Brain Perfusion SPECT in Juvenile Neuronal Ceroid Lipofuscinosis

J. Launes; H. Heiskala; P. Nikkinen; P. Santavuori

doi:10.1055/s-2007-973754

Neuropediatrics, Table of Contents

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

Original Articles

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

Abstract

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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

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