Brain, Vol 121, Issue 2 243-252, Copyright © 1998 by Oxford University Press
H Manji, RS Howard, DH Miller, NP Hirsch, L Carr, K Bahtia, N Quinn and CD Marsden
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.
ARTICLES
Status dystonicus: the syndrome and its management
Royal Free Hospital, London, UK.
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