Age: 74
Sex: Male
Indication: Right-sided jerking movements
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Nonketotic Hyperglycemic Hemichorea-Hemiballism
Findings
- T1 signal hyperintensity in the left putamen
- No evidence of acute infarct, mass effect, or hydrocephalus
- Scattered T2/FLAIR hyperintensities in the bilateral subcortical and periventricular white matter
- Bilateral basal ganglia calcifications
- Small mucous retention cysts in the right maxillary sinus
- 15 mm T2 hyperintense structure in the left parapharyngeal space
Diagnosis
- Nonketotic hyperglycemic hemichorea-hemiballism
Sample Report
T1 signal hyperintensity in the left putamen, which given clinically reported contralateral jerking movements is favored to represent nonketotic hyperglycemic hemichorea-hemiballism. Other toxic/metabolic disorders associated with T1 hyperdensity in the basal ganglia are considered unlikely given profound asymmetry.
No evidence of acute infarct, mass effect, or hydrocephalus.
Scattered T2/FLAIR hyperintensities in the bilateral subcortical and periventricular white matter, which though nonspecific likely relate to chronic small vessel disease.
15 mm T2 hyperintense structure in the left parapharyngeal space which may represent a cyst, nerve sheath tumor, or salivary gland neoplasm. Recommend ENT referral for further evaluation.
Discussion
- Nonketotic hyperglycemic hemichorea-hemiballism is a rare movement disorder seen in patients with uncontrolled hyperglycemia
- The diagnosis is usually made clinically, but imaging findings can be supportive
- The most reliable imaging finding is intrinsic T1 signal hyperintensity, though the involved basal ganglia may also be hyperdense on CT, may have T2 signal hypointensity, and may have associated susceptibility artifact or restricted diffusion
- The putamen is involved more frequently than the globus pallidus or caudate
- Symptoms typically rapidly improve with correction of hyperglycemia with imaging findings resolving more slowly over 2-11 months
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