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LETTER TO THE EDITOR Table of Contents  
Ahead of print publication
Chorea in moyamoya disease with ipsilateral basal ganglia atrophy and mineralization

1 Department of Neuroradiology, School of Excellence in Neurosurgery, Super Speciality Hospital, Netaji Subhash Chandra Bose Medical College Jabalpur, Madhya Pradesh, India
2 Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
3 Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India

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Date of Submission02-Mar-2022
Date of Decision10-Jul-2022
Date of Acceptance01-Aug-2022
Date of Web Publication31-Jan-2023

How to cite this URL:
Yadav N, Pendharkar H, Prasad C, Kenchaiah R, Kamble N. Chorea in moyamoya disease with ipsilateral basal ganglia atrophy and mineralization. Ann Mov Disord [Epub ahead of print] [cited 2023 Jun 1]. Available from: https://www.aomd.in/preprintarticle.asp?id=368612

Chorea is a rare presentation of Moyamoya disease (MMD) and is usually secondary to basal ganglia infarct or hypertrophied collaterals.[1] In this article, we present the case of a patient with MMD with choreiform movements and ipsilateral basal ganglia atrophy and mineralization.

A 19-year-old woman presented with recurrent transient episodes of weakness involving the left upper and lower limbs for 1 month, along with abnormal involuntary arrhythmic and quasi-purposive movements of the left upper limb. The movements were neither episodic nor intermittent, which differentiated them from limb-shaking transient ischemic attacks. The patient has a past history of weakness and aphasia 1.5 years ago, with persistent weakness in the right upper and lower limbs. Examination revealed right hemiparesis and left upper limb chorea. Hemogram, erythrocyte sedimentation rate, and liver and renal function tests were normal. Vasculitis panel including antineutrophil antibodies, cytoplasmic antineutrophil antibodies, perinuclear antineutrophil antibodies, anti-double stranded DNA antibodies were negative.

Magnetic resonance imaging revealed deep watershed infarcts in the bilateral centrum semiovale and corona radiata. The left caudate nucleus and putamen showed T2/fluid-attenuated inversion recovery hyperintense signal with atrophy and blooming on susceptibility-attenuated imaging. Time-of-flight magnetic resonance angiography and digital subtraction angiography showed narrowing of the supraclinoid internal carotid arteries with net-like collaterals in the basal cisterns, suggestive of MMD [Figure 1] and [Figure 2]. No area of the acute infarct was noted. Arterial spin labeling perfusion images revealed hypoperfusion in the bilateral frontal (right > left) and parietal lobes and left basal ganglia. The patient was maintained on aspirin and started on carbamazepine for chorea,[2],[3] which was discontinued due to an allergic reaction. Chorea subsided after 2 weeks.
Figure 1: (a, b) Fluid-attenuated inversion recovery axial images showing watershed infarcts in the bilateral centrum semiovale (arrows). (c) Time-of-flight magnetic resonance angiography image showing narrowing of the bilateral supraclinoid internal carotid arteries (right > left). (d) T2-weighted axial image showing thin net-like collaterals in the basilar cistern. (e) T2-weighted axial image showing left basal ganglia atrophy; no infarct/collaterals/flow void noted in the basal ganglia. (f) Susceptibility-weighted imaging axial image showing mineralization (blooming) involving left basal ganglia. (g) Arterial spin labeling perfusion cerebral blood flow map showing right frontal and bilateral parietal hypoperfusion. (h) Arterial spin labeling perfusion cerebral blood flow map showing bilateral frontal hypoperfusion (right > left) and left basal ganglia hypoperfusion (arrow)

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Figure 2: Right internal carotid artery (ICA) injection antero-posterior view (a) and lateral view (b) showing occlusion of the right supraclinoid ICA with fetal posterior cerebral artery; few pial collaterals noted from branches of the posterior cerebral artery. (c) Left ICA injection antero-posterior view showing narrowing of the left supraclinoid ICA; flow is noted across anterior communicating artery, leading to opacification of the right anterior and middle cerebral arteries (attenuated)

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Chorea, which is reported in 3–6% of MMD cases,[4],[5] is attributed to basal ganglia infarct or hypertrophied collaterals in the basal ganglia.[4] None of these symptoms were observed in our patient; she had chronic deep watershed infarcts in the bilateral centrum semiovale. In addition, Mengwei Hao et al.[6] reported a case of hemichorea–hemiballism following acute watershed infarcts in the bilateral centrum semiovale. They attributed ischemia of the basal ganglia to intracranial atherosclerosis as the cause of chorea, which was mild enough to not be viewed on imaging.[6]

Disruption of excitatory connections from the cerebral cortex to the basal ganglia can cause functional derangement of the striatum. The cortical areas may contribute to the pathogenesis of cortical hemichorea–hemiballismus even without remarkable involvement of the basal ganglia and thalamus.[7] Disruption of the connections between the frontal and parietal cortices may be another pathomechanism of cortical hemichorea–hemiballismus,[7] as our patient had frontoparietal hypoperfusion.

