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Blepharospasm and orofacial‐cervical dystonia: Clinical and pharmacological findings in 100 patients
Author(s) -
Jankovic Joseph,
Ford Janet
Publication year - 1983
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410130406
Subject(s) - blepharospasm , trihexyphenidyl , cervical dystonia , dystonia , medicine , focal dystonia , spasmodic torticollis , spasmodic dysphonia , botulinum toxin , movement disorders , dermatology , pediatrics , surgery , anesthesia , psychiatry , disease
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.