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Kazuya Yoshida - One of the best experts on this subject based on the ideXlab platform.
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prevalence and incidence of Oromandibular Dystonia an oral and maxillofacial surgery service based study
Clinical Oral Investigations, 2021Co-Authors: Kazuya YoshidaAbstract:Oromandibular Dystonia is a focal Dystonia characterized by sustained or intermittent contractions of the masticatory and/or tongue muscles. This epidemiological study aimed to estimate the prevalence and incidence of Oromandibular Dystonia in Kyoto (population: 1,465,701). The population sample was citizens of Kyoto who visited our department between 2015 and 2019 and were differentially diagnosed by an Oromandibular Dystonia specialist having idiopathic (primary) and acquired (secondary) Oromandibular Dystonia. A total of 144 patients (100 women and 44 men; mean age, 57.5 years) were analyzed for clinical features, and the prevalence (prevalence date, January 1, 2020) and annual incidence were estimated. The male-to-female ratio was 1:2.3 (p<0.001). Age at onset was significantly (p<0.01) earlier in men (47.5 years) than that in women (56.9 years). The crude prevalence of Oromandibular Dystonia was estimated at 9.8 per 100,000 persons (95% confidence interval: 8.3–11.6) (idiopathic Dystonia, 5.7 [4.6–7.1]; tardive Dystonia, 3.4 [2.5–4.5]) and incidence at 2.0 (1.3–2.8) per 100,000 person-years (idiopathic Dystonia, 1.2 [0.68–1.9], tardive Dystonia, 0.68 [0.32–1.3]). The prevalence was 13.0 (10.5–15.8) in women and 6.3 (4.6–8.5) in men. All age groups showed female predominance. The highest prevalence was 23.6 (14.4–36.5) in women aged 60–69 years. As this is an oral and maxillofacial surgery service–based study, the actual prevalence of Oromandibular Dystonia may be even higher. It was suggested that Oromandibular Dystonia might be more common than cervical Dystonia or blepharospasm.
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development and validation of a disease specific Oromandibular Dystonia rating scale omdrs
Frontiers in Neurology, 2020Co-Authors: Kazuya YoshidaAbstract:Background: Oromandibular Dystonia manifests with sustained or task-specific contractions of the masticatory, tongue, and/or other muscles in the stomatognathic system. Since its symptoms can vary, it has been difficult to objectively measure disease severity and post-treatment changes. Objective: To develop and validate a comprehensive measurement tool for Oromandibular Dystonia. Methods: An examiner-rated scale included three subscales for severity, disability, and pain, modified specifically for Oromandibular Dystonia from the Toronto Western Spasmodic Torticollis Rating Scale-2. To evaluate the severity of each subtype of Oromandibular Dystonia, four of the six items were selected according to the subtype (jaw closing Dystonia, tongue Dystonia, jaw opening Dystonia, jaw deviation [protrusion] Dystonia, and lip Dystonia). A patient-administered questionnaire based on clinical features and other relevant aspects associated with Oromandibular Dystonia was developed, which included five subscales: general, eating, speech, cosmetic, and social/family life. The questionnaire, examiner-rated scale, and four subscales (sleep, annoyance, mood, and psychosocial functioning) of the Cervical Dystonia Impact Profile-58 were combined to construct the Oromandibular Dystonia rating scale (OMDRS). The reliability and validity of the scale were assessed using clinimetric testing. Results: Six hundred and eighteen patients with Oromandibular Dystonia (394 women and 224 men; mean age, 51.7 years) were evaluated by the OMDRS. The overall OMDRS showed high-level internal consistency measured by Cronbach's alpha (0.95) with a logical factor structure. Cronbach's alpha for the subscales was satisfactory to excellent, ranging from 0.72 to 0.94. All items revealed acceptable inter-rater reliability (kappa > 0.4, interclass correlation coefficient > 0.6). Repeated ratings of videotapes revealed acceptable intra-rater reliability for all items (kappa > 0.76, interclass correlation coefficient > 0.86). Test-retest reliability showed a significant (p < 0.001) correlation efficiency. The OMDRS showed significant (p < 0.001) convergent and discriminant validity and significant (p < 0.001) sensitivity to changes after botulinum toxin therapy. Conclusion: The OMDRS can be useful for the comprehensive evaluation of disease severity, disability, psychosocial functioning, and impact on the quality of life as well as therapeutic changes in patients with Oromandibular Dystonia.
