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Toxins Apr 2020Blepharospasm and oromandibular dystonia are focal dystonias characterized by involuntary and often patterned, repetitive muscle contractions. There is a long history of... (Review)
Review
Blepharospasm and oromandibular dystonia are focal dystonias characterized by involuntary and often patterned, repetitive muscle contractions. There is a long history of medical and surgical therapies, with the current first-line therapy, botulinum neurotoxin (BoNT), becoming standard of care in 1989. This comprehensive review utilized MEDLINE and PubMed and provides an overview of the history of these focal dystonias, BoNT, and the use of toxin to treat them. We present the levels of clinical evidence for each toxin for both, focal dystonias and offer guidance for muscle and site selection as well as dosing.
Topics: Blepharospasm; Botulinum Toxins; Dystonic Disorders; Humans; Mandibular Diseases; Muscular Diseases; Neuromuscular Agents
PubMed: 32331272
DOI: 10.3390/toxins12040269 -
Industrial Psychiatry Journal 2016Meige's syndrome consists of idiopathic blepharospasm and oromandibular dystonia. The exact etiology is not known and various hypotheses have been proposed for its...
Meige's syndrome consists of idiopathic blepharospasm and oromandibular dystonia. The exact etiology is not known and various hypotheses have been proposed for its causation. The hypothesis suggesting dopaminergic and cholinergic hyperactivity is most widely accepted. There is no curative drug for Meige's syndrome although a variety of treatments have been proposed. We report a case which responded to tetrabenazine.
PubMed: 28659707
DOI: 10.4103/0972-6748.207853 -
Frontiers in Neurology 2021Meige syndrome (MS) is cranial dystonia characterized by the combination of upper and lower cranial involvement and including binocular eyelid spasms (blepharospasm;... (Review)
Review
Meige syndrome (MS) is cranial dystonia characterized by the combination of upper and lower cranial involvement and including binocular eyelid spasms (blepharospasm; BSP) and involuntary movements of the jaw muscles (oromandibular dystonia; OMD). The etiology and pathogenesis of this disorder of the extrapyramidal system are not well-understood. Neurologic and ophthalmic examinations often reveal no abnormalities, making diagnosis difficult and often resulting in misdiagnosis. A small proportion of patients have a family history of the disease, but to date no causative genes have been identified to date and no cure is available, although botulinum toxin A therapy effectively mitigates the symptoms and deep brain stimulation is gaining increasing attention as a viable alternative treatment option. Here we review the history and progress of research on MS, BSP, and OMD, as well as the etiology, pathology, diagnosis, and treatment.
PubMed: 33854473
DOI: 10.3389/fneur.2021.630221 -
Revue Medicale Suisse Dec 2017Characterized by an aplasia, hypoplasia or dysplasia of the lymphatic network, the primary lymphedema takes part of rare diseases. If 10 % of cases are congenital, the...
Characterized by an aplasia, hypoplasia or dysplasia of the lymphatic network, the primary lymphedema takes part of rare diseases. If 10 % of cases are congenital, the majority of them are detected before 35 years, most of the time due to an intercurrent event suh as a sprain or an infection. Although rarer, some primaries lymphedemas are family forms such the syndromes of Milroy and Meige. The primary lymphedema can also be a part of more complex malformative diseases such as Klinefelter, Turner or Noonan syndromes. If the lymphatic drainage associated with the elastic contention remains the basis care of lymphedema, we have to identify these patients as well as their family, sometimes requiring multidisciplinary cares.
Topics: Humans; Lymphatic Vessels; Lymphedema; Syndrome
PubMed: 29211371
DOI: No ID Found -
Neurologic Clinics May 2020The dystonias are a large and heterogenous group of disorders characterized by excessive muscle contractions leading to abnormal postures and/or repetitive movements.... (Review)
Review
The dystonias are a large and heterogenous group of disorders characterized by excessive muscle contractions leading to abnormal postures and/or repetitive movements. Their clinical manifestations vary widely, and there are many potential causes. Despite the heterogeneity, helpful treatments are available for the vast majority of patients. Symptom-based therapies include oral medications, botulinum toxins, and surgical interventions. For some subtypes of dystonia, specific mechanism-based treatments are available. Advances in understanding the biological basis for many types of dystonia have led to numerous recent clinical trials, so additional treatments are likely to become available in the very near future.
Topics: Dystonia; Humans
PubMed: 32279713
DOI: 10.1016/j.ncl.2020.01.003 -
Clinical Parkinsonism & Related... 2021Dyspnoea is rarely mentioned in the clinical description of adult-onset isolated dystonia. In this study, we present the clinical features of 13 patients with Meige...
BACKGROUND
Dyspnoea is rarely mentioned in the clinical description of adult-onset isolated dystonia. In this study, we present the clinical features of 13 patients with Meige syndrome (cranio-cervical dystonia) with breathing difficulties.
METHODS
A retrospective case note review was performed of patients presenting with Meige syndrome and shortness of breath, to a neuro-laryngology MDT clinic.
