Pantothenate kinase-associated neurodegeneration with increased lentiform nuclei cerebral blood flow.
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.
Koyama
M
M
Department of Nuclear Medicine, Cancer Institute Hospital, Tokyo, Japan.
Yagishita
A
A
D001921:Brain / Q000000981:diagnostic imaging* / Q000473:pathology; D002560:Cerebrovascular Circulation; D002648:Child; D003342:Corpus Striatum / Q000098:blood supply* / Q000000981:diagnostic imaging; D003545:Cysteine / Q000031:analogs & derivatives; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009154:Mutation; D009410:Nerve Degeneration / Q000000981:diagnostic imaging*; D015609:Organotechnetium Compounds; D006211:Pantothenate Kinase-Associated Neurodegeneration / Q000175:diagnosis / Q000000981:diagnostic imaging* / Q000235:genetics / Q000503:physiopathology; D017853:Phosphotransferases (Alcohol Group Acceptor) / Q000235:genetics*; D019275:Radiopharmaceuticals; D015899:Tomography, Emission-Computed, Single-Photon
D015609:Organotechnetium Compounds; D019275:Radiopharmaceuticals; D017853:Phosphotransferases (Alcohol Group Acceptor); C023088:pantothenate kinase; C059659:technetium Tc 99m bicisate; D003545:Cysteine