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LEUKODYSTROPHY, HYPOMYELINATING, 28(HLD28)

MedGen UID:
1054668
Concept ID:
CN378649
Disease or Syndrome
Synonym: HLD28
 
Gene (location): MAL (2q11.1)
 
OMIM®: 620978

Definition

Hypomyelinating leukodystrophy-28 (HLD28) is an autosomal recessive disorder characterized by developmental delay and nystagmus in infancy, followed by significant learning disabilities and progressive motor deterioration within the first decade (Elpidorou et al., 2022). For a discussion of genetic heterogeneity of hypomyelinating leukodystrophy, see 312080. [from OMIM]

Recent clinical studies

Etiology

Oikarainen JH, Knuutinen OA, Kangas SM, Rahikkala EJ, Pokka TM, Moilanen JS, Hinttala RM, Vieira PM, Uusimaa JM, Suo-Palosaari MH
Dev Med Child Neurol 2025 Feb;67(2):186-194. Epub 2024 Jul 30 doi: 10.1111/dmcn.16036. PMID: 39080972Free PMC Article
Yan H, Ji H, Kubisiak T, Wu Y, Xiao J, Gu Q, Yang Y, Xie H, Ji T, Gao K, Li D, Xiong H, Shi Z, Li M, Zhang Y, Duan R, Bao X, Jiang Y, Burmeister M, Wang J
J Hum Genet 2021 Aug;66(8):761-768. Epub 2021 Feb 18 doi: 10.1038/s10038-020-00896-5. PMID: 33597727Free PMC Article
Tonduti D, Vanderver A, Berardinelli A, Schmidt JL, Collins CD, Novara F, Genni AD, Mita A, Triulzi F, Brunstrom-Hernandez JE, Zuffardi O, Balottin U, Orcesi S
J Child Neurol 2013 Jun;28(6):795-800. Epub 2012 Jul 17 doi: 10.1177/0883073812450944. PMID: 22805248Free PMC Article
Boespflug-Tanguy O, Labauge P, Fogli A, Vaurs-Barriere C
Curr Neurol Neurosci Rep 2008 May;8(3):217-29. doi: 10.1007/s11910-008-0034-x. PMID: 18541117

Diagnosis

Oikarainen JH, Knuutinen OA, Kangas SM, Rahikkala EJ, Pokka TM, Moilanen JS, Hinttala RM, Vieira PM, Uusimaa JM, Suo-Palosaari MH
Dev Med Child Neurol 2025 Feb;67(2):186-194. Epub 2024 Jul 30 doi: 10.1111/dmcn.16036. PMID: 39080972Free PMC Article
Siori D, Vlachakis D, Makrythanasis P, Traeger-Synodinos J, Veltra D, Kampouraki A, Chrousos GP
Genes (Basel) 2024 Apr 23;15(5) doi: 10.3390/genes15050525. PMID: 38790154Free PMC Article
Yan H, Ji H, Kubisiak T, Wu Y, Xiao J, Gu Q, Yang Y, Xie H, Ji T, Gao K, Li D, Xiong H, Shi Z, Li M, Zhang Y, Duan R, Bao X, Jiang Y, Burmeister M, Wang J
J Hum Genet 2021 Aug;66(8):761-768. Epub 2021 Feb 18 doi: 10.1038/s10038-020-00896-5. PMID: 33597727Free PMC Article
Di Bella D, Magri S, Benzoni C, Farina L, Maccagnano C, Sarto E, Moscatelli M, Baratta S, Ciano C, Piacentini SHMJ, Draghi L, Mauro E, Pareyson D, Gellera C, Taroni F, Salsano E
Eur J Neurol 2021 Mar;28(3):934-944. Epub 2020 Dec 3 doi: 10.1111/ene.14646. PMID: 33190326
Boespflug-Tanguy O, Labauge P, Fogli A, Vaurs-Barriere C
Curr Neurol Neurosci Rep 2008 May;8(3):217-29. doi: 10.1007/s11910-008-0034-x. PMID: 18541117

Prognosis

Siori D, Vlachakis D, Makrythanasis P, Traeger-Synodinos J, Veltra D, Kampouraki A, Chrousos GP
Genes (Basel) 2024 Apr 23;15(5) doi: 10.3390/genes15050525. PMID: 38790154Free PMC Article
Tonduti D, Vanderver A, Berardinelli A, Schmidt JL, Collins CD, Novara F, Genni AD, Mita A, Triulzi F, Brunstrom-Hernandez JE, Zuffardi O, Balottin U, Orcesi S
J Child Neurol 2013 Jun;28(6):795-800. Epub 2012 Jul 17 doi: 10.1177/0883073812450944. PMID: 22805248Free PMC Article

Clinical prediction guides

Harting I, Garbade SF, Roosendaal SD, Fels-Palesandro H, Raudonat C, Mohr A, Wolf NI
Eur J Paediatr Neurol 2024 Sep;52:59-66. Epub 2024 Jul 27 doi: 10.1016/j.ejpn.2024.07.010. PMID: 39098096
Harting I, Garbade SF, Rosendaal SD, Mohr A, Sherbini O, Vanderver A, Wolf NI
Eur J Paediatr Neurol 2022 Nov;41:71-79. Epub 2022 Nov 4 doi: 10.1016/j.ejpn.2022.10.003. PMID: 36368233Free PMC Article
Fogli A, Schiffmann R, Hugendubler L, Combes P, Bertini E, Rodriguez D, Kimball SR, Boespflug-Tanguy O
Eur J Hum Genet 2004 Jul;12(7):561-6. doi: 10.1038/sj.ejhg.5201189. PMID: 15054402

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