Benign essential blepharospasm is a common focal dystonia characterized by involuntary eyelid closure. Its etiology, supported by animal models, appears to be multifactorial, representing the influence of a genetic background and an environmental trigger. The genetic background could be responsible for the reduced brain inhibition, identified with physiologic studies that would set up a permissive condition for increased brain plasticity. Reduced D2 receptors identified with PET might be an indicator of this reduced inhibition. The trigger could be repetitive use or local ocular disease. Although symptomatic therapy is available, better approaches are needed and will likely become available as the genetics and pathophysiology become well understood. PMID:12434791
Gilbert, AL; Dillon, WP; Horton, JC
Blepharospasm is rarely due to an identifiable etiology. In the majority of cases, imaging fails to reveal any structural lesion. Here we describe an otherwise healthy patient with blepharospasm who was found to have pontine capillary telangiectasia. We propose a potential association between blepharospasm and pontine capillary telangiectasia. © 2012 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Hallett, Mark; Evinger, Craig; Jankovic, Joseph; Stacy, Mark
This review updates understanding and research on blepharospasm, a subtype of focal dystonia. Topics covered include clinical aspects, pathology, pathophysiology, animal models, dry eye, photophobia, epidemiology, genetics, and treatment. Blepharospasm should be differentiated from apraxia of eyelid opening. New insights into pathology and pathophysiology are derived from different types of imaging, including magnetic resonance studies. Physiologic studies indicate increased plasticity and tr...
Pikielny, R T; Micheli, F E; Fernández Pardal, M M; Casas Parera, I; Giannaula, R J; Gatto, M
Blepharospasm is a relatively frequent cranial dystonia which may be seen either alone or related to orofacial-mandibular dystonia (Meige's syndrome). In its maximum degree it can cause functional blindness.Twelve patients with blepharospasm (4 essential and 8 Meige's syndrome) who had been previously treated unsuccessfully with drugs (trihexyphenidyl, biperiden, carbamazepine, lithium, baclofen, lisuride, imipramine, clonazepam and butyrophenones) were treated for 12 months with periocular injections of botulinum toxin (BOTOX). A "low" dose of 12,5 U per eye was employed. With this dose, eleven out of twelve patients experienced significant improvement which lasted from five to fifteen weeks. The only nonresponder obtained complete relief upon duplicating the dose. The only side effect was uni or bilateral ptosis in six patients which improved completely in seven to twenty one days. One patient developed a peripheral facial palsy with complete remission in nineteen days. No systemic side effects were noted. There was only one desertion from this study due to depression enhanced by prolonged (21 days) ptosis. All patients (including the deserter) agreed that treatment with BOTOX provided more relief than any other previous therapeutic method. Our results confirm those obtained by others but a more prolonged study is needed to better evaluate long term effects. PMID:2101846
Murai, Hideki; Suzuki, Yukihisa; Kiyosawa, Motohiro; Wakakura, Masato; Mochizuki, Manabu; Ishiwata, Kiichi; Ishii, Kenji
A 43-year-old woman with drug-related blepharospasm was followed up for 22 months. She had undergone etizolam treatment for 19 years for indefinite complaints. We examined her cerebral glucose metabolism 5 times (between days 149 and 688 since presentation), using positron emission tomography, and identified regions of interest in the thalamus, caudate nucleus, putamen, and primary somatosensory area on both sides. The severity of the blepharospasm was evaluated by PET scanning using the Wakakura classification. Sixteen women (mean age 42.4 ± 11.7 years) were examined as normal controls. The thalamic glucose metabolism in our patient was significantly increased on days 149, 212, and 688. The severity of the blepharospasm was positively correlated with the thalamic glucose metabolism, suggesting that the severity of blepharospasms reflects thalamic activity. PMID:22110436
Murai, Hideki; Suzuki, Yukihisa; Kiyosawa, Motohiro; Wakakura, Masato; Mochizuki, Manabu; Ishiwata, Kiichi; Ishii, Kenji
A 43-year-old woman with drug-related blepharospasm was followed up for 22 months. She had undergone etizolam treatment for 19 years for indefinite complaints. We examined her cerebral glucose metabolism 5 times (between days 149 and 688 since presentation), using positron emission tomography, and identified regions of interest in the thalamus, caudate nucleus, putamen, and primary somatosensory area on both sides. The severity of the blepharospasm was evaluated by PET scanning using the Waka...
Full Text Available Zolpidem is usually used for the treatment of insomnia as a hypnotic drug. It was also suggested to be effective in the treatment of dystonia in some studies. A 74-year-old woman had been suffering from frequent and intense bilateral spasms of the eyelids for 20 years. She has been treated with botulinum toxin injection and taken some medications. But, she experienced a little effect and was not satisfied with those treatments. Her symptom was improved after taking Zolpidem which had been prescribed for insomnia by her primary physician. She did not show any improvement after placebo injection and neostigmine test. This is the first report which shows improvement of isolated blepharospasm by Zolpidem in Korea. Zolpidem can be one of useful alternative pharmacological treatments for blepharospasm. Further randomized, blinded, placebo-controlled studies are needed to validate this finding.
Corridan, P.; Nightingale, S; Mashoudi, N.; Williams, A C
Botulinum toxin inhibits acetylcholine release and therefore could cause mydriasis. We report a case of acute angle-closure glaucoma which occurred shortly after a series of injections of botulinum toxin round the eyelids for blepharospasm.
Valls-Sole, Josep; Defazio, Giovanni
Blepharospasm (BSP) is a rather distressing form of focal dystonia. Although many aspects of its pathophysiological mechanisms are already known, we lack fundamental evidence on etiology, prevention, and treatment. To advance in our knowledge, we need to review what is already known in various aspects of the disorder and use these bases to find future lines of interest. Some of the signs observed in BSP are cause, while others are consequence of the disorder. Non-motor symptoms and signs may be a cue for understanding better the disease. Various cerebral sites have been shown to be functionally abnormal in BSP, including the basal ganglia, the cortex, and the cerebellum. However, we still do not know if the dysfunction or structural change affecting these brain regions is cause or consequence of BSP. Further advances in neurophysiology and neuroimaging may eventually clarify the pathophysiological mechanisms implicated. In this manuscript, we aim to update what is known regarding epidemiology, clinical aspects, and pathophysiology of the disorder and speculate on the directions of research worth pursuing in the near future. PMID:27064462
Fezza, John; Burns, John; Woodward, Julie; Truong, Daniel; Hedges, Thomas; Verma, Amit
To characterize satisfaction with current standard-of-care botulinum neurotoxin type A (BoNT/A) treatment for blepharospasm, we performed a cross-sectional, structured survey in subjects with blepharospasm who had received ≥2 BoNT/A cycles. Subjects were interviewed immediately before re-injection to evaluate treatment satisfaction, time course of treatment effects, preferred injection intervals, Jankovic Rating Scale (JRS), and Blepharospasm Disability Index (BSDI). Subjects' (n=114) last treatment was onabotulinumtoxinA (n=78), incobotulinumtoxinA (n=35), or abobotulinumtoxinA (n=1). The most frequent injection interval was 12weeks (46.5% subjects); 30.7% had an interval >12weeks. The main rationale for interval choice was "to maintain treatment efficacy" (44.7%). However, 36.6% reported that treatment effects usually declined within 8weeks; 69.6% within 10weeks. JRS and BSDI scores indicated re-emergence of symptoms before re-injection, with 70.2% and 73.7% of subjects reporting difficulties to drive and read, respectively. Overall, treatment satisfaction was high, but declined at the end of the cycle. Many subjects (52.3%) would prefer an injection interval of <12weeks; 30.6% of <10weeks. In conclusion, the survey results indicate that blepharospasm symptoms, such as difficulties to drive and read, re-emerge at the end of a BoNT treatment cycle and that flexible, individualized treatment intervals may improve treatment satisfaction and outcomes. PMID:27423565
Moon, Nam Ju; Lee, Hyeon IL; Kim, Jae Chan
To determine if the involuntary contractions of eyelids may have any effects on the development of corneal astigmatism, we performed this prospective study which includes 19 patients with either essential blepharospasm or hemifacial spasm. In hemifacial spasm, the degree of corneal astigmatism was evaluated between two eyes. Then the topographic changes were checked using vector analysis technique before and after passively opening the eyelids. They were also measured before and at 1 and 6 mo...
María G. Cersósimo
Full Text Available Blepharospasm is a focal dystonia characterized by involuntary eye closure due to abnormal contraction of orbicular eyelid muscles. When blepharospasm is associated to the presence of involuntary oromandibular movements, it is termed Meige syndrome. The aim of this study was to investigate the presence of deglutition alterations in patients with concurrent blepharospasm and Meige syndrome. Twenty consecutive patients were studied by video fluoroscopy using a barium technique. The 4 stages of deglutition were investigated. Ninety percent of patients (18 cases presented deglutition disorders. The more commonly found alterations were premature food drop, 15 cases (83% and vallecuale residuals, 14 cases (78%. Sixty seven percent of abnormal findings occurred in the third stage of deglutition. Eighty-nine percent of patients (16 presented more than one swallowing alteration. There was a positive and significant correlation between the number of alterations and patient's age or disease duration. Prevalence of swallowing disorders in the healthy elderly population is reported to be 44%. In our series it reached 90%, suggesting that our findings might be related not only with age but also with a more widespread dystonia exceeding the orofacial muscles.El blefaroespasmo es una distonía focal caracterizada por el cierre involuntario de los ojos debido a la contracción anormal de los músculos orbiculares de los párpados. Cuando el blefaroespasmo se asocia a la presencia de movimientos involuntarios oromandibulares se denomina síndrome de Meige. El objetivo de este estudio fue investigar la presencia de alteraciones deglutorias en pacientes con blefaroespasmo y síndrome de Meige. Se incluyeron 20 pacientes consecutivos que fueron estudiados mediante vídeo fluoroscopia con técnica de bario. Se investigaron las 4 etapas de la deglución. El 90% de los pacientes (18 casos presentó trastornos en la deglución. Las alteraciones más comúnmente halladas
Fidias E. León-Sarmiento
Full Text Available El blefaroespasmo esencial benigno cursa con movimientos repetitivos anormales del cierre de los párpados y espasmo del músculo orbicular de los ojos. Modernas teorías postulan que este trastorno del movimiento se origina por alteraciones en el procesamiento de la información aferente, con posterior desintegración de dicha información a nivel del programa neural sensorimotor que existe en el sistema nervioso central, que se manifiesta luego como movimiento anormal en los individuos genéticamente susceptibles. Diferentes investigaciones que incluyen estudios imagenológicos, genéticos y neurofisiológicos han proporcionado nuevos hallazgos acerca de las áreas neurales involucradas en esta patología y la forma como se genera este trastorno. Dentro de estas investigaciones sobresale el estudio del reflejo del parpadeo obtenido eléctricamente, el cual consta de tres respuestas llamadas no-nociceptiva (R1, nociceptiva (R2 y ultranociceptiva (R3. Dicho reflejo y, principalmente, la respuesta refleja ultranociceptiva (R3 parece ser muy útil para entender más profundamente la fisiopatología de esta distonía focal y realizar la endofenotipificación funcional y seguimiento en la neuro rehabilitación correspondiente de este complejo problema neurológico.Benign essential blepharospasm is characterized by abnormal repetitive movements of lid closure and spasm of the orbiculari oculi muscles. Modern theories postulate that this movement disorder originates by abnormal processing of afferent information with further disintegration of the sensorimotor neural program at central levels of the nervous system all of which is seen as dystonic movements in genetically susceptible people. Different investigations including neuroimagin, genetic and neurophysiological studies have discovered new findings on what structures are involved and how this abnormal movement is generated. Among these research is noteworthy the study of electrically elicited
目的：观察舒经汤合复方樟柳碱治疗眼睑痉挛的临床疗效。方法62例眼睑痉挛患者随机分为两组，对照组采用复方樟柳碱治疗，治疗组采用舒经汤结合复方樟柳碱治疗，比较两组患者的治疗效果、复发情况、不良反应等。结果治疗组总有效率明显高于对照组（P＜0．05）；治疗组复发率明显低于对照组（P＜0．05）。结论舒经汤结合复方樟柳碱治疗眼睑痉挛效果显著，不良反应少。%s:Objective To observe the clinical efficacy of Shujing decoction combined with compound anisodine in treatment of blepharo-spasm.Methods 62casesofblepharospasmwererandomlydivideintotwogroups,thecontrolgroupreceivedcompoundanisodine,thetreat-ment group was given Shujing decoction and compound anisodinethe,the treatment effect, recurrence and adverse reactions of two groups were compared.Results The total effective rate of treatment group were significantly higher than control group (P<0.05);the recurrence rate of treatment group was significantly lower than the control group (P<0.05).Conclusion Shujing decoction combined with compound anisod-ine in treatment of blepharospasm has significantly effect and less adverse reaction.
