Jaw-opening oromandibular dystonia secondary to Wilson's Disease treated with botulinum toxin type A

Abstract

We have reported a case series of five patients with jaw-opening oromandibular dystonia secondary to Wilson's disease (WD), in which the patients were treated with botulinum toxin type A (BTX-A). In all cases, dystonia score was partially reduced three weeks after injections. The most common side effect was transient mild dysphagia. This preliminary study showed that jaw-opening oromandibular dystonia in WD may be partially responsive to the use of BTX-A.

Neurological manifestations in Wilson’s disease –possible treatment options for symptoms

“…In cases not responding to pharmacological therapy, neurosurgical treatment should be considered. [6,24,27,37] The pharmacological agents used in the treatment of dystonia include (1) anticholinergics, (2) baclofen, (3) benzodiazepines, (4) dopaminergic compounds (levodopa or dopamine agonists), (5) dopamine-depleting drugs, and (6) antiepileptic drugs (carbamazepine/oxcarbamazepine). [24,28] The data on the efficacy of this kind of treatment in patients with WD are limited but encouraging.…”

“…BTX injections used in focal dystonias were effective in 8/9 patients with WD (the remaining patient had a slight improvement), as reported separately by three different centers. [32,[37][38][39] In the case of dystonia affecting more body regions (i.e. segmental, multisegmental, or generalized dystonia), there are only few published reports on treatment of symptoms.…”

Psychogenic (Functional) Jaw-Opening Dystonia: Three Case Reports and a Comparison of Symptomatic Jaw-Opening Dystonia Associated with Organic Causes in the Literature

“…The pathophysiology and etiology of jaw-opening dystonia remain unclear, but some possible pathophysiological mechanisms or peripheral causes have been suggested for some cases [2,4]. Moreover, in some cases, the jaw-opening dystonia presumably had a central origin, e.g., focal brain lesion, ischemic stroke, neurodegenerative disease, or metabolic or genetic disorders with structural brain involvement [10][11][12][13][14][15][16][17]. However, secondary jaw-opening dystonia associated with a central lesion is extremely rare, and psychogenic (functional) movement disorder initially presenting as jaw-opening dystonia has not been reported in the literature.…”

Brief considerations on the dispensation profile of the botulinum toxin type A by the Brazilian Unified Health System for treatment of dystonias: Datasus data

“…Type A BT (BTA) has proved effective for various forms of dystonia [6] , particularly, blepharospasm [7] , [8] , [9] , cervical dystonia [10] , oromandibular dystonia [11] , [12] , bucolingual dyskinesia [13] and laryngeal dystonia [14] , [15] . Several studies have confirmed its long-term efficacy [16] , [17] , [18] .…”