Status dystonicus : the syndrome and its management

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...

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Bibliographic Details
Published in:Brain (London, England : 1878) England : 1878), 1998-02, Vol.121 (2), p.243-252
Main Authors: MANJI, H, HOWARD, R. S, MILLER, D. H, HIRSCH, N. P, CARR, L, BAHTIA, K, QUINN, N, MARSDEN, C. D
Format: Article
Language:English
Subjects:
Adolescent
Adult
Biological and medical sciences
Child
Critical Care
Dystonia - complications
Dystonia - physiopathology
Dystonia - therapy
Female
Humans
Male
Medical sciences
Muscle Rigidity - complications
Muscle Rigidity - physiopathology
Muscle Rigidity - therapy
Nervous system (semeiology, syndromes)
Nervous system as a whole
Neurology
Syndrome
Treatment Outcome
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Summary: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.
ISSN:0006-8950
1460-2156
1460-2156
DOI:10.1093/brain/121.2.243