Several triggers were reported to elicit or exacerbate abnormal movements, including emotion (all patients), intercurrent illnesses (patients 1, 2, and 7), high temperature (patient 1), intention, and purposeful movements (patients 1 and 7). Attacks frequently presented in clusters, lasting minutes (patient 1), hours or weeks (patient 2), or months (patient 7) (videos 1, 2, and 5). Such exacerbations were pharmacoresistant to standard therapies. Patients 1, 2, and 7 also exhibited associated autonomic instability, sweating, dehydration, and rise in CK, frequently requiring hospitalization and intensive care and, often, administration of anesthetic agents (patients 2 and 7). Worsening of baseline dystonia with hyperkinetic movements and loss of fine motor skills were reported in patient 7 after each episode. One such exacerbation determined a 3-month admission to the intensive care unit and anesthetic agents. The emergency placement of a deep brain stimulator (DBS) device bilaterally into the globus pallidus interna was transformative and resulted in almost complete remission of the pronounced hyperkinesia, although residual generalized dystonia persisted.
