvement was seen in 90% of patients both with both brands. Injection doses and treatment responses were consistent during the study with both drugs. No major side effects were reported, and relatively few minor adverse events were reported, with clear reduction from the titration period (6.1%), to the remainder of the study (3.9%).Botulinum toxin (BTX-A) is a satisfactory long-term treatment without need for dose increase over. Both Botox and Dysport were effective when used interchangeably.

A novel FBXO7-R345P mutation in a Chinese family with autosomal recessive parkinsonian-pyramidal syndrome.

BACKGROUND: Mutations in the F-box protein 7 (FBXO7) gene is one of the genetic causes of early-onset Parkinson's disease, which usually presents as autosomal recessive early-onset parkinsonian-pyramidal syndrome (PPS). Herein, we report a Chinese PPS family with a novel FBXO7 homozygous mutation. METHODS: Clinical data of the proband and his affected sister manifesting as early-onset parkinsonism combined with pyramidal signs were collected. DNAs of the two affected siblings, an unaffected sibling and their unaffected mother were isolated. Whole-exome sequencing (WES) was performed for the proband. After bioinformatic analysis, targeted variants were validated by Sanger sequencing in the family members available for DNAs. RESULTS: The proband began to walk unsteadily at 30-year-old and developed mild parkinsonism and stiffness in both lower extremities 4 years later. His older sister also manifested as early-onset parkinsonism with stiffness in both lower limbs and postural instability. Both the proband and his older sister carried a novel homozygous FBXO7 mutation in exon 7 (c.1034G > C, p. R345P). The homozygous mutation co-segregated with disease in this pedigree. The mutation located at a highly conserved amino acid residue in the F-box domain, which was predicted to be damaging in silico. CONCLUSIONS: Our study expands the mutational spectrum of autosomal recessive early-onset Parkinson's disease (PARK15) caused by FBXO7 mutations.

Voxel-Wise Brain-Wide Functional Connectivity Abnormalities in Patients with Primary Blepharospasm at Rest.

Background: Primary blepharospasm (BSP) is one of the most common focal dystonia and its pathophysiological mechanism remains unclear. An unbiased method was used in patients with BSP at rest to observe voxel-wise brain-wide functional connectivity (FC) changes. Method: A total of 48 subjects, including 24 untreated patients with BSP and 24 healthy controls, were recruited to undergo functional magnetic resonance imaging (fMRI). The method of global-brain FC (GFC) was adopted to analyze the resting-state fMRI data. We designed the support vector machine (SVM) method to determine whether GFC abnormalities could be utilized to distinguish the patients from the controls. Results: Relative to healthy controls, patients with BSP showed significantly decreased GFC in the bilateral superior medial prefrontal cortex/anterior cingulate cortex (MPFC/ACC) and increased GFC in the right postcentral gyrus/precentral gyrus/paracentral lobule, right superior frontal gyrus (SFG), and left paracentral lobule/supplement motor area (SMA), which were included in the default mode network (DMN) and sensorimotor network. SVM analysis showed that increased GFC values in the right postcentral gyrus/precentral gyrus/paracentral lobule could discriminate patients from controls with optimal accuracy, specificity, and sensitivity of 83.33%, 83.33%, and 83.33%, respectively. Conclusion: This study suggested that abnormal GFC in the brain areas associated with sensorimotor network and DMN might underlie the pathophysiology of BSP, which provided a new perspective to understand BSP. GFC in the right postcentral gyrus/precentral gyrus/paracentral lobule might be utilized as a latent biomarker to differentiate patients with BSP from controls.

Novel compound heterozygous FBXO7 mutations in a family with early onset Parkinson's disease.