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Oromandibular Dystonia screening questionnaire for differential diagnosis
Clinical Oral Investigations, 2019Co-Authors: Kazuya YoshidaAbstract:Oromandibular Dystonia, which is characterized by stereotypic, task-specific, or sustained contractions of masticatory and/or lingual muscles, is frequently misdiagnosed as tempOromandibular disorders or psychogenic disease. Diagnostic delay in Oromandibular Dystonia is not acceptable; thus, a screening tool that can distinguish this condition from a tempOromandibular disorder may be helpful for medical professionals unfamiliar with involuntary movements or tempOromandibular disorders. A questionnaire that included questions on the clinical features of Oromandibular Dystonia, such as stereotypy, task-specificity, sensory tricks, and morning benefit, and included questions to rule out tempOromandibular disorders (total point range 0–40) was administered to 553 patients suspected to have involuntary movements. Based on a careful examination and the differential diagnosis, the patients were divided into four groups: Oromandibular Dystonia (n = 385), oral dyskinesia (n = 84), psychogenic (functional) movement disorder (n = 50), and tempOromandibular disorders (n = 34). The questionnaire had a high level of internal consistency as measured by the Cronbach’s α (0.91), and item-total correlation was significant (p < 0.001). The test-retest reliability on two separate occasions showed a significant correlation (p < 0.001). Mean total scores of the questionnaire significantly differed among Oromandibular Dystonia (32.0), tempOromandibular disorders (10.4; one-way analysis of variance, p < 0.001), oral dyskinesia (21.0; p < 0.001), and psychogenic (functional) movement disorder (13.7; p < 0.001). Findings of this study suggest that the present questionnaire is a simple diagnostic tool that is useful for tentative differentiation of Oromandibular Dystonia from tempOromandibular disorders. This screening tool can be used to distinguish Oromandibular Dystonia from tempOromandibular disorders.
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multilingual website and cyberconsultations for Oromandibular Dystonia
Neurology International, 2018Co-Authors: Kazuya YoshidaAbstract:Oromandibular Dystonia is a focal Dystonia that manifests as involuntary masticatory and/or tongue muscle contractions. This movement disorder is frequently misdiagnosed as a tempOromandibular disorder. Hence, it would be useful to establish a method that makes it possible for patients with the condition to find appropriate medical institutions by themselves. The author produced a website Involuntary movements of the stomatognathic region (https://sites. google.com/site/OromandibularDystoniaenglish/) for patients with Oromandibular Dystonia, which is available in twenty languages. It has been viewed more than 1,000,000 times by individuals from all over the world. The visitors to the site have completed questionnaires and/or sent images or videos of their involuntary movements over the internet. Cyberconsultations (remote diagnosis) were also performed via Skype™. Approximately 1000 patients with involuntary stomatognathic movements visited our department. Only 12.5% of the patients had previously been diagnosed with or were suspected to have Dystonia. The findings of this study suggest that the multilingual website has contributed to increasing awareness of Oromandibular Dystonia and that the provision of basic telemedicine via the internet can aid the diagnosis and treatment of Oromandibular Dystonia.
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sensory trick splint as a multimodal therapy for Oromandibular Dystonia
Journal of Prosthodontic Research, 2017Co-Authors: Kazuya YoshidaAbstract:Abstract Purpose Many patients with Oromandibular Dystonia, which is characterized by involuntary masticatory, lower facial, and/or tongue muscle contractions, experience relief of symptoms through sensory tricks such as eating chewing gum or candy. The aim of this study was to identify the factors influencing the effects of splints in patients with Oromandibular Dystonia. Methods Occlusal splints were inserted in 128 patients (89 women, 39 men) with Oromandibular Dystonia (102 with jaw closing Dystonia, 20 with lingual Dystonia, 5 with jaw deviation Dystonia, 4 with jaw opening Dystonia, 3 with lip Dystonia, and 2 with jaw protrusion Dystonia). Patients who showed improvement with the use of splints and continued to wear them for at least 3 months were defined as responders. In contrast, patients who showed little or no effect and/or were unable to insert splints were defined as non-responders. Differences in demographic and clinical data were statistically compared between responders and non-responders. Results Ninety-eight patients (76.6%) were responders (subjective improvement: 30.5%). Thirty patients (23.4%) were non-responders (subjective improvement: 7.2%). The responders were significantly older than the non-responders (53.8 years vs 47.0 years; p Conclusions The sensory trick splint is especially helpful for patients with jaw closing Dystonia. It is useful, although partially effective, as an alternative therapy in patients for whom other therapies have been unsatisfactory.
