Subthalamic nucleus deep brain stimulation in primary Meige syndrome: motor and non-motor outcomes.

BACKGROUND AND PURPOSE: Deep brain stimulation (DBS) has emerged as a promising treatment for movement disorders. This prospective study aims to evaluate the effects of bilateral subthalamic nucleus DBS (STN-DBS) on motor and non-motor symptoms in patients with primary Meige syndrome. METHODS: Thirty patients who underwent bilateral STN-DBS between April 2017 and June 2020 were included. Standardized and validated scales were utilized to assess the severity of dystonia, health-related quality of life, sleep, cognitive function and mental status at baseline and at 1 year and 3 years after neurostimulation. RESULTS: The Burke-Fahn-Marsden Dystonia Rating Scale movement scores showed a mean improvement of 63.0% and 66.8% at 1 year and 3 years, respectively, after neurostimulation. Similarly, the Burke-Fahn-Marsden Dystonia Rating Scale disability scores improved by 60.8% and 63.3% at the same time points. Postoperative quality of life demonstrated a significant and sustained improvement throughout the follow-up period. However, cognitive function, mental status, sleep quality and other neuropsychological functions did not change after 3 years of neurostimulation. Eight adverse events occurred in six patients, but no deaths or permanent sequelae were reported. CONCLUSIONS: Bilateral STN-DBS is a safe and effective alternative treatment for primary Meige syndrome, leading to improvements in motor function and quality of life. Nevertheless, it did not yield significant amelioration in cognitive, mental, sleep status and other neuropsychological functions after 3 years of neurostimulation.

Bilateral Pallidal Deep Brain Stimulation in Meige Syndrome: Effects on Motor Function, Neuropsychological Status, and Mood.

BACKGROUND: Bilateral pallidal deep brain stimulation (DBS) has been broadly accepted as a feasible surgical procedure for treating various forms of dystonia, but its effects on motor function, neuropsychological status, and mood in patients with Meige syndrome have rarely been examined. OBJECTIVE: To evaluate the effects of bilateral globus pallidus internus DBS (GPi-DBS) on the motor performance, quality of life, neuropsychological status, and mood of patients with primary Meige syndrome. METHODS: Between January 2015 and April 2019, the database of 35 patients with Meige syndrome who underwent bilateral GPi-DBS in our institution was retrospectively reviewed. The severity of dystonia, health-related quality of life, cognitive function, and mood were assessed using standardized and validated rating scales at baseline. Repeat assessment of the same domains was performed at 1 year and 3 years after neurostimulation in a similar manner. RESULTS: One year and 3 years after bilateral GPi-DBS, Burke-Fahn-Marsden Dystonia Rating Scale movement scores were improved by 65% and 72% and Burke-Fahn-Marsden Dystonia Rating Scale disability scores were improved by 49% and 57%, respectively. The significant improvement in health-related quality of life observed at 1 year was sustained at 3 years. Relative to baseline and to the 1-year assessment, cognitive functions and mood remained stable after 3 years of neurostimulation. No deaths or life-threatening events were reported over the study period. CONCLUSION: Bilateral GPi-DBS is a safe and effective approach for medically refractory Meige syndrome that can improve motor function and quality of life without cognitive and mood side effects.

Low-voltage bilateral pallidal stimulation for severe meige syndrome in a patient with primary segmental dystonia: case report.

BACKGROUND AND IMPORTANCE: Meige syndrome (MS) is an adult-onset segmental dystonia characterized by the combination of upper and lower cranial involvement. Its treatment is challenging and the use of oral medication or of botulinum neurotoxin treatment is not decisive. Deep brain stimulation of the globus pallidus internum (GPi DBS) has been used occasionally in severe cases. CLINICAL PRESENTATION: We report the long-term efficacy of low-voltage chronic bilateral GPi DBS in a patient with segmental dystonia featuring severe MS and cervical brachial involvement. The patient received a bilateral ventroposterolateral GPi implant. Postoperative 3-dimensional reconstruction allowed checking of the definitive position of the electrode and contacts within the targeted nucleus. Following implant, the patient received bilateral low-voltage stimulation, consisting in amplitude of 1.3 V with a pulse width of 90 microseconds and a frequency of 130 Hz, yielding a current of 23 microA. Clinical follow-up for 38 months showed a progressive and sustained improvement of dystonia with unchanged electrical settings throughout the observation period. The patient again undertook normal life activities. CONCLUSION: Bilateral low-voltage GPi stimulation allowed efficient control of MS symptoms and the associated brachial cervical dystonia.

