Apraxia Of Eyelid Opening Research Articles

Myotomy In Situ for Essential Blepharospasm Refractory to Botulinum Toxin.

Full myectomy is recommended for benign essential blepharospasm (BEB) refractory to botulinum toxin (BT) treatment, but long-term swelling, scar contracture, hollow appearance, and unnatural contour of the eyelids are common postoperative complications. We present myotomy in situ to minimize these adverse outcomes. The redundant eyelid skin with its underlying muscle is resected first, and myotomy in situ is performed by completely cutting the residual orbicularis oculi muscles into multiple cubes and down to the subcutaneous layer, and then cutting the procerus and corrugator muscles down to the periosteum. Patient demographics, medical treatment history, BT injection history, blepharoptosis correction techniques, associated surgical procedures, and aesthetic outcomes were analyzed. Preoperative and postoperative BT injection dosage and frequency, as well as modified disability scores, were compared using paired Student t tests. Twenty-five patients underwent this technique. Their average ± SD age was 64.4 ± 8.9 years, with average postoperative follow-up occurring 17.0 ± 8.0 months. Associated diseases included blepharoptosis (88%) and apraxia of lid opening (44%). There were no postoperative hematoma, seroma, scar contracture, and depressed hollow eyelid contours. Preoperative to postoperative assessments revealed improvements in mean BT injection interval (10.4 ± 2.1 to 14.6 ± 2.9 weeks, P < 0.001), BT injection dosage (44.4 ± 13.3 to 28.1 ± 6.7 units, P < 0.001), and modified disability score (15.3 ± 3.0 to 2.8 ± 2.2, P < 0.001). All patients were highly satisfied with functional and aesthetic surgical outcomes (4.5 ± 0.6 on Likert scale). Myotomy in situ is effective for patients with BEB who are refractory to BT treatment, with therapeutic benefits similar to that of full myectomy with the ability to maintain favorable cosmetic results. Relative high incidence of blepharoptosis and apraxia of lid opening in patients with refractory BEB was reported. Simultaneous correction of the ptosis can further optimize outcomes.

Functional blindness: Blepharospasm with apraxia of eyelid opening

Purpose: The purpose was to evaluate the effectiveness of protractor myectomy surgery in patients with severe blepharospasm whose daily life is severely impaired. Materials and Methods: Records of fifty patients with benign, essential blepharospasm were examined retrospectively and seven were included in the study. All patients underwent a complete ophthalmological and neurological examination. The severity and frequency of spasm was determined by Jankovic Rating Scale, ranging from 0 to 4, and six daily activities were determined by Blepharospasm Disability Index. The primary indication for protractor myectomy was poor response to serial botulinum toxin injections. The main outcome measure was based on whether the spasms were fully healed or the need for additional botox application. Results: Fourteen eyes of seven patients with significant functional disability were included in the study. Excessive blinking and visual disability were the most common symptoms seen in all patients, and all had previously used one or more medications. Myectomy surgery was performed in all patients who were resistant to serial botulinum toxin injections. Of the 14 eyelids, 85.7% showed resolution of spasms and no longer required botulinum toxin treatment following surgery. One patient with keratitis sequelae and dry eye was given botulinum 1 month after the surgery. One patient with residual blepharospasm in the lower eyelid underwent lower eyelid myectomy and blepharoplasty. Transient periorbital edema, hematoma, and echymosis developed following surgery. The patients were followed up for 2 years, and excellent cosmetic result with no contracture was observed in all patients. Conclusion: Protractor myectomy is an effective treatment modality in cases of blepharospasm with severe functional disability.

Kurcz powiek – od rozpoznania do właściwego leczenia

Background: Blepharospasm is a movement disorder characterized by involuntary spasms of eye-closing muscles. This second most common adult-onset focal dystonia still raises many doubts among ophthalmologists and neurologists. Aim of the study: To present current knowledge about blepharospasm with historical background of the disease. Methods: Literature review regarding diagnosis, pathophysiology and treatment of blepharospasm. Results: Different types of eye-closing spasms, the spread of dystonia, co-occurrence of eyelid opening apraxia, enhanced blinking, sensory trick and psychiatric disorders define blepharospasm as a heterogeneous condition. Actual genetic, electrophysiological and neuroimaging studies reveal complex and multifocal involuntary movements generators defining blepharospasm and other focal dystonias as a ”communication node disorders” where botulinum toxin acting at the end of all pathways is the first-line treatment. Conclusions: The history of blepharospasm as a neurological condition that affects the eye shows that the most important discoveries regarding the diagnosis, pathophysiology and treatment of this disease have occurred owing to ophthalmologists and neurologists. Therefore, the future of blepharospasm will also depend on this cooperation.

