Hyperglycemic choreoathetosis: role of the putamen in pathogenesis.
Hyperglycemic choreoathetosis (HC) is an uncommon syndrome often associated with hyperintensity of the basal ganglia on MRI. We performed a retrospective review of cases with HC to characterize the clinical, biochemical, and neuroimaging (CT, MRI, and MR spectroscopy) findings and to propose a mechanism for this syndrome. Seven HC patients with a mean age of 75.1 years, mean blood glucose of 27.4 mmol/L, and mean plasma osmolarity of 313.4 mmol/L were studied. All had MR-T1 hyperintensity of the putamen on the side contralateral to the choreoathetosis. Two patients had additional hyperintensity of the globus pallidus while one also had involvement of the caudate. On MR-T2, 2 patients showed hyperintensity, 2 isointensity, and 3 hypointensity in the putamen. MR spectroscopy showed elevated choline and reduced N-acetylaspartate; two patients also had elevated myoinositol levels. Our findings suggest that the putamen has a central role in HC, and MR spectroscopy supports neuronal dysfunction in the putamen. Biochemical and neuroimaging findings support hyperviscosity as the most plausible mechanism.
Kandiah
Nagaendran
N
Department of Neurology, National Neuroscience Institute, 11 Jalan Tan Tock Seng, Singapore. nagaendran_kandiah@nni.com.sg
Tan
Kevin
K
Lim
C C Tchoyoson
CC
Venketasubramanian
Narayanaswamy
N
D000368:Aged; D000369:Aged, 80 and over; D002819:Chorea / Q000150:complications / Q000473:pathology*; D038524:Diffusion Magnetic Resonance Imaging / Q000379:methods; D005260:Female; D006801:Humans; D006943:Hyperglycemia / Q000150:complications / Q000473:pathology*; D009682:Magnetic Resonance Spectroscopy / Q000379:methods; D008297:Male; D011699:Putamen / Q000473:pathology*; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed / Q000379:methods