Posttraumatic mid-facial pain and Meige's syndrome relieved by pressure on the nasion and retrocollis.

A 42-year-old farmer developed persistent mid-facial segmental pain and Meige's syndrome several months after suffering facial trauma and a fracture of the nose. He was not afflicted by systemic ailments, had no family history of movement disorder and no history of exposure to neuroleptic drugs. He was capable of suppressing his facial pain by performing a ritual that included forcefully tilting his head backwards, lowering of his eyelids and applying strong pressure to his nasion. Exceptionally dystonic movements and elaborate behavioral rituals may serve as a mechanism of pain suppression.

Oromandibular dystonia: A dental approach

Oromandibular dystonia consists of prolonged spasms of contraction of the muscles of the mouth and jaw. Primaryidiopathic forms and secondary forms exist. Secondary dystonia develops due to environmental factors;some cases of cranial dystonia after dental procedure have been reported, but the causal relationship betweenthese procedures and dystonia remains unclear. Traumatic situations in the mouth, such as poor aligned denturesor multiple teeth extractions may cause an impairment of proprioception of the oral cavity, leading to subsequentdevelopment of dystonia. The clinical characteristics of oromandibular dystonia are classified according to theaffected muscles. The muscles involved may be the muscles of mastication, muscles of facial expression, or themuscles of the tongue. At present, there is no known cure for OMD. The mainstay of treatment for most focaldystonia is botulinum toxin injections. It is important for the dentist to be familiar with oromandibular dystonia,as it can develop after dental treatment and is often misdiagnosed as a dental problem (AU)

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Oromandibular dystonia: a dental approach.

Oromandibular dystonia consists of prolonged spasms of contraction of the muscles of the mouth and jaw. Primary idiopathic forms and secondary forms exist. Secondary dystonia develops due to environmental factors; some cases of cranial dystonia after dental procedure have been reported, but the causal relationship between these procedures and dystonia remains unclear. Traumatic situations in the mouth, such as poor aligned dentures or multiple teeth extractions may cause an impairment of proprioception of the oral cavity, leading to subsequent development of dystonia. The clinical characteristics of oromandibular dystonia are classified according to the affected muscles. The muscles involved may be the muscles of mastication, muscles of facial expression, or the muscles of the tongue. At present, there is no known cure for OMD. The mainstay of treatment for most focal dystonia is botulinum toxin injections. It is important for the dentist to be familiar with oromandibular dystonia, as it can develop after dental treatment and is often misdiagnosed as a dental problem.

Oromandibular dystonia and persistent psychiatric symptoms in extra-pontine myelinolysis.

A 32 years female presented with gradually progressive dysarthria, dysphagia, oromandibular dystonia and mild generalized weakness. She had several episodes of acute psychotic behavior. She had abnormal saccadic eye movements, generalized hypertonia and exaggerated jerks in upper limbs. She was previously treated in a peripheral hospital for severe vomiting and diarrhea. MRI of brain revealed symmetrical T-2 weighted hyperintensities in bilateral putaminal and caudate region along with pons and midbrain suggesting demyelination due to a metabolic insult. Her power improved gradually over days and the dysarthria, dysphagia and oromandibular dystonia improved gradually over several weeks with supportive measures but the psychiatric manifestations are still persisting.

Clinical profile of various sub-types of writer's cramp.

We profiled 141 consecutive patients of writer's cramp (WC). Simple WC (n=108) had a mean (SD) disease duration of 3.65 (2.7) years and a severity score of 2.8 (0.91) by Burke-Fahn-Marden (BFM) scale (handwriting score: 0-4 ), whereas complex WC (n=33) had a disease duration of 5.8 (2.81) years and severity score of 3.6 (0.52). Compared with focal WC, generalized WC cases had longer disease duration, severer disability, and were older. Mirror dystonia was seen in 65 patients (46.1%). Co-existent Meige's syndrome (n=4) and cervical dystonia (n=6) were seen. Some patients had abnormal posturing of the neck (n=5) and of the oromandibular region (n=3) only while writing. Those receiving botulinum toxin fared significantly better than those on oral therapy, at 1 month post treatment.

The natural history of embouchure dystonia.