Joseph Jankovic - One of the best experts on this subject based on the ideXlab platform.
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tardive and idiopathic Oromandibular Dystonia a clinical comparison
Journal of Neurology Neurosurgery and Psychiatry, 2000Co-Authors: Engking Tan, Joseph JankovicAbstract:OBJECTIVE Most patients with tardive Dystonia have a focal onset involving the cranial-cervical region. Because of its resemblance to idiopathic cranial Dystonia, a common form of Dystonia, it often poses a diagnostic problem. To compare clinical features and response to botulinum toxin (BTX) injections between patients with tardive and idiopathic Oromandibular Dystonia (OMD). METHODS Patients seen in a movement disorder clinic who satisfied the inclusion criteria for tardive or idiopathic OMD were studied. The clinical variables and responses to BTX between the two groups of patients were compared. In the tardive group, we also compared the clinical variables between those with oro-facial-lingual stereotypies, and those without. RESULTS Twenty four patients with tardive OMD and 92 with idiopathic OMD were studied. There were no differences in the demographic characteristics. Most were women, with duration of symptoms longer than 8 years. The mean duration of neuroleptic exposure was 7.1 (SD 7.9) years. Jaw closure was the most frequent subtype of OMD (tardive=41.7%, idiopathic=51.1%). Idiopathic patients were more likely to have coexistent cervical Dystonia (p v 31.5%, p CONCLUSIONS Oro-facial-lingual stereotypies were significantly more frequent in the tardive than the idiopathic group. Presence of stereotypic movements in the limbs, akathisia, and respiratory dyskinesias in patients with OMD strongly suggests prior neuroleptic exposure. Dystonia in tardive OMD is more likely to be restricted to the Oromandibular region, whereas in patients with idiopathic OMD, there is often coexistent cervical Dystonia. BTX is equally effective in both groups of patients.
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botulinum toxin a in patients with Oromandibular Dystonia long term follow up
Neurology, 1999Co-Authors: Engking Tan, Joseph JankovicAbstract:Objective: To study the safety and efficacy of botulinum toxin A (BTX) in patients with Oromandibular Dystonia (OMD) and to compare the treatment results of the various subtypes of OMD. Background: OMD is one of the most challenging forms of Dystonia to treat. Pharmacologic therapies are generally not effective, and there are no surgical alternatives. Methods: Of 202 patients diagnosed clinically to have OMD in a movement disorders clinic over a period of 10 years, 162 patients satisfied the study inclusion criteria. The masseters and submentalis complex were the only two muscle groups injected with BTX in this group of patients. Results: The mean age was 57.9 ± 15.3 years and the mean follow-up period was 4.4 ± 3.8 years. More than half the patients had jaw-closing (JC) Dystonia. A total of 2,529 BTX treatments were administered into the masseter muscles, submentalis complex, or both during a total of 1,213 treatment visits. The mean doses of BTX (per side) were 54.2 ± 15.2 U for the masseters and 28.6 ± 16.7 U for the submentalis complex. The mean total duration of response was 16.4 ± 7.1 weeks. The mean global effect of BTX was 3.1 ± 1.0 (range, 0 to 4, where 4 equals the complete abolition of the Dystonia), with the JC Dystonia patients responding best. Fifty-one patients (31.5%) reported adverse effects with BTX in at least one visit. Complications such as dysphagia and dysarthria were reported in 135 (11.1%) of all treatment visits. Conclusions: BTX is a safe and effective long-term treatment for OMD. JC Dystonia responds better than jaw-opening or mixed Dystonias, and the treatment of the latter types of OMD are more likely associated with dysphagia and dysarthria. Jaw-opening Dystonia can be treated successfully by injecting the submentalis complex.
