Cerebral blood flow in dystonia due to pantothenate kinase-associated neurodegeneration.
Pantothenate kinase-associated neurodegeneration
cerebral blood flow
globus pallidus
tiger’s eye
Journal
The neuroradiology journal
ISSN: 2385-1996
Titre abrégé: Neuroradiol J
Pays: United States
ID NLM: 101295103
Informations de publication
Date de publication:
Dec 2020
Dec 2020
Historique:
pubmed:
28
8
2020
medline:
8
9
2021
entrez:
28
8
2020
Statut:
ppublish
Résumé
The aim of this study was to look for deviations of cerebral perfusion in patients suffering from pantothenate kinase-associated neurodegeneration, where the globus pallidus is affected by severe accumulation of iron. Under resting conditions, cerebral blood flow was measured by the magnetic resonance imaging technique of arterial spin labelling in cortical areas and basal ganglia in eight pantothenate kinase-associated neurodegeneration patients and 14 healthy age-matched control subjects and correlated to T2* time of these areas and - in patients - to clinical parameters. Despite highly significant differences of T2* time of the globus pallidus (20 vs 39 ms, The absence of any obvious deviations of perfusion in the group of patients during a resting condition does not support the view that (non-functional) vascular pathology is a major pathogenic factor in pantothenate kinase-associated neurodegeneration in the younger age group. The findings underline the value of the arterial spin technique to measure cerebral blood flow in areas of disturbed susceptibility.
Sections du résumé
BACKGROUND AND PURPOSE
OBJECTIVE
The aim of this study was to look for deviations of cerebral perfusion in patients suffering from pantothenate kinase-associated neurodegeneration, where the globus pallidus is affected by severe accumulation of iron.
MATERIAL AND METHODS
METHODS
Under resting conditions, cerebral blood flow was measured by the magnetic resonance imaging technique of arterial spin labelling in cortical areas and basal ganglia in eight pantothenate kinase-associated neurodegeneration patients and 14 healthy age-matched control subjects and correlated to T2* time of these areas and - in patients - to clinical parameters.
RESULTS
RESULTS
Despite highly significant differences of T2* time of the globus pallidus (20 vs 39 ms,
CONCLUSION
CONCLUSIONS
The absence of any obvious deviations of perfusion in the group of patients during a resting condition does not support the view that (non-functional) vascular pathology is a major pathogenic factor in pantothenate kinase-associated neurodegeneration in the younger age group. The findings underline the value of the arterial spin technique to measure cerebral blood flow in areas of disturbed susceptibility.
Identifiants
pubmed: 32851917
doi: 10.1177/1971400920943967
pmc: PMC7788684
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
479-485Références
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