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Oromandibular Dystonia in Cerebrotendinous Xanthomatosis
Author(s) -
Benson Brian E.,
Guss Joel,
Blitzer Andrew
Publication year - 2009
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1002/lary.20502
Subject(s) - cerebrotendinous xanthomatosis , dystonia , medicine , psychiatry , cholesterol
She was treated with speech therapy and serial titrated electromyography-guided BTX injections (Figure 5). A maximum of 15 units of BTX were administered to each of the external pterygoid muscles and 5 units of toxin were administered to each anterior belly of the digastric muscles. Following chemodenervation of these muscles, the patient experienced decreased frequency, intensity, and duration of the dyskinesias. Her speech articulation and fluency improved. Her ability to masticate and swallow both solids and liquids improved, although she remained PEG dependent. She is currently working with a nutritionist to maximize her oral caloric intake, with the goal of reducing or eliminating her PEG dependence. Oromandibular dystonia (OMD) is a neurologic disorder characterized by involuntary movements of the masticatory, lingual, and pharyngeal muscles. Oral pharmacologic agents have limited efficacy in alleviating the symptoms of OMD. OMD complicated by recurrent temporomandibular joint dislocation has previously been described in a patient with CTX4. BTX is a safe and effective treatment for OMD5. In addition to directly weakening the affected muscles, some authors suggest that BTX also modulates inhibitory and excitatory intracortical pathways6.