Use of botulinum toxin in Parkinson's disease

Botulinum toxin has emerged as an important therapeutic intervention within the realm of movement disorders, especially for focal and generalized dystonias. Botulinum toxin has additionally been used for a variety of symptoms associated with parkinsonism. In this review, we will specifically evaluate use of botulinum toxin in idiopathic Parkinson's disease. We will discuss symptoms including sialorrhea, limb, dystonia, tremor, dyskinesias, freezing of gait, camptocormia, pisa syndrome, urinary dysfunction, constipation, dysphagia, eyelid opening apraxia, and blepharospasm.

Sleep-Induced Apraxia of Eyelid Opening.

Apraxia of eyelid opening (AEO) primarily has been described as bilateral loss of volitional ability to open the eyes at certain times and often associated with neurodegenerative disease. Rarely, it can occur in isolation and as an idiopathic phenomenon. There are a few reports of unilateral AEO only on awakening from sleep. We report an additional 11 patients with this unusual variation of AEO. Retrospective, observational case series of patients collected from 3 separate neuro-ophthalmology practices. All 11 patients were Caucasian women with a mean age of 59 years (range 35-80 years). All experienced AEO on awakening from sleep. Eight patients had unilateral AEO, and 3 had bilateral symptoms. The duration of episodes ranged from 3 weeks to several years. Ten of the patients reported manually elevating the eyelid to open it, while 1 patient waited for the eyelid to open spontaneously. After initial manual elevation, all patients reported normal function and position of the eyelids for the remainder of the day. Seven patients had a history of autoimmune disease. Slit-lamp and fundus examinations were negative for ocular pathology to explain the patients' symptoms, and 9 patients had unremarkable brain imaging. AEO occurring only on awakening from sleep is a rare entity. Neuroimaging and extensive laboratory testing are not indicated without associated neurologic or ocular findings. There may be a Caucasian female preponderance and autoimmune link in patients with AEO, but further studies are required.

Apraxia of lid opening with frontal lobe dysfunction after traumatic brain injury: A slowly progressive delayed complication

Background: Apraxia of lid opening (ALO) has been suggested to be a dysfunction of the supranuclear control of the levator palpebrae superioris caused mainly by basal ganglial lesion. The hypometabolism of the medial frontal lobe may be a one of pathophysiologic mechanism in ALO with a frontal cognitive impairment.

Botulinum toxin in parkinsonism: The when, how, and which for botulinum toxin injections

The aim of this article is to provide a review of the use of injections of botulinum toxin in the management of selected symptoms and signs of Parkinson's disease and other forms of parkinsonism. Sialorrhea is defined as inability to control oral secretions, resulting in excessive saliva in the oropharynx. There is a high level of evidence for the treatment of sialorrhea in parkinsonism with injections of different forms of botulinum toxin type A as well as botulinum toxin type B. Tremor can be improved by the use of botulinum toxin injections but improved tremor control often leads to concomitant motor weakness, limiting its use. Levodopa induced dyskinesias are difficult to treat with botulinum toxin injections because of their variable frequency and direction. Apraxia of eyelid opening, a sign more commonly seen in progressive supranuclear palsy and other tauopathies, often improves after botulinum toxin injections. Recent data suggest that regardless of the underlying mechanism, pain in parkinsonism can be alleviated by botulinum toxin injections. Finally, freezing of gait, camptocormia and Pisa syndrome in parkinsonism almost invariably fail to respond to botulinum toxin injections.

FRONTAL COGNITIVE DYSFUNCTION WITH APRAXIA OF EYELID OPENING AFTER TRAUMATIC BRAIN INJURY: A SLOWLY PROGRESSIVE DELAYED COMPLICATION

Apraxia of eyelid opening (AEO) has been suggested to be a dysfunction of the supranuclear control of the levator palpebrae superioris caused mainly by basal ganglial lesion. The hypometabolism of the medial frontal lobe may be a pathophysiologic mechanism in frontal lobe dysfunction and AEO. We report two patients who developed frontal cognitive dysfunction with AEO, as a delayed complication, 4–6 years after traumatic brain injury (TBI) progressively. Their MRI showed encephalomalacia with low signal intensity in medial frontal cortex which suggests loss of cortico-spinal tract fibers in the medial frontal cortex, which was not shown in initial brain CT scans. Delayed pathologic changes after TBI may contribute to the development of frontal cognitive dysfunction and AEO in these cases. Therefore we need to make close observation if the patients have TBI, especially severe enough to loss of consciousness even their brain imaging scans do not show abnormal finding initially.

