We evaluated prospectively 100 patients, the largest reported series, with
blepharospasm and orofacial-
cervical dystonia, or
Meige syndrome. The mean age at onset was 51.7 years, and 81% presented between the ages of 40 and 70. Women outnumbered men three to two.
Blepharospasm was the initial symptom in 58 patients, but only 23 had
involuntary movements localized to the orbicularis oculi. Sixty-one patients had the complete syndrome,
blepharospasm and oromandibular
dystonia, and 60 had neck or generalized
dystonia in addition to the orofacial movements. Twenty-one patients with spasmodic
dysphonia were included; in 12 of these patients, spasmodic
dysphonia was part of the complete (
Meige) syndrome, and 16 of these patients had neck or generalized
dystonia or
essential tremor. An organic cause of
Meige syndrome is supported by a high correlation with
essential tremor and other
movement disorders and by positive family history in some patients. Response to medication was inconsistent, but 69% of patient trials resulted in some improvement; in 22% the benefit was marked and persistent.
Tetrabenazine,
lithium, and
trihexyphenidyl were most useful for the treatment of oromandibular
dystonia, and
clonazepam was useful in some patients with
blepharospasm.