This is a unique case of nonketotic hyperglycemic (NKH) chorea in a 34-year-old white male. The patient had a poorly controlled type 2 diabetes mellitus (DM) due to medication incompliance. He complained of polyuria, polydipsia, and weight loss of 20 pounds within a month before presentation. T2-weighted (T2W) MRI showed hyperintensity in the left basal ganglion. Glycated hemoglobin (HBA1c) was 13.6%. The patient was started on insulin and clonazepam and the chorea resolved after proper control of the glucose level. To our knowledge, this is the first reported case of NKH chorea in a young white male with high T2-weighted (T2W) magnetic resonance signal in the basal ganglia. 1. Introduction Chorea is an irregular, poorly patterned, involuntary movement disorder. Various conditions such as cerebrovascular insufficiency, neurodegenerative diseases, neoplastic diseases, immunological diseases, infectious diseases, and metabolic diseases are known as secondary causes of this rare disorder [1]. Hyperglycemia is the most common metabolic cause of chorea-ballism [2]. NKH chorea is more common in elderly patients [2–5] especially females from East Asian origin [3, 5]. Proper control of DM, with or without neuroleptic drugs, is the key for treatment. 2. Case A 34-year-old white male presented to our hospital complaining of 1 month of flailing-like movements of his right upper extremity that progressed to the whole right side of the body and his neck. He also complained of a 20-pound weight loss within a month before presentation accompanied by polyuria and polydipsia. The patient denied any loss of consciousness, weakness, difficulty walking, headaches, blurry vision, slurred speech, fever, chills, or recent flu-like symptoms. He also denied taking any neuroleptic drugs. His past medical history was significant for type 2 DM for which he was on insulin but had not been taking it for a year because of financial problems. Physical exam showed mild hypotonia in the right upper extremity with no weakness in all extremities. Laboratory investigations showed blood sugar around 230？mg/dL, with no anion gap. HBA1c was 13.6%. Urine drug screening was negative. CT scan of the head did not show any abnormalities. MRI of the brain (Figures 1, 2, 3, 4, and 5) showed high T2W signal in both putamina of the basal ganglia and low T1 weighted (T1W) signal in the left putamen of the basal ganglion with no restricted diffusion on the axial diffusion weighted imaging (DWI). Thyroid stimulating hormone, antinuclear antibody, antiphospholipid antibody, liver function tests,
References
[1]
R. B. Dewey and J. Jankovic, “Hemiballism-hemichorea. Clinical and pharmacologic findings in 21 patients,” Archives of Neurology, vol. 46, no. 8, pp. 862–867, 1989.
[2]
S. Lee, J. Shin, J. Kim et al., “Chorea-ballism associated with nonketotic hyperglycaemia or diabetic ketoacidosis: characteristics of 25 patients in Korea,” Diabetes Research and Clinical Practice, vol. 93, no. 2, pp. e80–e83, 2011.
[3]
A. C. Felicio, C. V. Chang, C. Godeiro-Junior, M. P. Okoshi, and H. B. Ferraz, “Hemichorea-hemiballism as the first presentation of type 2 diabetes mellitus,” Arquivos de Neuro-Psiquiatria A, vol. 66, no. 2, pp. 249–250, 2008.
[4]
C. Song, X. Yang, G. Xing, et al., “Hemichorea associated with nonketotic hyperglycemia in a female,” Neuro Endocrinology Letter, vol. 33, no. 5, pp. 489–492, 2012.
[5]
C. V. Chang, A. C. Felicio, C. Godeiro et al., “Chorea-ballism as a manifestation of decompensated type 2 diabetes mellitus,” American Journal of the Medical Sciences, vol. 333, no. 3, pp. 175–177, 2007.
[6]
S. F. Bedwell, “Some observations on hemiballismus,” Neurology, vol. 10, pp. 619–622, 1960.
[7]
J. L. Hsu, H. Wang, and W. Hsu, “Hyperglycemia-induced unilateral basal ganglion lesions with and without hemichorea: a PET study,” Journal of Neurology, vol. 251, no. 12, pp. 1486–1490, 2004.
[8]
S. Ohara, S. Nakagawa, K. Tabata, and T. Hashimoto, “Hemiballism with hyperglycemia and striatal T1-MRI hyperintensity: an autopsy report,” Movement Disorders, vol. 16, no. 3, pp. 521–525, 2001.
[9]
S. Oh, K. Lee, J. Im, and M. Lee, “Chorea associated with non-ketotic hyperglycemia and hyperintensity basal ganglia lesion on T1-weighted brain MRI studya meta-analysis of 53 cases including four present cases,” Journal of the Neurological Sciences, vol. 200, no. 1-2, pp. 57–62, 2002.
[10]
C. Battisti, F. Forte, E. Rubenni et al., “Two cases of hemichorea-hemiballism with nonketotic hyperglycemia: a new point of view,” Neurological Sciences, vol. 30, no. 3, pp. 179–183, 2009.
[11]
M. Wintermark, N. J. Fischbein, P. Mukherjee, E. L. Yuh, and W. P. Dillon, “Unilateral putaminal CT, MR, and diffusion abnormalities secondary to nonketotic hyperglycemia in the setting of acute neurologic symptoms mimicking stroke,” American Journal of Neuroradiology, vol. 25, no. 6, pp. 975–976, 2004.
[12]
D. Branca, O. Gervasio, E. le Piane, C. Russo, and U. Aguglia, “Chorea induced by non-ketotic hyperglycaemia: a case report,” Neurological Sciences, vol. 26, no. 4, pp. 275–277, 2005.
[13]
C. Nagai, T. Kato, T. Katagiri, and H. Sasaki, “Hyperintense putamen on T1-weighted MR images in a case of chorea with hyperglycemia,” American Journal of Neuroradiology, vol. 16, no. 6, pp. 1243–1246, 1995.
[14]
R. P. Newman and W. R. Kinkel, “Paroxysmal choreoathetosis due to hypoglycemia,” Archives of Neurology, vol. 41, no. 3, pp. 341–342, 1984.
[15]
Z. Zaitout, “CT and MRI findings in the basal ganglia in non-ketotic hyperglycaemia associated hemichorea and hemi-ballismus (HC-HB),” Neuroradiology, vol. 54, no. 10, pp. 1119–1120, 2012.
