MRI of neurodegeneration with brain iron accumulation.

Stéphane Lehéricy, Emmanuel Roze, Cyril Goizet, Fanny Mochel
Current Opinion in Neurology. 2020-08-01; 33(4): 462-473
DOI: 10.1097/wco.0000000000000844

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1. Curr Opin Neurol. 2020 Aug;33(4):462-473. doi: 10.1097/WCO.0000000000000844.

MRI of neurodegeneration with brain iron accumulation.

Lehéricy S(1)(2)(3), Roze E(1)(4), Goizet C(5)(6), Mochel F(1)(7)(8).

Author information:
(1)Paris Brain Institute, Institut du Cerveau et de la Moelle épinière – ICM,
INSERM U 1127, CNRS UMR 7225, Sorbonne Université, Team ‘Movement Investigations
and Therapeutics’ (MOV’IT).
(2)ICM, Centre de NeuroImagerie de Recherche – CENIR.
(3)Department of Neuroradiology, Pitié-Salpêtrière hospital (AP-HP), Paris.
(4)Department of Neurology, Pitie-Salpetrire Hospital, Public Assistance – Paris
Hospitals (AP-HP), Paris.
(5)Reference Center for Rare ‘Neurogenetic’ Diseases, Department of Medical
Genetics, Pellegrin Hospital, Bordeaux University Hospital.
(6)Rare Diseases Laboratory: Genetics and Metabolism (MRGM), INSERM U1211,
Bordeaux University, Bordeaux, France.
(7)Department of Genetics, Pitié -Salpêtrière Hospital, Public Assistance – Paris
Hospitals (AP-HP), Paris.
(8)Reference Center for Neurometabolic diseases, Pitié -Salpêtrière Hospital,

PURPOSE OF REVIEW: The diagnosis of neurodegeneration with brain iron
accumulation (NBIA) typically associates various extrapyramidal and pyramidal
features, cognitive and psychiatric symptoms with bilateral hypointensities in
the globus pallidus on iron-sensitive magnetic resonance images, reflecting the
alteration of iron homeostasis in this area. This article details the
contribution of MRI in the diagnosis by summarizing and comparing MRI patterns of
the various NBIA subtypes.
RECENT FINDINGS: MRI almost always shows characteristic changes combining iron
accumulation and additional neuroimaging abnormalities. Iron-sensitive MRI shows
iron deposition in the basal ganglia, particularly in bilateral globus pallidus
and substantia nigra. Other regions may be affected depending on the NBIA
subtypes including the cerebellum and dentate nucleus, the midbrain, the
striatum, the thalamus, and the cortex. Atrophy of the cerebellum, brainstem,
corpus callosum and cortex, and white matter changes may be associated and worsen
with disease duration. Iron deposition can be quantified using R2 or quantitative
susceptibility mapping.
SUMMARY: Recent MRI advances allow depicting differences between the various
subtypes of NBIA, providing a useful analytical framework for clinicians.
Standardization of protocols for image acquisition and analysis may help
improving the detection of imaging changes associated with NBIA and the
quantification of iron deposition.

DOI: 10.1097/WCO.0000000000000844
PMID: 32657887 [Indexed for MEDLINE]

Auteurs Bordeaux Neurocampus