Executive functioning in patients with blepharospasm in comparison with patients with hemifacial spasm Funções executivas em pacientes com blefaroespasmo em comparação a pacientes com espasmo hemifacial
Fernando Machado Vilhena Dias
Full Text Available BACKGROUND: Non-motor symptoms have been described in primary dystonia, but the results on cognitive impairment in this condition are discordant. Blepharospasm (BM is a type of primary focal dystonia characterized by recurrent and involuntary eye blinking. Hemifacial spasm (HS, a condition with different pathophysiology, constitutes an adequate control group when investigating non-motor disturbances in BM. OBJECTIVE:To compare the performance of patients with BM and HS in the Frontal Assessment Battery (FAB. METHOD: Twenty-two patients with BM and 29 patients with HS were submitted to the FAB and the Mini-Mental State Examination (MMSE. FAB scores were compared between the two groups. Correlations between FAB and sociodemographic and clinical variables were calculated. RESULTS: BM group was not different from HS in relation to gender, age, length of symptoms, educational level and performance in the MMSE. FAB scores were also similar in both groups. FAB scores correlated negatively with age and positively with educational level and MMSE scores. CONCLUSION: Executive functioning as assessed by FAB is not altered in BM in comparison with HS.INTRODUÇÃO: Alterações não-motoras são descritas na distonia primária, embora sejam conflitantes os resultados sobre prejuízo cognitivo nessa condição. Blefaroespasmo (BE é um tipo de distonia primária focal caracterizada por contrações recorrentes e involuntárias das pálpebras. Espasmo hemifacial (EH, cuja fisiopatologia é distinta do BE, constitui bom grupo controle quando se investiga alterações não-motoras no BE. OBJETIVO: Comparar o desempenho de pacientes com BE e com EH na Bateria de Avaliação Frontal (BAF. MÉTODO: BAF e Mini-Exame do Estado Mental (MEEM foram administrados a 22 pacientes com BE e 29 com EH. Os escores da BAF foram comparados entre os dois grupos. Correlações entre resultados na BAF e variáveis sócio-demográficas e clínicas foram calculadas. RESULTADOS: Os
Toxina botulínica no blefaroespasmo, no espasmo hemifacial e na distonia cervical: resultados em 33 pacientes Botulinum toxin in blepharospasm, hemifacial spasm and cervical dystonia: results in 33 patients
Sérgio Ap. Novis
Full Text Available Avaliamos os resultados terapêuticos obtidos com o emprego de toxina botulínica do tipo A em 33 pacientes com distonia (12 com blefaroespamo; 10 com espasmo hemifacial e 11 com torcicolo espasmódico. Utilizamos uma escala de pontuação de gravidade antes de cada aplicação, sendo reavaliados duas semanas após, seguindo a mesma escala. Entre os com blefaroespasmo, oito eram mulheres e quatro homens; a média de idade foi 57,7 anos; a média do tempo de doença de quatro anos; três tinham história similar na família; nove eram essenciais e três fizeram uso de neurolépticos (distonia tardia. A dose média empregada ficou em 51,3 U, com a duração média do efeito benéfico de 2,8 meses. Do total de 22 aplicações (injeções e reinjeções, 14 (63,7% tiveram resultado ótimo, 5 (22,7% bom e três (13,6% nulo. Naqueles com espasmo hemifacial, oito eram mulheres e dois homens; a média de idade foi 52,6 anos; a média do tempo de doença 7,4 anos; oito eram essenciais e dois pós-páralíticos. A dose média empregada ficou em 32 U. Do total de 15 aplicações, todos (100% tiveram resultado ótimo, com a duração média do efeito benéfico de 3,4 meses. Nos pacientes com distonia cervical, oito eram homens e três mulheres; a média de idade foi 44,2 anos; a média do tempo de doença 12,2 anos; seis eram essenciais, três fizeram uso de neuroléptico e dois tinham história familiar. A dose média empregada ficou em 238,6 U, com a duração média do efeito benéfico de 4,7 meses. Do total de 20 aplicações, 18 (90% tiveram resultado bom, 1 (5% regular e 1 (5% nulo. Ptose palpebral, paresia facial e disfagia foram os efeitos colaterais mais encontrados. Concluímos que a toxina botulínica revelou-se eficaz no tratamento destas condições.The effects of botulinum toxin type A were studied in 33 patients with dystonia (12 blepharospasms, 10 hemifacial spasms and 11 spasmodic torticollis. A rate scale was used to evaluate the severity
Lange, Florian; Seer, Caroline; Dengler, Reinhard; Dressler, Dirk; Kopp, Bruno
Objectives Although primary dystonia is typically characterized as a movement disorder, it is also associated with cognitive alterations in the domain of executive functioning which may arise from changes in cortico-basal ganglia circuits. Specifically, in comparison to healthy controls, patients with dystonia show deficits in neuropsychological tests of cognitive flexibility. However, it is unclear whether cognitive inflexibility is caused by the pathomechanisms underlying primary dystonia or by confounding factors such as depression or symptom-related distraction.Methods The present study aimed to eliminate these confounds by examining cognitive flexibility in dystonia patients and in patients with similar motor symptoms but without a comparable central pathophysiology. Eighteen patients with primary blepharospasm, a common form of dystonia affecting the muscles around the eyes, and 19 patients with hemifacial spasm, a facial nerve disorder causing similar eyelid spasms, completed a computerized version of the Wisconsin Card Sorting Test (cWCST). The two groups were further compared on tests of global cognitive functioning, psychiatric symptoms, health status, and impulsiveness. Results Blepharospasm patients committed significantly more errors on the cWCST than patients with hemifacial spasm. Group differences were most pronounced with regard to integration errors, a measure of rule-inference processes on the cWCST. Integration errors were also associated with impulsiveness in patients with blepharospasm. Conclusions Primary blepharospasm is related to deficits in cognitive flexibility, even when blepharospasm patients are compared with patients who suffer from motor symptoms of non-dystonic origin. Our results support the possibility that cognitive inflexibility results from the specific pathophysiological processes underlying primary dystonia. (JINS, 2016, 22, 662-670). PMID:27333537
Canan Aslı Utine; İsmet Durak
Recently, pepper spray has been used by both civilians, as a non-lethal personal defense spray, and law enforcement agencies in population movements. People exposed to pepper spray experience panic and disorientation due to acute severe pain, blepharospasm, tearing and sometimes blurred vision and even temporary blindness. Having knowledge about the properties and mechanism of action of pepper spray and the different aspects of this exposure from other ocular chemical injuries is imp...
Harrison, Andrew R; Berbos, Zachary; Zaldivar, Renzo A.; Anderson, Brian C.; Semmer, Mollie; Lee, Michael S.; McLoon, Linda K.
While botulinum toxin injections are effective in reducing muscle spasms in blepharospasm and hemifacial spasm patients, they have a relatively short duration of action due to formation of new functional neuromuscular junctions. Co-injection of botulinum toxin–treated eyelids with corticotrophin-releasing factor or antibody to insulin growth factor–receptor prevented neuromuscular junction increases. The long-term goal is to increase the duration of effectiveness of botulinum toxin and reduce...
Mastrolilli, F.; Benvenga, A.; Di Biase, L; Giambattistelli, F.; Trotta, L.; Salomone, G.; Quintiliani, L.; Landi, D.; Melgari, J. M.; Vernieri, F.
Corticobasal degeneration (CBD) is an uncommon, sporadic, neurodegenerative disorder of mid- to late-adult life. We describe a further example of the pathologic heterogeneity of this condition. A 71-year-old woman initially presented dysarthria, clumsiness, progressive asymmetric bradykinesia, and rigidity in left arm. Rigidity gradually involved ipsilateral leg; postural instability with falls, blepharospasm, and dysphagia subsequently developed. She has been previously diagnosed as unrespon...
Punga, Anna Rostedt; Eriksson, Annika; Alimohammadi, Mohammad
Despite the extensive use of botulinum toxin A (BoNTA) in medical and cosmetic treatments, the potential spreading of BoNTA to surrounding tissues remains unknown. A patient with hemifacial paralysis upon blepharospasm treatment with low dose of BoNTA, prompted us to investigate the spreading effect. A randomised, double-blind study was conducted in which 5 healthy women (33-52 years) were treated with different doses of onabotulinum toxin unilaterally in the corrugator muscle. Parameters of ...
Joohi Jimenez-ShahedDepartment of Neurology, Baylor College of Medicine, Houston, TX, USAAbstract: Dystonia is a movement disorder of uncertain pathogenesis that is characterized by involuntary and inappropriate muscle contractions which cause sustained abnormal postures and movements of multiple or single (focal) body regions. The most common focal dystonias are cervical dystonia (CD) and blepharospasm (BSP). The first-line recommended treatment for CD and BSP is injection with botulinum tox...
Full Text Available Wolfgang H Jost,1 Reiner Benecke,2 Dieter Hauschke,3 Joseph Jankovic,4 Petr Kaňovský,5 Peter Roggenkämper,6 David M Simpson,7 Cynthia L Comella81Department of Neurology, University of Freiburg, Freiburg, Germany; 2Clinic and Policlinic for Neurology, University of Rostock, Rostock, Germany; 3Institute of Medical Biometry and Medical Informatics, University of Freiburg, Freiburg, Germany; 4Department of Neurology, Baylor College of Medicine, Houston, TX, USA; 5Department of Neurology, Palacky University Olomouc, Faculty of Medicine and Dentistry and University Hospital, Olomouc, Czech Republic; 6University Eye Clinic of Bonn, Bonn, Germany; 7Icahn School of Medicine at Mount Sinai, New York, NY, USA; 8Rush University Medical Center, Chicago, IL, USABackground: IncobotulinumtoxinA (Xeomin® is a purified botulinum neurotoxin type A formulation, free from complexing proteins, with proven efficacy and good tolerability for the treatment of neurological conditions such as blepharospasm, cervical dystonia (CD, and post-stroke spasticity of the upper limb. This article provides a comprehensive overview of incobotulinumtoxinA based on randomized controlled trials and prospective clinical studies.Summary: IncobotulinumtoxinA provides clinical efficacy in treating blepharospasm, CD, and upper-limb post-stroke spasticity based on randomized, double-blind, placebo-controlled trials with open-label extension periods (total study duration up to 89 weeks. Adverse events were generally mild or moderate. The most frequent adverse events, probably related to the injections, included eyelid ptosis and dry eye in the treatment of blepharospasm, dysphagia, neck pain, and muscular weakness in patients with CD, and injection site pain and muscular weakness when used for treating spasticity. In blepharospasm and CD, incobotulinumtoxinA was investigated in clinical trials permitting flexible intertreatment intervals based on the individual patient’s clinical need
Geraldo de Barros Ribeiro
Full Text Available PURPOSE: Crotoxin is the main neurotoxin of South American rattlesnake Crotalus durissus terrificus. The neurotoxic action is characterized by a presynaptic blockade. The purpose of this research is to assess the ability of crotoxin to induce temporary paralysis of extraocular and facial muscles in humans. METHODS: Doses of crotoxin used ranged from 2 to 5 units (U, each unit corresponding to one LD50. We first applied 2U of crotoxin in one of the extraocular muscles of 3 amaurotic individuals to be submitted to ocular evisceration. In the second stage, we applied crotoxin in 12 extraocular muscles of 9 patients with strabismic amblyopia. In the last stage, crotoxin was used in the treatment of blepharospasm in another 3 patients. RESULTS: No patient showed any systemic side effect or change in vision or any eye structure problem after the procedure. The only local side effects observed were slight conjunctival hyperemia, which recovered spontaneously. In 2 patients there was no change in ocular deviation after 2U crotoxin application. Limitation of the muscle action was observed in 8 of the 12 applications. The change in ocular deviation after application of 2U of crotoxin (9 injections was in average 15.7 prism diopters (PD. When the dose was 4U (2 applications the change was in average 37.5 PD and a single application of 5U produced a change of 16 PD in ocular deviation. This effect lasted from 1 to 3 months. Two of the 3 patients with blepharospasm had the hemifacial spasm improved with crotoxin, which returned after 2 months. CONCLUSIONS: This study provides data suggesting that crotoxin may be a useful new therapeutic option for the treatment of strabismus and blepharospasm. We expect that with further studies crotoxin could be an option for many other medical areas.
