sign in sign up
<<
Home , Dyskinesia, Hemiballismus

International 2020; volume 12:8328

Clinical features of hemichorea- despite the absence of a specific and reliably hemiballism: A -related predictive anatomical location. We examined Correspondence: Nobuko Shiraiwa, Course of the clinical course of three HCHB cases Neurology, Department of Health, Faculty of contralateral to the vascular insult. Health Sciences, Tsukuba University of Technology, 4-12-7 Kasuga, Tsukuba, Ibaraki, Nobuko Shiraiwa,1,2 Sachiko Hoshino,2 305-8521, Japan. Tel.: +81-29-858-9538. E-mail: [email protected]. Go Saito,3 Akira Tamaoka,4 Norio Ohkoshi1,5 Materials Key words: Hemichorea-hemiballism, Stroke- 1Course of Neurology, Department of We examined the age, sex, MRI results, related movement disorders, . Health Sciences, Tsukuba University of vascular risk factors, management, and Acknowledgments: We would like to thank Technology; 2Department of Neurology, outcomes for three cases of HCHB. Editage (www.editage.jp) for English lan- Tsukuba Memorial Hospital; 3Primary Ballism is a condition that causes a guage editing. Care and Medical Education, Graduate violent, irregular, large-amplitude, Contributions: NS designed the study and School of Comprehensive Human involuntary movement that primarily involves the proximal extremities. It can be wrote the initial draft of the manuscript. NO Sciences, University of Tsukuba; contributed to the analysis and interpretation categorized as a fast form of .1-2 4Department of Neurology, Graduate of data, and assisted in the preparation of the Chorea presents as a condition that School of Comprehensive Human manuscript. All other authors have contributed causes rapid, irregular, and involuntary Science, University of Tsukuba; to data collection and interpretation, and criti- movement that typically involves both 5Department of Radiological cally reviewed the manuscript. All authors proximal and distal muscles.1-2 approved the final version of the manuscript Technology, Tsukuba International and agree to be accountable for all aspects of University, Japan the work; this ensures that questions related to the accuracy or integrity of the work are Case Report #1 appropriatelyonly investigated and resolved. Abstract A 74-year-old man presented with Conflict of interest: The authors declare no continuous hemichoreic movements on his conflicts of interest. We examined pathogenesis and clinical left arm, left leg, and tongue for ten to Availability of data and materials: All data are features of three hemichorea-hemiballism fourteen days before visiting our clinic.use His available within the text. (HCHB) cases. We studied their age, blood sugar (BS) level on the first visit was magnetic resonance imaging results, 151 mg/dl. Brain MRI showed an old Ethics approval and consent to participate: vascular risk factors, management, and cerebral infarct in the right , Study complies with the ethical procedure. outcomes. One man and two women (aged which has been present for the past four Informed consent: Informed consent has been 74-86 years) demonstrated acute onset of years (Figure 1A,B). In addition, he had a received. HCHB, lasting for at least several months. transient cerebral ischemic attack that Patients had one or more vascular risk affected his left arm eight years ago. His Received for publication: 30 September 2019. factors, including hypertension and diabetes. vascular risk factors included hypertension Revision received: 13 December 2019. All patients presented subacute or old and diabetes (BS=143 mg/dl, Accepted for publication: 10 February 2020. infarction in the basal ganglia with HbA1c=7.0%). Clonazepam (0.5 mg/day) contralateral symptoms. We administered administration was ineffective. His This work is licensed under a Creative Commons Attribution NonCommercial 4.0 clonazepam (0.5-1 mg/day), ceased three and a half months License (CC BY-NC 4.0). (0.375-0.75 mg/day), or both as necessary after onset once the clonazepam dose was and observed symptom-control. Vascular increased up to 1.0 mg/day and haloperidol ©Copyright: the Author(s), 2019 lesions in the basal ganglia were a (0.75 mg/day) was added (Figure 2A). Licensee PAGEPress, Italy contributing factor. Symptoms were Neurology International 2020; 12:8328 controlled using pharmacotherapyNon-commercial with doi:10.4081/ni.2020.8328 gamma-aminobutyric acid-agonist (clonazepam) or anti- Case Report #2 (haloperidol) medication. An 81-year-old woman suddenly its onset, we reduced the dose without presented with continuous hemiballism/ relapsing the previous symptoms; however, choreic movements on her left arm and leg she continued to demonstrate oral dyskinesia for one week before visiting our clinic. Brain Introduction (Figure 2B). MRI taken seven days after the onset showed and movement disorders are subacute cerebral infarcts in the right globus relatively common and demand the attention pallidus (Figure 1C-E). Her vascular risk of neurologists and primary care physicians. factors included hypertension but no Case Report #3 However, stroke-related movement diabetes. Her symptoms ameliorated after disorders, such as hemichorea-hemiballism Haloperidol (0.75 mg/day) administration. An 86-year-old woman presented with (HCHB), occur only in a small percentage of Eighteen months later, she presented continuous hemiballism/choreic movement on cases.1-3 Although its pathogenesis remains symptoms of oral dyskinesia. We attempted her left leg and her left arm for one to two unclear, research previously conducted in the to eliminate haloperidol to treat her months before visiting our clinic. Brain MRI field suggests that the mechanisms involve condition but could not prevent her showed periventricular hyperdensity, along the motor circuitry of the basal ganglia, symptoms from relapsing. Three years after with old infarcts in the right globus pallidus and

[page 6] [Neurology International 2020; 12:8328] Case Report (Figure 1F-H). She had hypertension and vascular but no diabetes. Her symptoms completely disappeared after receiving clonazepam (0.5 mg/day) administration. We aimed to reduce its dose from daily to every other day (0.25 mg/day) for several months after the onset of her condition due to her drowsiness (Figure 2C).

