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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 27:212-213, January 2006
© 2006 American Society of Neuroradiology

CASE REPORT
PEDIATRICS

Pantothenate Kinase-Associated Neurodegeneration with Increased Lentiform Nuclei Cerebral Blood Flow

M. Koyama^a and A. Yagishita^b

^a Department of Nuclear Medicine, Cancer Institute Hospital, Tokyo, Japan
^b Department of Neuroradiology, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan

Address correspondence to Masmichi Koyama, MD, Department of Nuclear Medicine, Cancer Institute Hospital, - 3-10-6, Ariake, Koto-ku, Tokyo 135-8550, Japan

Summary: We report a case of pantothenate kinase-associated neurodegeneration with increased regional cerebral blood flow (rCBF) in bilateral lentiform nuclei on technetium Tc-99m ethyl cysteinate dimer single-photon emission CT (ECD-SPECT). A 6-year-old boy presented with opisthotonus. T2*-weighted MR images revealed areas of marked hypointensity with a hyperintense focus in bilateral globus pallidus, creating the characteristic eye-of-the-tiger appearance. ECD-SPECT showed increased rCBF in bilateral lentiform nuclei.

This article has been cited by other articles:

				A. Gregory and S.J. Hayflick PANK2 Mutation Screening Recommended to Confirm Diagnosis of Pantothenate Kinase-Associated Neurodegeneration. AJNR Am. J. Neuroradiol., May 1, 2006; 27(5): 951 - 951. [Full Text] [PDF]