Moyamoya and progressive myoclonic epilepsy secondary to

ANCL ANCL, adult neuronal ceroid lipofuscinosis BMIPB, the Brain Injury Rehabilitation Trust Memory and Information Processing Battery CLN6 Kufs disease LINCL, late-infantile neuronal ceroid lipofuscinosis MERRF, mitochondrial epilepsy with ragged red fibres Moyamoya NCL NCL, neuronal ceroid lipofuscinosis Neuronal ceroid lipofuscinosis PPT1, palmitoyl-protein thioesterase 1 SEP, somatosensory evoked potentials TPP1, tripeptidyl peptidase 1 WAIS-IV, Wechsler Adult Intelligence Scale (4th edition) Wiegl, Weigl Color Form Sorting Test mtDNA, mitochondrial DNA

Journal

Epilepsy & behavior reports
ISSN: 2589-9864
Titre abrégé: Epilepsy Behav Rep
Pays: United States
ID NLM: 101750909

Informations de publication

Date de publication:
2020
Historique:
received: 20 05 2020
revised: 29 07 2020
accepted: 01 08 2020
entrez: 7 10 2020
pubmed: 8 10 2020
medline: 8 10 2020
Statut: epublish

Résumé

The neuronal ceroid lipofuscinoses (NCL) are a collection of lysosomal storage diseases characterised by the accumulation of characteristic inclusions containing lipofuscin in various tissues of the body and are one of the causes of progressive myoclonic epilepsy. Mutations in at least thirteen genes have been identified as causes of NCL, which can present as infantile, late-infantile, juvenile or adult forms.

Identifiants

pubmed: 33024953
doi: 10.1016/j.ebr.2020.100389
pii: S2589-9864(20)30037-X
pmc: PMC7528204
doi:

Types de publication

Case Reports

Langues

eng

Pagination

100389

Subventions

Organisme : Wellcome Trust
Pays : United Kingdom

Informations de copyright

© 2020 The Authors.

Déclaration de conflit d'intérêts

None.

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Auteurs

Jamie Talbot (J)

Department of Clinical and Experimental Epilepsy, UCL Queen Square Institute of Neurology, Queen Square, London WC1N 3BG, UK.

Priyanka Singh (P)

Department of Clinical and Experimental Epilepsy, UCL Queen Square Institute of Neurology, Queen Square, London WC1N 3BG, UK.

Clinda Puvirajasinghe (C)

Rare & Inherited Disease Laboratory, North Thames Genomic Laboratory Hub, Great Ormond Street Hospital for Children NHS Foundation Trust, Levels 4-6 Barclay House, 37, Queen Square, London WC1N 3BH, UK.

Sanjay M Sisodiya (SM)

Department of Clinical and Experimental Epilepsy, UCL Queen Square Institute of Neurology, Queen Square, London WC1N 3BG, UK.

Fergus Rugg-Gunn (F)

Department of Clinical and Experimental Epilepsy, UCL Queen Square Institute of Neurology, Queen Square, London WC1N 3BG, UK.

Classifications MeSH