Dystonia as a patterned motor malflow

The definition of dystonia has been renewed repeatedly but always within the context of overactive muscle contractions. In dystonia the muscles unnecessary for a motion fail to be adequately suppressed because of the loss of central motor control, resulting in inappropriate movements or abnormal postures (“overflow phenomenon”). This represents, however, only one side of the disrupted motor control. Some patients complain of the inability to activate muscles necessary for the intended task despite the lack of motor paresis, as best exemplified by the apraxia of eyelid opening. The author has ever coined the term “negative dystonia” for this phenomenon, which occurs hypothetically as a result of failed central motor flow and is often co-existent with overflow. Negative dystonia has a fixed pattern and sensory trick may be effective to ameliorate the symptom just as in the case of conventional dystonia. Hence we can speculate that the overflow and flow failure are the two sides of the same coin, both being caused by the loss of motor control. The current hypothesis is that dystonia is a representation of the patterned motor malflow (malicious flow) due to the loss of motor control.

123I-FP-CIT SPECT imaging in blepharospasm

IntroductionBlepharospasm is a focal dystonia characterized by involuntary cocontraction of the eyelid protractors, causing spasmodic closure of the eyelids. Apraxia of eyelid opening is caused by an inability to initiate lid opening without paralytic abnormality. Some studies suggest that patients with either pure blepharospasm or blepharospasm associated with apraxia of eyelid opening are more prone to developing Parkinson's disease. MethodsIn our study, 123I-FP-CIT (DAT) SPECT was performed in 24 patients with either pure blepharospasm or blepharospasm associated with apraxia of eyelid opening and no signs of parkinsonism to identify dopaminergic dysfunction. ResultsDAT-SPECT was abnormal in 11 (46%) cases (five patients with isolated blepharospasm and six patients with blepharospasm associated with apraxia of eyelid opening) whose mean disease duration was 11 years. ConclusionOur study revealed presynaptic dopaminergic dysfunction, as determined by 123I-FP-CIT or DAT-SPECT, in nearly half of our blepharospasm patients (with or without apraxia of eyelid opening). Thus, the presence of blepharospasm might be an early sign of a parkinsonian syndrome.

A 72-year-old with eyelid opening apraxia in Steele-Richardson-Olszewski syndrome.

Key Clinical MessageIn our patient with Steele–Richardson–Olszewski syndrome presenting with apraxia of eyelid opening, there is a possibility to be readily confused with conditions presenting in similar pattern such as blepharospasm and eyelid ptosis. Therefore, it is essential to correlate the clinical findings with electromyographic recordings and dopamine transporter scan (DaT).

Frontalis suspension surgery to treat patients with blepharospasm and eyelid opening apraxia: long-term results.

Blepharospasm may be accompanied by eyelid opening apraxia (EOA) reducing the efficacy of botulinum toxin (BT) therapy. The frontalis suspension operation (FSO) is then the only effective treatment option available. We want to report the first long-term results with FSO. We studied 15 patients with blepharospasm and EAO unresponsive to BT therapy (9 females, 6 males, age 61.9±11.5years). FSO was performed by applying 2 polytetrafluoroethylene threads (PTFE, Gore-Tex®) per eye connecting the frontalis muscle to the upper eye lid. Tension of the two carrés was set to produce a palpebral fissure width of 2-3mm. Therapy outcome was monitored by a quality-of-life questionnaire (QoL-Q) and a self-assessment calendar reviewing postoperative days 0-9 (T1), 10-89 (T2), 90-179 (T3), 180-365 (T4), and >365days (T5). Altogether, 40 FSO were performed. Postoperatively, all patients reported improved eyelid opening, 4 (27%) complete remission of symptoms. At T1, this improvement was 74.6±26.4% on the self-assessment scale, after 1year 68.2±27.5%. Throughout the observation period (T1-T5), the improvement was 71.9±25.6%. All 19 items on the QoL-Q (except for presence of involuntary eye lid closure) showed postoperative improvement. Adverse effects included circumscript upper eyelid haematomas, suture extrusion, suture granuloma, lacrimation, and infections. In all patients, BT therapy had to be continued to treat orbicularis oculi contractions. Our first long-term results demonstrate that FSO is a benign procedure producing robust and stable therapeutic effects on EOA in blepharospasm.

