Implementing strabismus-specific psychosocial questionnaires in everyday clinical practice: mental health and quality of life in the context of strabismus surgery.

BACKGROUND: Strabismus can have a great negative impact on the quality of life and the well-being of affected patients. In the past, these aspects were often neglected and, compared with somatic functioning, placed in the background. The aim of our study is to elicit factors influencing satisfaction with strabismus surgery, quality of life and expectations of surgery in order to better predict who will benefit the most and who may need further support. METHODS: We made a selection of suitable questionnaires to assess psychosocial aspects of strabismus and decided for Adult Strabismus 20 Questionnaire, Amblyopia and Strabismus Questionnaire, Diplopia Questionnaire, Expectations of Strabismus Surgery Questionnaire and Hospital Anxiety and Depression Scale. We then translated these measures (if not available in German). The patients filled out these forms as part of their preoperative orthoptic and ophthalmological assessment as well as approximately 3 months after strabismus surgery. RESULTS: We enrolled 59 patients in this study. Postoperative strabismus-related quality of life was higher after surgery and anxiety and depression levels were lower. Satisfaction with surgery was lower with higher postoperative angle and diplopia; the latter was also a determinant of lower postoperative quality of life. Higher expectations of strabismus surgery were present with higher depression levels and higher preoperative strabismus angle. CONCLUSION: Our data indicate that strabismus surgery may cause a significant improvement in several psychosocial domains. There is evidence that psychosocial factors can have significant impact on expectations with surgery. Hence, it is important to consider mental health aspects of this disease in order to treat patients in the best possible way.

Low-dose AtropIne for Myopia Control in Children (AIM): protocol for a randomised, controlled, double-blind, multicentre, clinical trial with two parallel arms.

INTRODUCTION: Myopia is a major cause of degenerative eye disease and increases the risk of secondary visual impairment. Mitigating its progression therefore has great potential of clinically relevant benefit as shown by using highly diluted atropine eye drops in children of Asian origin. However, limited evidence is available regarding the efficacy and safety of low-dose atropine therapy in non-Asian populations. Hence, the Low-dose AtropIne for Myopia Control in Children (AIM) study will test the efficacy and safety of 0.02% atropine vs placebo in a German population. METHODS AND ANALYSIS: AIM is a national, multicentre, prospective, randomised, placebo-controlled, double-blind trial with two parallel arms. The primary objective is to assess the efficacy of atropine 0.02% eyedrops for myopia control in children of Caucasian origin. The primary outcome is the change in cycloplegic refraction after 1 year of treatment (D/year). Secondary and tertiary outcome measures comprise the change in axial length (mm/year) in children treated with 0.02% atropine compared with placebo, the myopic progression of participants treated with 0.01% compared with 0.02% atropine (D/year and mm/year), and the safety profile of both 0.02% and 0.01% atropine. Furthermore, the myopic progression 1 year after cessation of therapy with 0.02% atropine will be evaluated. Inclusion criteria are an age of 8-12 years and myopia of -1 D to -6 D with an estimated annual myopia progression of ≥0.5 D. After randomisation, patients will receive either atropine 0.02% (arm A) or placebo eye drops (arm B) in the first year of treatment. In the second year, they will continue to receive atropine 0.02% (arm A) or switch to atropine 0.01% (arm B). In the third year, they will switch to placebo (arm A) or continue with atropine 0.01% (arm B). To achieve a statistical power of 80%, the calculated sample size is 300. The trial has started in October 2021 with a planned recruitment period of 18 months. ETHICS AND DISSEMINATION: AIM has been approved by the Central Ethics Committee of the University Medical Center Freiburg (21-1106), local ethics committees of each participating centre and the German Federal Institute for Drugs and Medical Devices (61-3910-4044659). It complies with the Declaration of Helsinki, local laws and ICH-GCP. Results and underlying data from this trial will be disseminated through peer-reviewed publications and conference presentations. TRIAL REGISTRATION NUMBER: NCT03865160.

[Progression of Myopia - which Preventive Measures Can be Recommended?] / Myopie(progression) ­ welche präventiven Ansätze sind sinnvoll?

