Non-ketotic hyperosmolar hyperglycemic chorea

Matheus Ferreira Gomes Euripedes Gomes de Carvalho Neto Fernando Kowacs Carlos R. M. Rieder About the authors

KEYWORDS:
Chorea; Dyskinesias; Hyperglycemia

PALAVRAS CHAVE:
Coreia; Discinesias; Hiperglicemia

INTRODUCTION

Chorea is a type of hyperkinesia characterized by the presence of involuntary, brief and unsustained movements caused by irregular sequential muscle activation that flows continuously in a disorderly and unpredictable way.11. Cardoso F, Seppi K, Mair KJ, Wenning GK, Poewe W. Seminar on choreas. Lancet Neurol. 2006;5(7):589-602. It can be a manifestation of a primary neurologic genetic disorder, such as Huntington disease, or may occur as a neurologic complication of a systemic, toxic or metabolic cause, e.g., hypo/hypercalcemia or hyperglycemia.22. Cardoso F. Chorea: non-genetic causes. Curr Opin Neurol. 2004;17(4):433-6. Bedwell33. Bedwell SF. Some observations on hemiballismus. Neurology. 1960;10:619-22., in 1960, was the first author to describe the rare clinical syndrome of nonketotic hyperosmolar hyperglycemic (NKHH) chorea. Although rare, it is a treatable condition and, therefore, should be recognized.

OBJECTIVES

To describe the clinical presentation and neuroradiologic findings of a typical case of NKHH chorea.

CASE

An 80-year-old man was admitted after the abrupt onset of involuntary movements that affected his whole body three months before admission. Medical history revealed diabetes mellitus and systemic arterial hypertension with irregular follow-up and poor glycemic control.

Neurological examination showed orofacial dyskinesias and gait associated choreoatheteroid movements affecting mainly the left lower limb. Hemoglucotest was 462mg/dL on admission and brain MRI showed non-specific hyperintense areas in the basal ganglia on T1 (FIGURE).

FIGURE
AXIAL (A) AND SAGITTAL (B) T1-WEIGHTED IMAGES SHOWING BILATERAL SPONTANEOUS HYPERINTENSE AREAS IN THE BASAL GANGLIA

The diagnosis of chorea secondary to nonketotic hyperosmolar hyperglycemic state was established through clinical and imaging findings, and the patient was managed with intensive diabetes control and haloperidol 5mg orally twice daily. There was a remarkable improvement in the next few days, and the patient was subsequently discharged with almost no symptoms.

DISCUSSION

Chorea pathophysiology is still not widely understood. However, unlike in parkinsonism and dystonia, intracortical inhibition of the motor cortex is normal.44. Hanajima R, Ugawa Y, Terao Y, Furubayashi T, Machii K, Shiio Y, et al. Intracortical inhibition of the motor cortex is normal in chorea. J Neurol Neurosurg Psychiatry. 1999;66(6):783-6. Semiquantitative analysis of single photon emission computed tomography in patients with hemichorea due to various causes suggests that there is an increase in activity in the contralateral thalamus, possibly due to disinhibition as a result of loss of normal pallidal inhibitory input.55. Kim JS, Lee KS, Lee KH, Kim YI, Kim BS, Chung YA, et al. Evidence of thalamic disinhibition in patients with hemichorea: semiquantitative analysis using SPECT. J Neurol Neurosurg Psychiatry. 2002;72(3):329-33.

Non-ketotic hyperglycemia-induced chorea occurs more often in women and is usually associated with very high blood glucose66. Oh SH, Lee KY, Im JH, Lee MS. Chorea associated with non-ketotic hyperglycemia and hyperintensity basal ganglia lesion on T1-weighted brain MRI study: a meta-analysis of 53 cases including four present cases. J Neurol Sci. 2002;200(1-2):57-62.. The exact pathophysiology of NKHH chorea remains unclear. However, many hypotheses as blood hyperviscosity, petechial hemorrhage, depletion of gamma-aminobutyric acid (GABA) and cerebral vascular insufficiency have been suggested.77. Abdelghany M, Massoud S. Nonketotic hyperglycemic chorea. Case Rep Neurol Med. 2014;2014:128037. The correction of the metabolic abnormality usually is curative, but it can rarely continue for months after resolution of hyperglycemia.88. Ahlskog JE, Nishino H, Evidente VG, Tulloch JW, Forbes GS, Caviness JN, et al. Persistent chorea triggered by hyperglycemic crisis in diabetics. Mov Disord. 2001;16(5):890-8. Striatal permanent vascular changes may mean persistence of chorea for long periods.99. Walker RH. Differential diagnosis of chorea. Curr Neurol Neurosci Rep. 2011;11(4):385-95.

