Adrenocorticotropic Hormone Induced Status Dystonicus in a Child with West Syndrome
Jasmine Singh, Roosy Aulakh
Keywords :
Adrenocorticotropic hormone, Status dystonicus, West syndrome
Citation Information :
Singh J, Aulakh R. Adrenocorticotropic Hormone Induced Status Dystonicus in a Child with West Syndrome. Indian J Crit Care Med 2022; 26 (8):961-962.
Dystonia is a movement disorder characterized by involuntary sustained or intermittent muscle contraction causing repetitive twisting movements and abnormal postures. Status dystonicus (SD) is an enigmatic disease of cryptic etiology. We hereby report a child with West syndrome (WS) who went on to develop SD following intramuscular adrenocorticotropic hormone (ACTH) injection. An 11-month-old male child presented with complaints of flexor spasms for 2 months. The diagnosis of WS was confirmed by electroencephalography (EEG), which showed hypsarrhythmia. Intramuscular ACTH was added, and oral trihexyphenidyl was started for dystonia. On day 7 of ACTH, the child developed frequent opisthotonic posturing. Management protocol for grade IV SD was initiated. Administration of N-terminal of ACTH in rat locus coeruleus has been shown to produce human dystonia-like movement and abnormal posturing.
Sanger TD, Chen D, Fehlings DL, Hallett M, Lang AE, Mink JW, et al. Definition and classification of hyperkinetic movements in childhood. Mov Disord 2010;25(11):1538–1549. DOI: 10.1002/mds.23088.
Thakkar P, Naveen BP, Yoganathan S, John JA, Thomas M. Status dystonicus: diagnosis and management of a rare and challenging entity. J Pediatr Rehabil Med 2019;12(1):1–4. DOI: 10.3233/PRM-170510.
Burke RE, Fahn S, Marsden CD, Bressman SB, Moskowitz C, Friedman J. Validity and reliability of a rating scale for the primary torsion dystonias. Neurology 1985;35(1):73–77. DOI: 10.1212/wnl.35.1.73.
Manji H, Howard RS, Miller DH, Hirsch NP, Carr L, Bhatia K, et al. Status dystonicus: the syndrome and its management. Brain 1998;121 (Pt 2):243–252. DOI: 10.1093/brain/121.2.243.
Mishra D, Singhal S, Juneja M. Status dystonicus a rare complication of dystonia. Indian Pediatr 2010;47(10):883–885. DOI: 10.1007/s13312-010-0138-7.
Thakkar P, Naveen BP, Yoganathan S, John JA, Thomas M. Status dystonicus: diagnosis and management of a rare and challenging entity. J Pediatr Rehabil Med 2019;12:71–74. DOI: 10.3233/PRM-170510.
Angurana SK, Muralidharan J, Dayal D, Ismail J. Status dystonicus in a child with familial idiopathic hypoparathyroidism. The Indian Journal of Pediatrics 2017;84(5):405–407. DOI: 10.1007/s12098-017-2295-3.
Allen NM, Lin JP, Lynch T, King MD. Status dystonicus: a practice guide. Dev Med Child Neurol 2014;56(2):105–112. DOI: 10.1111/dmcn.12339.
Fasano A, Ricciardi L, Bentivoglio AR, Canavese C, Zorzi G, Petrovic I, et al. Status dystonicus: predictors of outcome and progression patterns of underlying disease. Mov Disord 2012;27(6):783–788. DOI: 10.1002/mds.24981.
Jacquet Y. A dystonia-like syndrome after neuropeptide (MSH/ACTH) stimulation of the rat locus ceruleus. Adv Neurol 1988;50:299–311. PMID: 2840807.
Hoshino H, Kanemura H. ACTH for epileptic spasms in Leigh syndrome with SLC19A3 mutation can induce status dystonicus. Epileptic Disord 2022;24(1):171–175. DOI: 10.1684/epd.2021.1374.