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peripherally induced Oromandibular Dystonia
Journal of Neurology Neurosurgery and Psychiatry, 1998Co-Authors: Charulata Sankhla, Eugene C Lai, Joseph JankovicAbstract:OBJECTIVES—Oromandibular Dystonia (OMD) is a focal Dystonia manifested by involuntary muscle contractions producing repetitive, patterned mouth, jaw, and tongue movements. Dystonia is usually idiopathic (primary), but in some cases it follows peripheral injury. Peripherally induced cervical and limb Dystonia is well recognised, and the aim of this study was to characterise peripherally induced OMD. METHODS—The following inclusion criteria were used for peripherally induced OMD: (1) the onset of the Dystonia was within a few days or months (up to 1 year) after the injury; (2) the trauma was well documented by the patient's history or a review of their medical and dental records; and (3) the onset of Dystonia was anatomically related to the site of injury (facial and oral). RESULTS—Twenty seven patients were identified in the database with OMD, temporally and anatomically related to prior injury or surgery. No additional precipitant other than trauma could be detected. None of the patients had any litigation pending. The mean age at onset was 50.11 (SD 14.15) (range 23-74) years and there was a 2:1 female preponderance. Mean latency between the initial trauma and the onset of OMD was 65 days (range 1 day-1 year). Ten (37%) patients had some evidence of predisposing factors such as family history of movement disorders, prior exposure to neuroleptic drugs, and associated Dystonia affecting other regions or essential tremor. When compared with 21 patients with primary OMD, there was no difference for age at onset, female preponderance, and phenomenology. The frequency of dystonic writer's cramp, spasmodic dysphonia, bruxism, essential tremor, and family history of movement disorder, however, was lower in the post-traumatic group (p<0.05). In both groups the response to botulinum toxin treatment was superior to medical therapy (p<0.005). Surgical intervention for tempOromandibular disorders was more frequent in the post-traumatic group and was associated with worsening of Dystonia. CONCLUSION—The study indicates that Oromandibular-facial trauma, including dental procedures, may precipitate the onset of OMD, especially in predisposed people. Prompt recognition and treatment may prevent further complications.
Andrew Blitzer - One of the best experts on this subject based on the ideXlab platform.
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Oromandibular Dystonia long term management with botulinum toxin
Laryngoscope, 2013Co-Authors: Catherine F Sinclair, Lowell E Gurey, Andrew BlitzerAbstract:Objectives/Hypothesis To review the long-term management of patients with Oromandibular Dystonia (OMD) treated using botulinum toxin. Study Design Retrospective chart review at a clinical research center. Methods Between 1995 and 2011, 59 patients with a diagnosis of OMD were treated with botulinum toxin. Data were collected on patient demographics, disease characteristics, and long-term treatment outcomes. Differences in management between an earlier published series of the first 20 OMD patients treated with botulinum toxin at this center and subsequent patients were analyzed. Results Patients were more commonly female (72% vs. 28%) with an average age at first botulinum treatment of 56.6 years. The median number of treatments was five (range, 1–35 treatments). Average time between treatments was 3.8 months (±5.2). Overall, 47.5%, had the jaw-closing form of OMD, which was associated with a preferential deviation to one side in 53.6%. These patients received initial injections to the masseter ± temporalis muscle; the external pterygoid was injected for associated lateral jaw deviation. Internal pterygoid injections were rarely used (3.4%). For the jaw-opening form, injections were initially administered to the external pterygoid, with the addition of anterior digastric for ongoing symptoms. When compared with patients in the older series, more patients since 1988 had treatments to the external pterygoid (P = .001) and anterior digastric (P = .006) in accordance with an increase in the diagnosis of jaw-opening OMD (P = .05). Conclusions Long-term management of OMD with botulinum toxin has minimal morbidity and is useful for all clinical forms. Injections can be titrated by dose and location to address the predominant muscle groups involved. Level of Evidence: 4 Laryngoscope, 123:3078–3083, 2013
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botulinum toxin injections for the treatment of Oromandibular Dystonia
