Chorea-ballismus with nonketotic hyperglycemia in primary diabetes mellitus.
OBJECTIVE
To describe the neuroimaging (Ct, MR, and single-photon emission CT [SPECT]) findings in a series of patients with chorea-ballismus associated with nonketotic hyperglycemia in primary diabetes mellitus and to correlate the imaging findings with the clinical presentation.


METHODS
The neuroimaging and clinical data from 10 patients with chorea-ballismus associated with nonketotic hyperglycemia in primary diabetes mellitus were evaluated. Family and drug histories, as well as other causes of chorea, were excluded. All 10 patients had CT, 5 also had MR imaging, and 3 had SPECT examinations. Three had follow-up CT and MR imaging studies, and MR findings were correlated with CT findings in 5 cases. Two experienced neuroradiologists, aware of the diagnosis but blinded to the clinical status of the patients, evaluated all images and reached a consensus as to the final interpretation.


RESULTS
CT studies in 9 of 10 patients showed a hyperdense putamen and/or caudate nucleus; in 1, the CT findings were normal. T1-weighted MR images in all 5 patients who had MR imaging (including the patient with a normal CT study) showed hyperintense lesions without significant T2 signal alternation at the basal ganglia. In all 3 of the patients who had SPECT studies of the brain, the scans revealed hypoperfusion at corresponding areas. All 3 follow-up studies depicted resolution of the lesions in the abnormal basal ganglia. Increased hypointensity on T2-weighted and gradient-echo T2*-weighted images was also observed in the sequential MR images. In all patients, the initial side of involvement correlated well with the neuroimaging findings. The chorea resolved within 2 days after treatment of the hyperglycemia in 9 patients.


CONCLUSIONS
In patients with chorea-ballismus associated with nonketotic hyperglycemia in primary diabetes mellitus, CT and T1-weighted MR images show unilateral or bilateral lesions of the putamen and/or caudate. SPECT scans show hypoperfusion. These findings may be related to petechial hemorrhage and/or myelin destruction. Early recognition of these imaging characteristics may facilitate diagnosis of primary diabetes mellitus with hyperglycemia and prompt appropriate therapy.
Lai
P H
PH
Department of Radiology, Veterans General Hospital- Kaohsiung, National Yang Ming University, Taiwan, Republic of China.
Tien
R D
RD
Chang
M H
MH
Teng
M M
MM
Yang
C F
CF
Pan
H B
HB
Chen
C
C
Lirng
J F
JF
Kong
K W
KW
D000368:Aged; D001480:Basal Ganglia Diseases / Q000175:diagnosis*; D001928:Brain Diseases, Metabolic / Q000175:diagnosis*; D002545:Brain Ischemia / Q000175:diagnosis; D002421:Caudate Nucleus / Q000473:pathology; D002819:Chorea / Q000175:diagnosis*; D004292:Dominance, Cerebral / Q000502:physiology; D006801:Humans; D006944:Hyperglycemic Hyperosmolar Nonketotic Coma / Q000175:diagnosis*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination; D011699:Putamen / Q000473:pathology; D012189:Retrospective Studies; D015899:Tomography, Emission-Computed, Single-Photon; D014057:Tomography, X-Ray Computed