The prevalence of myopia shows a worldwide increase with large regional differences. Especially high myopia enhances the risk of irreversible vision loss due to myopic maculopathy or other secondary effects. Reducing the prevalence and progression of myopia in schoolchildren is therefore a main goal in ophthalmology. Spending at least two hours a day outdoors is the easiest way to reduce myopia progression. Another modifiable factor is to reduce continuous near work with distances of less than 30 cm. Low-dose atropine eye drops administered once daily over two or more years have been shown to reduce myopia progression. Optical interventions which have been effective are multifocal contact lenses or orthokeratology contact lenses, but these have the risk of microbial keratitis. Whereas neither under- nor overcorrection of myopia have been proven effective, new so-called multisegment glasses have reduced myopia progression. Most of the studies concerning atropine and optic interventions have been performed in groups of Asian children, which are known to have more severe myopia progression, although actually there are many studies being conducted on Caucasian children. Still, there is also a lack of studies contrasting pharmacologic against optic interventions and comparing these with a combination of methods. The decision to start optic or pharmacologic measures can therefore only be an individual decision and is mainly based on age, refraction and progression in the past, while environmental factors should be assessed first.

Frequency of Hemorrhagic Side Effects of Botulinum Neurotoxin Treatment in Patients with Blepharospasm and Hemifacial Spasm on Antithrombotic Medication.

The aim of this study was to investigate the frequency of hemorrhagic side effects of botulinum neurotoxin A injections (BoNT/A) for the treatment of benign essential blepharospasm (BEB) and hemifacial spasm (HFS) in patients taking antithrombotic drugs (ATD). A total of 140 patients were included (female: 65%; BEB: 75%; mean age: 70 ± 12 years). According to their current antithrombotic medication, participants were either assigned to the ATD group (41%), or to the control group (59%). The ATD group was further divided into subgroups depending on the medication administered: acetylsalicylic acid, ADP receptor antagonists, direct oral anticoagulants, vitamin-K antagonists, or dual antiplatelet therapy. The frequency of hemorrhagic side effects was recorded by retrospective analysis of past treatments as documented in the patient's file set in relation to the number of past treatments (hematoma frequency of past treatments, HFretro) as well as by a prospective survey capturing the side effects of one single treatment (hematoma frequency of actual treatment, HFactual). There was no significant difference in hematoma frequency between the ATD group and the control group, neither for past (HFretro: ATD: 2%; 45/2554; control: 4%; 109/2744) nor for the current BoNT/A treatments (HFactual: ATD: 30%; 16/53; control: 31%; 22/72). Even between ATD subgroups, hematoma frequency did not differ significantly. Overall, hemorrhagic side effects of the BoNT/A treatment for BEB and HFS were mild and non-disabling.

Ultra-Long-Term Therapy of Benign Essential Blepharospasm with Botulinumtoxin A-30 Years of Experience in a Tertiary Care Center.

Aim of this study was to investigate the long-term results of botulinum toxin A (BoNT-A) injections for the treatment of benign essential blepharospasm (BEB) and to report our experience with (ultra-)long-term treatment with onabotulinumtoxin-A. We conducted a retrospective cross-sectional analysis at a university hospital. Patients with BEB and BoNT-A treatment were assigned to the Total Blepharospasm Group, patients with ≥21 onabotulinumtoxin-A injections to the Ona Long-Term Group. The Total Blepharospasm Group (n = 1940) included 33,933 BoNT-A injections. The age of patients at symptom onset was (mean ± SD) 58.0 ± 13.1 years, and 70.4% were female. The Ona long-term group (n = 234) included 10,632 onabotulinumtoxin-A injections. In this group, patients received 45.4 ± 22.9 injections with a mean dose of 22.2 IU ± 0.5. The duration of treatment was 12.6 ± 5.4 years, ranging from 2.9 to 30.0 years. The effect-duration-dose quotient did not change during long-term treatment. The observed side effects were comparable in type and frequency to other studies, even with the (ultra-)long treatment with onabotulinumtoxin-A. Our results, based on one of the largest patient populations and a treatment duration of up to 30 years, impressively demonstrate that onabotulinumtoxin-A is a safe and effective therapy for essential blepharospasm, even in the ultra-long term.

