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Alternating hemiplegia of childhood

MedGen UID:
90925
Concept ID:
C0338488
Disease or Syndrome
Synonym: Alternating hemiplegia syndrome
SNOMED CT: Alternating hemiplegia of childhood (230466004)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0016241
OMIM® Phenotypic series: PS104290
Orphanet: ORPHA2131

Definition

A rare, genetic, neurodevelopmental disorder characterized by early-onset of recurrent, transient episodes of hemiplegia (including quadriplegia), which typically disappear upon sleep. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAlternating hemiplegia of childhood
Follow this link to review classifications for Alternating hemiplegia of childhood in Orphanet.

Professional guidelines

PubMed

Mikati MA, Panagiotakaki E, Arzimanoglou A
Eur J Paediatr Neurol 2021 May;32:A4-A5. Epub 2021 May 4 doi: 10.1016/j.ejpn.2021.05.004. PMID: 33975787
Samanta D
Pediatr Neurol 2020 Feb;103:12-20. Epub 2019 Nov 1 doi: 10.1016/j.pediatrneurol.2019.10.003. PMID: 31836335
Cross JH
Semin Fetal Neonatal Med 2013 Aug;18(4):192-5. Epub 2013 Apr 30 doi: 10.1016/j.siny.2013.04.003. PMID: 23642846

Recent clinical studies

Etiology

Poole J, Zagaglia S, Demurtas R, Farrell F, Walker MC, Sisodiya SM, Balestrini S, Vivekananda U
PLoS One 2022;17(9):e0268720. Epub 2022 Sep 30 doi: 10.1371/journal.pone.0268720. PMID: 36178910Free PMC Article
Uchitel J, Abdelnour E, Boggs A, Prange L, Pratt M, Bonner M, Jasien J, Dawson G, Abrahamsen T, Mikati MA
Dev Med Child Neurol 2020 Jul;62(7):820-826. Epub 2020 Feb 7 doi: 10.1111/dmcn.14473. PMID: 32031250
Severino M, Pisciotta L, Tortora D, Toselli B, Stagnaro M, Cordani R, Morana G, Zicca A, Kotzeva S, Zanaboni C, Montobbio G, Rossi A, De Grandis E; IBAHC Consortium
J Neurol 2020 May;267(5):1300-1311. Epub 2020 Jan 16 doi: 10.1007/s00415-020-09698-3. PMID: 31950366
Heinzen EL, Arzimanoglou A, Brashear A, Clapcote SJ, Gurrieri F, Goldstein DB, Jóhannesson SH, Mikati MA, Neville B, Nicole S, Ozelius LJ, Poulsen H, Schyns T, Sweadner KJ, van den Maagdenberg A, Vilsen B; ATP1A3 Working Group
Lancet Neurol 2014 May;13(5):503-14. doi: 10.1016/S1474-4422(14)70011-0. PMID: 24739246Free PMC Article
Sasaki M, Ishii A, Saito Y, Morisada N, Iijima K, Takada S, Araki A, Tanabe Y, Arai H, Yamashita S, Ohashi T, Oda Y, Ichiseki H, Hirabayashi S, Yasuhara A, Kawawaki H, Kimura S, Shimono M, Narumiya S, Suzuki M, Yoshida T, Oyazato Y, Tsuneishi S, Ozasa S, Yokochi K, Dejima S, Akiyama T, Kishi N, Kira R, Ikeda T, Oguni H, Zhang B, Tsuji S, Hirose S
Neurology 2014 Feb 11;82(6):482-90. Epub 2014 Jan 15 doi: 10.1212/WNL.0000000000000102. PMID: 24431296

Diagnosis

Ananthavarathan P, Kamourieh S
Handb Clin Neurol 2023;198:221-227. doi: 10.1016/B978-0-12-823356-6.00005-6. PMID: 38043964
Vezyroglou A, Akilapa R, Barwick K, Koene S, Brownstein CA, Holder-Espinasse M, Fry AE, Németh AH, Tofaris GK, Hay E, Hughes I, Mansour S, Mordekar SR, Splitt M, Turnpenny PD, Demetriou D, Koopmann TT, Ruivenkamp CAL, Agrawal PB, Carr L, Clowes V, Ghali N, Holder SE, Radley J, Male A, Sisodiya SM, Kurian MA, Cross JH, Balasubramanian M
Neurology 2022 Oct 4;99(14):e1511-e1526. Epub 2022 Jul 18 doi: 10.1212/WNL.0000000000200927. PMID: 36192182Free PMC Article
Blumenfeld AE, Victorio MC, Berenson FR
Semin Pediatr Neurol 2016 Feb;23(1):18-22. Epub 2016 Jan 22 doi: 10.1016/j.spen.2016.01.007. PMID: 27017017
Gergont A, Kaciński M
Neurol Neurochir Pol 2014;48(2):130-5. Epub 2014 Feb 15 doi: 10.1016/j.pjnns.2013.05.003. PMID: 24821639
Kansagra S, Mikati MA, Vigevano F
Handb Clin Neurol 2013;112:821-6. doi: 10.1016/B978-0-444-52910-7.00001-5. PMID: 23622289