Operative Techniques in Otolaryngology-head and Neck Surgery, 2004Co-Authors: Anthony Cultrara, Ajay E Chitkara, Andrew BlitzerAbstract:Oromandibular Dystonia is a neurologic disorder that manifests as involuntary repetitive spasms of the masticatory, lingual, and pharyngeal musculature, resulting in uncontrolled jaw and tongue movements. As with most Dystonias, these actions are typically action induced and may cause considerable social distress to affected persons. In some cases, progressive disease may lead to significant dysphagia and/or dysarthria. Before the introduction of botulinum toxin injection, oral pharmacological agents provided limited treatment. This article reviews the assessment of Oromandibular Dystonia, techniques for intramuscular botulinum toxin injection, and possible posttreatment complications. Dystonia is a neurological phenomenon characterized by repetitive involuntary sustained (tonic) or spasmodic (clonic) muscle contractions that cause abnormal body posturing. 1 The dystonic movements are typically action induced; therefore, the involved body part may appear normal at rest. Dystonia can be categorized as focal, involving one particular small group of muscles; as segmental, involving neighboring groups of muscles; or as generalized, with widespread muscle involvement. However, the craniocervical region is more likely to be affected. 2 The etiologies of Dystonia are varied, with the principal cause being idiopathic. Dystonia may also be the result of trauma, tardive syndromes, neurologic insult, neurodegenerative disease, or inherited disorders. Because the etiologies of Dystonia may be diverse, consultation with a neurologist is prudent before rendering treatment. Cranial‐ cervical Dystonia involves the musculature of the face, periorbita, masticatory complex, tongue, larynx, pharynx, and neck. 3 In this section we will focus on a subset of cranial‐ cervical Dystonia known as Oromandibular Dystonia (OMD). OMD consists of involuntary spasms of masticatory, lingual, perioral, and pharyngeal muscles and may be classified as jaw opening, jaw closing, jaw deviation, and/or tongue protrusion type. When these symptoms are found in conjunction with blepharospasm, the condition is referred to as Meige’s syndrome. These involuntary postures of the tongue and jaw may lead to dysphagia, dysarthria, bruxism, and/or tempOromandibular joint subluxation and damage. 4 In addition to physical impairment, the involuntary facial contortions may result in significant social impairment. 5
Engking Tan - One of the best experts on this subject based on the ideXlab platform.
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levodopa induced Oromandibular Dystonia in progressive supranuclear palsy
Clinical Neurology and Neurosurgery, 2003Co-Authors: Engking Tan, L L Chan, M C WongAbstract:Levodopa-induced dyskinesias have been reported in Parkinson's disease and multiple system atrophy. Cranial Dystonias are rare in patients with progressive supranuclear palsy (PSP). In this report we describe an unusual case of reversible levodopa-induced Oromandibular Dystonia (OMD) in a PSP patient to highlight the importance of recognizing this drug related complication in the management of PSP, and discuss the possible underlying pathophysiology.
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tardive and idiopathic Oromandibular Dystonia a clinical comparison
Journal of Neurology Neurosurgery and Psychiatry, 2000Co-Authors: Engking Tan, Joseph JankovicAbstract:OBJECTIVE Most patients with tardive Dystonia have a focal onset involving the cranial-cervical region. Because of its resemblance to idiopathic cranial Dystonia, a common form of Dystonia, it often poses a diagnostic problem. To compare clinical features and response to botulinum toxin (BTX) injections between patients with tardive and idiopathic Oromandibular Dystonia (OMD). METHODS Patients seen in a movement disorder clinic who satisfied the inclusion criteria for tardive or idiopathic OMD were studied. The clinical variables and responses to BTX between the two groups of patients were compared. In the tardive group, we also compared the clinical variables between those with oro-facial-lingual stereotypies, and those without. RESULTS Twenty four patients with tardive OMD and 92 with idiopathic OMD were studied. There were no differences in the demographic characteristics. Most were women, with duration of symptoms longer than 8 years. The mean duration of neuroleptic exposure was 7.1 (SD 7.9) years. Jaw closure was the most frequent subtype of OMD (tardive=41.7%, idiopathic=51.1%). Idiopathic patients were more likely to have coexistent cervical Dystonia (p v 31.5%, p CONCLUSIONS Oro-facial-lingual stereotypies were significantly more frequent in the tardive than the idiopathic group. Presence of stereotypic movements in the limbs, akathisia, and respiratory dyskinesias in patients with OMD strongly suggests prior neuroleptic exposure. Dystonia in tardive OMD is more likely to be restricted to the Oromandibular region, whereas in patients with idiopathic OMD, there is often coexistent cervical Dystonia. BTX is equally effective in both groups of patients.
