This document discusses Meige syndrome, which involves involuntary contractions of the muscles around the eyes (blepharospasm) and mouth (oromandibular dystonia). It can be primary or secondary to conditions like neuroleptic medication use or brain injuries. Pathophysiology may involve dopaminergic/cholinergic hyperactivity or decreased inhibitory neurons in the cortex. Diagnosis involves tests like EMGs and imaging to rule out other causes. Treatment options include anticholinergics, dopamine antagonists, Botox injections, and deep brain stimulation. Differential diagnoses include other movement disorders or neurological/psychiatric conditions.

1. Ade Wijaya, MD – November 2021

2.  Introduction / Clinical Presentation
 Etiology
 Pathophysiology
 Diagnosis
 Management
 Differential diagnosis

3. blepharospasm
Oromandibular
dystonia
MEIGE
Syndrome
Jahngir MU, Ameer MA, Patel BC. Meige syndrome. StatPearls [Internet]. 2021 Feb 25.

4.  Primary
 Secondary:
- Neuroleptic medication
- Head trauma, stroke, demyelination of brain stem
region, normal pressure hydrocephalus, cerebral
hypoxia, postoperative (bilateral thalamotomy),
kernicterus, space-occupying lesions, post-
encephalitis.
- Meige syndrome can be associated with other
movement disorders, like Parkinson disease,
Wilson disease, olivopontocerebellar atrophy, or
Lewy body diseas
Jahngir MU, Ameer MA, Patel BC. Meige syndrome. StatPearls [Internet]. 2021 Feb 25.

5.  Dopaminergic and cholinergic hyperactivity
 Decreased functioning of inhibitory neurons
(for example, GABAergic neurons) in the
cortex
 Various environmental and genetic factors
 In 33% of patients with Meige syndrome,
emotional stress may be a factor as their
primary symptoms are anxiety, depression,
and sleep problems
Jahngir MU, Ameer MA, Patel BC. Meige syndrome. StatPearls [Internet]. 2021 Feb 25.

6.  Facial electromyography
 MRI/CT brain to rule out the stroke or any
other brain lesion
 Serum SSA/SSB level
 Serum Cu and ceruloplasmin level
 Beck's depression inventory
 Serum drug screen
Jahngir MU, Ameer MA, Patel BC. Meige syndrome. StatPearls [Internet]. 2021 Feb 25.

7.  Drug of choice: anticholinergics (e.g.,
trihexyphenidyl), dopamine antagonists (e.g.,
tiapride, tetrabenazine), GABA receptor agonists
(e.g., benzodiazepines, baclofen)
 Others: antiepileptics (e.g., valproic acid), and a
number of psychoactive drugs. Eszopiclone,
nitrazepam, zolpidem
 Botulinum A injection
 Deep brain stimulation of globus pallidus interna
Jahngir MU, Ameer MA, Patel BC. Meige syndrome. StatPearls [Internet]. 2021 Feb 25.

8.  Xeromas
 Spinocerebellar ataxia
 Progressive supranuclear palsy
 Wilson disease
 Ischemic stroke
 Autoimmune/inflammatory disease (multiple
sclerosis, lupus erythematosus, Behcet disease)
 Metabolic (hypoxia, pontine myelinolysis)
 Neoplasm (meningioma, metastatic tumor)
 Myoclonus-dystonia syndrome
 Psychogenic craniocervical dystonia
 Generalized anxiety disorder