How Do I Examine Blepharospasm?

Blepharospasm, which is the most frequent cranial dystonia, is characterized clinically by bilateral, synchronous, and symmetric involuntary orbicularis oculi muscle contractions. Assessment of motor abnormalities in patients with blepharospasm is an important issue in the clinical practice of movement disorders. This video highlights the most important aspects in the clinical evaluation of blepharospasm. We will show how we approach the main motor abnormalities related to blepharospasm. Additional features that often characterize blepharospasm, such as increased blinking, sensory tricks that can transiently improve muscle spasms, and apraxia of eyelid opening will also be discussed. Then, we will summarize the main aspects that differentiate patients with blepharospasm from other conditions characterized by eyelid disturbances. Finally, we will take into account the possible therapeutic implications of an accurate clinical examination of patients.

Aripiprazole Can Improve Apraxia of Eyelid Opening in Parkinson's Disease

We herein report three cases of Parkinson's disease associated with difficulty in eyelid opening, referred to as apraxia of eyelid opening (AEO), which improved after aripiprazole treatment. In case 1, aripiprazole was administered as a psychiatric treatment. It proved to be effective in AEO with blepharospasm. In case 2 and case 3, the patients experienced AEO without blepharospasm, and a significant improvement was observed after aripiprazole treatment. In this study, the aripiprazole dosage ranged between 3 and 9 mg/day. This is the first report of aripiprazole as a potentially effective treatment for AEO in Parkinson's disease.

Botulinum Toxin: A Clinical Update on Ophthalmic Uses

Aim: To review the role of botulinum toxin in ophthalmology; its indications and side effects. Method: A literature search on pubmed was performed and articles explaining the role of botulinum toxin in ophthalmology were reviewed Result: Botulinum toxin is being widely used in ophthalmology for various indications like Essential Blepharospasm, Myokymia, Eyelid apraxia, Strabismus, Hyperlacrimation, Spastic entropion and Cosmetic uses. Complications of the injection include local effects like ecchymosis, pain or infection and spill over effects like ptosis, diplopia, lagophthalmos, mid facial weakness and dry eyes. Conclusion: There are various implications of botulinum toxin in ophthalmology. When considering its application in clinical practice, one should be careful of the indications, risks and benefits of the procedure. When properly delivered it is a potential, efficacious, minimally-invasive treatment modality. Key Words: Apraxia of Eyelid Opening, Botulinum toxin, Essential Blepharospasm, Eyelid myokymia, Spastic Entropion

Five-Year Outcomes of Bilateral Subthalamic Nucleus Stimulation in Japanese Patients with Parkinson’s Disease

Background: Deep brain stimulation (DBS) of the subthalamic nucleus (STN) is widely performed for medically refractory Parkinson’s disease (PD). Several western studies have examined the long-term outcomes of STN DBS. However, the long-term outcomes in Japanese patients have not been reported. Methods: We studied the long-term outcomes of STN DBS in Japanese patients with PD. Fifty-five consecutive patients treated with bilateral STN DBS were followed for 5 years after surgery. Each patient underwent Unified Parkinson’s Disease Rating Scale assessments preoperatively and 1 and 5 years after surgery. Results: Twelve patients (22%) were lost to follow up within 5 years. Among them, 7 died and 5 became bed ridden because of PD deterioration. In the 43 patients followed for 5 years, STN DBS significantly improved motor function. The cardinal motor symptoms of tremor, rigidity, and bradykinesia in medication-on periods were significantly better than baseline 5 years after DBS. However, axial motor symptoms of speech, gait and postural stability gradually deteriorated and significantly worsened 5 years after DBS. Motor complications, including dyskinesia and motor fluctuations, significantly improved after DBS with a marked reduction in dopaminergic medication. These effects were maintained 5 years after DBS. Frequently, persisting adverse effects included apraxia of eyelid opening and dysarthria. Conclusions: STN DBS significantly improved motor symptoms in patients with advanced PD. These effects were maintained over 5 years in most patients. However, some showed rapid PD progression even after STN DBS. Other treatments for the axial symptoms and disease progression are needed in long-term PD treatment.

Frontalis suspension surgery to treat patients with essential blepharospasm and apraxia of eyelid opening-technique and results.

