Hyperglycemic hemichorea

Epidemiology

  • More common in hyperglycemic females

Clinical presentation

  • Acute onset of involuntary movements involving one side of the body.
  • Bilateral chorea is less common: in one retrospective study, only 6 of 53 patients experiencing abnormal movements had bilateral symptoms.
  • Blood and urine ketones are not elevated, but there is high serum osmolality (hyperosmolar hyperglycemic state)

Imaging features

  • Imaging findings are generally contralateral to the side of the body experiencing chorea.
  • The putamen is most commonly involved, but other parts of basal ganglia can be affected as well.
  • Noncontrast CT typically: unilateral hyperintensity of the basal ganglia.

    Right hyperdense basal ganglia

    • MRI:
      – T1 hyperintensity, with more variable signal changes on T2 sequence.
      – Restricted diffusion and hypointensity on susceptibility-weighted imaging also have been reported.

      T1 hyperintense right basal ganglia


      T2* sequence (GRE)


      SWI sequence shows increased susceptibility in right basal ganglia.

  • Signal abnormalities on MRI usually resolves several months to a year after chorea had subsided.

Pathophysiology

Capture

Treatment

  • Correction of hyperglycemia.
  • Symtoms usually resolves within days to weeks of blood glucose normalization (a lag between symptom resolution and radiologic finding resolution)
  • For patients with symptoms that persist despite glucose control, dopamine receptor blockers can be used.

More on basal ganglia abnormalities please visit this link:
https://medicalrojak.wordpress.com/2015/10/19/differential-diagnosis-of-bilateral-abnormalities-of-basal-ganglia-and-thalamus/

References:

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