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Neuropediatrics 1996; 27(2): 84-87
DOI: 10.1055/s-2007-973754
Original Articles

© Hippokrates Verlag GmbH Stuttgart

Brain Perfusion SPECT in Juvenile Neuronal Ceroid Lipofuscinosis

J. Launes1 , H. Heiskala2 , P. Nikkinen3 , P. Santavuori2
  • 1Departments of Neurology, University Central Hospital, Helsinki, Finland
  • 2Departments of Child Neurology, University Central Hospital, Helsinki, Finland
  • 3Central Laboratory, Division of Nuclear Medicine, University Central Hospital, Helsinki, Finland
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Publication Date:
13 March 2007 (online)

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Abstract

The juvenile neuronal ceroid-lipofuscinosis (JNCL) is a recessively inherited progressive encephalopathy. We studied 21 JNCL patients with a duration of illness of 1 to 17 years by 99mTc-HM-PAO single photon emission computed tomography (SPECT) and correlated the findings with clinical parameters. All patients had at least one hypoperfused brain area, the median number of such areas was 5 per patient. Parietally, occipitally, and in the cerebellar lobes hypoperfusion was usually mild whereas it was temporally more severe. Right parietal hypoperfusion correlated to neurological dysfunction.

Key words

Juvenile neuronal ceroid lipofuscinosis - Single photon emission computed tomography