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Journal of Clinical Movement Disorders

, 4:8

Advances in neuroimaging: Insights into neurological and psychiatric disease


Chorea is a hyperkinetic movement disorder consisting of involuntary irregular, flowing movements of the trunk, neck or face. Although Huntington’s disease is the most common cause of chorea in adults, chorea can also result from many other neurodegenerative, metabolic, and autoimmune conditions. While the pathophysiology of these different conditions is quite variable, recent advances in functional imaging have enabled the development of new methods for analysis of brain activity and neuronal dysfunction. In this paper we review the growing body of functional imaging data that has been performed in chorea syndromes and identify particular trends, which can be used to better understand the underlying network changes within the basal ganglia. While it can be challenging to identify whether changes are primary, secondary, or compensatory, identification of these trends can ultimately be useful in diagnostic testing and treatment in many of the conditions that cause chorea.

KeywordsChorea Functional imaging Positron emission tomography Functional MRI Single-photon emission tomography Huntington Acanthocytosis Sydenham AbbreviationsC-CFT11-carbon-2 carbomethoxy-3-4-18F-fluorophenyltropane

CFLB 457S-N-1-ethyl-2-pyrrolidinylmethyl-5- bromo-2,3-dimethoxybenzamide

CSCH 23390R-+-8-Chloro-2,3,4,5-tetrahydro-3-11Cmethyl-5-phenyl-1H-3-benzazepin-7-ol


FMNI-65992-2-3-4-2-Ffluoroethoxyphenyl- 7-methyl-4-oxo-3,4-dihydroquinazolin-2-ylethyl-4- isopropoxyisoindoline-1,3-dione

TcTRODAT99mTc-22-3-4-chlorophenyl-8-methyl-8-azabicyclo1, 2, 3-oct-2-yl-methyl2-mercaptoeythlaminoethylaminoethane-thiolato3-N2,N2’, S2,S2oxo-1R-exo-exo

Tc-ECDTc –ethyl cysteinate dimer


BHCBenign hereditary chorea

BOLDBlood-oxygen-level dependent


DaTSCAN-I-FP-CITI-2B-carbomethoxy-3B-4-iodophenyl-N-3-fluoro-propyl nortropane

DRPLADentatorubropallidoluysian atrophy


fMRIFunctional magnetic resonance imaging

HDHuntington’s disease

MRSMagnetic resonance spectroscopy


PDE10Phosphodiesterase 10

PETPositron emission tomography

PKCParoxysmal kinesigenic choreoathetosis

RS-fMRIResting state fMRI

SCA17Spinocerebellar ataxia 17

SPECTSingle Photon Emission Computed Tomography

UHDRSUnified Huntington’s Disease Rating Scale

Autor: Debra J. Ehrlich - Ruth H. Walker


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