Meige Syndrome
Conditions
Keywords
DBS
Brief summary
Meige syndrome is a rare and debilitating movement disorder characterized by blepharospasm, oromandibular dystonia, and cervical muscle contractions, often refractory to pharmacological therapies. Deep brain stimulation (DBS), initially developed to treat essential tremor and Parkinson's disease, has been explored in recent years as a potential treatment for various dystonias, including Meige's syndrome. This clinical study aims to evaluate the safety and efficacy of bilateral single-electrode ventralis oralis (VO) nucleus stimulation combined with subthalamic nucleus deep brain stimulation (STN-DBS) in patients with Meige syndrome.
Detailed description
While deep brain stimulation (DBS) targeting the subthalamic nucleus (STN) or the globus pallidus interna (GPi) has shown moderate efficacy, incomplete symptom relief and high stimulation thresholds with associated side effects remain significant limitations. Emerging evidence suggests that dual-target neuromodulation combining STN with ventralis oralis (VO) nucleus stimulation may synergistically modulate hyperactive basal ganglia-thalamocortical circuits, potentially enhancing therapeutic outcomes.The study will involve patients diagnosed with Meige syndrome who are eligible for DBS therapy. Participants will be randomly assigned to one of two groups.The primary outcome measure is the improvement in motor symptoms, assessed using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) before and after treatment. Secondary outcomes will include changes in quality of life, anxiety and depression scores, and any adverse effects related to the DBS procedure.The results of this study will be used to guide future clinical trials and inform the treatment options for patients with Meige syndrome.
Interventions
DEVICEdeep brain stimulation
bilateral single-electrode VO combined with STN target implantation
Sponsors
Huashan Hospital
Study design
Allocation
RANDOMIZED
Intervention model
PARALLEL
Primary purpose
TREATMENT
Masking
QUADRUPLE (Subject, Caregiver, Investigator, Outcomes Assessor)
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Must meet the clinical diagnostic criteria for Meige syndrome * Only includes patients with: Blepharospasm-type Meige syndrome and Blepharospasm with lower jaw muscle dystonia-type * All patients must have undergone at least one year of systematic and standard treatment prior to surgery
Exclusion criteria
* History of neurological diseases other than Meige syndrome * Severe cognitive impairment * Severe psychiatric disorders
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Improvement of primary symptoms | 1 year after stimulation | Scale title: Burke-Fahn-Marsden Dystonia Rating Scale Section I: Motor subscale (0-120) Section II: Disability scale (0-30) Higher scores: Indicate worse outcomes (more severe dystonia symptoms). |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Improvement of symptoms | 1 year after stimulation | Scale title: Jankovic Rating Scale Minimum value: 0 (No dystonia) Maximum value: 8 (Severe dystonia) Higher scores: Indicate worse outcomes (more severe dystonia symptoms). |
| Psychological assessment | 1 year after stimulation | Scale title: Hamilton Anxiety Rating Scale Minimum value: 0 (No anxiety) Maximum value: 56 (Severe anxiety) Higher scores: Indicate worse outcomes (more severe anxiety). |
| Cognitive assessment | 1 year after stimulation | Scale title: Mini-Mental State Examination Minimum value: 0 (Severe cognitive impairment) Maximum value: 30 (No cognitive impairment) Higher scores: Indicate better outcomes (better cognitive function). |
| health-related quality of life | 1 year after stimulation | Scale title:Short Form 36 Health Survey The SF-36 is a widely used tool to assess general health status and quality of life. It includes 36 items that measure eight health domains: Physical functioning Role limitations due to physical health problems Role limitations due to emotional problems Energy/fatigue Emotional well-being Social functioning Pain General health perceptions Each of the domains is scored separately, and the overall score can reflect a person's physical and mental health. Minimum value: 0 (Worst health status) Maximum value: 100 (Best health status) Higher scores: Indicate better outcomes (better health-related quality of life). |
Countries
China
Outcome results
None listed