New study findings challenge age-associated beliefs in Dementia

A recent study has produced novel insights into changes in brains with Krabbe disease that are similar to those changes seen in dementia with Lewy bodies and Parkinson’s Disease.

Krabbe disease is a rare autosomal recessive neurodegenerative childhood disorder, affecting about 1 in 100,000 individuals. The observational study investigated whether proteins found in Krabbe disease had pathological similarities to those found in Lewy body.

The study’s findings were recently published in the journal, Brain. They have shown for the first time that proteins linked with age-associated neurodegenerative diseases, like dementia with Lewy bodies, shared key qualities with those found in Krabbe disease. This research has important implications for understanding the mechanisms underlying Lewy body formation in Lewy body disease.

Dr Erskine, Alzheimer’s Research UK Research Fellow at Newcastle University, who is an expert in Lewy bodies and led the study commented,

“These findings challenge the view that changes to the brain that underlie these forms of dementia are merely age-associated.”

“This is an important message, as while we cannot stop ageing, we can potentially fix something that is not working properly.”

The research, funded by Alzheimer’s Research UK and led by Newcastle University, was supported by a range of partners, including the Newcastle Brain Tissue Resource and the NIHR Newcastle Biomedical Research Centre (BRC).

Information above curtesy of Medscape, read full article here

Christopher Hatton et al. Prion-like α-synuclein pathology in the brain of children with Krabbe disease, Brain (2022). DOI: 10.1093 / brain / awac002