Canan Aslı Utine
Full Text Available Recently, pepper spray has been used by both civilians, as a non-lethal personal defense spray, and law enforcement agencies in population movements. People exposed to pepper spray experience panic and disorientation due to acute severe pain, blepharospasm, tearing and sometimes blurred vision and even temporary blindness. Having knowledge about the properties and mechanism of action of pepper spray and the different aspects of this exposure from other ocular chemical injuries is important for timely and correct interventions and appropriate management of the condition by the ophthalmologists in emergency care. (Turk J Oph thal mol 2012; 42: 294-7
Dystonia is a movement disorder of uncertain pathogenesis that is characterized by involuntary and inappropriate muscle contractions which cause sustained abnormal postures and movements of multiple or single (focal) body regions. The most common focal dystonias are cervical dystonia (CD) and blepharospasm (BSP). The first-line recommended treatment for CD and BSP is injection with botulinum toxin (BoNT), of which two serotypes are available: BoNT type A (BoNT/A) and BoNT type B (BoNT/B). Con...
Full Text Available OBJECTIVE: Local injection of botulinum toxin is a highly effective treatment option for a wide range of movement disorders and there are reliable sources of information on its indications, effects and safety in clinical practice. In this study, we report our experience with botulinum toxin in the treatment of facial region disorders. METHODS: Patients who had been followed in the Botulinum Toxin Outpatient Clinic of the Neurology Department were retrospectively evaluated. Two preparations of botulinum toxin type A (BT-A were used. The efficacy of BT-A injections was rated according to the improvement in symptoms as follows: marked - 75-100% improvement, good - 50-74%, moderate - 25-49%, and insufficient - less than 25% symptom relief. RESULTS: One hundred eighty-two patients (73 male, 109 female with various facial region disorders were included. The efficacy rates for patients who had very good and good improvement were high in the treatment of blepharospasm, hemifacial spasm, facial synkinesis, and Meige syndrome and moderate for oromandibular dystonia and hypersalivation. Ptosis was the most common side effect. CONCLUSION: According to our results, botulinum toxin was very effective treatment for blepharospasm, Meige syndrome, hemifacial spasm and facial synkinesis, whereas it demonstrated good efficacy in oromandibular dystonia and hypersalivation.
Brin, Mitchell F
Since the late 1970s, local injections of BoNT have provided clinical benefit for patients with inappropriately contracting muscles with or without pain or sensory disturbance. Marketing authorization for some BoNTs, depending on country, include core indications of dystonia (blepharospasm and cervical dystonia), large muscle spastic disorders (not yet approved in the United States, e.g., adult post-stroke spasticity and equinus foot deformity), hyperhidrosis and aesthetic. Subsequent development has extended to selected conditions characterized by recurrent or chronic pain (migraine headache), and urologic indications (neurogenic/idiopathic overactive bladder; prostate hyperplasia), with multiple additional opportunities available. Portfolio management requires a careful individual opportunity assessment of scientific and technical aspects (basic science foundation, potential to treat unmet medical need, product-specific risk in specific populations, therapeutic margin/safety profile, and probability of successful registration pathway). This article describes ongoing development targets for BOTOX. PMID:19470342
Wirth, M; Bonnemains, C; Auger, J; Raffo, E; Leheup, B
Sandifer's syndrome is a dystonic movement disorder in infants with gastroesophageal reflux (GER). It is probably misdiagnosed as epileptic seizures. We report the case of a 5-month-old infant with no past medical history admitted to a pediatric unit for suspicion of infantile spasms. She presented with dystonic movements of the upper left limb with left blepharospasm and an occasional dystonic head posture. Physical examination, EEG, brain MRI, and blood analysis were normal. Since the baby experienced regurgitations, Sandifer's syndrome was suspected and confirmed by 24-h esophageal pH monitoring that documented pathological GER. The dystonic symptoms quickly disappeared under treatment with thickened infant formula and sodium alginate. Infantile spasms remain the first diagnosis to explore with axial or para-axial dystonic postural events. Sandifer's syndrome should be retained when neurological investigations are normal and abnormal movements disappear under treatment of proven GER. Prognosis is excellent. PMID:26697813
Chaudhry, Shabana; Shoaib, Khawaja Khalid; Hing, Stephen; Smith, James
Iris cysts can arise from iris pigment epithelium or stroma. We present 3 cases of iris cysts which have been managed in different ways. In a one-month neonate, cyst was punctured with keratome and gentle diode laser endophotocoagulation was applied to the base. A2.5-month infant presented with watering and blepharospasm since birth. Clear fluid was aspirated from the cyst with a 27-gauge needle and Ethanol 96% (ETOH) was injected into the cyst and then aspirated. It was followed by injection/aspiration of 0.3 ml of balanced salt solution thrice. Cyst wall was excised. A13-month toddler presented with 4-month history of intermittent irritation and photophobia. The cyst was aspirated with a 25-gauge needle and the cyst walls were nibbled with 20-gauge vitrectomy cutter. Excision is better than injection of sclerosing solutions. The aim is to remove the whole cyst to avoid recurrence and to prevent amblyopia. PMID:27376231
Lupton, H W; Reed, D E
Experimental infection of eastern cottontail rabbits (Sylvilagus floridanus) with infectious bovine rhinotracheitis virus caused acute keratoconjunctivitis and a fatal systemic infection. The clinical syndrome was characterized initially by blepharospasm and ocular discharge. The rabbits were markedly depressed on post-exposure day (PED) 5 and were dead or moribund on PED 6. The virus was readily recovered from liver and adrenal gland tissue on PED 6 and from conjunctival swabs on PED 1 to 6. Histopathologic studies revealed a few necrotic foci in the liver and multiple focal to diffuse necrosis of the adrenal glands. Viral isolation and immunofluorescence tests were used to demonstrate a direct association between infectious bovine rhinotracheitis viral antigens and the lesions. PMID:230773
Full Text Available Botulinum toxin (BTX, a potent neurotoxin which is produced by the bacterium Clostridium botulinum, consists of eight distinct neurotoxin serotypes referred to as (BTX type-A [BTX-A], B, C, D, E, F, G, H all of which inhibit acetylcholine release at the neuromuscular junction. BTX-A, by blocking acetylcholine release at neuromuscular junctions, accounts for its therapeutic action to relieve dystonia, spasticity, and related disorders. A wide variety of medical conditions such as bruxism, hyperhidrosis, achalasia, focal dystonia, upper motor neuron syndrome, blepharospasm, and chronic migraine are now treated with BTX. The cosmetological applications include correction of lines, creases, and wrinkling all over the face, chin, neck, and chest. Side effects are generally rare and minimal. Injections with BTX-A are well-tolerated. Discovery of further newer indications of this neurotoxin can enlighten the path of research in the field of neuroscience.
To the Editor:The apraxia of eyelid opening (ALO) refers to a nonparalytic movement disorder of eyelid characterized bv transient difficulty in voluntarily initiating the act of lid elevation in spite of preserved alertness and language comprehension.1 Thetypical freezing of gait (FOG) is characterized by an abrupt difficulty in starting or continuing rhythmic and repetitive movements.The frequency of ALO was 0.3％-0.7％ in Parkinson's disease (PD),2,3 and it almost concurrently occurred with blepharospasm (BPS) or other dystonia.Yoon et al3 reported 3 ALO cases with BPS out of 913 PD patients.It is rare that a PD patient presents isolated ALO (not with BPS or other dystonia) as well as FOG.We reported here a case of PD with isolated ALO and FOG.
Full Text Available Abstract Background In this review article different interdisciplinary relevant applications of botulinum toxin type A (BTA in the head and face region are demonstrated. Patients with head and face disorders of different etiology often suffer from disorders concerning their musculature (example: synkinesis in mimic muscles or gland-secretion. This leads to many problems and reduces their quality of life. The application of BTA can improve movement disorders like blepharospasm, hemifacial spasm, synkinesis following defective healing of the facial nerve, palatal tremor, severe bruxism, oromandibular dystonias hypertrophy of the masseter muscle and disorders of the autonomous nerve system like hypersalivation, hyperlacrimation, pathological sweating and intrinsic rhinitis. Conclusion The application of botulinum toxin type A is a helpful and minimally invasive treatment option to improve the quality of life in patients with head and face disorders of different quality and etiology. Side effects are rare.
Muller, J; Wissel, J; Kemmler, G; Voller, B; Bodner, T; Schneider, A; Wenning, G; Poewe, W
Objective: To develop and test a questionnaire for measuring quality of life in patients with craniocervical dystonia. Methods: A 29-item pool was developed based on semi-structured interviews of patients with cervical dystonia (CD) and blepharospasm (BSP). This preliminary questionnaire was administered to 203 consecutive patients with CD and BSP from Austrian dystonia and botulinum toxin outpatient clinics. For scale generation, a combination of exploratory factor and cluster analysis was applied. This resulted in the 24-item version of the instrument (CDQ-24) based on five subscales: Stigma, Emotional wellbeing, Pain, Activities of daily living, and Social/family life. The validity and reliability of the CDQ-24 was assessed in 231 consecutive patients with CD and BSP different from those examined with the preliminary questionnaire. This second survey included the CDQ-24, a generic QoL instrument (SF-36) and clinical rating scales. Sensitivity to change was analysed in 51 previously untreated (de novo) patients four weeks and one year following the first botulinum toxin treatment. Results: Internal consistency reliability was satisfactory for all subscales, with values of Cronbach's α ranging from 0.77 to 0.89. The CDQ-24 subscales showed moderate to high correlations with those SF-36 subscales measuring similar aspects (Pearson's correlation r = 0.50–0.73; p<0.001, each). Sensitivity to change was confirmed by highly significant improvements of all CDQ-24 subscores in the de novo patients from baseline to four week follow up. One year follow up data revealed a stable improvement. Conclusion: The CDQ-24 is the first fully validated and disease specific questionnaire to evaluate quality of life of patients with cervical dystonia and blepharospasm and we propose its use in clinical trials as well as in daily clinical practice. PMID:15090572
Adult-onset primary torsion dystonia (AOPTD) is an autosomal dominant disorder with markedly reduced penetrance. Sensory abnormalities are present in AOPTD and also in unaffected relatives, possibly indicating non-manifesting gene carriage (acting as an endophenotype). The temporal discrimination threshold (TDT) is the shortest time interval at which two stimuli are detected to be asynchronous. We aimed to compare the sensitivity and specificity of three different TDT tasks (visual, tactile and mixed\\/visual-tactile). We also aimed to examine the sensitivity of TDTs in different AOPTD phenotypes. To examine tasks, we tested TDT in 41 patients and 51 controls using visual (2 lights), tactile (non-painful electrical stimulation) and mixed (1 light, 1 electrical) stimuli. To investigate phenotypes, we examined 71 AOPTD patients (37 cervical dystonia, 14 writer\\'s cramp, 9 blepharospasm, 11 spasmodic dysphonia) and 8 musician\\'s dystonia patients. The upper limit of normal was defined as control mean +2.5 SD. In dystonia patients, the visual task detected abnormalities in 35\\/41 (85%), the tactile task in 35\\/41 (85%) and the mixed task in 26\\/41 (63%); the mixed task was less sensitive than the other two (p = 0.04). Specificity was 100% for the visual and tactile tasks. Abnormal TDTs were found in 36 of 37 (97.3%) cervical dystonia, 12 of 14 (85.7%) writer\\'s cramp, 8 of 9 (88.8%) blepharospasm, 10 of 11 (90.1%) spasmodic dysphonia patients and 5 of 8 (62.5%) musicians. The visual and tactile tasks were found to be more sensitive than the mixed task. Temporal discrimination threshold results were comparable across common adult-onset primary torsion dystonia phenotypes, with lower sensitivity in the musicians.