Focal task-specific dystonias are unusual disorders of motor control, often affecting individuals who perform complex repetitive movements. Musicians are especially prone to develop these disorders because of their training regimens and intense practice schedules. Task-specific dystonia occurring in keyboard or string instrumentalists usually affects the hand. In contrast, there have been few descriptions of musicians with task-specific dystonia affecting the muscles of the face and jaw. We report detailed clinical observations of 26 professional brass and woodwind players afflicted with focal task-specific dystonia of the embouchure (the pattern of lip, jaw, and tongue muscles used to control the flow of air into a mouthpiece). This is the largest and most comprehensively studied series of such patients. Patients developed embouchure dystonia in the fourth decade, and initial symptoms were usually limited to one range of notes or style of playing. Once present, dystonia progressed without remission and responded poorly to oral medications and botulinum toxin injection. Patients with embouchure dystonia could be separated by the pattern of their abnormal movements into several groups, including embouchure tremor, involuntary lip movements, and jaw closure. Dystonia not infrequently spread to other oral tasks, often producing significant disability. Effective treatments are needed for this challenging and unusual disorder.

Meige syndrome secondary to basal ganglia injury: a potential cause of acute respiratory distress.

BACKGROUND: Meige syndrome is a movement disorder that includes blepharospasm and oromandibular dystonias. Its etiology may be idiopathic (primary) or it may arise secondary to focal brain injury. Acute respiratory distress as a feature of such dystonias occurs infrequently. A review of the literature on Meige syndrome and the relationship between dystonias and respiratory compromise is presented. METHODS: A 60-year-old woman suffered a cerebral anoxic event secondary to manual strangulation. She developed progressive blepharospasm combined with oromandibular and cervical dystonias. Neuroimaging demonstrated bilateral damage localized to the globus pallidus. Years later, she presented to the emergency department in intermittent respiratory distress associated with facial and cervical muscle spasms. RESULTS: Increasing frequency and severity of the disorder was noted over years. The acute onset of respiratory involvement required intubation and eventual tracheotomy. A partial therapeutic benefit of tetrabenazine was demonstrated. CONCLUSION: This case highlights two interesting aspects of Meige's syndrome: (1) Focal bilateral basal ganglia lesions appear to be responsible for this patient's movement disorder which is consistent with relative overactivity of the direct pathway from striatum to globus pallidus internal and substantia nigra pars reticularis; (2) Respiratory involvement in a primarily craniofacial dystonia to the point of acute airway compromise.

[Blepharospasm and Meige's syndrome--a contribution to it's pathogenesis]. / Kurcz powiek i zespól Meige'a--przyczynek do patogenezy.

Two cases with blepharospasm followed by Meige syndrome were described. In the first magnetic resonants revealed bilateral hypodensive foci in thalamus. During the few years general dystonia has been developed after several relapses of disease diagnosed as multiple infarcts followed by aphasia, hemiparesis and asynergia. In the other one, who died, loss of neurons in striatum, especially in caudate nuclei was found. It is concluded that blepharospasm could be a syndrome of different origin and only the phase of Meige syndrome.

Case-control study of Meige's syndrome. Result of a pilot study.

A pilot case-control study was conducted to identify possible risk factors for Meige's syndrome. Patients with Meige's syndrome and age- and sex-matched controls suffering from other neurological diseases were recruited from the Movement Disorders Clinic and Neurology Outpatient Department of the All India Insititute of Medical Sciences. All participants were interviewed and information regarding psychiatric and medical illnesses, use of medications, exposure to fumes, dust and pets, characteristics such as marital status, socio-economic status, alcohol, tea/coffee use, tobacco use, betel nut chewing and family history of neurodegenerative diseases among first-degree relatives was ascertained. We found that betel nut with tobacco chewing was a significant predictor for Meige's syndrome (adjusted odds ratio 7.4, 95% confidence interval = 1.0-59. 82). The role of local irritation or the effect of some chemicals in tobacco and betel nuts needs further evaluation of the pathogenesis of Meige's syndrome.

Peripherally induced oromandibular dystonia.

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.

Clinical and demographic features of Meige's syndrome.

Fifty five patients with Meige's syndrome were examined for clinical and demographic features. The mean age of onset was 52.3 years. The peak age of onset was in the sixth decade with a male to female ratio of 1.11:1. The mean duration of illness was 3.7 years. Commonest initial symptom was increased blinking, seen in 30 cases (54.5%). Twenty five patients (45.4%) had complete syndrome of blepharospasm with oromandibular dystonia, whereas 24 patients (43.6%) had blepharospasm alone and the rest (6 patients, 10.9%) had oromandibular dystonia. The extension of spasm beyond cranial muscles was observed in 10 patients (18.1%). Eleven patients had family history of dystonia or other extrapyramidal disorders. Incidence of depression was high in these cases.