Journal of the Neurological Sciences 393 (2018) 113–115 Contents lists available at ScienceDirect Journal of the Neurological Sciences journal homepage: www.elsevier.com/locate/jns Letter to the Editor Hemichorea-hemiballism by branch atheromatous disease with a unique cerebral blood flow abnormality T A R T I C LE I N FO Keywords: Hemichorea Hemiballism Branch atheromatous disease Putamen Single-photon emission computed tomography Ischemic symptoms Basal ganglia and cerebellar loops Dear Editor, A summary of his clinical course is shown in Fig. 1O. Hemichorea-hemiballism (HCHB) is a rare manifestation caused by hyperglycemia or stroke [1,2]. The predominantly affected sites are the basal ganglia and thalamus [3,4]. However, why HCHB appears in only a few cases remains unclear. We herein report a case involving a man with branch atheromatous disease (BAD) in the internal putamen with the development of a cerebral blood flow (CBF) abnormality beyond the ischemic lesion and resultant HCHB. 2. Discussion 1. Case A 60-year-old right-handed man suddenly developed pure left hemichorea-hemiballism and was transferred to our hospital 199 min after onset. He had medically treated hypertension, and his initial blood pressure was 132/62 mmHg. At admission, the patient exhibited left HCHB with no other neurological disturbances. His initial National Institutes of Health Stroke Scale score was 0. The left HCHB disappeared 30 min after admission. Laboratory examination showed a mildly elevated blood glucose concentration (146 mg/dl). Although cranial magnetic resonance imaging 246 min after symptom onset showed no acute ischemic change (Fig. 1A–F), we suspected ischemic stroke and thus initiated treatment with antiplatelet agents. During his hospital stay, the patient developed no recurrence of his neurological symptoms, including HCHB. Furthermore, atrial fibrillation was not detected on long-term electrocardiographic monitoring, and transesophageal echocardiography showed no significant findings. However, cranial magnetic resonance imaging 3 days after onset revealed a vertically long ischemic lesion (BAD) of the right middle cerebral artery branch, which was likely the lenticulostriate artery (Fig. 1G–L). Three-dimensional stereotactic surface projection analysis of 123I-N-isopropyl-p-iodoamphetamine single-photon emission computed tomography (123I-IMP SPECT) 8 days after onset revealed decreased CBF in the right premotor area with an obvious bilateral difference (right > left) in the cerebellum (Fig. 1M). However, magnetic resonance angiography showed no stenosis (Fig. 1N). The patient was discharged 10 days after onset with a modified Rankin scale score of 0. The present case suggests that HCHB is associated with CBF abnormalities in the premotor area and cerebellar cortex, resulting from BAD in the putamen. The basal ganglia, cerebellar cortex, and motor cortex form a discrete circuit termed the basal ganglia and cerebellar loops [5], which controls both self-initiated and externally triggered movement [6,7]. A recent focus of study is participation of the putamen in the motor cortex in some movement disorders [8]. The lenticular fasciculus, part of the pallidofugal motor fiber system, projects to the ventral thalamic nuclei, which also projects to the motor cortex. The dentate nucleus of the cerebellum also projects to the ventral thalamic nuclei and affects the motor cortex. In the present case, we speculate that putamen ischemia induced a decrease in the CBF in the premotor area and a secondary increase in the CBF in the cerebellar cortex for compensation, which disturbed the function of the basal ganglia and cerebellar loops and resulted in HCHB. Ischemia of the basal ganglia was the likely cause of damage to this circuit in our patient. Moreover, development of the ischemic lesion was gradual, which may be why the HCHB disappeared in the short term. This case furthers our understanding of the relationship of the clinical course of HCHB with CBF abnormalities and suggests that damage to the basal ganglia and cerebellar loops plays a role in HCHB. Funding