CEREBRAL HYPOPERFUSION AND HYPOMETABOLISM WITH ALTERED STRIATAL SIGNAL INTENSITY IN CHOREA-ACANTHOCYTOSIS - A COMBINED PET AND MRI STUDY

We studied cerebral perfusion and oxygen metabolism in three patients with chorea-acanthocytosis using positron-emission tomography and oxyg en-15 labeled O-2 and CO2. High-field magnetic resonance imaging also was performed, Regional cerebral blood flow (rCBF) and oxygen metaboli sm (rCMRO(2)) were significantly reduced in the caudate and putamen wh en compared with seven control subjects. Mild but significant reductio ns of rCBF (lower than the normal control values -2 SD) were found in the bilateral frontal, left temporal and parietal, and bilateral thala mic areas; rCMRO(2) was reduced in the bilateral frontal and left temp oral arena. Magnetic resonance imaging showed increased signal intensi ty accompanied by scattered bright spots in the caudate bead and putam en on T2-weighted images; decreased signal intensity was shown at thes e sites on T1-weighted images. These findings were not observed in 10 neurologically normal volunteers and are rare in the common hyperkinet ic form of Huntington's disease. Reduced cerebral perfusion and oxygen metabolism seem to be related to the intellectual and personality cha nges that occur in chorea-acanthocytosis. Combined positron-emission t omography and magnetic resonance imaging studies may improve diagnosti c accuracy in patients with chorea-acanthocytosis and related disorder s.