Periorbital injections of botulinum toxin a: a novel therapeutic option for convergence spasm in neuropsychiatric disorders.

PURPOSE: Convergence spasm (CS, spasm of near reflex) is characterized by transient attacks of convergence, miosis and accommodation, often associated with functional neurological disorders. To date, no simple and efficient treatment option is available for CS. This study investigates whether periorbital botulinum toxin injections as used in essential blepharospasm are also a treatment option in these patients. METHODS: All patients with convergence spasm having been treated with periorbital BoNTA injections in the department of neuro-ophthalmology were identified. Data were extracted from patient files concerning details and subjective effectiveness of botulinum toxin injections and relation to psychiatric or neurological disorders. Patients reporting with a history of closed-head trauma or organic neurologic pathologies possibly causing CS were excluded. A telephone assessment with a standardized questionnaire was performed to evaluate mental health issues as a trigger, as well as the long-term effect and satisfaction with periorbital injections. RESULTS: Of 16 patients treated with periorbital botulinum toxin injections for convergence spasm, 9 patients reported depression and/or anxiety disorders ongoing or in the past. A median number of 3 injections (range 1-13) was administered with a variable effect (relief of symptoms) between no effect and effect of up to more than 12 weeks. A longitudinal follow-up revealed ongoing symptoms in five patients. CONCLUSIONS: Periorbital botulinum toxin injections are less invasive than injections in the medial rectus muscle and can be a bridging therapeutic option in patients with CS. Mental health exploration is important due to psychiatric comorbidity.

Learning curve evaluation upskilling retinal imaging using smartphones.

Smartphone-based fundus imaging (SBFI) is a low-cost approach for screening of various ophthalmic diseases and particularly suited to resource limited settings. Thus, we assessed how best to upskill alternative healthcare cadres in SBFI and whether quality of obtained images is comparable to ophthalmologists. Ophthalmic assistants and ophthalmologists received a standardized training to SBFI (Heine iC2 combined with an iPhone 6) and 10 training examinations for capturing central retinal images. Examination time, total number of images, image alignment, usable field-of-view, and image quality (sharpness/focus, reflex artifacts, contrast/illumination) were analyzed. Thirty examiners (14 ophthalmic assistants and 16 ophthalmologists) and 14 volunteer test subjects were included. Mean examination time (1st and 10th training, respectively: 2.17 ± 1.54 and 0.56 ± 0.51 min, p < .0001), usable field-of-view (92 ± 16% and 98 ± 6.0%, p = .003) and image quality in terms of sharpness/focus (p = .002) improved by the training. Examination time was significantly shorter for ophthalmologists compared to ophthalmic assistants (10th training: 0.35 ± 0.21 and 0.79 ± 0.65 min, p = .011), but there was no significant difference in usable field-of-view and image quality. This study demonstrates the high learnability of SBFI with a relatively short training and mostly comparable results across healthcare cadres. The results will aid implementing and planning further SBFI field studies.

Validation of a new hemifacial spasm grading questionnaire (HFS score) assessing clinical and quality of life parameters.

Validation of the new hemifacial spasm (HFS) questionnaire "HFS score" that captures both clinical (HFS clinical) and health-related quality of life (HRQOL) parameters (HFS subjective) in one tool and comparison with a global HRQOL questionnaire. Cross sectional, prospective validation study including 143 subjects (62 HFS patients and 81 healthy volunteers) from the University Eye Hospital Bonn. Patients were interviewed with the new HFS score and the SF-12 questionnaire prior to and 3 weeks after Botulinum neurotoxin A (BoNT-A) injection. All HRQOL-related questions (HFS subjective) were answered on a visual analogue scale (VAS) ranging from 0 (no complaints) to 100% (maximum complaints) by the patients themselves. Reproducibility was tested in a study extension with 10 patients by repeat interviews (telephone/personal). The new HFS score questionnaire provided a reliable clinical assessment and demonstrated that BoNT-A therapy significantly reduced frequency and severity of eye and cheek spasms (p < 0.001; Wilcoxon test). Relevant aspects of HRQOL of HFS patients were assessed with high accuracy and sensitivity. Significant improvements were achieved after BoNT-A injection in five out of eight HRQOL parameters (p ≤ 0.02; Wilcoxon test). Cronbach's alpha of 0.818 demonstrated good internal consistency. Telephone survey provided comparable results to personal interviews. This new sensitive and specific HFS score seems a reliable instrument to monitor BoNT therapy and customize it to the needs of the individual HFS patient-in clinical studies and daily clinical practice.

