Meige’s syndrome

This chapter deals with botulinum toxin type A (BOTOX) in the treatment of strabismus, blepharospasm, and related disorders. Botulinum toxin type A (BOTOX) has been used to treat strabismus, blepharospasm, Meige s syndrome, and spasmodic torticollis. By preventing acetylcholine release at me neuromuscular junction, botulinum toxin A usually causes a temporary paralysis of the locally injected muscles. The variability in duration of paralysis may be related to me rate of developing antibodies to me toxin, upregulation of nicotinic cholinergic postsynaptic receptors, and aberrant regeneration of motor nerve fibers at me neuromuscular junction. Complications related to this toxin include double vision (diplopia) and lid droop (ptosis). 

The differential diagnosis includes idiopathic torsion dystonia, parkinsonism, idiopathic torticollis, Huntington s disease, Wilson s disease, and Meige s syndrome (blepharospasm, oromandibular dystonia). A retrospective evaluation of the records of patients with idiopathic cervical dystonias (n = 82) and tardive cervical dystonias (n = 20) has been performed, in a search for clinical features that could help separate these closely related disorders (332). Despite the overall similarity, the presence of a dystonic head tremor was strongly suggestive of the idiopathic form, which was present in 42% and did not occur at all in the tardive group. A family history of dystonia (10%) was also exclusive to the idiopathic group. 

Meige s syndrome is characterized by blepharospasm and spasm of the lower facial or oromandibular muscles (395). 

A 52-year-old woman developed Meige s syndrome 2 days after the appearance of akathisia. She had taken neuroleptic drugs for years, but her current medication had been changed to bromperidol 18 mg/day and trihexyphenidyl (benzhexol) 6 mg/day 2 days later she developed akathisia. Oral perphenazine 12 mg and trihexyphenidyl 6 mg dose-dependently reduced the frequency of blepharospasm the dosages of bromperidol and trihexyphenidyl were gradually reduced to 8 mg/ day and 3 mg/day respectively over 3 months, by which time her symptoms had completely disappeared. 

Hayashi T, Furutani M, Taniyama J, Kiyasu M, Hikasa S, Horiguchi J, Yamawaki S. Neuroleptic-induced Meige s syndrome following akathisia pharmacologic characteristics. Psychiatry Clin Neurosci 1998 52(4) 445-8. 

However, there was no evidence of a beneficial effect of clozapine in primary dystonia, the most common form of dystonia and a difficult disorder to treat, until the report of a 56-year-old woman with severe and persistent primary cranial dystonia (Meige s syndrome), who responded to clozapine (50-100 mg) (94). 

Sieche A, Giedke H. Treatment of primary cranial dystonia (Meige s syndrome) with clozapine. J Clin Psychiatry 115. 2000 61(12) 949. 

When blepharospasm is accompanied by periodic lower fecial movement, the disorder is referred to as Meige s syndrome or idiopathic orofacial dystonia. If the mandible also becomes involved, the disorder is referred to as Breughel s syndrome or oromandibular dystonia. When several cranial nerves are involved, the disorder is called segmental cranial dystonia. Although often discussed as separate entities, these dystonic syndromes may be the same disease process with variable clinical manifestations. 

Kebapci M, Aslan 0, Kaya T, Yalcin OT, Ozalp S (2002) Pedunculated uterine leiomyoma associated with pseudo-Meigs syndrome and elevated CA-125 level CT features. Eur Radiol 12[Suppl 3] S127-129... 

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