Full Text Available Joohi Jimenez-ShahedDepartment of Neurology, Baylor College of Medicine, Houston, TX, USAAbstract: Dystonia is a movement disorder of uncertain pathogenesis that is characterized by involuntary and inappropriate muscle contractions which cause sustained abnormal postures and movements of multiple or single (focal body regions. The most common focal dystonias are cervical dystonia (CD and blepharospasm (BSP. The first-line recommended treatment for CD and BSP is injection with botulinum toxin (BoNT, of which two serotypes are available: BoNT type A (BoNT/A and BoNT type B (BoNT/B. Conventional BoNT formulations include inactive complexing proteins, which may increase the risk for antigenicity, possibly leading to treatment failure. IncobotulinumtoxinA (Xeomin®; Merz Pharmaceuticals GmbH, Frankfurt, Germany is a BoNT/A agent that has been recently Food and Drug Administration-approved for the treatment of adults with CD and adults with BSP previously treated with onabotulinumtoxinA (Botox®; Allergen, Inc, Irvine, CA – a conventional BoNT/A. IncobotulinumtoxinA is the only BoNT product that is free of complexing proteins. The necessity of complexing proteins for the effectiveness of botulinum toxin treatment has been challenged by preclinical and clinical studies with incobotulinumtoxinA. These studies have also suggested that incobotulinumtoxinA is associated with a lower risk for stimulating antibody formation than onabotulinumtoxinA. In phase 3 noninferiority trials, incobotulinumtoxinA demonstrated significant improvements in CD and BSP symptoms in both primary and secondary measures, compared with baseline, and met criteria for noninferiority versus onabotulinumtoxinA. In placebo-controlled trials, incobotulinumtoxinA also significantly improved the symptoms of CD and BSP, with robust outcomes in both primary and secondary measures. The use of incobotulinumtoxinA has been well tolerated in all trials, with an adverse event profile similar
Bloem, B R; Voermans, N C; Aerts, M B; Bhatia, K P; van Engelen, B G M; van de Warrenburg, B P
The rapid advances in modern neurology have led to increased specialisation in clinical practice. Being an expert in a neurology subspecialty offers advantages for diagnosing and managing specific disorders. However, specialisation also risks tunnel vision: interpreting symptoms and signs within one's own framework of reference, while ignoring differential diagnostic options from other subspecialties. This is particularly relevant when the patient's presentation potentially belongs to different neurological subspecialties. We illustrate this challenge by highlighting a series of clinical features that partially overlap between two common subspecialties: movement disorders and neuromuscular disorders. An overlap in clinical presentation is not rare, and includes, for example, involuntary eyelid closure (which could be active eye closure due to blepharospasm, or ptosis due to weakness). Other overlapping features include abnormal postures, involuntary movements and gait changes. We describe two of these overlapping features in more detail and emphasise the possible consequences of 'looking through the wrong end of the telescope' in such patients, as this may lead to a wrong differential diagnosis, unnecessary investigations and a delayed treatment start. PMID:26965497
Full Text Available This study was carried out to describe the ultrasonographic findings in relation to the clinical symptoms of some common ocular conditions in sheep and goats. Fifty animals (32 goats and 18 sheep with different ocular problems were examined. Ultrasonographic examination was performed using a B-mode ocular ultrasound unit, and the structure of the globe was evaluated at a depth of 4-6 cm. Early cases (n=35, 70% showed varying ocular conditions; hypopyon, (n=8, 16%, stromal abscesses, (n=4, 8%, and anterior uveitis (n=23, 46%. Hypopyon appeared clinically as a white or yellowish material in the anterior chamber, and ultrasonographically as a hyperechoic mass in the anterior chamber. Severe iridocyclitis was noticed in acute cases of infectious keratoconjunctivitis (IKC accompanied by blepharospasm, photophobia, excessive tearing and eyelid margin crust formation. Ultrasonographically, the pupil appeared constricted with increased hyperechoic thickening of the ciliary body. In chronic cases of IKC, corneal pigmentation (n=5, 10% and cataract (n=10, 20% were seen. Ultrasonographically the type and degree of cataract were diagnosed. The present study provides an inside view of the inner ocular structures during the course of certain eye diseases where ophthalmoscopic examination is not possible. Our findings, although preliminary, are relevant for the more complete diagnosis of certain external ocular conditions in sheep and goat herds.
Full Text Available Objective: To evaluate the effect of riboflavin-ultraviolet-A-induced cross-linking (CXL following corneal alkali burns in rabbits. Methods: The right corneas and limbi of ten rabbits were burned using a 1N solution of NaOH and the animals were then divided into two groups: a control group submitted to clinical treatment alone and an experimental group that was treated 1 h after injury with CXL, followed by the same clinical treatment as administered to the controls. Clinical parameters were evaluated post-injury at 1, 7, 15, and 30 days by two independent observers. Following this evaluation, the corneas were excised and examined histologically. Results: There were no statistically significant differences in clinical parameters, such as hyperemia, corneal edema, ciliary injection, limbal ischemia, secretion, corneal neovascularization, symblepharon, or blepharospasm, at any of the time-points evaluated. However, the size of the epithelial defect was significantly smaller in the CXL group (p<0.05 (day 15: p=0.008 and day 30: p=0.008 and the extent of the corneal injury (opacity lesion was also smaller (day 30: p=0.021. Histopathology showed the presence of collagen bridges linking the collagen fibers in only the CXL group. Conclusions: These results suggest that the use of CXL may improve the prognosis of acute corneal alkali burns.
Aguilar, Mariela C.; Gonzalez, Alex; Rowaan, Cornelis; De Freitas, Carolina; Rosa, Potyra R.; Alawa, Karam; Lam, Byron L.; Parel, Jean-Marie A.
As there is no clinically available instrument to systematically and reliably determine the photosensitivity thresholds of patients with dry eyes, blepharospasms, migraines, traumatic brain injuries, and genetic disorders such as Achromatopsia, retinitis pigmentosa and other retinal dysfunctions, a computer-controlled optoelectronics system was designed. The BPEI Photosensitivity System provides a light stimuli emitted from a bi-cupola concave, 210 white LED array with varying intensity ranging from 1 to 32,000 lux. The system can either utilize a normal or an enhanced testing mode for subjects with low light tolerance. The automated instrument adjusts the intensity of each light stimulus. The subject is instructed to indicate discomfort by pressing a hand-held button. Reliability of the responses is tracked during the test. The photosensitivity threshold is then calculated after 10 response reversals. In a preliminary study, we demonstrated that subjects suffering from Achromatopsia experienced lower photosensitivity thresholds than normal subjects. Hence, the system can safely and reliably determine the photosensitivity thresholds of healthy and light sensitive subjects by detecting and quantifying the individual differences. Future studies will be performed with this system to determine the photosensitivity threshold differences between normal subjects and subjects suffering from other conditions that affect light sensitivity.
Payne, Susannah; Tisch, Stephen; Cole, Ian; Brake, Helen; Rough, Judy; Darveniza, Paul
Laryngeal dystonia is a movement disorder of the muscles within the larynx, which most commonly manifests as spasmodic dysphonia (SD). Rarer reported manifestations include dystonic respiratory stridor and dyscoordinate breathing. Laryngeal dystonia has been treated successfully with botulinum neurotoxin (BTX) injections since 1984. We reviewed prospectively collected data in a consecutive series of 193 patients with laryngeal dystonia who were seen at St. Vincent's Hospital between 1991 and 2011. Patient data were analyzed in Excel, R, and Prism. Laryngeal dystonia manifested as SD (92.7%), stridor (11.9%), dystonic cough (6.2%), dyscoordinate breathing (4.1%), paroxysmal hiccups (1.6%), and paroxysmal sneezing (1.6%). There were more women (68.4%) than men (31.6%), and the average age at onset was 47 years. A positive family history of dystonia was present in 16.1% of patients. A higher incidence of extra-laryngeal dystonia (ie, torticollis and blepharospasm) and concurrent manifestations of laryngeal dystonia were present in patients with dystonic cough, dyscoordinate breathing, paroxysmal sneezing, and hiccups than in other patients (P = 0.003 and P Technical failures were rare (1.1%). Dysphonia secondary to vocal cord paresis followed 38.7% of treatments. Laryngeal dystonia manifests predominantly as SD, but other manifestations include stridor, dyscoordinate breathing, paroxysmal cough, hiccups, and sneezing. BTX injections are very effective across all subgroups. Severe adverse events are rare. PMID:24753288
Sadar, Miranda J; Guzman, David Sanchez-Migallon; Burton, Andrew G; Byrne, Barbara A; Wiggans, K Tomo; Hollingsworth, Steven R
A 1.5-year-old, intact female khaki Campbell duck (Anas platyrhynchos domesticus) was evaluated for lethargy and a swollen left eye (OS). Mucoid discharge, chemosis, and conjunctival hyperemia with trace aqueous flare, indicating anterior uveitis, in the anterior chamber were evident on ophthalmic examination. There was no fluorescein stain uptake by the cornea. Initial topical antibiotic therapy and systemic anti-inflammatory treatments were unsuccessful, and the lesion progressed to a diffuse, yellow-white plaque, which covered 90%-95% of the cornea 4 days later. There was moderate blepharospasm, mild blepharedema, and epiphora OS. The mobility of the nictitating membrane was impaired because of the presence of the plaque over the cornea. Cytologic examination of a corneal scraping revealed fungal hyphae, and aerobic culture confirmed Aspergillus species. Treatment with topical voriconazole (1 drop OS q4h-q6h) was initiated and was switched to oral voriconazole (20 mg/kg PO q12h) 6 days after initiating treatment. The ocular disease improved during the antifungal treatment period. Eighty-four days after initial presentation (9 days after discontinuation of treatment), there was no clinical evidence of mycotic keratitis on ophthalmic examination. PMID:25843471
Kelly Cristine de Sousa Pontes
Full Text Available The aim of this study was to evaluate the effects of canine amniotic membrane, previously preserved in glycerin, used as a patch on the repair of experimentally-made superficial corneal ulcers and to compare corneal epithelization between the treated and non-treated groups. Xenogeneic amniotic membranes were collected aseptically and preserved in 99% glycerin at room temperature. Each animal was anesthetized and submitted to superficial corneal keratectomy of the left eye. The treated group received a fragment of canine amniotic membrane as a patch, while the control group had no treatment. The treated group showed blepharospasm, ocular discharge and conjunctival congestion. The membrane accelerated corneal repair in the beginning of the process, however, it delayed its conclusion (p<0.05. Treated eyes showed greater vessel formation and decreased corneal transparency (p<0.05. The stroma of the control group was thicker than that of the treated group (p<0.05. We suggest that amniotic membrane used in this manner can be applied as a therapy for superficial corneal ulcers in the beginning phases of the repair process.
Full Text Available Corticobasal degeneration (CBD is an uncommon, sporadic, neurodegenerative disorder of mid- to late-adult life. We describe a further example of the pathologic heterogeneity of this condition. A 71-year-old woman initially presented dysarthria, clumsiness, progressive asymmetric bradykinesia, and rigidity in left arm. Rigidity gradually involved ipsilateral leg; postural instability with falls, blepharospasm, and dysphagia subsequently developed. She has been previously diagnosed as unresponsive Parkinson's Disease. At our clinical examination, she presented left upper-arm-fixed-dystonia, spasticity in left lower limb and pyramidal signs (Babinski and Hoffmann. Brain MRI showed asymmetric cortical atrophy in the right frontotemporal cortex. Neuropsychological examination showed an impairment in visuospatial functioning, frontal-executive dysfunction, and hemineglect. This case demonstrates that association of asymmetrical focal cortical and subcortical features remains the clinical hallmark of this condition. There are no absolute markers for the clinical diagnosis that is complicated by the variability of presentation involving also cognitive symptoms that are reviewed in the paper. Despite the difficulty of diagnosing CBD, somatosensory evoked potentials, motor evoked potentials, long latency reflexes, and correlations between results on electroencephalography (EEG and electromyography (EMG provide further support for a CBD diagnosis. These techniques are also used to identify neurophysiological correlates of the neurological signs of the disease.
Punga, Anna Rostedt; Eriksson, Annika; Alimohammadi, Mohammad
Despite the extensive use of botulinum toxin A (BoNTA) in medical and cosmetic treatments, the potential spreading of BoNTA to surrounding tissues remains unknown. A patient with hemifacial paralysis upon blepharospasm treatment with low dose of BoNTA, prompted us to investigate the spreading effect. A randomised, double-blind study was conducted in which 5 healthy women (33-52 years) were treated with different doses of onabotulinum toxin unilaterally in the corrugator muscle. Parameters of efficacy and diffusion (CMAP; EMG and jitter analysis) in both glabellar and frontalis muscles were assessed at baseline, 2 and 4 weeks following BoNTA injection. CMAP of the treated glabellar muscles was reduced to approximately 40% in all dose groups. Additionally, contralateral CMAP reduction was observed in 3 of 5 subjects. These data confirm regional diffusion of BoNTA in facial muscle application, which raises question on the reliability of split-face models in BoNTA studies. PMID:25766591
Full Text Available Background and Purpose: Primary focal or segmental dystonia is a rare clinical condition. The clinical features of dystonia have not been evaluated in China. We performed a study to investigate the epidemiology of primary dystonia and its clinical variants in an adult population. Methods: A Botulinum Toxin Clinic-based study was conducted in the period 18 May through 8 October 2010 in East China. We identified 523 dystonia patients from the Movement disorders and Botulinum Toxin clinic Cases. Results: The most common focal dystonia were blepharospasm (59%, cervical dystonia (35%, limb dystonia (3%, oromandibular dystonia (2% and laryngeal dystonia (1%. Males with primary dystonia were noted to have earlier age of onset. A female predominance was noted for most of the primary dystonias with a male to female ratio (M : F ranging from 1 : 1.48 to 1 : 3. Conclusions: The epidemiological features of dystonia in East China we collected were similar to the report in Japan which contrasts partly with that reported in Europe.
