Differential involvement of hippocampal subfields in Niemann-Pick type C disease: a case-control study.
Pierre WibawaFlorian KurthEileen LudersChristos PantelisVanessa L CropleyMaria A Di BiaseDennis VelakoulisMark WalterfangPublished in: Metabolic brain disease (2021)
Hippocampal brain regions are strongly implicated in Niemann Pick type C disease (NPC), but little is known regarding distinct subregions of the hippocampal complex and whether these are equally or differentially affected. To address this gap, we compared volumes of five hippocampal subfields between NPC and healthy individuals using MRI. To this end, 9 adult-onset NPC cases and 9 age- and gender-matched controls underwent a 3 T T1-weighted MRI scan. Gray matter volumes of the cornu ammonis (CA1, CA2 and CA3), dentate gyrus (DG), subiculum, entorhinal cortex and hippocampal-amygdalar transition area were calculated by integrating MRI-based image intensities with microscopically defined cytoarchitectonic probabilities. Compared to healthy controls, NPC patients showed smaller volumes of the CA1-3 and DG regions bilaterally, with the greatest difference localized to the left DG (Cohen's d = 1.993, p = 0.008). No significant associations were shown between hippocampal subfield volumes and key clinical features of NPC, including disease duration, symptom severity and psychosis. The pattern of hippocampal subregional atrophy in NPC differs from those seen in other dementias, which may indicate unique cytoarchitectural vulnerabilities in this earlier-onset disorder. Future MRI studies of hippocampal subfields may clarify its potential as a biomarker of neurodegeneration in NPC.
Keyphrases
- cerebral ischemia
- contrast enhanced
- temporal lobe epilepsy
- magnetic resonance imaging
- end stage renal disease
- computed tomography
- chronic kidney disease
- deep learning
- ejection fraction
- subarachnoid hemorrhage
- brain injury
- machine learning
- protein kinase
- blood brain barrier
- white matter
- peritoneal dialysis
- patient reported outcomes
- network analysis