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Article

The Unified Batten Disease Rating Scale (UBDRS): Validation and reliability in an independent CLN3 disease sample.

European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society - 1 May 2022

Wibbeler Eva, Nickel Miriam, Schwering Christoph, Schulz Angela, Mink Jonathan W

Abstract excerpt

BACKGROUND: The neuronal ceroid lipofuscinoses (NCLs) are a group of disorders characterized by neurodegeneration and intracellular accumulation of an auto-fluorescent lipopigment. Together, NCLs represent the most common cause of cerebral neurodegenerative disease in children. CLN3 disease, the classic juvenile-onset form (JNCL) due to mutations in CLN3, is characterized by progressive vision loss, epilepsy,...

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Identifiers

PMID: 35427884
DOI: 10.1016/j.ejpn.2022.03.005
PMCID: PMC9879304

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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