Causes of Meige Syndrome
Meige syndrome is an idiopathic cranial dystonia with no clearly determined etiology, though the most widely accepted hypothesis suggests dopaminergic and cholinergic hyperactivity in the basal ganglia as the underlying pathophysiological mechanism. 1
Primary Etiology
The exact cause remains unknown, but Meige syndrome is considered by many investigators to be a form of idiopathic torsion dystonia affecting cranial muscles. 2
The pathophysiology is not clearly determined, though dysfunction in basal ganglia circuits controlling movement is implicated. 3
Dopaminergic and cholinergic hyperactivity represents the most widely accepted mechanistic hypothesis for symptom generation. 1
Secondary Associations
While Meige syndrome is primarily idiopathic, it has been identified in specific patient populations:
Parkinson's disease patients may develop Meige syndrome as an associated feature of their underlying neurodegenerative condition. 4
Atypical Parkinsonism syndromes can present with cranial dystonia resembling Meige syndrome. 4
Essential tremor patients have been identified with concurrent Meige syndrome. 4
Clinical Context
Typical presentation occurs in middle-aged females, with symptoms beginning as isolated blepharospasm before progressing to include oromandibular dystonia. 5
Symptoms are highly variable and affected by psychological factors, which can lead to misdiagnosis as conversion disorder or anxiety disorder, particularly since symptoms can be voluntarily inhibited to some extent. 3
Progressive nature is characteristic, with patients often starting with increased blinking frequency and difficulty keeping eyelids open, then developing jaw clenching, shoulder shrugging, and involuntary head movements over time. 4
Important Clinical Pitfall
Do not misdiagnose Meige syndrome as a psychiatric disorder (conversion disorder, anxiety disorder) simply because symptoms fluctuate with stress or can be partially suppressed voluntarily—this is an inherent feature of the dystonia itself, not evidence of psychogenic origin. 3