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Meige’s Syndrome
Meige’s syndrome is a type of adult-onset dystonia that was first described by Henry Meige in 1910. Primary Meige’s syndrome is characterized by a combination of oromandibular dystonia (OMD) and idiopathic or essential blepharospasm. Secondary Meige’s syndrome can be encountered in the context of several different neurodegenerative disorders, in the chronic administration of neuroleptics, and in patients with focal brain lesions. These secondary cases suggest that dysfunction of the basal ganglia or of the mesencephalic/diencephalic region plays an important role in this syndrome.1
The pathophysiology of this syndrome, like all segmental dystonias, is not well understood. Various hypotheses have been formulated, although none has been universally accepted. A recent study using functional magnetic resonance imaging (MRI) showed reduced activation of the primary motor and ventral precortex during involuntary oromandibular movements, a phenomenon that probably reflects reduced cortical inhibition in motor and premotor areas. The same study revealed an increased activation of the somatosensory areas, which indicates that an altered somatosensory representation may be responsible for the manifestations of this syndrome.2
The onset of symptoms typically occurs between 30 and 70 years of age, most often in the sixth decade. It is twice as common in women as in men. In most cases, the involuntary facial and mandible movements gradually progress in intensity, as well as in the number of affected muscle groups, over a period of 1 to 4 years after onset, after which they usually stabilize. The orofacial contractions are bilateral, symmetric, and nonrhythmic spasms of various muscle groups. They typically last from a few seconds to minutes and can be triggered by certain actions, and especially by psychosocial stressors.
In order of decreasing frequency the following types of spasms may be seen: eye closure (blepharospasm), mouth retraction, eyebrow elevation and frowning, platysmal contractions, neck spasms (flexion, extension, and lateral rotation), jaw-opening spasms, soft palate spasms, mouth-clenching spasms, lip pursing, inspiratory spasms, lip-tightening spasms, contraction of nasal and floor-of-mouth muscles, spasms of tongue protrusion, and spasms of shoulder elevation. Several types of orbicularis oculi spasms occur in Meige’s syndrome: brief clonic spasms, prolonged dystonic spasms, constant tonic contraction, and “apraxia” of lid opening.3
Historically, these spasms were considered psychogenic in origin and patients were prescribed psychiatric treatment. Even after recognition of the neurologic basis of segmental dystonias, medical therapy with various agents has historically been the first-line treatment. Such agents as dopaminergic/anticholinergics, tetrabenazine, benzodiazepines, and baclofen have been used with varying success. Botulinum neurotoxin (BoNT) injections with electromyography (EMG) guidance in selected muscles have also been used successfully to control the spasms associated with this syndrome. Corrective ablative surgery (eyelid myectomy, blepharoplasty, and lid lifts) has been described, but due to lack of convincing efficacy data, it currently plays little, if any, role in therapy. Globus pallidus deep brain stimulation (DBS) is an established treatment for medically refractory primary forms of dystonia. Given the dramatic clinical improvement seen in DBS patients with primary generalized dystonia, trials of pallidal DBS have been performed for the treatment of segmental dystonia as well.4
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