Xin-hua Wan; Kevin Dat Vuong; Joseph Jankovic
Objective To evaluate efficacy and safety of botulinum toxin type B (BTX-B) in treatment of movement disorders including blepharospasm, oromandibular dystonia, hemifacial spasm, tremor, tics, and hypersecretory disorders such as sialorrhea and hyperhidrosis.Methods A retrospective study of BTX-B injections in treatment of 58 patients with various neurological disorders was performed. The mean follow-up time was 0.9 ± 0.8 years. Results of the first and last treatment of patients with at least 3injection sessions were compared.Results The response of 58 patients to a total of 157 BTX-B treatment sessions was analyzed. Of the 157 treatment sessions, 120 sessions (76.4%) resulted in moderate or marked improvement while 17 sessions (10.8%) had no response.The clinical benefits after BTX-B treatment lasted an average of 14 weeks. Of the 41 patients with at least 3 injection sessions (mean 10 ± 8.6), most patients needed increased dosage upon the last session compared to the first session. Nineteen patients (32.8%) with 27 sessions (17.2%) reported adverse effects with BTX-B treatment.Conclusios Though most patients require increased dosage to maintain effective response after repeated injections,BTX-B is an effective and safe treatment drug for a variety of movement disorders, as well as drooling and hyperhidrosis.
万新华; 汤晓芙; 王荫椿
Objective. To introduce the properties of Chinese type A botulinum toxin (CBTXA, made by Lanzhou Institute of Biological Products), and its long-term effect for focal dystonia and hemifacial spasm. Method. The purity and recovery of crude and crystalline toxin were tested. Long-term data from 305 patients with hemifacial spasm (HFS), blepharospasm (BS) and cervical dystonia (CD) were evaluated and subgroups of patients received CBTXA injections between 1994 and 2000 in at least six separate treatment sessions, with follow up for 2-8 years. The therapeutic results of the last session CBTXA injections were analyzed in comparison with the first session. Result. CBTXA purity was high[(2.55～2.60)×107D50/mgPr, A260/A280 ≤0.55, high molecular substance accounted for 99.2% of total proteins]. Long term treatment with CBTXA in patients with focal dystonia and HFS was not associated with any decline in benefit, and efficacy may improve slightly with repeat treatments. CBTXA is an excellent long-term treatment of HFS, BS and CD. Conchusion. We conclude that Chinese type A botulinum toxin is of botulinum toxin therapy quality standard according to results obtained from the basic study and long-term clinical applications. The reinjection of CBTXA significantly improves the quality of life of most patients and is a safe, effective and comparatively economical treatment for patients with focal dystonia and HFS.
Full Text Available Manipulation of the optic nerve can lead to irreversible vision changes. We present a patient with a past medical history of skin allergy and allergic conjunctivitis (AC who presented with insidious unexplained unilateral vision loss. Physical exam revealed significant blepharospasm, mild lid edema, bulbar conjunctival hyperemia, afferent pupillary defect, and slight papillary hypertrophy. Slit lamp examination demonstrated superior and inferior conjunctival scarring as well as superior corneal scarring but no signs of external trauma or neurological damage were noted. Conjunctival cultures and cytologic evaluation demonstrated significant eosinophilic infiltration. Subsequent ophthalmoscopic examination revealed optic nerve atrophy. Upon further questioning, the patient admitted to vigorous itching of the affected eye for many months. Given the presenting symptoms, history, and negative ophthalmological workup, it was determined that the optic nerve atrophy was likely secondary to digital pressure from vigorous itching. Although AC can be a significant source of decreased vision via corneal ulceration, no reported cases have ever described AC-induced vision loss of this degree from vigorous itching and chronic pressure leading to optic nerve damage. Despite being self-limiting in nature, allergic conjunctivitis should be properly managed as extreme cases can result in mechanical compression of the optic nerve and compromise vision.
Full Text Available Pier Luigi Dodi Department of Veterinary Medicine Sciences, University of Parma, Parma, Italy Abstract: Keratoconjunctivitis sicca (KCS is a frequent canine ophthalmic disease, resulting from the deficiency of one or more elements in the precorneal tear film. There are different known causes of KCS in dogs, including congenital, metabolic, infectious, drug induced, neurogenic, radiation, iatrogenic, idiopathic, and immune mediated, though the last one is the most prevalent form in dogs. Initially, clinical signs of KCS include blepharospasm caused by ocular pain, mucoid to mucopurulent ocular discharge, and conjunctival hyperemia; secondary bacterial infection may also occur, with chronicity, corneal epithelial hyperplasia, pigmentation, neovascularization, and corneal ulceration. The diagnosis of KCS is based on the presence of consistent clinical signs and measurement of decreased aqueous tear production using the Schirmer tear test. Therapy is based on administering the following topical drugs: ocular lubricant, mucolytics, antibiotics, corticosteroids, pilocarpine, and immunomodulators. These last drugs (eg, cyclosporine, pimecrolimus, and tacrolimus have immunosuppressive activity and stimulate tear production. Furthermore, the nerve growth factor is a new subject matter of the research. Although these therapies are advantageous, stimulation of natural tear production seems to provide the highest recovery in clinical signs and prevention of vision loss. The goal of the following article is to describe the recent developments about KCS in dogs emphasizing the use of new therapies. Keywords: dogs, keratoconjunctivitis sicca, treatment, NGF
Sherman, Amanda; Daniels, Joshua B; Wilkie, David A; Lutz, Elizabeth
A 5-year-old spayed female diabetic mixed-breed dog underwent phacoemulsification and intraocular lens implantation to correct bilateral hypermature cataracts. Two months postsurgery, the patient presented with ulcerative keratitis and multifocal stromal abscessation OD, which was controlled, but never resolved, with topical fluoroquinolone therapy. The patient re-presented 2 months later with a new, raised, white gritty corneal opacity associated with hyperemia, chemosis, and blepharospasm OD. Cytology of the right cornea revealed filamentous bacteria, suggestive of Actinomyces spp. Actinomyces bowdenii was subsequently isolated in pure culture and identified via 16s rDNA sequencing. Actinomyces bowdenii has never before been described as a cause of ocular infection. An immunosuppressed corneal environment likely contributed to this opportunistic Actinomycosis. The infection was not controlled with fluoroquinolone therapy, and the isolate, in vitro, was resistant to three fluoroquinolones (ciprofloxacin, ofloxacin, and levofloxacin), which also has not been previously reported for this species of Actinomyces. A superficial keratectomy with conjunctival graft was employed to successfully manage the infection. PMID:23121462
Full Text Available Avaliaram-se os aspectos clínicos do entrópio de desenvolvimento em filhotes de cães da raça Shar Pei. Utilizaram-se 50 animais com idades entre 18 e 128 dias, que apresentavam graus variáveis de inversão palpebral e lesões oculares, as quais foram classificadas de acordo com um modelo específico proposto. As lesões encontradas foram fotofobia, epífora, inversão da margem palpebral, blefarospasmo, conjuntivite, quemose, edema, erosão e vascularização da córnea e phthisis bulbi.The clinical aspects of entropion in puppies of the Shar Pei breed were studied using 50 animals, 18 to 128 day-old, that presented variable degrees of palpebral inversion and ocular lesions. The ocular lesions were classified according to a specific model. Photophobia, epiphora, palpebral margins inversion, blepharospasm, conjuntivitis, quemosis, corneal edema, erosion and vascularization and phthisis bulbi were observed.
Ryle, James P.; Al-Kalbani, Mohammed; Gopinathan, Unnikrishnan; Boyle, Gerard; Coakley, Davis; Sheridan, John T.
Ocular microtremor (OMT) is a physiological high frequency (up to 150Hz) low amplitude (150-2500nm) involuntary tremor of the human eye. It is one of the three fixational ocular motions described by Adler and Fliegelman in 1934 as well as microsaccades and drift. Clinical OMT investigations to date have used eye-contacting piezoelectric probes or piezoelectric strain gauges. Before contact can be made, the eye must first be anaesthetised. In some cases, this induces eyelid spasms (blepharospasm) making it impossible to measure OMT. Using the contact probe method, the eye motion is mechanically damped. In addition to this, it is not possible to obtain exact information about the displacement. Results from clinical studies to date have given electrical signal amplitudes from the probe. Recent studies suggest a number of clinical applications for OMT, these include monitoring the depth of anaesthesia of a patient in surgery, prediction of outcome in coma, diagnosis of brainstem death. In addition to this, abnormal OMT frequency content is present in patients with neurological disorders such as Multiple sclerosis and Parkinson's disease. However for ongoing clinical investigations the contact probe method falls short of a non-contact accurate measurement solution. In this paper, we design a compact non contact phase modulating optical fiber speckle interferometer to measure eye motions. We present our calibration results using a calibrated piezoelectric vibration simulator. Digital signal processing is then performed to extract the low amplitude high frequency displacement information.
Extracapsular cataract extraction in horses by nuclear fragmentation using adapted instruments Facectomia extracapsular em eqüinos mediante núcleo fragmentação, com o emprego original de instrumentais adaptados
Full Text Available It was studied the nuclear fragmentation technique for lens extraction in six adult horses using adapted surgical instruments. Postoperative assessment revealed a reduction of intraocular pressure in all animals during the first postoperative days, as well as varying degrees of photophobia, blepharospasm, corneal edema, and iridocyclitis. Fibrin formation mainly located in the axial portion of the pupilla was noted in most cases, affecting or impairing vision.Estudou-se a técnica de núcleo-fragmentação para a extração da lente em seis eqüinos adultos, utilizando-se instrumentais cirúrgicos adaptados. Nas avaliações pós-operatórias, verificou-se diminuição da pressão intra-ocular, em todos os animais, nos primeiros dias de pós-operatório e, ainda, fotofobia, blefarospasmo, edema de córnea e iridociclites, em graus diversos. Observou-se produção de fibrina que, na maioria dos casos, localizava-se na porção axial da pupila, dificultando ou impedindo a visão.
Full Text Available Myotonia congenita (MC is a skeletal muscle channelopathy characterized by inability of the muscle to relax following voluntary contraction. Worldwide population prevalence in humans is 1:100,000. Studies in mice, dogs, humans and goats confirmed myotonia associated with functional defects in chloride channels and mutations in a skeletal muscle chloride channel (CLCN1. CLCN1 encodes for the most abundant chloride channel in the skeletal muscle cell membrane. Five random bred cats from Winnipeg, Canada with MC were examined. All cats had a protruding tongue, limited range of jaw motion and drooling with prominent neck and proximal limb musculature. All cats had blepharospasm upon palpebral reflex testing and a short-strided gait. Electromyograms demonstrated myotonic discharges at a mean frequency of 300 Hz resembling the sound of a 'swarm of bees'. Muscle histopathology showed hypertrophy of all fiber types. Direct sequencing of CLCN1 revealed a mutation disrupting a donor splice site downstream of exon 16 in only the affected cats. In vitro translation of the mutated protein predicted a premature truncation and partial lack of the highly conserved CBS1 (cystathionine β-synthase domain critical for ion transport activity and one dimerization domain pivotal in channel formation. Genetic screening of the Winnipeg random bred population of the cats' origin identified carriers of the mutation. A genetic test for population screening is now available and carrier cats from the feral population can be identified.
