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Episodic hemiplegia

MedGen UID:
350828
Concept ID:
C1863061
Finding
Synonym: Hemiplegia, episodic
 
HPO: HP:0012194

Definition

Transient episodes of weakness of the arm, leg, and in some cases the face on one side of the body. [from HPO]

Term Hierarchy

Conditions with this feature

Alternating hemiplegia of childhood 1
MedGen UID:
762361
Concept ID:
C3549447
Disease or Syndrome
Alternating hemiplegia of childhood is a rare syndrome of episodic hemi- or quadriplegia lasting minutes to days. Most cases are accompanied by dystonic posturing, choreoathetoid movements, nystagmus, other ocular motor abnormalities, autonomic disturbances, and progressive cognitive impairment (Mikati et al., 1992). The disorder may mimic or overlap with other disorders, including familial hemiplegic migraine (FHM1; 141500) and GLUT1 deficiency syndrome (606777) (Rotstein et al., 2009). Genetic Heterogeneity of Alternating Hemiplegia of Childhood See also AHC2 (614820), caused by mutation in the ATP1A3 gene (182350).

Professional guidelines

PubMed

Samanta D
Pediatr Neurol 2020 Feb;103:12-20. Epub 2019 Nov 1 doi: 10.1016/j.pediatrneurol.2019.10.003. PMID: 31836335
Brockmann K
Curr Neurol Neurosci Rep 2013 Oct;13(10):379. doi: 10.1007/s11910-013-0379-7. PMID: 23963607
Neville BG, Ninan M
Dev Med Child Neurol 2007 Oct;49(10):777-80. doi: 10.1111/j.1469-8749.2007.00777.x. PMID: 17880649

Recent clinical studies

Etiology

Rosewich H, Thiele H, Ohlenbusch A, Maschke U, Altmüller J, Frommolt P, Zirn B, Ebinger F, Siemes H, Nürnberg P, Brockmann K, Gärtner J
Lancet Neurol 2012 Sep;11(9):764-73. Epub 2012 Jul 30 doi: 10.1016/S1474-4422(12)70182-5. PMID: 22850527

Diagnosis

Luan H, Zhang L, Zhang S, Zhang M
Medicine (Baltimore) 2021 Dec 23;100(51):e28141. doi: 10.1097/MD.0000000000028141. PMID: 34941060Free PMC Article
Rosewich H, Thiele H, Ohlenbusch A, Maschke U, Altmüller J, Frommolt P, Zirn B, Ebinger F, Siemes H, Nürnberg P, Brockmann K, Gärtner J
Lancet Neurol 2012 Sep;11(9):764-73. Epub 2012 Jul 30 doi: 10.1016/S1474-4422(12)70182-5. PMID: 22850527

Therapy

Luan H, Zhang L, Zhang S, Zhang M
Medicine (Baltimore) 2021 Dec 23;100(51):e28141. doi: 10.1097/MD.0000000000028141. PMID: 34941060Free PMC Article

Prognosis

Luan H, Zhang L, Zhang S, Zhang M
Medicine (Baltimore) 2021 Dec 23;100(51):e28141. doi: 10.1097/MD.0000000000028141. PMID: 34941060Free PMC Article
Jen J, Yue Q, Nelson SF, Yu H, Litt M, Nutt J, Baloh RW
Neurology 1999 Jul 13;53(1):34-7. doi: 10.1212/wnl.53.1.34. PMID: 10408533

Clinical prediction guides

Luan H, Zhang L, Zhang S, Zhang M
Medicine (Baltimore) 2021 Dec 23;100(51):e28141. doi: 10.1097/MD.0000000000028141. PMID: 34941060Free PMC Article
Jen J, Yue Q, Nelson SF, Yu H, Litt M, Nutt J, Baloh RW
Neurology 1999 Jul 13;53(1):34-7. doi: 10.1212/wnl.53.1.34. PMID: 10408533

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