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AJHM Volume 2 Issue 3 (July-Sept 2018) CASE REPORT

CASE REPORT

Non-ketotic Presenting with Acute Hemichorea and Ballism Pradeep C. Bollu MD1

1Department of , University of Missouri, Columbia, Missouri

Corresponding author: Pradeep C. Bollu, MD. One Hospital Dr. Columbia, MO 65212 [email protected]

Received: October 8, 2017 Accepted: May 10, 2018 Published: August 17, 2018

Am J Hosp Med 2018 Jul;2(3):2018.019 https://doi.org/10.24150/ajhm/2018.019

Non-ketotic hyperglycemia is a complication of poorly controlled diabetes mellitus. Rarely, it can present like an acute neurological syndrome with unilateral choreiform and ballistic movements. Such a presentation usually raises the suspicion of a cerebrovascular event and prompts more workup. Moreover, the neuroimaging in this condition also suggests a variety of potential possibilities. Identification of this rare presentation of non-ketotic hyperglycemia helps with the appropriate management and avoid unnecessary investigations. In this case report, we report the case of an elderly woman who presented with hemichorea-ballism due to non-ketotic hyperglycemia and discuss the literature on this presentation. We also highlighted the differential diagnosis based on neuroimaging.

INTRODUCTION CASE PRESENTATION

Hemichorea/hemiballism is a neurological A 64-year-old Caucasian female presented syndrome characterized by violent to the outpatient neurology clinic with an involuntary movements on one side of the almost eight-month history of abnormal body. These movements mainly involve the movements of the left upper and lower upper extremity. This syndrome is usually extremities that started suddenly while she associated with acute vascular insults in the was at church. They were present constantly vicinity of the . Lesions during the daytime and would subside in different sites of the striatum were also during sleep. These movements were reported to result in a similar syndrome1. In choreiform in nature most of the time and the last three decades, several patients with occasionally would become ballistic with this syndrome associated with elevated reports of self-injury during the flailing. blood sugars were reported. Most of these Patient reported that her diabetes was under patients have a fairly quick recovery from poor control before and around the time her their symptoms with the correction of clinical symptoms started. Her other hyperglycemia. However, we report a case significant medical problems included of prolonged hemichorea/hemiballism that chronic kidney disease and peripheral started in the setting of poorly controlled neuropathy. diabetes mellitus and associated with T1- Her examination did not show weighted putaminal hyperintensity on rigidity or bradykinesia in the limbs. She magnetic resonance imaging (MRI) scan of also did not have any focal weakness. The her brain. deep tendon reflexes were slightly

Bollu, Pradeep www.ajhm.org 1 AJHM Volume 2 Issue 3 (July-Sept 2018) CASE REPORT diminished in the lower extremities abnormal movements but caused severe symmetrically which may have been due to sleepiness and the medication was stopped. her peripheral neuropathy. The MRI of the She was started on aripiprazole which also brain showed a T1 hyperintensity in the helped significantly with the abnormal right without any evidence of acute movements. During this time, the patient infarction or hemorrhage (figure 1). started to get her blood sugars under control. Magnetic resonance angiography did not Repeat MRI of the brain in three months show any significant stenosis of the brain or showed reduction in the T1 hyperintense neck vasculature. A trial of signal in the right putamen (figure 2). Her resulted in significant improvement of her neuroleptic treatment was complicated by

Figure 1. Initial MRI scan at the time of presentation showing T1 weighted hyperintensity in the right putamen.

Bollu, Pradeep www.ajhm.org 2 AJHM Volume 2 Issue 3 (July-Sept 2018) CASE REPORT

Figure 2. Follow up MRI scan (3 months after the initial scan) showing resolution of the T1 weighted hyperintensity in the right putamen. the appearance of buccolingual and DISCUSSION copulatory that required gradual discontinuation of the neuroleptic therapy. Although rare, nonketotic hyperglycemic Her symptoms gradually resolved in the next hemichorea-ballism is a potential few months only to become manifest during complication of poorly controlled blood times of emotional stress as the patient went sugars and usually presents in patients with off the medication completely. no previous history of diabetes or hyperglycemia2. Sometimes, the same presentation can be seen in the setting of