RESULTS
Some patients were severely limited by their breathlessness, but others did not volunteer these symptoms. The majority of patients were referred with the assumption that the larynx was the cause of the problem; however half the patients did not have evidence of laryngeal involvement. Of the patients who had laryngeal involvement, injecting the larynx alone did not always relieve the dyspnoea. The majority of our patients responded to injection of the suprahyoid muscles, including genioglossus, digastric and mylohyoid.
CONCLUSION
We recommend routinely establishing if the patient with Meige syndrome has signs or symptoms of breathlessness, and establishing the level of the problem, as this can be treated successfully.
PubMed: 34541486
DOI: 10.1016/j.prdoa.2021.100106 -
Neurologic Clinics Feb 2015The dystonias are a group of disorders characterized by excessive involuntary muscle contractions leading to abnormal postures and/or repetitive movements. A careful... (Review)
Review
The dystonias are a group of disorders characterized by excessive involuntary muscle contractions leading to abnormal postures and/or repetitive movements. A careful assessment of the clinical manifestations is helpful for identifying syndromic patterns that focus diagnostic testing on potential causes. If a cause is identified, specific etiology-based treatments may be available. In most cases, a specific cause cannot be identified, and treatments are based on symptoms. Treatment options include counseling, education, oral medications, botulinum toxin injections, and several surgical procedures. A substantial reduction in symptoms and improved quality of life is achieved in most patients by combining these options.
Topics: Dystonia; Humans
PubMed: 25432724
DOI: 10.1016/j.ncl.2014.09.002 -
Journal of the Neurological Sciences Jan 2017'Meige's syndrome' is a type of cranial dystonia characterized by blepharospasm and oromandibular dystonia and can be associated with complex movement of lower facial... (Review)
Review
'Meige's syndrome' is a type of cranial dystonia characterized by blepharospasm and oromandibular dystonia and can be associated with complex movement of lower facial muscles, mouth, jaw, tongue, pharyngeal and cervical muscles. Frequently, blepharospasm is the earliest clinical manifestation, which spreads over a period of time to involve other cranial and extra-cranial muscles. Common characteristics of this syndrome are well known, but their variety is wide. Different eponyms such as "Breughel syndrome", "Wood syndrome", "Blepharospasm plus", "Segmental cranial dystonia" and "Segmental cranio-cervical dystonia" have been used to describe this entity with numerous anatomical variations. In the majority of the patients Meige's syndrome is primary or idiopathic, where the cause of spasm is not known, however secondary cases can occur following prolonged use of neuroleptics or secondary to underlying brain disorders. This syndrome has also been described in patients with essential tremor, Parkinson's disease and atypical Parkinsonism. Neurophysiological features are similar to other focal dystonia characterized by abnormal plasticity and impaired inhibition. Most of the patients are successfully treated with injection of botulinum toxin, however deep brain stimulation has emerged as a good therapeutic option in intractable patients. The objective of this review is to understand whether patients who develop Meige's syndrome are different from patients who manifest blepharospasm or oromandibular dystonia alone.
Topics: History, 19th Century; History, 20th Century; History, 21st Century; Humans; Meige Syndrome
PubMed: 28017205
DOI: 10.1016/j.jns.2016.11.053 -
Scientific Reports Aug 2021To contribute to the understanding of the aetiology and pathogenesis of Meige syndrome, the metabolic networks of patients with Meige syndrome were investigated using...
To contribute to the understanding of the aetiology and pathogenesis of Meige syndrome, the metabolic networks of patients with Meige syndrome were investigated using 18F-fluoro-D-glucose positron emission tomography (18F-FDG-PET) imaging of cerebral glucose metabolism. Fifty right-handed and unmedicated primary Meige syndrome patients enrolled between September 2017 and September 2020 at the Department of Neurosurgery, Peking University People's Hospital, and 50 age- and sex-matched healthy control subjects participated in the study. Metabolic connectivity and graph theory analysis were used to investigate metabolic network differences based on 18F-FDG-PET images. Glucose hypometabolism was detected in the left internal globus pallidus and parietal lobe, right frontal lobe and postcentral gyrus, and bilateral thalamus and cerebellum of patients with Meige syndrome. Clustering coefficients (Cps) (density threshold: 16-28%; P < 0.05) and shortest path lengths (Lps) (density threshold: 10-15%; P < 0.05) were higher in Meige syndrome patients than in healthy controls. Small-worldness was lower in Meige syndrome patients than in healthy controls, and centrality was significantly lower in the right superior occipital gyrus and pallidum and higher in the right thalamus. Hypometabolism in the globus pallidus and thalamus may indicate basal ganglia-thalamocortical motor circuit abnormalities as a pathogenic mechanism of Meige syndrome, providing a possible explanation for the efficacy of deep brain stimulation (DBS) in improving symptoms. Meige syndrome patients had abnormal small-world properties. Centrality changes in the right pallidus and thalamus verified the important roles of these regions in the pathogenesis of Meige syndrome.
Topics: Adult; Aged; Brain; Case-Control Studies; Female; Glucose; Humans; Male; Meige Syndrome; Metabolic Networks and Pathways; Metabolome; Middle Aged; Positron-Emission Tomography; Tomography, X-Ray Computed
PubMed: 34344985
DOI: 10.1038/s41598-021-95333-8