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Blepharospasm and orofacial‐cervical dystonia: Clinical and pharmacological findings in 100 patients

Identifieur interne : 00D290 ( Main/Exploration ); précédent : 00D289; suivant : 00D291

Blepharospasm and orofacial‐cervical dystonia: Clinical and pharmacological findings in 100 patients

Auteurs : Joseph Jankovic [États-Unis] ; Janet Ford

Source :

RBID : ISTEX:AB71A428870EC10BBB903DD6F09299B8AD90819A

Descripteurs français

English descriptors

Abstract

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.

Url:
DOI: 10.1002/ana.410130406


Affiliations:


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Le document en format XML

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<term>Basal ganglia</term>
<term>Blepharospasm</term>
<term>Brainstem</term>
<term>Clonazepam</term>
<term>Dopamine</term>
<term>Dopaminergic</term>
<term>Dyskinesia</term>
<term>Dysphonia</term>
<term>Dystonia</term>
<term>Dystonic</term>
<term>Dystonic spasms</term>
<term>Essential blepharospasm</term>
<term>Essential tremor</term>
<term>Facial</term>
<term>Facial dystonia</term>
<term>Facial movements</term>
<term>Facial muscles</term>
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<term>Female preponderance</term>
<term>Generalized dystonia</term>
<term>Head tremor</term>
<term>High incidence</term>
<term>Huntington disease</term>
<term>Hypothyroidism</term>
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<term>Palatal myoclonus</term>
<term>Parkinson disease</term>
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<term>Spasmodic</term>
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<term>Tardive</term>
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<div type="abstract" xml:lang="en">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.</div>
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