title: Status dystonicus: the syndrome and its management.
abstract: Patients with dystonic syndromes sometimes develop increasingly frequent and relentless episodes of devastating generalized dystonia which we call status dystonicus. Twelve cases of status dystonicus, of various underlying aetiologies, are presented. Possible precipitating factors were identified in only five cases: intercurrent infection (one); discontinuation of lithium (one) and tetrabenazine (one); and the introduction of clonazepam (two). Nine patients required mechanical ventilation and three others were sedated with intravenous chlormethiazole. Drug therapy used included benzhexol, tetrabenazine, pimozide, baclofen, chlorpromazine, haloperidol, carbamazepine and acetozolamide. Two patients underwent thalamotomies, one of whom improved. Two patients died, five returned to their pre-status dystonicus condition, two eventually made a full recovery and three were worse. Patients with status dystonicus should be managed on an intensive care unit as they may develop bulbar and respiratory complications which may require ventilation. Metabolic problems encountered can include rhabdomyolysis with acute renal failure. Drug therapy with benzhexol, tetrabenazine and pimozide or haloperidol may be beneficial in some cases.
authors.affiliation: Royal Free Hospital, London, UK.
authors.forename: H
authors.initials: H
authors.lastname: Manji
authors.forename: R S
authors.initials: RS
authors.lastname: Howard
authors.forename: D H
authors.initials: DH
authors.lastname: Miller
authors.forename: N P
authors.initials: NP
authors.lastname: Hirsch
authors.forename: L
authors.initials: L
authors.lastname: Carr
authors.forename: K
authors.initials: K
authors.lastname: Bhatia
authors.forename: N
authors.initials: N
authors.lastname: Quinn
authors.forename: C D
authors.initials: CD
authors.lastname: Marsden
authors.forename: K
authors.initials: K
authors.lastname: Bahtia
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D003422:Critical Care; D004421:Dystonia / Q000150:complications* / Q000503:physiopathology / Q000628:therapy*; D005260:Female; D006801:Humans; D008297:Male; D009127:Muscle Rigidity / Q000150:complications* / Q000503:physiopathology / Q000628:therapy*; D013577:Syndrome; D016896:Treatment Outcome