Jaw deviation dystonia evaluated by movement-related cortical potentials and treated with muscle afferent block

Abstract

Jaw-deviation dystonia is characterized by the lateral shift of the mandible due to involuntary masticatory muscle contraction, causing difficulties in speech or mastication. We evaluated a patient with jaw-deviation dystonia by recording movement-related cortical potentials (MRCPs) and treated with muscle afferent block (MAB). MRCP associated with mandibular movements (mouth opening, closing, and left and right lateral movements) was recorded in the patient and ten age-matched healthy subjects. In the control subjects, the amplitude of Bereitschaftspotential (BP)/negative slope (NS') was significantly higher for left lateral movements than for the mouth closing. The cortical map of BP/NS' prior to mouth opening and closing showed symmetric distribution, whereas those of lateral movements showed a slight predominance in the ipsilateral hemisphere. The patient showed lower amplitude as compared with control subjects. The right lateral movement (homonymous task) showed task-specific markedly reduced potentials. After MAB by intramuscular injection of lidocaine and ethanol to the inferior head of the left lateral pterygoid muscle, the deviation abolished and severity in speech and mastication was significantly improved. This study suggests that jaw-deviation dystonia might have the same etiology as other focal dystonias.