Therapy

Patel S, Maney K, Morris L, Papadopoulou MT, Prange L, Boggs A, Hunanyan A, Megvinov A, Vavassori R, Panagiotakaki E, Mikati MA
Eur J Paediatr Neurol 2024 Mar;49:55-59. Epub 2024 Feb 12 doi: 10.1016/j.ejpn.2024.02.004. PMID: 38367370
Samanta D
Pediatr Neurol 2020 Feb;103:12-20. Epub 2019 Nov 1 doi: 10.1016/j.pediatrneurol.2019.10.003. PMID: 31836335
Uchitel J, Helseth A, Prange L, McLean M, Ghusayni R, Sachdev M, Hunanyan A, Mikati MA
Neurology 2019 Sep 24;93(13):e1248-e1259. Epub 2019 Sep 4 doi: 10.1212/WNL.0000000000008159. PMID: 31484714
Kansagra S, Mikati MA, Vigevano F
Handb Clin Neurol 2013;112:821-6. doi: 10.1016/B978-0-444-52910-7.00001-5. PMID: 23622289
Gordon N
Dev Med Child Neurol 1995 May;37(5):464-8. doi: 10.1111/j.1469-8749.1995.tb12030.x. PMID: 7768346

Prognosis

Poole J, Zagaglia S, Demurtas R, Farrell F, Walker MC, Sisodiya SM, Balestrini S, Vivekananda U
PLoS One 2022;17(9):e0268720. Epub 2022 Sep 30 doi: 10.1371/journal.pone.0268720. PMID: 36178910Free PMC Article
Pavone P, Pappalardo XG, Ruggieri M, Falsaperla R, Parano E
Medicine (Baltimore) 2022 Aug 5;101(31):e29413. doi: 10.1097/MD.0000000000029413. PMID: 35945798Free PMC Article
Vetro A, Nielsen HN, Holm R, Hevner RF, Parrini E, Powis Z, Møller RS, Bellan C, Simonati A, Lesca G, Helbig KL, Palmer EE, Mei D, Ballardini E, Van Haeringen A, Syrbe S, Leuzzi V, Cioni G, Curry CJ, Costain G, Santucci M, Chong K, Mancini GMS, Clayton-Smith J, Bigoni S, Scheffer IE, Dobyns WB, Vilsen B, Guerrini R; ATP1A2/A3-collaborators
Brain 2021 Jun 22;144(5):1435-1450. doi: 10.1093/brain/awab052. PMID: 33880529
Kansagra S, Mikati MA, Vigevano F
Handb Clin Neurol 2013;112:821-6. doi: 10.1016/B978-0-444-52910-7.00001-5. PMID: 23622289
Gordon N
Dev Med Child Neurol 1995 May;37(5):464-8. doi: 10.1111/j.1469-8749.1995.tb12030.x. PMID: 7768346

Clinical prediction guides

Vezyroglou A, Akilapa R, Barwick K, Koene S, Brownstein CA, Holder-Espinasse M, Fry AE, Németh AH, Tofaris GK, Hay E, Hughes I, Mansour S, Mordekar SR, Splitt M, Turnpenny PD, Demetriou D, Koopmann TT, Ruivenkamp CAL, Agrawal PB, Carr L, Clowes V, Ghali N, Holder SE, Radley J, Male A, Sisodiya SM, Kurian MA, Cross JH, Balasubramanian M
Neurology 2022 Oct 4;99(14):e1511-e1526. Epub 2022 Jul 18 doi: 10.1212/WNL.0000000000200927. PMID: 36192182Free PMC Article
Poole J, Zagaglia S, Demurtas R, Farrell F, Walker MC, Sisodiya SM, Balestrini S, Vivekananda U
PLoS One 2022;17(9):e0268720. Epub 2022 Sep 30 doi: 10.1371/journal.pone.0268720. PMID: 36178910Free PMC Article
Vetro A, Nielsen HN, Holm R, Hevner RF, Parrini E, Powis Z, Møller RS, Bellan C, Simonati A, Lesca G, Helbig KL, Palmer EE, Mei D, Ballardini E, Van Haeringen A, Syrbe S, Leuzzi V, Cioni G, Curry CJ, Costain G, Santucci M, Chong K, Mancini GMS, Clayton-Smith J, Bigoni S, Scheffer IE, Dobyns WB, Vilsen B, Guerrini R; ATP1A2/A3-collaborators
Brain 2021 Jun 22;144(5):1435-1450. doi: 10.1093/brain/awab052. PMID: 33880529
Pratt M, Uchitel J, McGreal N, Gordon K, Prange L, McLean M, Noel RJ, Rikard B, Rogers Boruta MK, Mikati MA
Orphanet J Rare Dis 2020 Sep 3;15(1):231. doi: 10.1186/s13023-020-01474-w. PMID: 32883312Free PMC Article
Uchitel J, Helseth A, Prange L, McLean M, Ghusayni R, Sachdev M, Hunanyan A, Mikati MA
Neurology 2019 Sep 24;93(13):e1248-e1259. Epub 2019 Sep 4 doi: 10.1212/WNL.0000000000008159. PMID: 31484714

Recent systematic reviews

Motavasseli D, Delorme C, Bayle N, Gracies JM, Roze E, Baude M
Toxins (Basel) 2024 Sep 13;16(9) doi: 10.3390/toxins16090392. PMID: 39330850Free PMC Article

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