Hemichorea-Hemiballismus Due to Non-Ketotic Hyperglycemia

Yıl 2008, Cilt: 2 Sayı: 1, 264 - 268, 23.03.2008

Hayat Güven

Öz

Non-ketotic hyperglycemia is a rare cause of hemichorea-hemiballismus. Hemichorea-hemiballismus associated with non-ketotic hyperglycemia, is usually seen in old and diabetic women who have unregulated glucose regimen. The disorder has characteristic cerebral imaging findings and prognosis is generally good. The pathophysiology of the disorder and the nature of the findings seen in imaging studies are not clear yet. While alterations of neurotransmitters due to metabolic derangement and cerebral ischemia are considered to be responsible in pathogenesis; calcifications, petechial hemorragies, myelinolysis and presence of abundant gemistocytes have been suggested as possible mechanisms of the lesions seen in imaging studies. In this paper, we describe a diabetic 80 year old woman who presented with right hemichorea-hemiballismus associated with non-ketotic hyperglycemia. There was a hyperintense lesion at contralateral striatum on T1 weighted magnetic resonance images and the involuntary movements of the patient didn’t respond to the medical theraphy.

Anahtar Kelimeler

Chorea; hyperglycemia;, magnetic, resonance imaging

Non-Ketotik Hiperglisemiye Bağlı Hemikore-Hemiballismus

Öz

Non-ketotik hiperglisemi, hemikore-hemiballismusun nadir bir nedenidir. Nonketotik hiperglisemiye bağlı hemikore-hemiballismus, genellikle yaşlı ve kan şekeri regüle olmayan diabetik kadınlarda görülür. Karakteristik görüntüleme bulgularına sahiptir ve sıklıkla iyi prognozludur. Hastalığın patofizyolojisi ve görüntüleme çalışmalarındaki bulguların doğası henüz tam aydınlatılamamıştır. Metabolik yetmezlik sonucunda gelişen nörotransmitter değişiklikleri ve serebral iskemi hastalığın patogenezinde sorumlu tutulurken; görüntüleme çalışmalarında saptanan lezyonların kalsifikasyonlardan, peteşiyal hemorajilerden, myelinolizisden veya abondan gemistositlerin varlığından kaynaklanabileceği ileri sürülmüştür. Bu makalede; sağ hemikore-hemiballismus ile başvuran ve etiyolojisinde non-ketotik hiperglisemi saptanan 80 yaşında diabetik bir kadın hasta sunulmuştur. Hastanın Tl ağırlıklı magnetik rezonans görüntülemesinde kontrlateral striatumda hiperintens lezyon belirlenmiş ve istemsiz hareketler medikal tedaviye direnç göstermiştir.

Anahtar Kelimeler

Kore; hiperglisemi; manyetik rezonans görüntüleme, mr, kore,

Kaynakça

• 1. Vidakovic A, Dragasevic N, Kostic VS. Hemiballism: report of 25 cases. J Neurol Neurosurg Psychiatry 1994;57:945-9.
• 2. Lee BC, Hwang SH, Chang GY. Hemiballis-mus-hemichorea in older diabetic women: A clinical syndrome with MRI correlation. Neurology 1999;52: 646-8.
• 3. Lee EJ, Choi JY, Lee SH, Song SY, Lee YS. Hemichorea-hemiballism in primary diabetic patients: MR Correlation. J Comput Assist To-mogr 2002;26:905-11.
• 4. 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;200:57-62.
• 5. Ifergane G, Masalha R, Herishanu YO. Transient hemichorea/hemiballismus associated with new onset hyperglycemia. Can J Neurol Sci 2001; 28: 365-8.
• 6. Lai PH, Tien RD, Chang MH, Teng MM, Yang CF, Pan HB, Chen C, Lirng JF, Kong KW. Chorea-ballismus with nonketotic hyperglycemia in primary diabetes mellitus. AJNR Am J Neuroradiol 1996;17:1057-64.
• 7. Shan DE, Ho DM, Chang C, Pan HC, Teng MM. Hemichorea-hemiballism: an explanation for MR signal changes. AJNR Am J Neuroradiol 1998; 19: 863-70.
• 8. Oerlemans WGH, Moll LCM. Non-ketotic hyperglycemia in a young woman, presenting as hemiballism-hemichorea. Acta Neurol Scand 1999; 100: 411-4.
• 9. Hsu JL, Wang HC, Hsu WC. Hyperglycemia-induced unilateral basal ganglion lesion with and without hemichorea a PET study. J Neurol 2004; 251:1486-90.
• 10. Duckrow RB, Beard DC, Brennan RW. Regional cerebral blood flow decreases during hyperglycemia. Ann Neurol 1985;17:267-72.
• 11. Nagai C, Kato T, Katagiri T, Sasaki H. Hyperintense putamen on T1-weighted MR images in a case of chorea with hyperglycemia. AJNR Am J Neuroradiol 1995;16:1243-6.
• 12. Shan DE. Hemichorea-hemiballism associated with hyperintense putamen on T1-weighted MR images: An update and a hypothesis. Acta Neurol Taiwan 2004;13:170-7.
• 13. Broderick JP, Hagen T, Brott T, Tomsick T. Hyperglycemia and hemorrhagic transformation of cerebral infarcts. Stroke 1995; 26:484-7.
• 14. Ohara S, Nakagawa S, Tabata K, Hashimoto T. Hemiballism with hyperglycemia and striatal T1-MRI hyperintensity: an autopsy report. Mov Disord 2001; 16: 521-5.
• 15. Lai SL, Tseng YL, Hsu MC, Chen SS. Magnetic resonance imaging and single- photon emission computed tomography changes in hypoglycemia-induced chorea. Mov Disord 2004; 19:475-8.
• 16. ChuK, 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: 448-52.