Browse by author
Lookup NU author(s): Dr Grainne Gorman,
Professor Rita Horvath,
Professor Andrew Blamire,
Professor Patrick Chinnery
Full text for this publication is not currently held within this repository. Alternative links are provided below where available.
Neuroferritinopathy is an autosomal dominant extrapyramidal movement disorder, caused by FTL gene mutations. Iron decreases the MR T2* decay time, therefore increasing the R2* (R2* = 1 /T2*), which correlates with brain tissue iron content. 31 structural and quantitative MR imaging assessment of R2* in 10 patients with neuroferritinopathy demonstrated a unique pattern of basal ganglia cavitation involving the substantia nigra in older patients and increasing thalamic R2* signal intensity detectable during 6 months. Increasing R2* signal intensity in the thalamus correlated with progression on a clinical rating scale measuring dystonia severity. Thalamic R2* signal intensity is a clinically useful method of objectively tracking disease progression in this form of neurodegeneration with brain iron accumulation.
Author(s): McNeill A, Gorman G, Khan A, Horvath R, Blamire AM, Chinnery PF
Publication type: Article
Publication status: Published
Journal: American Journal of Neuroradiology
Print publication date: 01/10/2012
ISSN (print): 0195-6108
ISSN (electronic): 1936-959X
Publisher: American Society of Neuroradiology
Altmetrics provided by Altmetric