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botulinum toxin a in patients with Oromandibular Dystonia long term follow up
Neurology, 1999Co-Authors: Engking Tan, Joseph JankovicAbstract:Objective: To study the safety and efficacy of botulinum toxin A (BTX) in patients with Oromandibular Dystonia (OMD) and to compare the treatment results of the various subtypes of OMD. Background: OMD is one of the most challenging forms of Dystonia to treat. Pharmacologic therapies are generally not effective, and there are no surgical alternatives. Methods: Of 202 patients diagnosed clinically to have OMD in a movement disorders clinic over a period of 10 years, 162 patients satisfied the study inclusion criteria. The masseters and submentalis complex were the only two muscle groups injected with BTX in this group of patients. Results: The mean age was 57.9 ± 15.3 years and the mean follow-up period was 4.4 ± 3.8 years. More than half the patients had jaw-closing (JC) Dystonia. A total of 2,529 BTX treatments were administered into the masseter muscles, submentalis complex, or both during a total of 1,213 treatment visits. The mean doses of BTX (per side) were 54.2 ± 15.2 U for the masseters and 28.6 ± 16.7 U for the submentalis complex. The mean total duration of response was 16.4 ± 7.1 weeks. The mean global effect of BTX was 3.1 ± 1.0 (range, 0 to 4, where 4 equals the complete abolition of the Dystonia), with the JC Dystonia patients responding best. Fifty-one patients (31.5%) reported adverse effects with BTX in at least one visit. Complications such as dysphagia and dysarthria were reported in 135 (11.1%) of all treatment visits. Conclusions: BTX is a safe and effective long-term treatment for OMD. JC Dystonia responds better than jaw-opening or mixed Dystonias, and the treatment of the latter types of OMD are more likely associated with dysphagia and dysarthria. Jaw-opening Dystonia can be treated successfully by injecting the submentalis complex.
Stewart A Factor - One of the best experts on this subject based on the ideXlab platform.
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Oromandibular Dystonia a clinical examination of 2 020 cases
Frontiers in Neurology, 2021Co-Authors: Laura Scorr, Stewart A Factor, Sahyli Perez Parra, Rachel Kaye, Randal C Paniello, Scott A Norris, Joel S Perlmutter, Tobias Baumer, Tatiana Usnich, Brian BermanAbstract:Objective: The goal of this study is to better characterize the phenotypic heterogeneity of Oromandibular Dystonia (OMD) for the purpose of facilitating early diagnosis. Methods: First, we provide a comprehensive summary of the literature encompassing 1,121 cases. Next, we describe the clinical features of 727 OMD subjects enrolled by the Dystonia Coalition (DC), an international multicenter cohort. Finally, we summarize clinical features and treatment outcomes from cross-sectional analysis of 172 OMD subjects from two expert centers. Results: In all cohorts, typical age at onset was in the 50s and 70% of cases were female. The Dystonia Coalition cohort revealed perioral musculature was involved most commonly (85%), followed by jaw (61%) and tongue (17%). OMD more commonly appeared as part of a segmental Dystonia (43%), and less commonly focal (39%) or generalized (10%). OMD was found to be associated with impaired quality of life, independent of disease severity. On average, social anxiety (LSA score: 33 ± 28) was more common than depression (BDI II score: 9.7 ± 7.8). In the expert center cohorts, botulinum toxin injections improved symptom severity by more than 50% in ~80% of subjects, regardless of etiology. Conclusions: This comprehensive description of OMD cases has revealed novel insights into the most common OMD phenotypes, pattern of Dystonia distribution, associated psychiatric disturbances, and effect on QoL. We hope these findings will improve clinical recognition to aid in timely diagnosis and inform treatment strategies.