BACKGROUND: Mutations in the F-box protein 7 (FBXO7) gene result in autosomal recessive parkinsonism. This usually manifests as early-onset parkinsonian-pyramidal syndrome but patients exhibit high phenotypic variability. Here we describe the findings of a Yemeni family with two novel FBXO7 mutations. METHODS: Clinical data and DNA were available for three siblings with early-onset parkinsonism together with their parents and three unaffected siblings. A targeted next generation sequencing panel was used to screen the proband for mutations in 14 genes known to cause a parkinsonian disorder. In addition, SNCA, PARK2, PINK1, and PARK7 were screened for copy number variants. RESULTS: The proband carried two novel compound heterozygous FBXO7 mutations: a missense mutation in exon 1 (p.G39R; c.115G > A) and a frameshift mutation in exon 5 (p.L280fs; c.838del). The mutations segregated with disease in the family with the exception of a potentially pre-symptomatic individual whose age was below the age of onset in two of their three affected siblings. P.G39R occurred at a highly conserved amino acid residue and both mutations were predicted to be deleterious in silico. In contrast to most reported families, the phenotype in this pedigree was consistent with clinically typical Parkinson's disease (PD) with a lack of pyramidal signs and good response to dopaminergic therapy. CONCLUSIONS: Our study expands the phenotype associated with FBXO7 to include early-onset PD and broadens the list of causative mutations. These data suggest that FBXO7 should be included in clinical genetic testing panels for PD, particularly in patients with early onset or a recessive inheritance pattern.

Basal ganglia and cerebellar circuits have distinct roles in blepharospasm.

INTRODUCTION: To identify areas of brain activity associated with involuntary muscle contractions in patients with blepharospasm using functional MRI. METHODS: 15 patients with blepharospasm underwent 8-min resting state scans with spontaneous orbicularis oculi muscle contractions simultaneously recorded using MRI-compatible surface electromyography. Spasm severity and spasm onset/offset were modeled using the amplitude of the electromyography signal (EMG-Amp) and its first temporal derivative (EMG-Onset), respectively, and included in a multiple regression functional MRI analysis using SPM12. Primary outcome was within-group blood-oxygen-level dependent activations that co-varied with EMG-Amp and EMG-Onset following correction for multiple comparisons for an overall cluster corrected p < 0.05. Secondary analyses included testing for correlations between imaging findings and symptom severity, as measured by clinical dystonia rating scales, using an uncorrected voxel-level threshold of p < 0.001. RESULTS: Imaging data from one subject were excluded due to excessive movement. EMG-Amp co-activated within the left sensorimotor cortex and cerebellum, as well as right lingual gyrus and superior temporal gyrus. EMG-Onset co-activated within the left posterior putamen/pallidum and a frontal eye field region in the left superior frontal gyrus. Symptom severity and EMG-Amp significantly co-varied in a small cluster within the left cerebellum. CONCLUSION: Our preliminary findings here suggest that cerebello-cortical circuits in blepharospasm could drive the intensity of eyelid spasms while basal ganglia circuits are associated with the triggering of spasms. This supports the network model for dystonia and identifies specific areas of involvement consistent with known brain regions responsible for control of movement.

Botulinum Toxin Effects on Sensorimotor Integration in Focal Dystonias.

(1) Background: In dystonia, the somatosensory temporal discrimination threshold (STDT) is abnormally increased at rest and higher and longer-lasting during movement execution in comparison with healthy subjects (HS), suggesting an abnormal sensorimotor integration. These abnormalities are thought to depend on abnormal proprioceptive input coming from dystonic muscles. Since Botulinum toxin-A (BT-A) reduces proprioceptive input in the injected muscles, our study investigated the effects of BT-A on STDT tested at rest and during voluntary movement execution in patients with focal dystonia. (2) Methods: We enrolled 35 patients with focal dystonia: 14 patients with cervical dystonia (CD), 11 patients with blepharospasm (BSP), and 10 patients with focal hand dystonia (FHD); and 12 age-matched HS. STDT tested by delivering paired stimuli was measured in all subjects at rest and during index finger abductions. (3) Results: Patients with dystonia had higher STDT values at rest and during movement execution than HS. While BT-A did not modify STDT at rest, it reduced the abnormal values of STDT during movement in CD and FHD patients, but not in BSP patients. (4) Conclusions: BT-A improved abnormal sensorimotor integration in CD and FHD, most likely by decreasing the overflow of proprioceptive signaling from muscle dystonic activity to the thalamus.

Pretarsal blepharospasm: Clinical and electromyographic characteristics.

