Co-Occurrence of Seizure and Chorea in a Patient with Nonketotic Hyperglycemia

2005 
roleptic drugs. She had no family history of movement disorders. On admission, she had chorea involving the left arm and leg. The chorea was continuous and could not be suppressed voluntarily, although it disappeared during sleep. It gradually worsened over the following days, involving the left face, left arm, left leg, and the right leg to a lesser degree. The patient also showed frequent, recurrent episodes of reduced attentiveness and responsiveness, rambling speech, irritability, and visual hallucinations, which consisted of several onions painted in all the colors of the rainbow. The level of fasting blood glucose was 473 mg/dl and glycosylated hemoglobin A Ic was 18%. No ketones were detected in urinalysis. Other routine blood tests and specialized tests including thyroid test, antistreptolysin O, rheumatoid factor, lupus anticoagulant, antiphospholipid antibody, antinuclear antibody, anti-ds-DNA, and antineutrophil cytoplasmic antibody were all normal. Neurological examination revealed only mildly decreased deep tendon refl exes and sensation of vibration on both lower legs. Her gait was markedly impaired due to severe chorea. A brain CT performed 10 days after symptom onset showed slightly high densities in the bilateral basal ganglia ( fi g. 1 a). The level of hyperattenuation decreased in Dear Sir, Chorea and striatal hyperintensity on T 1 -weighted MRI associated with nonketotic hyperglycemia (NKH) have been recognized as a peculiar disorder [1, 2] . It tends to occur in elderly people. Most reported cases are in people of east Asian origin, which suggests a possible genetic disposition to the disorder [3] . The etiological relationship between chorea and striatal hyperintensity, and the pathogenesis of striatal lesions remains unknown. Seizures associated with NKH have also been reported [4, 5] . However, the coexistence of seizure and chorea in a patient with NKH has not been previously reported. We describe here a patient who concurrently developed seizure and chorea associated with NKH.
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