MRI data confirm the selective involvement of thalamic and amygdalar nuclei in amyotrophic lateral sclerosis and primary lateral sclerosis

Data in Brief

卷:32

Efstratios Karavasilis¹  Russell L. McLaughlin²  Alice Vajda³  Mark A. Doherty⁴  Peter Bede⁴  Colette Donaghy⁴  Jennifer C. Hengeveld⁴  Mary Clare McKenna⁵  Kai Ming Chang⁵  We Fong Siah⁵  Rangariroyashe H. Chipika⁵  Stacey Li Hi Shing⁵  Eoin Finegan⁵  Orla Hardiman⁶  Siobhan Hutchinson⁷  Foteini Christidi⁸ 

[1] Electronics and Computer Science, University of Southampton, United Kingdom;

[2] Social Care Trust, Belfast, Northern Ireland, United Kingdom;

[3] 2nd Department of Radiology, Attikon University Hospital, University of Athens, Athens, Greece;

[4] Complex Trait Genomics Laboratory, Smurfit Institute of Genetics, Trinity College Dublin, Ireland;

[5] Computational Neuroimaging Group, Biomedical Sciences Institute, Trinity College Dublin, Ireland;

[6] Department of Neurology, St James's Hospital Dublin, Ireland;

[7] Department of Neurology, Western Health &

[8] First Department of Neurology, Aeginition Hospital, National and Kapodistrian University of Athens, Greece;

关键词: Motor neuron disease;    Amyotrophic lateral sclerosis;    Primary lateral sclerosis;    Thalamus;    Neuroimaging;   

DOI  :  

来源: DOAJ

【 摘 要 】

A standardised imaging protocol was implemented to evaluate disease burden in specific thalamic and amygdalar nuclei in 133 carefully phenotyped and genotyped motor neuron disease patients. “Switchboard malfunction in motor neuron diseases: selective pathology of thalamic nuclei in amyotrophic lateral sclerosis and primary lateral sclerosis” [1] “Amygdala pathology in amyotrophic lateral sclerosis and primary lateral sclerosis” [2] Raw volumetric data, group comparisons, effect sizes and percentage change are presented. Both ALS and PLS patients exhibited focal thalamus atrophy in ventral lateral and ventral anterior regions revealing extrapyramidal motor degeneration. Reduced accessory basal nucleus and cortical nucleus volumes were noted in the amygdala of C9orf72 negative ALS patients compared to healthy controls. ALS patients carrying the GGGGCC hexanucleotide repeats in C9orf72 exhibited preferential pathology in the mediodorsal-paratenial-reuniens thalamic nuclei and in the lateral nucleus and cortico-amygdaloid transition area of the amygdala. Considerable thalamic atrophy was observed in the sensory nuclei and lateral geniculate region of PLS patients. Our data demonstrate genotype-specific patterns of thalamus and amygdala involvement in ALS and a distinct disease-burden pattern in PLS. The dataset may be utilised for validation purposes, meta-analyses and the interpretation of thalamic and amygdalar profiles from other ALS genotypes.

【 授权许可】

Unknown