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delineation of the clinical features and treatment response of Oromandibular Dystonia a multicenter summary of 2 057 cases
medRxiv, 2020Co-Authors: Laura Scorr, Stewart A Factor, Sahyli Perez Parra, Rachel Kaye, Randal C Paniello, Scott A Norris, Joel S Perlmutter, Tobias Baumer, Tatiana Usnich, Brian BermanAbstract:Abstract Objective To better characterize Oromandibular Dystonia (OMD) to facilitate early diagnosis and test the hypothesis that botulinum toxin treatment alleviates symptoms, regardless of etiology, to provide guidance on treatment strategies. Methods To better characterize this condition we utilize a three-pronged approach. First, we provide a comprehensive summary of the world’s literature encompassing 1157 cases in 27 separate manuscripts. Next, we describe the clinical features of 727 OMD subjects enrolled by the Dystonia Coalition (DC), an international multicenter database. Finally, we provide details of the treatment approach and response from two expert centers where large numbers of OMD patients are followed. Cases from expert centers were utilized to analyze whether response to botulinum toxin varied by etiology of OMD. Results In all cohorts, typical age at onset was in the 50s and approximately 70 % of cases were female. Although the literature OMD more commonly described as a focal Dystonia, analysis of the DC database revealed it more commonly appears as part of a segmental Dystonia. Expert center review of 173 cases revealed botulinum toxin injections improved symptom severity by more than 50% in approximately 78% of subjects. Among the patients at expert centers, analysis revealed that treatment response did not vary by etiology. Conclusions Botulinum toxin injections are an effective treatment for OMD, regardless of etiology. By providing a more comprehensive description of OMD and the therapeutic efficacy of botulinum toxin for this type of Dystonia, we hope to improve clinical recognition to aid in timely diagnosis and inform treatment strategies.
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treatment of Oromandibular Dystonia
2019Co-Authors: Stewart A Factor, Laura ScorrAbstract:Oromandibular Dystonia (OMD) is a rare form of Dystonia that affects the masticatory, lower facial, and lingual muscles. OMD is often idiopathic and can present as a focal Dystonia or as part of a segmental or generalized pattern of Dystonia. The movement phenotypes of OMD include jaw opening, jaw closing, lateral jaw deviation, lingual protrusion, and mixed presentations. Diagnosis and treatment initiation are often delayed because OMD is under-recognized. Meanwhile these patients endure significant disability, because eating and speaking are impaired, which has a negative impact on quality of life. Botulinum toxin injections are the most effective therapeutic strategy. The technique and onabotulinumA toxin doses for jaw injection are described.
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Pilot Single-Blind Trial of AbobotulinumtoxinA in Oromandibular Dystonia
Neurotherapeutics, 2018Co-Authors: Laura Scorr, Michael R. Silver, John Hanfelt, Elaine Sperin, Alan Freeman, H. A. Jinnah, Stewart A FactorAbstract:Oromandibular Dystonia (OMD) causes involuntary movements of masticatory and lingual muscles impairing eating, speaking, and swallowing. Treatment options are limited. The objective of this study was to determine the safety and efficacy of abobotulinumtoxinA (aboBoNTA) in OMD. A dose-finding study (phase 1) followed by a single session, prospective, single-blind trial (phase 2) was carried out. OMD subjects were evaluated at baseline, 6 and 12 weeks. Muscles injected were tailored to individual symptoms using EMG guidance, but the aboBoNTA dose for each muscle was pre-specified based on phase 1 results. Evaluations were Global Dystonia Rating Scale (GDS), Unified Dystonia Rating Scale (UDRS), Clinical Global Impression (CGI) improvement and severity, and quality of life (OMDQ-25). Adverse events were monitored. The lowest dosage in phase 1 resulted in adverse effects in two of three patients and thus was used in phase 2. In phase 2, adverse effects were observed in 50% of subjects including dysphagia, voice change, and soft palate weakness. Most were mild. Significant improvement was seen in quality of life (OMDQ-25), speech (BFMq21), and change in GDS, UDRS, CGI severity assessed by the unblinded investigator, but not in blinded video ratings. We conclude that aboBoNTA therapy in this study was associated with improved quality of life and was generally well tolerated in OMD, but occurrence of dysphagia dictated the importance of using low genioglossus dosing. Face to face assessment appears to be more sensitive than video assessment for change in OMD severity. Consideration of the disability in OMD places constraints on traditional placebo-control trial design. Development of novel trial designs is warranted.
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secondary nonresponsiveness to botulinum toxin type a in patients with Oromandibular Dystonia
Movement Disorders, 2002Co-Authors: Charles H Adler, Stewart A Factor, Mitchell Brin, Kapil Dev SethiAbstract:Intramuscular injection of botulinum toxin type A is the treatment of choice for most cases of Oromandibular Dystonia. We report on five patients with Oromandibular Dystonia that developed secondary nonresponsiveness to botulinum toxin type A following multiple injections over a 6-year period.
