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Updated: Jan 11, 2026

An In Vitro Model for the Study of Cellular Pathophysiology in Globoid Cell Leukodystrophy

An In Vitro Model for the Study of Cellular Pathophysiology in Globoid Cell Leukodystrophy

Published on: October 21, 2014

Cognitive and intellectual functioning in leukodystrophy patients: a systematic review.

Wietske H M Grol^1,2,3, Marjo S van der Knaap^1,2,4, Nicole I Wolf^1,2

¹Department of Child Neurology, Emma's Children's Hospital, Amsterdam UMC, Amsterdam, The Netherlands.

Orphanet Journal of Rare Diseases

|November 11, 2025

Summary

Leukodystrophies, genetic white matter disorders, cause cognitive decline. This review highlights that while intelligence and processing speed are often affected, specific cognitive profiles remain unclear, necessitating further research for better patient guidance.

Keywords:

Cognitive functioning Intelligence Leukodystrophies Neuropsychological tests

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Published on: October 13, 2016

Area of Science:

Neuroscience
Genetics
Neurology

Background:

Leukodystrophies are genetic white matter disorders causing progressive neurological deterioration.
Cognitive decline, mood changes, and motor dysfunction are common, potentially leading to underestimation of deficits.
Lack of understanding hinders timely interventions and patient guidance.

Conclusions:

Cognition and intelligence are affected in leukodystrophies, with information processing speed frequently impaired.
Specific cognitive and intellectual profiles remain unclear, hindering effective patient guidance.
Further well-designed studies are needed to clarify profiles, inform testing guidelines, and develop outcome measures for therapies.