Results Among the three HCHB cases, all cases demonstrated a subacute or old infarction in the basal ganglia contralateral to the symptoms. Due to the severity of daily life disruptions caused by HCHB, which lasted at least several months in one case, we treated them with clonazepam (0.5-1 mg/day), haloperidol (0.375-0.75 mg/day), or both as necessary to control the observed symptoms. Symptoms were controlled using pharmacotherapy with gamma-aminobutyric acid (GABA)-agonist (clonazepam) or anti- dopaminergic (haloperidol) activity. We only attempted withdrawal when the symptoms were brought under control due to the observed side effects, such as oral dyskinesia and drowsiness, but encountered difficulties use due to recurrence. Figure 1. Magnetic resonance image findings for the three hemichorea-hemiballism cases. In case 1, brain magnetic resonance image (MRI) shows the old cerebral infarct in the right globus pallidus present for the past four years (A: T2 weighted MRI image (T2WI)at the onset, B: T2WI at the four years before the onset). In case 2, MRI shows Discussion the subacute cerebral infarcts in the right globus pallidus (C; Diffusion weighted image(DWI), D; T2WI, and E; Fluid attenuated inversion recovery(FLAIR)). In case 3, HCHB is a hyperkinetic movement MRI shows periventricular hyperdensity (PVH), old infarcts in right globus pallidus and disorder characterized by unilateral putamen (F; FLAIR, G; T2WI, and H; T2WI). involuntary movement. Apart from cerebral

Non-commercial

Figure 2. Clinical courses of the three hemichorea-hemiballism (HCHB) cases (A; case 1, B; case 2, C; case 3) X means the year when HCHB occurred.

[Neurology International 2020; 12:8328] [page 7] Case Report vascular , HCHB occurs unilaterally that HCHB is associated with basal ganglia, due to nonketotic .4-6 Among as demonstrated in our three cases. References the three patients, one had diabetes; In some cases, HCHB can occur 1. Mehanna R, Jankovic J. Movement however, his blood sugar levels were not immediately after an acute stroke, whereas disorders in cerebrovascular diseases. high enough to be considered HCHB- others can experience a progressive or Lancet Neurol 2013;12:597-608. associated. Therefore, our cases were delayed onset.1 Among our HCHB cases, the 2. Nakawah MO, Lai EC. Post-stroke considered purely stroke-related. MRI for case 2, which was taken seven days Neuropsychiatr Dis Treat Here, lesions in the globus pallidum or after the onset of HCHB, showed the 2016;12:2885-93. subacute cerebral infarcts in the globus putamen contralateral to the dyskinesia were 3. Laganiere S, Boes AD, Fox MD. pallidus contralateral to the symptoms, and observed in all cases. The pathogenesis for Network localization of hemichorea- in cases 1 and 3, old infarction was seen in stroke-related HCHB is unclear; previous Neurology 2016;86: the basal ganglia contralateral to the studies have suggested mechanisms 2187-95. symptoms. These observations suggest that involving the basal ganglia motor circuitry, 4. Hashimoto T, Hanyu N, Yahikozawa H, the onset of HCHB after stroke varies from despite the absence of a specific and reliably Yanagisawa N. Persistent hemiballism 1-3 7 case to case. predictive anatomical location. Grandas with striatal hyperintensity on T1- We successfully controlled the stated that in clinical radiological series of weighted MRI in a diabetic patient: a symptoms using pharmacotherapy with hemiballism, which are classically related to 6-year follow-up study. J Neurol Sci GABA-agonist (clonazepam) or anti- lesions in the , most 1999;165:178-81. dopaminergic (haloperidol) activity. patients had lesions outside this nucleus, 5. Kitagawa M, Yamanaka Y, Adachi T, et However, due to side effects, such as oral mainly in other basal ganglia structures. It al. Diabetic hemichorea-hemiballism dyskinesia and drowsiness, we attempted has also been suggested that abnormal after prompt improvement in withdrawal in situations where the neuronal firing patterns in the internal hyperglycemia. Intern Med 2017;56:3 symptoms were controlled but could not do segment of the globus pallidus may be 073-6. so due to recurrence. related to the pathogenesis of hemiballism.7 6. Son BC, Choi JG, Ko HC. Globus Furthermore, Zijlmans8 reviewed patients pallidusonly internus with vascular-related chorea affecting one for disabling diabetic hemiballism/ side of the body in which the lesions are Conclusions hemichorea. Case Rep Neurol Med most frequently found in the and 2017;2017:2165905. lentiform nucleus. Among the three HCHB patients,use all 7. Grandas F. Hemiballismus. Handb Clin HCHB is also known to be a showed subacute or old infarction in the Neurol 2011;100:249-60. hyperkinetic movement disorder in diabetic basal ganglia contralateral to the symptoms. 8. Zijlmans JC. Vascular chorea in adults patients. In diabetic hemichorea- Symptoms were controlled using and children. Handb Clin Neurol hemiballism, striatal hyperintensity is pharmacotherapy with GABA-agonist 2011;100:261-70. typically seen on T1-weighted magnetic (clonazepam) or anti-dopaminergic resonance imaging.4-6 These reports support (haloperidol) activity.

Non-commercial

[page 8] [Neurology International 2020; 12:8328]