Many of the metabolic choreas are associated with abnormalities on MRI scans. Nevertheless, the etiology of the MRI changes is not fully understood.1010. Stanley F, Jankovic, J, Hallett M. Principles and practice of movement disorders. 2nd ed. Saunders; 2011.p.335-48. Hepatocerebral degeneration and hyperglycemic chorea are often associated with high signal intensity on T1-weighted MRI involving the striatum and pallidum.88. Ahlskog JE, Nishino H, Evidente VG, Tulloch JW, Forbes GS, Caviness JN, et al. Persistent chorea triggered by hyperglycemic crisis in diabetics. Mov Disord. 2001;16(5):890-8.

Chu et al.1111. Chu K, Kang DW, Kim DE, Park SH, Roh JK. Diffusion-weighted and gradient echo magnetic resonance findings of hemichorea-hemiballismus associated with diabetic hyperglycemia: a hyperviscosity syndrome? Arch Neurol. 2002;59(3):448-52., in a report of two patients with hyperglycemic hemichorea-hemiballism, found high signal intensities on T1- and T2-weighted images as well as on diffusion-weighted MRI accompanied by a reduction in diffusion coefficient, suggestive of hyperviscosity, rather than petechial hemorrhages, as the mechanism of edema in the striatum. This is also corroborated by another study of seven patients with hyperglycemic choreoathetosis using MRI and MR spectroscopy.1212. Kandiah N, Tan K, Lim CC, Venketasubramanian N. Hyperglycemic choreoathetosis: role of the putamen in pathogenesis. Mov Disord. 2009;24(6):915-9. Interestingly, the presence of high counts of acanthocytes may predispose patients with diabetes to develop hyperglycemic chorea.1313. Pisani A, Diomedi M, Rum A, Cianciulli P, Floris R, Orlacchio A, et al. Acanthocytosis as a predisposing factor for non-ketotic hyperglycemia induced chorea-ballism. J Neurol Neurosurg Psychiatry. 2005;76(12):1717-9.

CONCLUSION

A thorough physical examination and compatible clinical history and imaging are essential tools for diagnosing and treating metabolic chorea. In this case of symptoms secondary to diabetes decompensation, glycemic control added to a central dopaminergic inhibitor were effective.

REFERENCES

  • 1
    Cardoso F, Seppi K, Mair KJ, Wenning GK, Poewe W. Seminar on choreas. Lancet Neurol. 2006;5(7):589-602.
  • 2
    Cardoso F. Chorea: non-genetic causes. Curr Opin Neurol. 2004;17(4):433-6.
  • 3
    Bedwell SF. Some observations on hemiballismus. Neurology. 1960;10:619-22.
  • 4
    Hanajima R, Ugawa Y, Terao Y, Furubayashi T, Machii K, Shiio Y, et al. Intracortical inhibition of the motor cortex is normal in chorea. J Neurol Neurosurg Psychiatry. 1999;66(6):783-6.
  • 5
    Kim JS, Lee KS, Lee KH, Kim YI, Kim BS, Chung YA, et al. Evidence of thalamic disinhibition in patients with hemichorea: semiquantitative analysis using SPECT. J Neurol Neurosurg Psychiatry. 2002;72(3):329-33.
  • 6
    Oh SH, Lee KY, Im JH, Lee MS. Chorea associated with non-ketotic hyperglycemia and hyperintensity basal ganglia lesion on T1-weighted brain MRI study: a meta-analysis of 53 cases including four present cases. J Neurol Sci. 2002;200(1-2):57-62.
  • 7
    Abdelghany M, Massoud S. Nonketotic hyperglycemic chorea. Case Rep Neurol Med. 2014;2014:128037.
  • 8
    Ahlskog JE, Nishino H, Evidente VG, Tulloch JW, Forbes GS, Caviness JN, et al. Persistent chorea triggered by hyperglycemic crisis in diabetics. Mov Disord. 2001;16(5):890-8.
  • 9
    Walker RH. Differential diagnosis of chorea. Curr Neurol Neurosci Rep. 2011;11(4):385-95.
  • 10
    Stanley F, Jankovic, J, Hallett M. Principles and practice of movement disorders. 2nd ed. Saunders; 2011.p.335-48.
  • 11
    Chu K, Kang DW, Kim DE, Park SH, Roh JK. Diffusion-weighted and gradient echo magnetic resonance findings of hemichorea-hemiballismus associated with diabetic hyperglycemia: a hyperviscosity syndrome? Arch Neurol. 2002;59(3):448-52.
  • 12
    Kandiah N, Tan K, Lim CC, Venketasubramanian N. Hyperglycemic choreoathetosis: role of the putamen in pathogenesis. Mov Disord. 2009;24(6):915-9.
  • 13
    Pisani A, Diomedi M, Rum A, Cianciulli P, Floris R, Orlacchio A, et al. Acanthocytosis as a predisposing factor for non-ketotic hyperglycemia induced chorea-ballism. J Neurol Neurosurg Psychiatry. 2005;76(12):1717-9.

Publication Dates

  • Publication in this collection
    Feb 2019

History

  • Received
    13 June 2018
  • Accepted
    20 June 2018
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