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Diabetic hemichorea-hemiballismus with nonketotic hyperglicemia: a rare cause of hyperkinetic movement disorders

Hemibalismo-hemicoreia associada à hiperglicemia não-cetótica: uma rara causa de distúrbios hipercinéticas do movimento

A 67-year-old woman was admitted with right hemichorea-hemiballismus. Blood glucose: 831 mg/dl. Magnetic resonance imaging (MRI) showed signal change in the left striatum (Figures 1 and 2).

Figure 1
A) Sagittal T1- weighted magnetic resonance imaging showing spontaneous diffuse high intensity of the left striatum (caudate nucleus and putamen). B and C) There is no significant enhancement on axial and coronal T1-weighted MRI after intravenous paramagnetic contrast administration.

Figure 2
A and B) Axial T2*-weighted gradient-echo MRI showing multiple confluent foci of hypointensities in left striatum probably due to petechial hemorrhage. C) There is no significant signal change on axial diffusion-weighted image.

This entity is characterized by hyperintensity confined to the striatum on T1-weighted MRI and contralateral hyperkinetic movement disorders in diabetic patients (type 2) with non-ketotic hyperglycemia11. Lai PH, Tien RD, Chang MH, Teng MM, Yang CF, Pan HB et al. Chorea-Ballismus with nonketotic hyperglycemia in primary diabetes mellitus. AJNR Am J Neuroradiol. 1996;17(6):1057-64.. T2*-weighted gradient-echo MRI can reveal low signal intensity related to petechial hemorrhage22. Suto Y, Mori M, Kagimoto H, Saito J. [A case of hemichorea with hyperglycemia presenting with low signal intensity in the striatum on T2*-weighted gradient-echo magnetic resonance imaging]. Rinsho Shinkeigaku. 2004;44(2):86-90. Japanese.. Pathological studies demonstrated selective neuronal loss, gliosis, reactive astrocytosis and hemorrhage33. Abe Y, Yamamoto T, Soeda T, Kumagai T, Tanno Y, Kubo J et al. Diabetic striatal disease: clinical presentation, neuroimaging, and pathology. Intern Med. 2009;48(13):1135-41. doi:10.2169/internalmedicine.48.1996.

The clinical symptoms usually improve markedly following the correction of hyperglycemia. Thus, the prompt recognition of this potentially treatable disease is of paramount importance.

REFERENCES

  • 1
    Lai PH, Tien RD, Chang MH, Teng MM, Yang CF, Pan HB et al. Chorea-Ballismus with nonketotic hyperglycemia in primary diabetes mellitus. AJNR Am J Neuroradiol. 1996;17(6):1057-64.
  • 2
    Suto Y, Mori M, Kagimoto H, Saito J. [A case of hemichorea with hyperglycemia presenting with low signal intensity in the striatum on T2*-weighted gradient-echo magnetic resonance imaging]. Rinsho Shinkeigaku. 2004;44(2):86-90. Japanese.
  • 3
    Abe Y, Yamamoto T, Soeda T, Kumagai T, Tanno Y, Kubo J et al. Diabetic striatal disease: clinical presentation, neuroimaging, and pathology. Intern Med. 2009;48(13):1135-41. doi:10.2169/internalmedicine.48.1996

Publication Dates

  • Publication in this collection
    Apr 2016

History

  • Received
    06 July 2015
  • Reviewed
    01 Oct 2015
  • Accepted
    20 Oct 2015
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