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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 -
The Canadian Journal of Neurological... Oct 2023
PubMed: 37830290
DOI: 10.1017/cjn.2023.298 -
Frontiers of Neurology and Neuroscience 2018Different eponyms such as "Wood syndrome," Meige syndrome, "Brueghel syndrome," "Blepharospasm plus syndrome" have been used to describe segmental craniocervical... (Review)
Review
Different eponyms such as "Wood syndrome," Meige syndrome, "Brueghel syndrome," "Blepharospasm plus syndrome" have been used to describe segmental craniocervical dystonias. These facial and/or oromandibular movement disorders are characterized by muscle contractions and spasms involving eyes, facial region, and sometimes pharynx, jaw, floor of the mouth, and tongue. The pathophysiology of craniocervical dystonia is poorly understood, but abnormal plasticity and impaired inhibition are suspected. Injection of botulinum toxin appears to be the best therapeutic option for treating segmental craniocervical dystonia. The objective of this chapter is to depict the history of segmental craniocervical dystonia in order to delineate the phenotypic spectrum of the disorders and to distinguish this entity from other facial and/or oromandibular movement disorders.
Topics: Humans; Meige Syndrome
PubMed: 29145188
DOI: 10.1159/000475701 -
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 -
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 -
Parkinsonism & Related Disorders Aug 2009Frequently, blepharospasm is associated with involuntary movements of the platysma, lower face and masticatory muscles. Similarly, masticatory dystonia may occur in... (Review)
Review
Frequently, blepharospasm is associated with involuntary movements of the platysma, lower face and masticatory muscles. Similarly, masticatory dystonia may occur in isolation or in combination with dystonia of other cranial and cervical muscles. The non-possessive and possessive forms of Meige and Brueghel syndromes have been variably and imprecisely ascribed to various anatomical variations of craniocervical dystonia. Herein, the origin of eponymic terms as applied to craniocervical dystonia is reviewed as support for proposed elimination of these eponyms from clinical usage. Although the term "segmental craniocervical dystonia" more accurately captures the combination of blepharospasm and dystonia of other head and neck muscles, delineation of craniocervical subphenotypes is essential for etiological/genetic and treatment studies. To conclude, the clinical features, epidemiology, pathophysiology and therapeutic management of segmental craniocervical dystonia are examined with a particular focus on "blepharospasm-plus" subphenotypes.
Topics: Anti-Dyskinesia Agents; Botulinum Toxins; Dystonia; Facial Muscles; History, 20th Century; History, 21st Century; Humans; Meige Syndrome
PubMed: 19457699
DOI: 10.1016/j.parkreldis.2009.04.006 -
Southern Medical Journal Jul 1980We present two women with Meige's syndrome, a condition in which the clinical presentation differs from tardive dyskinesia by the lack of exposure to neuroleptic drgus,...
We present two women with Meige's syndrome, a condition in which the clinical presentation differs from tardive dyskinesia by the lack of exposure to neuroleptic drgus, greater severity of blepharospasms, and more prolonged dystonic contractions of oromandibular muscles. In this condition we used triaxial accelerometry to detect dystonia, which may also appear in limb and respiratory muscles. Although psychologic factors may affect the symptoms, the basic pathogenesis of this syndrome does not seem to be psychogenic. We think that biochemical abnormalities in the basal ganglia are responsible for the dyskinesias and submit data suggesting a reduction of dopamine turnover in the central nervous system of one patient. Both patients have evidence of autoimmune diseases, and one patient's dystonic movements responded to immunosuppressive therapy, suggesting that autoimmune processes contribute to the pathogenic mechanism of Meige's syndrome in some instances.
Topics: Aged; Blepharospasm; Brain Diseases; Eyelid Diseases; Facial Muscles; Female; Humans; Mandibular Diseases; Middle Aged; Muscle Spasticity; Syndrome
PubMed: 7384844
DOI: 10.1097/00007611-198007000-00009 -
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 -
Journal of Neuroimmunology Feb 2024This report details a rare case where a patient simultaneously suffered from Sjogren's syndrome (SS) and Meige's syndrome (MS). SS, an autoimmune disorder, and MS, a...
This report details a rare case where a patient simultaneously suffered from Sjogren's syndrome (SS) and Meige's syndrome (MS). SS, an autoimmune disorder, and MS, a rare neurological condition characterized by involuntary eyelid closure, presented in a 73-year-old male. The patient had been experiencing dry eye symptoms for the past 5 years, with the onset of eyelid spasms and tetanic eye closure occurring 3 years ago. Traditional treatments, including subthalamic nucleus deep brain stimulation, provided only temporary relief. Diagnostic evaluations, including blood tests and imaging, confirmed SS and MS coexistence. Treatment involved a combination of steroids, immunosuppressants, and immunoglobulin, leading to significant symptom relief. This case suggests a potential association between SS and the development of MS, highlighting the importance of immunomodulatory therapy in managing neurological symptoms. Further research is needed to explore the relationship between these two conditions and to develop more effective treatment strategies.
Topics: Male; Humans; Aged; Sjogren's Syndrome; Meige Syndrome; Blepharospasm; Treatment Outcome
PubMed: 38134494
DOI: 10.1016/j.jneuroim.2023.578264