IntroductionWe describe the results of 15 patients suffering from essential blepharospasm with apraxia of eyelid opening who underwent frontalis suspension surgery.Material and methodsPatients with apraxia of eyelid opening and unresponsive to botulinum toxin injections were studied. Bilateral frontalis suspension surgery was performed (sling operation) using polytetrafluoroethylene (Gore-Tex®) sutures. The patients reported the degree of improvement using a subjective rating scale to evaluate the benefit of the operation at two times after surgery (0-10 days and 180-360 days).ResultsThe patients reported a high degree of subjective improvement. In the early postoperative period (0-10 days) the mean degree of subjective improvement was 74.6% (standard deviation (SD) 26.4%). At 180-360 days after surgery the mean improvement was 70.0% (SD 26.7%). Small hematomas of the upper lid occurred postoperatively in all patients. Other complications were suture extrusions (9.1%), suture granulomas (6.1%), lacrimation (5.0%) and local infections (7.5%). Postoperatively, all patients needed additional botulinum toxin injections for optimal outcome.ConclusionFrontalis suspension surgery is a minimally invasive and effective treatment option for apraxia of eyelid opening in patients with essential blepharospasm unresponsive to botulinum toxin injections alone.

Apraxia of eyelid opening after brain injury: a case report.

Apraxia of eyelid opening (AEO) is a syndrome characterized by the patient's difficulty in initiating eyelid elevation spontaneously. Most of the reported cases were associated with extrapyramidal diseases. We report a case of AEO presented after traumatic brain injury, not with extrapyramidal diseases, and improved by dopaminergic treatment. A 49-year-old man underwent a traffic accident and was transferred to the emergency room in an unconscious state. Brain computed tomography (CT) revealed a subdural and epidural hemorrhage at right temporal and bilateral frontal lobes, and he received burr-hole trephination. After receiving comprehensive treatment including occupational therapy, cognition and mobility gradually improved, but he could not open his eyes voluntarily. With dopaminergic treatment (levodopa/benserazide 200/50 mg), he started to open his eyes spontaneously, especially when eating and undergoing physical training. This case showed that AEO may occur after brain injury and that dopaminergic treatment is beneficial also in AEO patients without extrapyramidal diseases.

Pathology and sensitivity of current clinical criteria in corticobasal syndrome

The aim of this study was to investigate corticobasal syndrome with respect to underlying pathologies, the ability of current clinical criteria to detect early stages of disease, and symptoms and signs predicting background pathologies. We retrospectively analyzed the clinicopathological findings from patients with corticobasal syndrome. We also analyzed whether those findings fulfilled the diagnostic criteria for corticobasal degeneration (CBD). Finally, we investigated characteristic clinical features that are specific to each background pathology. Of 10 consecutive autopsied patients who had corticobasal syndrome (mean age ± standard deviation, 67.9 ± 9.3 years; male:female ratio, 6:4), three had corticobasal degeneration pathology, three had progressive supranuclear palsy, three had Alzheimer's disease, and one had atypical four-repeat tauopathy. Nine patients fulfilled Mayo criteria, and all 10 patients fulfilled modified Cambridge criteria at the later stage, but only two patients fulfilled either clinical criteria within 2 years of disease onset. Five patients fulfilled the clinical criteria for possible CBD (p-CBD), and one patient fulfilled the clinical research criteria for probable sporadic CBD (cr-CBD) at the later stage. Only two patients fulfilled the criteria for either p-CBD or cr-CBD within 2 years of disease onset. Although we could not find any predictive characteristic clinical features that were specific to CBD pathology, only patients with progressive supranuclear palsy developed apraxia of eyelid opening and cerebellar ataxia. Myoclonus and memory impairment, especially if they appear at an early stage of the disease, may predict Alzheimer's disease pathology. Sensitivity of the available clinical criteria for corticobasal syndrome was poor within 2 years of disease onset.

Myectomy for blepharospasm 2013

Botulinum toxin (BTX) injections are the main medical treatment of facial dystonias, but injections are ineffective in some patients. This review discusses the indications for myectomy and surgical technique for treating benign essential blepharospasm (BEB) and apraxia of eyelid opening (ALO). There are four reasons to consider myectomy for patients with BEB. The first is ALO associated with BEB. The second is patients with blepharospasm-associated deformities. The third is patients who are truly unresponsive to BTX. The fourth is patients who cannot afford or who refuse BTX injections. Myectomy remains essential for treating blepharospasm patients and the most common indication is those with associated ALO.