Yokoyama procedure for esotropia associated with high myopia: real-world data from a large-scale multicentre analysis.

PURPOSE: High myopic patients may develop strabismus due to globe dislocation out of the normal extraocular muscle cone. Surgical correction of this strabismus type is possible by joining the superior and lateral rectus muscles without the need for a scleral suture called the Yokoyama procedure. Data from large patient samples and the evaluation of a potential effect of an additional medial rectus recession (MRR) have been lacking so far. METHODS: We pooled retrospective patient data of 14 departments of ophthalmology in Germany and Switzerland and analysed determinants of postoperative results using multivariable regression models. RESULTS: We included 133 patients (mean age: 59.7 ± 13.4 years, surgery between 2008 and 2017) with a mean preoperative esotropia (both Yokoyama with and without MRR) of 23.8°±4.6°. The angle of preoperative esotropia increased with age. The postoperative esotropia was 8.7° ± 9.9°, and six patients were overcorrected. While preoperative esotropia was highly associated with postoperative results, we found no association of additional MRR with any of our postoperative outcome measures. The Yokoyama procedure had a higher absolute effect in patients with higher preoperative esotropia. CONCLUSION: Our study confirms the positive effect of the Yokoyama procedure on strabismus due to high myopia in large-scale real-world data. In some cases, MRR may be needed because of muscle contracture, although additional MRR statistically did not affect the postoperative outcome. In patients with bilateral high myopic strabismus, correction of both eyes seems beneficial. The effect size of the Yokoyama procedure appears to be mainly driven by preoperative esotropia.

Multi-scale image analysis and prediction of visual field defects after selective amygdalohippocampectomy.

Selective amygdalohippocampectomy is an effective treatment for patients with therapy-refractory temporal lobe epilepsy but may cause visual field defect (VFD). Here, we aimed to describe tissue-specific pre- and postoperative imaging correlates of the VFD severity using whole-brain analyses from voxel- to network-level. Twenty-eight patients with temporal lobe epilepsy underwent pre- and postoperative MRI (T1-MPRAGE and Diffusion Tensor Imaging) as well as kinetic perimetry according to Goldmann standard. We probed for whole-brain gray matter (GM) and white matter (WM) correlates of VFD using voxel-based morphometry and tract-based spatial statistics, respectively. We furthermore reconstructed individual structural connectomes and conducted local and global network analyses. Two clusters in the bihemispheric middle temporal gyri indicated a postsurgical GM volume decrease with increasing VFD severity (FWE-corrected p < 0.05). A single WM cluster showed a fractional anisotropy decrease with increasing severity of VFD in the ipsilesional optic radiation (FWE-corrected p < 0.05). Furthermore, patients with (vs. without) VFD showed a higher number of postoperative local connectivity changes. Neither in the GM, WM, nor in network metrics we found preoperative correlates of VFD severity. Still, in an explorative analysis, an artificial neural network meta-classifier could predict the occurrence of VFD based on presurgical connectomes above chance level.

Sonographic assessment of the optic nerve and the central retinal artery in idiopathic intracranial hypertension.

