Segmental Dystonia Treated with Deep Brain Stimulation
Dystonia causes sustained muscle contractions, repetitive twisting movements, and abnormal postures of the trunk, neck, face, or extremities (see Chap. 36). Dystonic movements can be either slow or rapid, change during different activities or postures, and may become fixed in advanced cases. Except for occasional tremor or myoclonus, the rest of the neurological examination is normal. Childhood onset generalized dystonia typically begins as a focal action dystonia, usually of one foot, and progresses over several years to become generalized (see Chap. 37). Cases presenting in adolescence more commonly begin in one upper extremity or in the cervical-thoracic region and are more slowly progressive (see Chap. 37)
KeywordsDeep Brain Stimulation Botulinum Toxin Globus Pallidus Advanced Case Cervical Dystonia
Segmental dystonia.mp4 (MP4 25,315KB)
Clip 1: while seated, the patient exhibits recurrent spasmodic left rotational torticollis and retrocollis together with adduction and extension posturing of his upper extremities. While walking, he exhibits jerky right lateral deviation of his upper trunk, recurrent jerky flexion at his right elbow, hyperextension and internal rotation of his left arm, and abortive attempts to stabilize his head with his hand. While writing, he exhibits perioral dystonia, repeated elevation of his right arm, slow writing, and mild spasmodic rotational torticollis to the left side. Clip 2: following GPi-DBS, while seated he displays less severe rotational torticollis and retrocollis with absence of upper limb dystonia. While walking, he displays right rotational torticollis without trunk or limb dystonia. He now writes faster and more easily without elevation of his right arm.