Guerreiro, Cleo E; Appelboam, Helen; Lowe, Robert C
A five-year-old entire male Tibetan Terrier was referred for left-sided periorbital swelling and blepharospasm 4 days following ipsilateral maxillary tooth extraction. Examination of the left eye revealed mild exophthalmos, pain on retropulsion, and absent menace response and pupillary light reflexes. Examination of the posterior segment was not possible owing to the anterior segment pathology. Differential diagnoses considered were iatrogenic globe penetration and peribulbar abscess/cellulitis. Ocular ultrasound was consistent with a penetrating wound to the globe. Treatment with systemic prednisolone and marbofloxacin, and topical atropine sulfate 1%, prednisolone acetate, and brinzolamide was started. Marked clinical improvement allowed visual confirmation of the perforation. Oral prednisolone was tapered over the following 10 weeks. At final re-examination (10 months), the patient was visual, and fundic examination revealed an additional chorioretinal scar, most likely an exit wound that was obscured by vitreal debris on initial examinations. Neither scar was associated with retinal detachment. To the authors' knowledge, this is the first reported case of successful medical management of iatrogenic globe penetration following exodontic procedures. PMID:23869648
Stades method for surgical correction of upper eyelid trichiasis-entropion: results and follow-up in 21 cases Método de Stades para a correção da triquíase-entrópio da pálpebra superior: resultados e acompanhamento de 21 casos
José Luiz Laus
Full Text Available Trichiasis is a condition in which lhe cuia and facial hairs grow toward lhe córnea or the conjunctiva. The hairs arising from normal sites are pointed aí an abnormal direction. This condition may be caused by prominent nasal folds, entropion, blepharospasm, slipped facial mask and dermoids. The upper eyelid trichiasis-entropion with lower eyelid entropionectropion frequentiy occurs in oíder English Cocker Spaniels. The ocular signs often are epiphora, blepharospasm, conjunctivitis, keratitis and comeal ulceratíon. Treatment depenas on the severity ofthe condition and must eliminate the ocular contact by misdirected cuia that irritate the eyeball. This report presents a retrospective study of21 patients with bilateral diffüse trichiasis (15 English Cocker Spaniels; 2 Basset hounds; l Bloodhound; l Fila Brasileiro and 2 mongrel dogs. The procedure described by Stades was employed m ali cases. Postoperatively, topical chioramphenicol oiníment (qid was appiied in the conjunctival soe and on the open woundfor 2 weeks. Sutures were removed 10 days after surgery. Correction ofpositioning ofthe upper eyelid was successfúl and its apposition to córnea was normal. In most of the cases the reepithelialiwtion was complete one month after surgery. No signs ofrecurrence werefound and there appeared to be no loss of normal fünction of the eyelid in the 21 dogs available for follow-up examination in a maximum period of 36 months.Triquíase é a condição na qual os cílios e os cabelos faciais crescem em direção à córnea ou conjuntiva. Os pêlos que surgem de locais normais estão apontados em uma direção anormal. Essa condição pode ser causada por dobras nasais proeminentes, entrópio, blefarospasmo, pele facial redundante e dennóides. A triquíase-entrópio da pálpebra superior associada ao entrópio-ectrópio da pálpebra inferior, frequentemente, ocorre em English Cocker Spaniels idosos. Os sinais oculares são frequentemente ep
Hertenstein, Elisabeth; Tang, Nicole K Y; Bernstein, Celia J; Nissen, Christoph; Underwood, Martin R; Sandhu, Harbinder K
Patients with primary dystonia, the third most prevalent movement disorder, suffer from a markedly reduced quality of life. This might, at least in part, be mediated by non-motor symptoms, including sleep disturbances. Characterising and treating sleep disturbances might provide new inroads to improve relevant patient-centred outcomes. This review evaluates the state of research on sleep in patients with dystonia and outlines an agenda for future research. A literature search was performed in July 2014 using PubMed, Medline via Ovid, PsycInfo, PsycArticles via Proquest and Embase via Ovid. Search results were screened for eligibility by two independent raters. Peer-reviewed publications reporting on sleep in patients with primary dystonia were included. Of 1445 studies identified through the search strategy, 18 met the inclusion criteria. In total, the included studies reported on 708 patients diagnosed with focal dystonia (cervical dystonia or blepharospasm), torsion dystonia, and dopa-responsive dystonia. The results indicate that at least half of the patients with focal cranial dystonia suffer from sleep disturbances, but excessive daytime sleepiness is uncommon. Sleep disturbance is associated with depressive symptoms. The frequency and duration of dystonic movements is markedly reduced during sleep. Reduced sleep quality appears to persist after treatment with botulinum toxin that successfully reduces motor symptoms. The findings are limited by a high clinical and methodological heterogeneity. Future research is needed to i) further characterize subjective and PSG sleep in patients with different types of dystonia, ii) determine the aetiology of sleep disturbances (e.g., abnormal brain function associated with dystonia, side effects of medication, psychological reasons), and iii) test whether targeted sleep interventions improve sleep and quality of life in patients with primary dystonia. PMID:26164369
Fidias E. Leon-Sarmiento
Full Text Available Blinking is a normal human phenomenom involving trigeminal and facial patways. To gain understanding on the neurobiology of blinking, five normal subjects were investigated before and after application of transdermal capsaicin at the forehead for two weeks. No effects of topical capsaicin were detected in eye blink rates. However, when capsaicin was applied to a female subject with blepharospasm, she showed a dramatic restoration of her vision subsequent to blinking modification. Deactivation of abnormal A-to-C fibers cross talks at the trigeminal-facial pathways seems to be the most likely mechanism of such improvement.El parpadeo es un fenómeno normal en los humanos que involucra las vías trigéminas faciales. Con el fin de conocer un poco más la neurobiología de este fenómeno estudiamos cinco individuos normales antes y después de aplicar capsaicina trasdérmica en la frente de cada uno de ellos, por dos semanas. La frecuencia de parpadeo no se alteró con la aplicación de capsaicina tópica. Sin embargo, cuando la misma sustancia se aplicó a una paciente con blefaroespasmo hubo dramática restauración de su visión, la cual fue secundaria a la modificación de la actividad muscular palpebral. La desactivación del cruce patológico de información que pasa de las fibras A a las fibras C, pertenecientes a las vías trigémino-faciales, parece ser el mecanismo de acción relacionado con la aplicación de capsaicina, el que estaría directamente relacionado con la recuperación clínica observada en la paciente con blefaroespasmo.
Ugarte, Marta; Teimory, Masoud
We describe eyelid movement abnormalities in an 80-year-old man with apraxia of lid opening (ALO), resulting from involuntary levator palpebrae inhibition (ILPI) and pretarsal orbicularis oculi (OO) contraction. He was unable to open his lids at will following closure. Attempted eye opening resulted in forceful contraction of the frontalis muscle, backward thrusting of the head and lengthened lid closure. The inability to reopen the lids was not evident during spontaneous reflex blinking and he had no difficulty in keeping the lids open once they had been manually lifted up. There were no episodes of involuntary drooping of the eyelids or spasmodic contraction of the OO causing involuntary eyelid closure. Pursuit eye movements were not restricted, the vestibulo-ocular reflex was preserved and both horizontal and vertical saccades were normal. Despite the clinically visible persistence of pretarsal OO activity, treatment with botulinum toxin injections in the pretarsal and preseptal portions of the muscle did not reduce his difficulty in initiating lid elevation but he found some benefit using lid crutches. ALO is thought to be due to an abnormality in the supranuclear control of eyelid movement. ILPI can present either isolated or combined with blepharospasm. The excitatory levator palpebrae response necessary to lift the lids up is likely to be in very close connection with the OO antagonistic inhibitory response. Alterations in one or another pre-motor structure may result in inability to raise the lids due to inhibition of the levator palpebrae as well as persistence of the pretarsal OO. PMID:17576707
Truong Daniel D
Full Text Available Abstract Background Although the c.904_906delGAG mutation in Exon 5 of TOR1A typically manifests as early-onset generalized dystonia, DYT1 dystonia is genetically and clinically heterogeneous. Recently, another Exon 5 mutation (c.863G>A has been associated with early-onset generalized dystonia and some ΔGAG mutation carriers present with late-onset focal dystonia. The aim of this study was to identify TOR1A Exon 5 mutations in a large cohort of subjects with mainly non-generalized primary dystonia. Methods High resolution melting (HRM was used to examine the entire TOR1A Exon 5 coding sequence in 1014 subjects with primary dystonia (422 spasmodic dysphonia, 285 cervical dystonia, 67 blepharospasm, 41 writer's cramp, 16 oromandibular dystonia, 38 other primary focal dystonia, 112 segmental dystonia, 16 multifocal dystonia, and 17 generalized dystonia and 250 controls (150 neurologically normal and 100 with other movement disorders. Diagnostic sensitivity and specificity were evaluated in an additional 8 subjects with known ΔGAG DYT1 dystonia and 88 subjects with ΔGAG-negative dystonia. Results HRM of TOR1A Exon 5 showed high (100% diagnostic sensitivity and specificity. HRM was rapid and economical. HRM reliably differentiated the TOR1A ΔGAG and c.863G>A mutations. Melting curves were normal in 250/250 controls and 1012/1014 subjects with primary dystonia. The two subjects with shifted melting curves were found to harbor the classic ΔGAG deletion: 1 a non-Jewish Caucasian female with childhood-onset multifocal dystonia and 2 an Ashkenazi Jewish female with adolescent-onset spasmodic dysphonia. Conclusion First, HRM is an inexpensive, diagnostically sensitive and specific, high-throughput method for mutation discovery. Second, Exon 5 mutations in TOR1A are rarely associated with non-generalized primary dystonia.
Full Text Available Purpose: To study the knowledge, attitude and practices (KAP towards computer vision syndrome prevalent in Indian ophthalmologists and to assess whether ′computer use by practitioners′ had any bearing on the knowledge and practices in computer vision syndrome (CVS. Materials and Methods: A random KAP survey was carried out on 300 Indian ophthalmologists using a 34-point spot-questionnaire in January 2005. Results: All the doctors who responded were aware of CVS. The chief presenting symptoms were eyestrain (97.8%, headache (82.1%, tiredness and burning sensation (79.1%, watering (66.4% and redness (61.2%. Ophthalmologists using computers reported that focusing from distance to near and vice versa ( P =0.006, χ2 test, blurred vision at a distance ( P =0.016, χ2 test and blepharospasm ( P =0.026, χ2 test formed part of the syndrome. The main mode of treatment used was tear substitutes. Half of ophthalmologists (50.7% were not prescribing any spectacles. They did not have any preference for any special type of glasses (68.7% or spectral filters. Computer-users were more likely to prescribe sedatives/ anxiolytics ( P = 0.04, χ2 test, spectacles ( P = 0.02, χ2 test and conscious frequent blinking ( P = 0.003, χ2 test than the non-computer-users. Conclusions: All respondents were aware of CVS. Confusion regarding treatment guidelines was observed in both groups. Computer-using ophthalmologists were more informed of symptoms and diagnostic signs but were misinformed about treatment modalities.
Xiao, Jianfeng; Bastian, Robert W; Perlmutter, Joel S; Racette, Brad A; Tabbal, Samer D; Karimi, Morvarid; Paniello, Randal C; Blitzer, Andrew; Batish, Sat Dev; Wszolek, Zbigniew K; Uitti, Ryan J; Hedera, Peter; Simon, David K; Tarsy, Daniel; Truong, Daniel D; Frei, Karen P; Pfeiffer, Ronald F; Gong, Suzhen; Zhao, Yu; LeDoux, Mark S
Background Although the c.904_906delGAG mutation in Exon 5 of TOR1A typically manifests as early-onset generalized dystonia, DYT1 dystonia is genetically and clinically heterogeneous. Recently, another Exon 5 mutation (c.863G>A) has been associated with early-onset generalized dystonia and some ΔGAG mutation carriers present with late-onset focal dystonia. The aim of this study was to identify TOR1A Exon 5 mutations in a large cohort of subjects with mainly non-generalized primary dystonia. Methods High resolution melting (HRM) was used to examine the entire TOR1A Exon 5 coding sequence in 1014 subjects with primary dystonia (422 spasmodic dysphonia, 285 cervical dystonia, 67 blepharospasm, 41 writer's cramp, 16 oromandibular dystonia, 38 other primary focal dystonia, 112 segmental dystonia, 16 multifocal dystonia, and 17 generalized dystonia) and 250 controls (150 neurologically normal and 100 with other movement disorders). Diagnostic sensitivity and specificity were evaluated in an additional 8 subjects with known ΔGAG DYT1 dystonia and 88 subjects with ΔGAG-negative dystonia. Results HRM of TOR1A Exon 5 showed high (100%) diagnostic sensitivity and specificity. HRM was rapid and economical. HRM reliably differentiated the TOR1A ΔGAG and c.863G>A mutations. Melting curves were normal in 250/250 controls and 1012/1014 subjects with primary dystonia. The two subjects with shifted melting curves were found to harbor the classic ΔGAG deletion: 1) a non-Jewish Caucasian female with childhood-onset multifocal dystonia and 2) an Ashkenazi Jewish female with adolescent-onset spasmodic dysphonia. Conclusion First, HRM is an inexpensive, diagnostically sensitive and specific, high-throughput method for mutation discovery. Second, Exon 5 mutations in TOR1A are rarely associated with non-generalized primary dystonia. PMID:19284587
Full Text Available Avram Fraint,1 Padmaja Vittal,2 Cynthia Comella2 1Department of Neurological Sciences, 2Section of Movement Disorders, Rush University Medical Center, Chicago, IL, USA Introduction: Botulinum toxin (BoNT is the treatment of choice for many neurologic movement disorders, including blepharospasm, hemifacial spasm, and cervical dystonia. There are two serotypes approved for use by the US Food and Drug Administration: three brands of serotype A and one of serotype B. Many attempts have been made at establishing dose conversion ratios between brands and serotypes. This review focuses on the existing data comparing different formulations of the same BoNT serotypes as well as that comparing different serotypes with one another. We focus on existing data regarding switching from one formulation or serotype to another and will also discuss the issue of immunogenicity of BoNT. With this information as a foundation, recommendations on safety of switching agents are addressed. Method: Literature review searching PubMed and Google Scholar using the search terms “switching botox”, “dosing equivalency in botox”, and “comparing botox”. Results/conclusion: Overall, there are many studies that demonstrate the efficacy and safety of each of the brands of BoNTs used in clinical practice. However, determination of dosing equivalencies among these brands and serotypes is complex with inconsistencies among the studies. When switching from one brand to another, the clinician should be aware of these issues, and not make the assumption that such ratios exist. Tailoring the dosage of each brand of BoNT to the clinical situation is the most prudent treatment strategy rather than focusing closely on conversion factors and concerns for immunogenicity. Keywords: botulinum toxin, BoNT, abobotulinumtoxin A, onabotulinumtoxin A, incobotulinumtoxin A, rimabotulinumtoxin B
In recent years, with the deepening study on the mechanism of botulinum toxin type A, the range of its applica -tions in the nervous system diseases continues to expand. Because botulinum toxin type A produces partial chemical dener-vation of the muscle resulting in a localized reduction in muscle activity, it can be used to treat hemifacial spasm, idio -pathic blepharospasm, oromandibular dystonia, spasmodic torticollis, tic disorder, limb spasticity after stroke and spastic cerebral palsy. In addition, it has recently begun to show some benefit in the control of hyperhidrosis ang excessive saliva -tion disease on account of its effects of the autonomic nervous system. It may also prove useful in treatment of a variety of headache and neuralgia owing to its analgesic effects. Therefore, botulinum toxin type A has become an important treatment option in the field of neurology.%近年来随着A型肉毒毒素作用机制研究的不断深入,其在神经系统疾病中的应用范围日渐扩大.因其肌肉化学性去神经支配效应可用于治疗面肌痉挛、特发性眼睑痉挛、口下颌肌张力障碍、痉挛性斜颈、抽动障碍、脑卒中后肢体痉挛和痉挛性脑瘫;因其植物神经系统效应可用于治疗多涎病症和多汗证;因其镇痛效应可用于治疗各种头痛和神经痛,故A型肉毒毒素已逐渐成为神经科领域一种重要的治疗手段.