This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. Authors' contributions K.M., M.S., K.F., and D.T. were the attending doctors of the present case. K.M. drafted the manuscript. Y.K., T.D., T.M., H.S., R.K., and R.T. helped to draft the manuscript. S.F. conceived the study, participated in its coordination, and helped to draft the manuscript. All authors read https://doi.org/10.1016/j.jns.2018.08.021 Received 14 June 2018; Received in revised form 18 August 2018; Accepted 21 August 2018 Available online 23 August 2018 0022-510X/ © 2018 Elsevier B.V. All rights reserved. Journal of the Neurological Sciences 393 (2018) 113–115 Letter to the Editor (caption on next page) 114 Journal of the Neurological Sciences 393 (2018) 113–115 Letter to the Editor Fig. 1. Cranial diffusion-weighted imaging (DWI) on the day of symptom onset in the axial (A–C; first column) and coronal (D–F; second column) views. There was no evidence of acute ischemic changes. However, cranial DWI at 3 days (axial views: G–I, third column; coronal views: J–L, fourth column) revealed branch atheromatous disease of the right putamen (arrows). 123I-N-isopropyl-p-iodoamphetamine single-photon emission computed tomography (123I-IMP SPECT) on day 8 (M; fifth column) showed decreased cerebral blood flow (CBF) in the right premotor area (arrowheads) and increased CBF in the right cerebellar cortex (arrows). There was no stenosis or obstruction on magnetic resonance angiography (N). A summary of the patient's clinical course showing the relationship between hemichoreahemiballism, changes in the National Institutes of Health Stroke scale score, examination findings, and treatment is shown in (O). and approved the final manuscript. [2] R. Bacchin, F. Macchione, D. Cardellini, et al., Levofloxacin-induced hemichoreahemiballism in a patient with previous thalamic infarction, Neurol. Sci. Mar. 6 (2018). [3] C.S. Kase, G.O. Maulsby, E. Dejuan, J.P. Mohr, Hemichorea-hemiballism and lacunar infarction in the basal ganglia, Neurology 31 (1981) 452–455. [4] L. D'Olhaberriague, A. Arboix, J.L. Marti-Vilalta, A. Moral, J. Massons, Movement disorders in ischemic stroke: clinical study of 22 patients, Eur. J. Neurol. 2 (1995) 553–557. [5] F.A. Middleton, P.L. Strick, Basal ganglia and cerebellar loops: motor and cognitive circuits, Brain Res. Brain Res. Rev. 31 (2000) 236–250. [6] T. Taniwaki, A. Okayama, T. Yoshiura, et al., Functional network of the basal ganglia and cerebellar motor loops in vivo: different activation patterns between self-initiated and externally triggered movements, NeuroImage 31 (2006) 745–753. [7] K. Takakusaki, Motor control by the basal ganglia, Rinsho Shinkeigaku 49 (2009) 325–334. [8] R. Yu, B. Liu, L. Wang, J. Chen, X. Liu, Enhanced functional connectivity between putamen and supplementary motor area in Parkinson's disease patients, PLoS One 8 (2013) e59717. Disclosure statement S.F. received honoraria from Mitsubishi Tanabe Pharma (manufacturer of edaravone) in 2016 and 2017. S.F. received a donation from Mitsubishi Tanabe Pharma (manufacturer of edaravone) in 2016. R.T. received honoraria from Mitsubishi Tanabe Pharma (manufacturer of edaravone) in 2016 and Sanofi K.K. (manufacturer of clopidogrel) in 2017. Acknowledgments We appreciate the cooperation of the patient. We also thank Angela Morben, DVM, ELS, from Edanz Group (www.edanzediting.com/ac), for editing a draft of this manuscript. ⁎ Kosuke Matsuzono , Masayuki Suzuki, Kohei Furuya, Dan Tomomasa, Younhee Kim, Tadashi Ozawa, Takafumi Mashiko, Haruo Shimazaki, Reiji Koide, Ryota Tanaka, Shigeru Fujimoto Division of Neurology, Department of Internal Medicine, Jichi Medical University School of Medicine, Tochigi, Japan. E-mail address: kmatsuzono51@jichi.ac.jp (K. Matsuzono) References [1] L.V. Kalia, L. Mozessohn, R.I. Aviv, et al., Hemichorea-hemiballism associated with hyperglycemia and a developmental venous anomaly, Neurology 78 (2012) 838–839. ⁎ Corresponding author at: Division of Neurology, Department of Internal Medicine, Jichi Medical University School of Medicine, Yakushiji 3311-1, Shimotsuke, Tochigi 329-0498, Japan. 115