OBJECTIVE: To describe the clinical and electromyographic characteristics of blepharospasm caused by selective involvement of the pars pretarsalis of the orbicularis oculi muscle. METHODS: Clinical assessment and simultaneous electromyographic recordings from levator palpebrae superioris and pars orbitaria and pretarsalis of orbicularis oculi muscles were performed in patients with blepharospasm and primary failure to botulinum toxin injections. Patients with selective abnormal electromyographic activity of the pars pretarsalis of the orbicularis oculi muscle were identified and treated with selective pretarsal injections of botulinum toxin. RESULTS: We found 24 patients with pretarsal blepharospasm confirmed by the electromyographic assessment. All of them were functionally blind. Three clinical-electromyographic patterns were identified: (a) Impairment of eyelid opening; (b) Increased blinking; (c) Spasms of eye closure combined with varying degrees of excessive blinking and impairment of eye-opening. Pretarsal injections of botulinum toxin induced a significant improvement in all patients and 50 % regained normal or near-normal vision. The clinical improvement was sustained after repeated pretarsal injections. CONCLUSIONS: Pretarsal blepharospasm can be suspected on clinical grounds and it can be confirmed by electromyographic recordings. SIGNIFICANCE: Recognition of this type of blepharospasm is important because of its excellent response to botulinum toxin injections applied into the pretarsal part of the orbicularis oculi muscle.

Sex, blinking, and dry eye.

Blinking sustains the corneal tear film generated by sexually dimorphic lacrimal and meibomian glands. Our study examines whether trigeminal control of blinking is also sexually dimorphic by investigating trigeminal reflex blinking, associative blink modification, and spontaneous blinking in male and female rats before and after unilateral dry eye caused by exorbital gland removal. Before gland removal, female rats exhibited a lower threshold for evoking trigeminal reflex blinks, a weaker effect of associative blink modification, and longer-duration spontaneous blinks than males. Spontaneous blink rate, reflex blink excitability, and occurrence of blink oscillations did not differ between the sexes. Reanalysis of previous data showed that humans showed the same blink sexual dimorphisms as rats. During the first 2 wk of dry eye, trigeminal blink circuit excitability and blink oscillations steadily rose in male rats, whereas excitability and blink oscillations did not change in females. Following dry eye, spontaneous blink duration increased for both males and females, whereas spontaneous blink rate remained constant for males but decreased for females. The associative modification treatment to depress trigeminal blink amplitude initially produced blink depression in males that converted to blink potentiation as trigeminal excitability rose, whereas females exhibited progressively more blink depression. These data indicated that dry eye increased excitability in male trigeminal reflex blink circuits at the expense of circuit modifiability, whereas trigeminal modifiability increased in females. This increased modifiability of female trigeminal blink circuits with dry eye may contribute to the preponderance of females developing the focal dystonia, benign essential blepharospasm.NEW & NOTEWORTHY All the elements controlling the corneal tear film are sexually dimorphic. Blinking, which smooths and maintains the tear film, also exhibits sex differences. Dry eye increases the sexual dimorphisms of blinking, including increased exaggeration of excitability in males and enhanced modifiability of the female trigeminal complex. This increased modifiability may explain female predominance in the development of the focal dystonia, benign essential blepharospasm.

Characteristics of tear abnormalities associated with benign essential blepharospasm and amelioration by means of botulinum toxin type A treatment.

PURPOSE: To investigate the characteristics of tear abnormalities with benign essential blepharospasm (BEB) and the effect of botulinum toxin type A (BTX-A) treatment. STUDY DESIGN: Prospective and clinical study. METHODS: Forty eyes of 40 patients (12 men and 28 women, ages 63.5 ±12.9) with BEB and tear abnormalities were enrolled. RESULTS: The average scores for subjective symptoms as evaluated by the visual analog scale (VAS) were 46.3 and Dry Eye-Related Quality-of-Life Score (DEQS) were 63.7. The fluorescein breakup time (FBUT) was 2.7 ± 1.6 sec. Among fluorescein breakup patterns (FBUPs), dimple break, with the corresponding mechanism of decreased wettability was the most frequent, observed in 29 eyes (73%). The NEI score was 0.4 ± 0.7 and the van Bijesterveld score was 0.6 ± 0.8; the Schirmer 1 test value was 13.1 ± 9.4 mm. Eighteen patients received BTX-A treatment, and significant improvement was found in severity of subjective symptoms both on VAS and DEQS as well as for FBUT. The main FBUPs changed from dimple break to random break. CONCLUSION: Tear abnormalities seen in BEB correspond to short BUT-type dry eye (DE), subclassified into decreased wettability DE in view of FBUPs.

Effect of light on blinking in patients with idiopathic isolated blepharospasm.