Bollu, Pradeep www.ajhm.org 3 AJHM Volume 2 Issue 3 (July-Sept 2018) CASE REPORT diabetic ketoacidosis. Most of the affected this condition gets triggered is not clear. The patients are in the 50-80 age range with condition is typically easily treatable by usually no history of diabetes mellitus2. The correcting blood sugar with rapid resolution usual onset of the symptoms is acute to of abnormal movements. Computed subacute and typically over a period of tomography (CT) scan of the brain hours. Hemichorea and ballism associated commonly shows hyperdensity of the with hyperglycemia was first described in putamen and sparing the 1960 by Bedwell3. Interestingly, patients internal capsule10 which sometimes can be affected by this disease entity are mostly mistaken for hemorrhage11. females and abnormal movements often get The MRI of the brain typically shows T1- significantly better with the correction of weighted hyperintensity in the lentiform hyperglycemia. A case of non-ketotic nucleus sparing the internal capsule with hyperglycemia with vague non-localizing some cases reported to be showing restricted symptoms without an associated movement diffusion of striatum12, high T2-weighted disorder has also been described in the past4. signal in , low T2-weighted Most of the cases reported in the past had signal in the striatum and contrast significant resolution of symptoms in 1-2 enhancement8. days. Our patient had her symptoms for Most patients with this condition almost 8 months which is unusual. recover with optimization of hyperglycemia The pathophysiology of this acute and the recovery ranges anywhere from one syndrome is unclear. It is thought that day to few months. A small minority of the hyperglycemia might result in a greater patients can have persistent chorea13. utilization of gamma-aminobutyric acid Radiological improvement is seen in almost (GABA) as an alternative energy source all the cases including those with persistent resulting in its depletion. GABA is a major . Along with management of blood inhibitory neurotransmitter in the brain and sugar, treatment options include D2 receptor the basal ganglia circuitry. This reduction in blockers like haloperidol though their GABA might disinhibit the which prolonged use is associated with increased can result in abnormal movements2. Various risk of tardive dyskinesias as seen in our other mechanisms were suggested that patient. has been successfully include acute basal ganglia dysfunction in used in some patients14 and is not associated the setting of hyperosmolar state5, with long term risk of tardive dyskinesias. hyperviscosity6, petechial hemorrhage7, Successful long-term treatment with inflammation8, and cerebrovascular thalamic (DBS) has insufficiency9. Post-menopausal alterations also been reported in a case of hemichorea- of the GABA and/or receptors in hemiballism in the setting of diabetes elderly females may be the reason for higher mellitus15. incidence in this group2. While the characteristic findings on CONCLUSION neuroimaging may vary to some degree, the pattern of basal ganglia involvement sparing Hemichorea/ballism in the setting of non- the internal capsule along with the sudden ketotic hyperglycemia is a rare but treatable onset of abnormal movements in the setting neurological condition. The sudden of markedly elevated blood sugars should appearance of unilateral symptoms usually prompt the consideration of this disease raises the suspicion of an acute vascular entity. The above which event which might result in unnecessary

Bollu, Pradeep www.ajhm.org 4 AJHM Volume 2 Issue 3 (July-Sept 2018) CASE REPORT diagnostic studies. Identification of the diabetic hyperglycemia: a hyperviscosity characteristic MRI findings in the syndrome? Archives of neurology. 2002;59(3):448-452. appropriate clinical setting will help in early 7. Chang M-H, Chiang H-T, Lai P-H, Sy C-G, recognition of this rare syndrome and Lee SS-J, Lo Y-Y. Putaminal petechial prompt the initiation of treatment. Compared haemorrhage as the cause of chorea: a to a cerebrovascular event causing a neuroimaging study. Journal of Neurology, , this syndrome usually Neurosurgery & Psychiatry. 1997;63(3):300-303. has a good prognosis with resolution of 8. Wang J-h, Wu T, Deng B-q, Zhang Y-w, symptoms after correction of elevated blood Zhang P, Wang Z-k. Hemichorea— sugar levels. hemiballismus associated with nonketotic hyperglycemia: A possible role of . Journal of the neurological Notes sciences. 2009;284(1):198-202. Financial support: Author declares that no financial 9. Shan D-E. Hemichorea-hemiballism assistance was taken from any source. associated with hyperintense putamen on Potential conflicts of interest: Author declares no T1-weighted MR images: an update and a conflicts of interest. hypothesis. Acta Neurol Taiwan. 2004;13(4):170-177. 10. Lai P-H, Tien RD, Chang M-H, et al. Chorea-ballismus with nonketotic References hyperglycemia in primary diabetes mellitus. 1. Dewey Jr RB, Jankovic J. Hemiballism- American Journal of Neuroradiology. hemichorea. Clinical and pharmacologic 1996;17(6):1057-1064. findings in 21 patients. Arch Neurol. 11. Wilson TJ, Than KD, Stetler WR, Heth JA. 1989;46(8):862-867. Non-ketotic hyperglycemic chorea– 2. Ifergane G, Masalha R, Herishanu Y. hemiballismus mimicking basal ganglia Transient hemichorea/hemiballismus hemorrhage. Journal of Clinical associated with new onset hyperglycemia. Neuroscience. 2011;18(11):1560-1561. Canadian journal of neurological sciences. 12. Narayanan S. Hyperglycemia-induced 2001;28(4):365-368. hemiballismus hemichorea: a case report 3. Bedwell SF. Some observations on and brief review of the literature. The hemiballismus. Neurology. 1960;10:619. Journal of emergency medicine. 4. Hansford BG, Albert D, Yang E. Classic 2012;43(3):442-444. neuroimaging findings of nonketotic 13. Oh S-H, Lee K-Y, Im J-H, Lee M-S. Chorea hyperglycemia on computed tomography associated with non-ketotic hyperglycemia and magnetic resonance imaging with and hyperintensity basal ganglia lesion on absence of typical movement disorder T1-weighted brain MRI study: a meta- symptoms (hemichorea-hemiballism). analysis of 53 cases including four present Journal of radiology case reports. cases. Journal of the neurological sciences. 2013;7(8):1. 2002;200(1):57-62. 5. Wintermark M, Fischbein NJ, Mukherjee P, 14. Sitburana O, Ondo WG. Tetrabenazine for Yuh EL, Dillon WP. Unilateral putaminal hyperglycemic‐ induced hemichorea– CT, MR, and diffusion abnormalities hemiballismus. Movement disorders. secondary to nonketotic hyperglycemia in 2006;21(11):2023-2025. the setting of acute neurologic symptoms 15. Nakano N, Uchiyama T, Okuda T, Kitano mimicking . American Journal of M, Taneda M. Successful long-term deep Neuroradiology. 2004;25(6):975-976. brain stimulation for hemichorea— 6. Chu K, Kang D-W, Kim D-E, Park S-H, hemiballism in a patient with diabetes: Case Roh J-K. Diffusion-weighted and gradient report. Journal of neurosurgery. echo magnetic resonance findings of 2005;102(6):1137-1141. hemichorea-hemiballismus associated with

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