Background: Corneal pannus is a disease which, if untreated, nearly always is progressive and may lead to blindness of the affected dog. A therapeutic standard is yet to be defined. Beta-ray irradiation with Sr-90 is often recommended on a casuistic basis, but systematic studies are sparse. The aim of the present study was to evaluate efficacy and to document side effects of radiotherapy with Sr-90. Material and Methods: 17 animals were treated. 13 of them received treatment of 15 Gy surface dose twice within 2 days with additional medical therapy with ciclosporin and prednisolon. Only the more affected eye was treated with radiation which was applied with an eye-applicator, the other eye served as control. Four animals with already advanced impairment of vision received keratectomy, afterwards radiation was applied on both sides. Results: Medical treatment alone led to deterioration in vascularization and spread of pigmentation in eleven of 13 (85%) of the control-eyes, density of pigmentation increased in eight of 13 (62%). After radiation therapy, almost all animals showed a marked initial improvement. Even if progressive disease occurred later on, further worsening as it happened in the control-eyes could be stopped in nine resp. ten of 13 eyes (69% and 77%). All animals with keratectomy and radiotherapy regained and preserved adequate vision. Besides short-term blepharospasm, no side effects were recorded. Conclusion: Corneal pannus is responsive to radiation therapy with Sr-90 and long-term benefit can be achieved. Side effects are minimal. Optimal sequencing of therapy and dosage still have to be examined. (orig.)
José Luiz Laus
Full Text Available Entropion is defined as the inward rolling of the eyelid margin in which the eyelashes and eyelid hair (frequently the lower lateral lid rub the cornea. Etiologies may be congenital, spastic, or cicatricial. This condition usually causes epiphora, blepharospasm, photophobia, conjunctivitis, purulent discharge, corneal vascularization, pigmentation and ulceration, if not surgically treated. Congenital entropion commonly affects dogs and is frequently hereditary in some breeds, whereas cats are uncommonly affected. A predilection for the Persian breed to present primary entropion has been suggested. The authors report two cases of entropion in Persian cats referred to the Ophthalmology Section of Veterinary College of São Paulo State University - UNESP, Jaboticabal - SP / Brazil. First case: a male Persian cat, 2 years old, with a history of bilateral ocular irritation and purulent discharge for 8 months. Ophthalmic examination revealed epiphora, blepharospasm, photophobia, bilateral entropion affecting the whole length of the lower eyelids, conjunctivitis and purulent discharge. Second case: a male Persian cat, 1 year old, with a history of bilateral ocular irritation and purulent discharge for 3 weeks. At ophthalmic examination the animal was presenting epiphora, blepharospasm, photophobia, bilateral entropion affecting the whole length of the lower lids, conjunctivitis, purulent discharge, corneal vascularization, superficial ulceration and edema. The entropion persisted after topical anaestesia in both cats. Surgical treatment was similar in both cases, based on the modified Holtz-Celsus procedure. Grid keratotomy procedure was also performed in the second case. Both cats had a satisfactory clinic evolution which was confirmed few days after surgery.O entrópio é caracterizado por uma inversão da margem palpebral, na qual os cílios e os pêlos da pálpebra (freqüentemente a porção lateral da pálpebra inferior atritam a córnea. A
Geraldo de Barros Ribeiro
Full Text Available PURPOSE: Crotoxin is the main neurotoxin of South American rattlesnake Crotalus durissus terrificus. The neurotoxic action is characterized by a presynaptic blockade. The purpose of this research is to assess the ability of crotoxin to induce temporary paralysis of extraocular and facial muscles in humans. METHODS: Doses of crotoxin used ranged from 2 to 5 units (U, each unit corresponding to one LD50. We first applied 2U of crotoxin in one of the extraocular muscles of 3 amaurotic individuals to be submitted to ocular evisceration. In the second stage, we applied crotoxin in 12 extraocular muscles of 9 patients with strabismic amblyopia. In the last stage, crotoxin was used in the treatment of blepharospasm in another 3 patients. RESULTS: No patient showed any systemic side effect or change in vision or any eye structure problem after the procedure. The only local side effects observed were slight conjunctival hyperemia, which recovered spontaneously. In 2 patients there was no change in ocular deviation after 2U crotoxin application. Limitation of the muscle action was observed in 8 of the 12 applications. The change in ocular deviation after application of 2U of crotoxin (9 injections was in average 15.7 prism diopters (PD. When the dose was 4U (2 applications the change was in average 37.5 PD and a single application of 5U produced a change of 16 PD in ocular deviation. This effect lasted from 1 to 3 months. Two of the 3 patients with blepharospasm had the hemifacial spasm improved with crotoxin, which returned after 2 months. CONCLUSIONS: This study provides data suggesting that crotoxin may be a useful new therapeutic option for the treatment of strabismus and blepharospasm. We expect that with further studies crotoxin could be an option for many other medical areas.OBJETIVO: A crotoxina é a principal neurotoxina da cascavel sul-americana Crotalus durissus terrificus e sua ação neurotóxica caracteriza-se por um bloqueio pr
sessões de 17,4 semanas (variação 16-18. Ela se tornou não-respondedora após a nona sessão. Primeiro tratamento com BTX-B, 6000U, recebeu escore 0. A segunda sessão, 12000U, produziu escore 4.Paciente 4- Com a idade de 69 anos este homem desenvolveu distonia cranial idiopática. Antes de ser atendido por mim, ele recebeu 6 sessões de BTX-A em outros serviços. Na minha instituição ele foi tratado com dose acumulada de 730U em 4 sessões com intervalo médio entre sessões de 16,3 semanas (variação 15-18, havendo perdido a resposta na sexta sessão. Tratamento com BTX-B, 12000U, produziu escore 4 e durou 20 semanas. Efeitos colaterais: dor local (todos os pacientes e ressecamento da boca e ptose palpebral (um paciente cada. CONCLUSÃO: Meus achados confirmam que injeções de BTX-B são eficazes e seguras para pacientes não-respondedores secundários a BTX-A. Os resultados também mostram ser necessário individualizar a dose de BTX-B para se alcançar melhores resultados.BACKGROUND: Botulinum toxin (BTX injection is the first choice treatment for focal dystonias. However 10% or more of patients who receive repetitive injections of BTX type A (BTX-A lose response (secondary non-responders. One of the strategies to manage such patients is to treat them with another serotype. The aim of this article is to describe my experience with BTX type B (BTX-B in the management of patients with focal dystonia who became secondary non-responders to BTX-A. METHOD: Open-label non-controlled use of BTX-B injections to treat dystonia patients who developed secondary nonresponse to BTX-A Response to treatment was rated on a 0-4 scale (Jankovic. RESULTS: Four patients entered the study. Pacient 1- At age 48 this man developed idiopathic cervical dystonia. Five years later he also presented with blepharospasm and idiopathic oromandibular dystonia. He was treated with 7604U of BTX-A along 23 sessions separated by a mean interval of 18.8 weeks (range 6-39. Loss of response was
Experimental lamellar keratoplasty in rabbits using microfibrilar cellulose membrane: clinical, morphological and immunohistochemical findings Ceratoplastia lamelar experimental em coelhos usando membrana microfibrilar de celulose: achados clínicos, morfológicos e imunoistoquímicos
Luciana Riacciardi Macedo
Full Text Available The clinical, histopathological and immunohistochemical features of the cornea were investigated in adult male New Zealand rabbits submitted to lamellar keratoplasty with microfibrillar cellulose membrane. Thirty animals were divided into five groups (n=6 and evaluated up to 60 days after surgery. Clinical examination revealed moderate manifestations of edema, blepharospasm and photophobia on the second day, which became mild or disappeared after the seventh day. This period was characterized clinically by repair of the corneal defect. Histopathological analysis showed the presence of a thin layer of squamous cells covering the damaged area as early as 7th day, accompanied by a mild infiltrate of polymorphonuclear cells. Blood vessels were observed in the epithelium after the 15th day, which had regressed by day 48. Ki67 antibody labeling showed an increase of proliferating cells in the epithelium by the 15th day and in the stroma by day 30. Remodeling and epithelial adhesion were observed during this period. Microfibrillar cellulose membrane (Bionext® used for lamellar keratoplasty was found to yield good results considering the good integration of the implant.Avaliaram-se aspectos clínicos, histopatógicos e imunoistoquímicos da córnes de coelhos da raça Nova Zelândia adultos e machos em ceratoplastias lamelares com membrana de celulose microfibrilar. Trinta animais distribuídos em cinco grupos (n=6 foram estudados por até 60 dias de pós-operatório. A avaliação clínica revelou manifestações moderadas de edema, blefaroespasmo e fotofobia ao segundo dia, evoluindo para formas discretas ou ausentes a partir do sétimo dia, período em que se observou, clinicamente, reparo do defeito corneal. A histopatologia revelou uma fina camada de células escamosas, recobrindo a área lesada já aos sete dias, com discreto infiltrado de células polimorfonucleares. Observaram-se vasos no epitélio a partir do 15o dia, com regressão ao 48o dia
Ghika, J; Ghika-Schmid, F; Fankhauser, H; Assal, G; Vingerhoets, F; Albanese, A; Bogousslavsky, J; Favre, J
The authors report the underestimated cognitive, mood, and behavioral complications in patients who have undergone bilateral contemporaneous pallidotomy, as seen in their early experience with functional neurosurgery for Parkinson's disease (PD) that is accompanied by severe motor fluctuations before pallidal stimulation. Four patients, not suffering from dementia, with advanced (Hoehn and Yahr Stages III-IV), medically untreatable PD featuring severe "on-off" fluctuations underwent bilateral contemporaneous posteroventral pallidotomy (PVP). All patients were evaluated according to the Core Assessment Program for Intracerebral Transplantations (CAPIT) protocol without positron emission tomography scans but with additional neuropsychological cognitive, mood, and behavior testing. For the first 3 to 6 months postoperatively, all patients showed a mean improvement of motor scores on the Unified Parkinson's Disease Rating Scale (UPDRS), in the best "on" (21%) and worst "off" (40%) UPDRS III motor subscale, a mean 30% improvement in the UPDRS II activities of daily living (ADL) subscore, and 60% on the UPDRS IV complications of treatment subscale. Dyskinesia disappeared almost completely, and the mean daily duration of the off time was reduced by an average of 60%. Despite these good results in the CAPIT scores, one patient experienced a partially regressive corticobulbar syndrome with dysphagia, dysarthria, and increased drooling. No emotional lability was found in this patient, but he did demonstrate severe bilateral postoperative pretarsal blepharospasm (apraxia of eyelid opening), which interfered with walking and which required treatment with high-dose subcutaneous injections of botulinum toxin. No patient showed visual field defects or hemiparesis, but postoperative depression, changes in personality, behavior, and executive functions were seen in two individuals. Postoperative abulia was reported by the family of one patient, who lost his preoperative
aspects were: ocular secretion, signs of discomfort, blepharospasm and cosmetic effect. Results turned out to be satisfactory in eight animals. It was concluded that acrylic resin could be an alternative prosthesis for intraocular implant.