OBJECTIVE: Melanopsin may be involved in the pathophysiology of photophobia in idiopathic isolated blepharospasm. We assessed the efficacy of blocking wavelengths of melanopsin absorption to reduce blinking in blepharospasm as a possible surrogate for photophobia. METHODS: Twenty-one participants (11 blepharospasm and 10 healthy controls) were studied. There were three sessions: (1) a baseline condition to measure the blink rate (BR) without intervention; (2) two conditions where the participants received intermittent light stimuli with high or low intensity without wearing study lenses; (3) four conditions in which the participants received intermittent light stimuli with high intensity while wearing one of four different lenses: tinted lenses with neutral gray or FL-41, or coated lenses that block 480-nm or 590-nm wavelength. The primary outcome measure was the BR. RESULTS: The blepharospasm group blinked more frequently than controls in dim room conditions. Patients reported greater photosensitivity compared to controls based on the questionnaire and exhibited a higher BR with intermittent light stimuli. The BR decreased for both groups when using 480-nm and 590-nm blocking lenses. In the patients, 480-nm and 590-nm blocking lenses reduced the mean BR by 9.6 blink/min and 10.3 blink/min, respectively, while in the control group, the mean BR decreased by 4.4 blink/min and 4.3 blink/min, respectively. CONCLUSIONS: Blepharospasm patients had increased BR with light stimuli which decreased with 590-nm and 480-nm blocking lenses. The 480-nm- and 590-nm- coated lenses might have therapeutic potential in treating photophobia although BR does not appear to be an optimal biomarker for photophobia.

Comparison of Safety and Efficacy of Botox and Neuronox in the Management of Benign Essential Blepharospasm: A Split-face Study.

PURPOSE: To compare the efficacy and safety of Botox and Neuronox in the management of benign essential blepharospasm (BEB). METHODS: We performed a triple-masked, randomized control study to compare Botox and Neuronox in 48 eyes of 24 patients with BEB. All 24 patients randomly received Botox or Neuronox in the periorbital region in a masked, randomized split-face manner, keeping the injection sites and doses uniform. The toxin preparation, injection, and clinical evaluations were done by three independent observers. Objective outcome measures included improvement in the severity of spasm, grading of the functional visual status, changes in palpebral fissure height, lagophthalmos, superficial punctate keratitis and Schirmer's test at 2 weeks, 6 weeks, and upon conclusion of the effect of the toxin. Subjective outcome measures included duration of the effect and a forced choice stating which half of the face was better. Evaluations were performed through clinical measurements, external digital photography, and high-definition videography. RESULTS: The mean duration of relief from spasms was 3.78 months (standard deviation, 1.58 months; range, 1 to 6 months). The improvement in the objective parameters like severity of spasm and functional visual status was statistically significant at the 2-week and 6-week follow-up visits (p < 0.001). The changes in palpebral fissure height, lagophthalmos, and superficial punctate keratitis were equally observed in both groups. At 2 and 6 weeks, three of 24 (12.5%) and one of 24 (4%) patients, respectively, reported an unequal effect between the two sides of the face, but this difference was not statistically significant. At final follow-up (conclusion of the toxin effect), patients reported equal effect with no preference for either hemiface. No statistically significant differences were found in the comparative analysis between the Neuronox and Botox groups. CONCLUSIONS: Neuronox and Botox are comparable in terms of their safety and efficacy in the management of BEB.

Glucose hypometabolism in the visual cortex proportional to disease severity in patients with essential blepharospasm.

Essential blepharospasm (EB) causes difficulty in eyelid opening because of involuntary movements of the orbicularis oculi muscle. Patients with EB have functional visual loss due to sustained eyelid closure. We examined cerebral glucose metabolism in 39 patients with EB (12 men and 27 women; mean age, 52.1 years) by using positron emission tomography with 18F-fluorodeoxyglucose. Forty-eight eye open healthy subjects and 48 eye close healthy subjects served as controls. We analyzed and compared the data between the patients and controls by using both statistical parametric mapping (SPM) and regions of interest (ROIs). We defined ROIs on both sides of the posterior striate cortex, anterior striate cortex, extrastriate cortex, and thalamus. In SPM analysis, glucose hypometabolism were observed in both sides of the extrastriate cortex compared to eye open controls but not to eye close controls. We also observed a significant negative correlation between the Jankovic Rating Scale (JRS) sum score and relative glucose metabolism level in the striate cortex of these patients. ROI analysis, a significant correlation was observed between the JRS sum score and glucose metabolism level in the posterior (right: r = -0.53, P = .0005; left: r = -0.65, P = .00001) and anterior (right: r = -0.33, P = .04; left: r = -0.37, P = .02) striate cortices of patients with EB. We surmise that the interruption of visual input cause glucose hypometabolism in the visual cortex of patients with EB.