PURPOSE: Transorbital sonography easily detects papilledema and enlarged optic nerve sheath diameters (ONSD) in IIH (idiopathic intracranial hypertension) patients. As the central retinal artery is located within the optic nerve, its hemodynamic properties might be affected by the increased pressure. In this study we assessed the diagnostic usefulness of transorbital sonography in IIH with a special focus on color Doppler imaging of the central retinal artery. IIH patients presented papilledema and enlarged ONSD. ONSD accurately predicted an increased intracranial pressure in IIH (cut-off: 5.8 mm, 81% sensitivity, 80% specificity). 24 h following therapeutic lumbar puncture ONSD diminished significantly, whereas papilledema was not changed. PSV (peak systolic velocity) and Vmean (mean flow velocity) of the central retinal artery were increased in IIH patients compared to controls. PSV accurately predicted an increase of intracranial pressure (cut-off: 11.0 cm/s, 70% sensitivity, 69% specificity). PI (pulsatility index), PSV and Vmean decreased following lumbar puncture. PSV and Vmean decreases were statistically significant for right eyes only in which the values changed to normal. In summary, besides ONSD enlargement and papilledema transbulbar sonography demonstrated an alteration of central retinal artery blood flow in IIH patients. Especially PSV might serve as valuable surrogate marker for intracranial pressure in IIH. Furthermore, the change of intra-individual central retinal arteries PI might be a valuable parameter to demonstrate response to lumbar puncture in IIH patients.

Distinct segregation of the pathogenic m.5667G>A mitochondrial tRNAAsn mutation in extraocular and skeletal muscle in chronic progressive external ophthalmoplegia.

Chronic progressive external ophthalmoplegia (CPEO) is a frequent clinical manifestation of disorders caused by pathogenic mitochondrial DNA mutations. However, for diagnostic purposes skeletal muscle tissue is used, since extraocular muscle tissue is usually not available for work-up. In the present study we aimed to identify causative factors that are responsible for extraocular muscle to be primarily affected in CPEO. We performed comparative histochemical and molecular genetic analyses of extraocular muscle and skeletal muscle single fibers in a case of isolated CPEO caused by the heteroplasmic m.5667G>A mutation in the mitochondrial tRNAAsn gene (MT-TN). Histochemical analyses revealed higher proportion of cytochrome c oxidase deficient fibers in extraocular muscle (41%) compared to skeletal muscle (10%). However, genetic analyses of single fibers revealed no significant difference either in the mutation loads between extraocular muscle and skeletal muscle cytochrome c oxidase deficient single fibers (extraocular muscle 86% ±â€¯4.6%; skeletal muscle 87.8 %±â€¯5.7%, p = 0.246) nor in the mutation threshold (extraocular muscle 74% ±â€¯3%; skeletal muscle 74% ±â€¯4%). We hypothesize that higher proportion of cytochrome c oxidase deficient fibers in extraocular muscle compared to skeletal muscle might be due to facilitated segregation of the m.5667G>A mutation into extraocular muscle, which may explain the preferential ocular manifestation and clinically isolated CPEO.

[Botulinumtoxin in Ophthalmology]. / Botulinumtoxin in der Augenheilkunde.

Botulinum toxin has been first applied in ophthalmology (as treatment for strabismus) and is nowadays widely used in many clinical areas. Currently the main application in ophthalmology is the treatment of essential blepharospasm and hemifacial spasm. Other important indications for botulinum toxin in ophthalmology are eyelid retraction in Graves' disease and the induction of protective ptosis in lagophthalmos or corneal diseases.

Stereoscopic Vision in Macular Telangiectasia Type 2.

PURPOSE: To investigate stereoscopic vision in patients with macular telangiectasia (MacTel) type 2 and correlate paracentral sensitivity loss to reduced stereoscopic function. METHODS: In a prospective single-center study, 50 patients with MacTel type 2 and 25 age-matched controls were investigated. Stereoscopic function was evaluated with Lang I, Titmus, and TNO tests. Sensitivity of the central 16° was tested using fundus-controlled perimetry (microperimetry). Functional loss was quantified as depth, size, and localization of scotomata. RESULTS: Both the Titmus and the TNO test revealed significantly reduced stereoscopic vision in patients compared to controls (p < 0.0001 for both). This applied even to patients with only relative or monocular paracentral scotoma. A strong correlation was observed for reduced stereoscopic vision with horizontal scotoma size and with the distance of scotomata from the foveal center. CONCLUSIONS: The results indicate that stereoscopic vision is impaired early in patients with MacTel type 2. A paracentral sensitivity loss, even if mild and limited to one eye, may considerably interfere with stereoscopic function despite normal visual acuity. Projection of paracentral scotomata within the patient's central visual field plays an important role in stereoscopic vision and should be considered when interpreting stereoscopic test results.