Full Text Available Giorgio Marchini, Marco Toscani, Francesca Chemello Eye Clinic, Department of Neurological and Movement Sciences, University of Verona, Verona, Italy Abstract: “Childhood glaucoma” is a heterogeneous group of severe pediatric conditions often associated with significant visual loss and characterized by elevated intraocular pressure (IOP and optic-disk cupping. Successful IOP control is crucial but challenging and most often achieved surgically, with medical therapy playing a supportive role. There are many classifications of childhood glaucoma, but they can simply be divided into primary, in which a developmental abnormality of the anterior chamber angle only exists, and secondary, in which aqueous outflow is reduced due to independent mechanisms that secondarily impair the function of the filtration angle. The worldwide prevalence of childhood blindness ranges from 0.03% in high-income countries to 0.12% in undeveloped countries. The majority of cases do not have an identified genetic mutation and, where the mutation is known, the genes often account for only a small proportion of cases. Several pathogenetic mechanisms are known to contribute to the development of childhood glaucoma. Whatever the cause, it results in a reduced aqueous outflow at the level of the trabecular meshwork. Age of onset and magnitude of the elevated IOP largely determine the clinical manifestation the high variability of clinical manifestations. Glaucoma from any cause in a neonate and infant is characterized by the classic triad of epiphora, photophobia, and blepharospasm, and could be associated with eye enlargement (buphthalmos and Haab striae. The eye examination, usually performed under general anesthesia, includes: tonometry, anterior-segment examination, gonioscopy, corneal diameter and axial length measurement, dilated fundoscopy with optic-nerve-head evaluation. Medical therapy, considering the high frequency of side effects, is generally used as
Avaliação do filme lacrimal de pacientes com distonia facial durante tratamento com toxina botulínica tipo A Lacrimal film evaluation of patients with facial dystonia during botulinum toxin type A treatment
Patricia Grativol Costa
Full Text Available OBJETIVO: Determinar o efeito da toxina botulínica no filme lacrimal em pacientes com distonia facial. MÉTODOS: Foram incluídos 24 pacientes portadores de blefaroespasmo essencial e espasmo hemifacial que receberam aplicação de toxina botulínica tipo A que foram submetidos à propedêutica do filme lacrimal previamente à aplicação e após, com 7 e 30 dias. RESULTADOS: Houve diminuição das queixas de olho seco trinta dias após a aplicação, entretanto, o tempo de ruptura do filme lacrimal e o teste de Schirmer não demonstraram variação significativa entre os períodos pré-tratamento e 1 mês da aplicação. Em relação ao teste de coloração com rosa bengala, todos os olhos que coraram no pré-tratamento, melhoraram na última avaliação. CONCLUSÃO: A injeção de toxina botulínica pode aliviar as queixas de olho seco nos pacientes com distonia facial pela provável ação de inibição do orbicular na sua função de bomba lacrimal.PURPOSE: To determine the effect of botulinum toxin injection in the eyelid on lacrimal film in patients with facial dystonia. METHODS: Twenty-four patients with essential blepharospasm and hemifacial spasm were submitted to botulinum toxin injection and lacrimal film tests were performed before the application and after seven and thirty days. RESULTS: There was improvement in symptoms of dry eye and rose bengal test, however, the breakup time and Schirmer's test did not show significant variation between pretreatment and after 1 month of follow-up. CONCLUSION: The dry eye symptoms in patients with facial dystonia may be attenuated by botulinum toxin due to its possible inhibitory effect on the orbicular muscle leading to a decrease in lacrimal pump.
Shuhui Wu; Huifang Shang; Xiaoyi Zou
etiopathogenesis of several primary dystonias has been observed at the molecular level. CONCLUSION: Various abnormalities of the dopaminergic system exist for different forms of dystonia; therefore, much more research is needed in this area. At the molecular level, relationships between abnormalities of the dopaminergic system and etiopathogenesis of the following syndromes have been observed: dopa-responsive dystonia, early-onset torsion dystonia, X-linked dystonia-parkinsonism syndrome, myoclonus dystonia syndrome, primary cervical dystonia, and focal dystonia blepharospasm.
Bentivoglio, Anna Rita; Del Grande, Alessandra; Petracca, Martina; Ialongo, Tamara; Ricciardi, Lucia
In humans, the therapeutic use of botulinum neurotoxin A (BoNT/A) is well recognized and continuously expanding. Four BoNTs are widely available for clinical practice: three are serotype A and one is serotype B: onabotulinumtoxinA (A/Ona), abobotulinumtoxinA (A/Abo) and incobotulinumtoxinA (A/Inco), rimabotulinumtoxinB (B/Rima). A/Abo, A/Inco, A/Ona and B/Rima are all licensed worldwide for cervical dystonia. In addition, the three BoNT/A products are approved for blepharospasm and focal dystonias, spasticity, hemifacial spasm, hyperhidrosis and facial lines, with remarkable regional differences. These toxin brands differ for specific activity, packaging, constituents, excipient, and storage. Comparative literature assessing the relative safety and efficacy of different BoNT products is limited, most data come from reports on small samples, and only a few studies meet criteria of evidence-based medicine. One study compared the effects of BoNT/A and BoNT/B on muscle activity of healthy volunteers, showing similar neurophysiological effects with a dose ratio of 1:100. In cervical dystonia, when comparing the effects of BoNT/A and BoNT/B, results are more variable, some studies reporting roughly similar peak effect and overall duration (at a ratio of 1:66, others reporting substantially shorter duration of BoNT/B than BoNT/A (at a ratio 1/24). Although the results of clinical studies are difficult to compare for methodological differences (dose ratio, study design, outcome measures), it is widely accepted that: BoNT/B is clinically effective using appropriate doses as BoNT/A (1:40-50), injections are generally more painful, in most of the studies on muscular conditions, efficacy is shorter, and immunogenicity higher. Since the earliest clinical trials, it has been reported that autonomic side effects are more frequent after BoNT/B injections, and this observation encouraged the use of BoNT/B for sialorrhea, hyperhidrosis and other non-motor symptoms. In these
Fábio Luiz da Cunha Brito
Full Text Available A case of a 3-month-old female mongrel dog with a history of apathy and previous contact with an alkaline (sodium hydroxide is reported. The dog was reluctant to open the right eye. Ophthalmic examination revealed blepharospasm, photophobia, epiphora, discrete chemosis, conjunctival hyperemia, and diffuse corneal edema involving the limbus. The fluorescein test was positive and the result of the Schirmer tear test was 32mm min-1. On the basis of these findings the diagnosis was alkali-induced ulceration and limbal autograft transplantation was performed. Corneal vascularization was observed by the third postoperative day, with intensification in vessel number and caliber on subsequent days and small areas of corneal transparency. The present results show that limbal autograft transplantation is a feasible procedure for the therapeutic management of alkali-induced corneal ulcers.Descreve-se um caso de um animal da espécie canina, fêmea, sem raça definida, de três meses de idade, com histórico de apatia, contato prévio com produto alcalino (hidróxido de sódio e relutância em abrir o olho direito. Ao exame oftálmico, foram observados blefarospasmo, fotofobia, epífora, quemose discreta, hiperemia conjuntival, e edema corneal difuso com comprometimento do limbo. Foram realizados o teste da fluoresceína positivo e o Teste Lacrimal de Schirmer 32mm min-1. Com base nos achados, firmou-se o diagnóstico de úlcera por álcali e realizou-se transplante autógeno do limbo. No pós-operatório, observou-se vascularização corneal a partir do terceiro dia e sua intensificação, em número e calibre, nos dias subseqüentes. Também foram observadas mínimas áreas de transparência corneal. Os resultados obtidos permitem admitir que o transplante autógeno de limbo é procedimento factível para o manejo da terapia de úlceras de córnea por álcali.
Czapliński, Adam; Steck, Andreas J; Fuhr, Peter
streptococcal infection in the pathogenesis of tics. The differential diagnosis of tics is reviewed in detail. Above all tics represent a social disability. The ability to tolerate tics varies greatly from one individual to another, and the need for treatment is better defined by the patient than by the physician. Mild cases do not need be treated. Ideally, management should be multidisciplinary and can range from educative to supportive means or to intricate pharmacological interventions. The major form of treatment of the motor or vocal symptoms continues to be based on high-potency "typical" neuroleptics (tiaprid, pimozide, haloperidol), which induce a wide range of potentially serious side effects. In everyday practice we prefer to start with an "atypical" neuroleptic drug--for example, olanzapin (5-10 mg/day), risperidone or clozapine. Other drugs, such as clonidin or pergolid are widely used but their efficiency is still questionable. SSRIs (sertaline, citalopram, fluoxetin, fluvoxamine) or other antidepressants (clomipramine) have been used in treatment of psychiatric comorbid conditions, too. Botulinum toxin injections have proved useful in tics, targeting at the symptoms of blepharospasm, in neck and facial muscles. PMID:12185806
Effects of topical 2% cyclosporine A on the corneas of dogs subjected to lamellar keratoplasty with a graft of equine pericardium preserved in glycerin: clinical and morphological evaluation Efeitos da aplicação tópica da ciclosporina A a 2% sobre a córnea de cães submetidos à ceratoplastia lamelar com implante de pericárdio de eqüino preservado em glicerina: avaliação clínica e morfológica
Roseli Borges Teixeira
Full Text Available The effects of topical 2% cyclosporine A on the cornea of dogs subjected to experimental lamellar keratoplasty with an equine pericardial graft were evaluated. Ten dogs were grouped to be evaluated 3, 7, 15, 30 and 60 days after surgery. Animals received bilateral grafts followed by the application of a 2% cyclosporine A ointment on the left eye (treated eye and the ointment base on the right eye (control eye twice a day. The ophthalmic evaluation showed profound bilateral blepharospasm, photophobia and a mucous secretion until the 7th day after surgery; corneal vascularization starting in the limbus was observed as early as the 3rd day in both eyes; opacification and vascularization were more intense in the treated cornea on days 15 and 30 after surgery. Vascularization was still evident on the 60th day, and looked similar in treated eyes and control eyes. The histologic evaluation showed a complete bilateral reepithelization and corneal vascularization three days after the surgery; intense vascularization in both eyes on days 15 and 30, that was much more pronounced in the treated cornea; and a bilateral predominance of polymorphonuclear cells until day 15, and mononuclear cells on day 30. Intact epithelium and stroma with new vessels, as well as graft absorption, in both eyes, were seen on the 60th day. The lamellar keratoplasty with equine pericardial graft was an effective model to study the inflammatory kinetics and corneal vascularization phenomenon. In this study, cyclosporine A did not inhibit corneal vascularization and it did not interfere in the corneal cicatricial process.Foram avaliados os efeitos da ciclosporina A a 2% sobre a córnea de cães submetidos à ceratoplastia lamelar experimental com implante de pericárdio de equino. Dez cães foram divididos em grupos para estudo aos três, sete, 15, 30 e 60 dias de pós-operatório, recebendo implantes bilaterais e em seguida aplicação da pomada com ciclosporina A a 2% no olho
ppm produced extreme discomfort with severe lacrimation, blepharospasm, and painful breathing. None of the concentrations tested impaired motor coordination or performance on neurological tests. The irritating effects subsided within 15 min to 24 h of removal from the inhalation chamber. The National Institute of Occupational Safety and Health (NIOSH) recommended an 8-h time-weighted average for occupational exposure of 100 ppm. A margin of safety of 500 was determined, based on a calculated exposure from the normal use of nail polish remover products (100% absorption) and the NOAEL for reproductive toxicity. The absorption of Methoxyisopropanol through the nail is likely to be low, suggesting this margin of safety is conservative. Because Methoxyisopropanol is volatile, exposure by inhalation is possible, but the odor becomes objectionable at 50 to 75 ppm in air. The Cosmetic Ingredient Review (CIR) Expert Panel concluded that Methoxyisopropanol and Methoxyisopropyl Acetate are safe for use in nail care products in the practices of use and concentration as described in this safety assessment. PMID:18830862