Ocular motor manifestations of movement disorders.

PURPOSE OF REVIEW: Impaired eye movements are frequently seen in ophthalmic and neurologic clinical practice, especially in individuals with movement disorders. Identification of the abnormal movement can aid initial diagnosis and improve understanding of the underlying disease pathophysiology. The present article reviews the ocular motor manifestations and recent research on them in common movement disorders. RECENT FINDINGS: Ocular motor manifestations and their pathophysiologic correlates are being defined. In particular, study of eye movements can help clarify the changing clinicopathologic spectrum of atypical parkinsonian disorders. The pathophysiology and natural history of blepharospasm are being elucidated. Recent research focuses on high-resolution imaging and other technological advances to improve the sensitivity of the ocular motility exam. Eye movements are being studied as biomarkers for diagnosis and progression in clinical care and trials. SUMMARY: The current review summarizes ocular motor manifestations in common movement disorders, and presents recent research investigating their cause and treatment.

A neural network-based software to recognise blepharospasm symptoms and to measure eye closure time.

Blepharospasm (BSP) is an adult-onset focal dystonia with phenomenologically heterogeneous effects, including, but not limited to, blinks, brief or prolonged spasms, and a narrowing or closure of the eyelids. In spite of the clear and well-known symptomatology, objectively rating the severity of this dystonia is a rather complex task since BSP symptoms are so subtle and hardly perceptible that even expert neurologists can rate the gravity of the pathology differently in the same patients. Software tools have been developed to help clinicians in the rating procedure. Currently, a computerised video-based system is available that is capable of objectively determining the eye closure time, however, it cannot distinguish the typical symptoms of the pathology. In this study, we attempt to take a step forward by proposing a neural network-based software able not only to measure the eye closure, time but also to recognise and count the typical blepharospasm symptoms. The software, after detecting the state of the eyes (open or closed), the movement of specific facial landmarks, and properly implementing artificial neural networks with an optimised topology, can recognise blinking, and brief and prolonged spasms. Comparing the software predictions with the observations of an expert neurologist allowed assessment of the sensitivity and specificity of the proposed software. The levels of sensitivity were high for recognising brief and prolonged spasms but were lower in the case of blinks. The proposed software is an automatic tool capable of making objective 'measurements' of blepharospasm symptoms.

A trial of a mechanical device for the treatment of blepharospasm.

BACKGROUND: Idiopathic blepharospasm (IB) is a rare but well-characterised adult onset focal dystonia that may cause severe visual disability. The most effective treatment is with periodic injections of botulinum toxin (BTX) into the pre-tarsal and/or pre-septal orbicularis oculi muscles bilaterally. However, even with treatment, practical visual function often remains compromised. A subset of IB sufferers find that eye opening improves with a focal unilateral digital pressure usually on a specific point on the temple. This is known as a 'sensory trick'. We have developed a spectacle mounted device ('Pressop') to apply continuous individually localised focal pressure on the temple to mimic the effect of finger pressure. The aim of the study was to determine if the 'sensory trick' could be replicated by Pressop and if the interval between BTX treatments could thereby be extended. SUBJECTS/METHODS: Study participants had three clinic visits-an initial screening assessment, a visit 2 weeks before the next injection was due when the device was fitted, and one 2 weeks later to assess the response to Pressop. A CDQ 24 and device-specific feedback questionnaire were completed and comparison photographs were taken. Repeat BTX injections were administered at the third visit. RESULTS: Of 58 patients with typical IB recruited to the trial, 39 reported an effective focal finger pressure sensory trick. 56 completed the trial, more than 50% of whom reported some benefit using Pressop; 18% had substantial improvement, sustained for up to 5 years. Improvement could occur in those without an effective sensory trick, therefore there was no significant correlation between using a sensory trick and benefiting from 'Pressop'. There was a trend towards the responders having greater improvement in CDQ24 total score than non-responders but this was not statistically significant. CONCLUSIONS: We recommend a trial of this simple safe device as a means of augmenting visual function in all IB patients.