Depression in blepharospasm: a question of facial feedback?

Depression is the most important nonmotor symptom in blepharospasm (BL). As facial expression influences emotional perception, summarized as the facial feedback hypothesis, we investigated if patients report fewer depressive symptoms if injections of botulinum neurotoxin (BoNT) include the "grief muscles" of the glabellar region, compared to treatment of orbicularis oculi muscles alone. Ninety BL patients were included, half of whom had BoNT treatment including the frown lines. While treatment pattern did not predict depressive symptoms overall, subgroup analysis revealed that in male BL patients, BoNT injections into the frown lines were associated with remarkably less depressive symptoms. We hypothesize that in BL patients presenting with dystonia of the eyebrow region, BoNT therapy should include frown line application whenever justified, to optimize nonmotor effects of BoNT denervation.

Vision after trans-sylvian or temporobasal selective amygdalohippocampectomy: a prospective randomised trial.

BACKGROUND: Selective amygdalohippocampectomy (SAH) is an accepted surgical procedure for treatment of pharmacoresistant mesial temporal lobe epilepsy, but it may lead to postoperative visual field deficits (VFDs). Here we present a prospective randomised trial comparing the postoperative VFDs after either a trans-sylvian or temporobasal approach for SAH. METHOD: Forty-eight patients were randomly assigned to trans-sylvian (n = 24) or temporobasal (n = 24) SAH. Postoperative VFD were quantitatively evaluated using automated static and kinetic perimetry. In 24 cases, diffusion tensor imaging-based deterministic fibre-tracking of the optic radiation was performed. The primary endpoint was absence of postoperative VFD. The secondary endpoint was seizure outcome and driving ability. RESULTS: Three patients (13 %) from the trans-sylvian group showed no VFD, compared to 11 patients (46 %) from the temporobasal group without VFD (p = 0.01, RR = 3.7; CI = 1.2-11.5). Fifteen patients from each group (63 %) became completely seizure-free (ILAE1). Among those seizure-free cases, five trans-sylvian (33 %) and ten temporobasal (66 %) patients could apply for a driving licence (NNT = 3) when VFDs were considered. Although the trans-sylvian group experienced more frequent VFDs, the mean functional visual impairment showed a tendency to be less pronounced compared with the temporobasal group. DTI-based tracking of the optic radiation revealed that a lower distance of optic radiation to the temporal base correlated with increased rate of VFD in the temporobasal group. CONCLUSIONS: Temporobasal SAH shows significantly fewer VFDs and equal seizure-free rate compared with the trans-sylvian SAH. However, in patients in whom the optic radiation is close to the temporal base, the trans-sylvian approach may be a preferred alternative.

Botulinum toxin therapy in congenital blepharospasm.

Botulinum toxin injections are the treatment of choice for the management of essential blepharospasm in adults. No cases of congenital blepharospasm have been described in the literature so far, and no cases of botulinum toxin injection in an infant have been reported. A 4-week-old girl was referred to our department with absent eye opening and spasmodically closed eyes. Pregnancy and delivery had been normal. A neuropediatric examination did not reveal useful findings. A periorbital injection of botulinum toxin was performed at the age of 2 months to prevent deprivation amblyopia. Four days later, clearly visible bilateral eye opening and commencement of eye contact were observed. At the age of 3 years, her eyelids remain open and no side effects of botulinum toxin therapy have occurred.

Botulinum toxin in hemifacial spasm: the challenge to assess the effect of treatment.

Hemifacial spasm is characterized by intermittent tonic or clonic contractions of the muscles supplied by the facial nerve. Although vision is less impaired than in patients with blepharospasm, the disease can impose significant psychosocial burden on patient's life. Botulinum toxin (BoNT) is the well-established pharmacotherapy of choice, but evidence from controlled clinical trials is sparse. There is a broad variety of rating scales used in clinical studies with BoNT and obviously no consensus has been reached how to assess treatment outcome in hemifacial spasm. Clinical rating scales focusing on objective function were used in a couple of controlled studies with BoNT and were appropriate to discriminate between BoNTA and placebo. But it has not been shown that they would be sensitive enough to detect minor differences between several BoNT formulations. Although most of the clinical scales consist of a five-point rating, the descriptors for the ordinal numbers are not necessarily the same so that the results of different clinical studies are not comparable to each other. The main disadvantage of clinical scales is that they do not take into account patient's perspective of disability and impact on daily life. For this reason some clinical studies applied health-related quality of life (HRQoL) questionnaires to assess efficacy, and one research group worked on the development of disease-specific tools. Although these HRQoL questionnaires have been validated and a good correlation to disease severity could be demonstrated, they are far from having become an established variable for efficacy assessment in hemifacial spasm trials. The challenge remains to establish tools which are appropriate to rate BoNT treatment effects in hemifacial spasm. Currently, it is virtually impossible to identify one rating scale which can cover all relevant aspects of the disorder. In consequence we recommend the implementation of a combination of different rating scales which address functional impairment as well as those issues which are most important to patients. Further research is needed to standardize and validate rating scales for hemifacial spasm in clinical studies.

Impaired sleep quality and restless legs syndrome in idiopathic focal dystonia: a controlled study.

While sleep disorders are common in Parkinson's disease and other basal ganglia disorders, information on sleep disturbances in dystonia is limited to generalized forms or Segawa disease. Although many patients with idiopathic cervical dystonia (CD) and blepharospasm (BL) report poor sleep, there are no data on frequency or interactions with well known symptoms like depression and pain. Standardized interviews and assessment instruments, clinical examinations, and self rating forms were applied in 221 patients with CD and BL, and in 93 neurologically healthy controls. Impaired sleep quality was found in 44% of CD patients, 46% of BL patients, and 20% of controls. In dystonia, it was associated with symptoms of depression (frequency of 26%; p < 0.001) and restless legs syndrome (RLS) (frequency of 19%; p < 0.01). Bruxism (in CD; p < 0.05), and female sex (in BL; p < 0.001) were identified as further risk factors, but not severity of dystonic symptoms. Excessive daytime sleepiness was rare in CD and BL (6%). With a frequency of 45%, impairment of sleep quality is common in focal dystonia and associated with symptoms of depression, bruxism, and RLS. Results in CD and BL patients are similar, pointing to an intrinsic mechanism of sleep disturbances rather than a direct effect of dystonic muscle activity. Further studies on sleep in focal dystonia, including polysomnographic recordings, are warranted.

Difficulties with differentiating botulinum toxin treatment effects in essential blepharospasm.

Blepharospasm is a focal dystonia in which the extraocular muscles contract repetitively, leading to excessive blinking and forced eyelid closure. Botulinum toxin type A (BoNTA) is the primary symptomatic treatment for blepharospasm and its effects have been evaluated using numerous rating scales. The main scales in use today were initially used to determine whether BoNTA treatment was superior to placebo, and most controlled trials have confirmed this. More recently, these scales have been used to determine whether there are efficacy differences between different BoNTs in blepharospasm. However, although the scales used in these trials are able to differentiate the effects of BoNT from placebo, they may not be sensitive enough to differentiate between BoNTs. Most of the scales include only four possible points for each item, which would necessitate a 25% greater improvement in one group than the other to detect any differences. Current scales are also relatively insensitive to patients with mild disability who may experience mainly psychosocial problems related to their blepharospasm. Clinical trials comparing BoNTs that include substantial numbers of mildly affected patients may be unlikely to find differences because the scales do not adequately measure mild symptoms. Additional challenges with evaluating blepharospasm include the lack of precision and objectivity of current measures, symptom variability, the need to evaluate aspects of the disorder that are most important to patients, and the different types of blepharospasm. Although no single scale may be able to capture all relevant aspects of blepharospasm, more sensitive and patient-centered scales are needed.