After anoxic-ischemic damage, there is interruption of normal axonal transportation of iron in the brain,[8] which may lead to central iron accumulation in the basal ganglia and white matter.[8] In our patient, hypoxic insult in the left cerebral hemisphere may have caused iron accumulation in the left basal ganglia.

Chorea was noted ipsilateral to the site of iron deposition in the basal ganglia. However, the patient had bilateral watershed infarcts in the centrum semiovale with right-sided weakness. This may have caused bilateral chorea, but due to right hemiparesis, only chorea on the left side may have manifested.

Few cases of hemichorea–hemiballismus with ipsilateral cerebral lesions have been described in [Table 1] (references in supplementary file). As mentioned earlier, our patient had hypoperfusion of the basal ganglia ipsilateral to the side of chorea (paragraph 3). In addition, Krauss et al.[9] described the occurrence of hemichorea–hemiballismus with contralateral hemiparesis and ipsilateral basal ganglia lesions in two patients (one case with ipsilateral basal ganglia astrocytoma and another case with ipsilateral basal ganglia hemorrhage). They proposed that contralateral hemiparesis may be a conditioning factor for hemichorea–hemiballismus ipsilateral to cerebral lesions. Furthermore, they observed that contralateral hemiparesis was present in the majority of the patients who were previously reported to have hyperkinesia ipsilateral to cerebral lesions.[8] These patients may have had bilateral chorea or ballism, if they did not have hemiparesis; this may have precluded the contralateral manifestation of the movement disorder.[9] We believe that this may be the possible pathomechanism of chorea ipsilateral to basal ganglia mineralization and atrophy in our case. Various proposed mechanisms have been summarized in [Figure 3].
Table 1: Previously described cases with chorea due to ipsilateral basal ganglia lesions

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Figure 3: Mechanisms causing movement disorders in Moyamoya disease

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In addition, non-decussation of the pyramidal tracts[10] and strong disinhibition of the thalamus, causing excitatory input to the ipsilateral cerebral cortex and channelling to the contralateral cerebral cortex via corpus callosum due to contralateral hemiparesis, has been proposed as an alternative hypothesis.[11] However, due to the bilateral hemispheric hypoperfusion state, the possibility remains that the ipsilateral basal ganglia lesion could be non-culpable.

Chorea is a rare manifestation in patients with MMD. To the best of our knowledge, mineralization and atrophy of the basal ganglia ipsilateral to the side of the chorea has not been previously described to date. In this case report, we have described this rare clinical presentation and imaging feature in patients with MMD and have attempted to explain its pathomechanism.

Informed consent

Informed consent was obtained from all individual participants included in the study. Consent to publish images was obtained from the patient.



Author contribution

  1. Dr. Nishtha Yadav: literature search, figures, data collection, data analysis, data interpretation, writing

  2. Dr. Hima Pendharkar: literature search, figures, study design, data analysis, data interpretation, revision and editing of manuscript

  3. Dr. Chandrajit Prasad: revision and editing of manuscript

  4. Dr. Raghavendra Kenchaiah: revision and editing of manuscript

  5. Dr. Nitish Kamble: revision and editing of manuscript

Ethical compliance statement

The study has been approved by the appropriate ethics committee and has therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  Supplementary references Top

1. Conos L. A propos dun cas díhemichoree posthemiplegique gauche avec une lesion homolateral du noyau lenticulaire. En&de 1911;6:153-160.

2. Kleist K. Die psychomotorischen Storungen und ihr Verhahnis zu den MotiIit;itsstiirungen bei Erkrankungen der Stammganglien. Monatschr. Psychiat. u. Neural. 1922; 52: 253-302.

3. Austregesilo A, GaBotti 0. Sur un cas díhemiparesie et díh Cmichoree avec lesion du noyau caude. Rev. Neural. 1924; 1: 41-43

4. Matzdorff P. Beitrlge zur Pathologie des extrapyramidalmotorischen Systems: das Syndrom des Corpus Luysi. Ztschr. ges. Neural. u. Psychiut. 1927; 109: 538-554.

5. Hampel E. HemibaBismus auf luischer Grundlage. Beitrag zur pathologischen Anatomie und Pathogenese des HemibaBismus. Dtsch. Ztschr. f. NetvenheiZk. 1936; 141: 77-96

6. Moersch FP, Kemohan JW. HemibaBismus. A clinicopathologic study. Arch. Neural. Psychiatry 1939; 41: 365-372.

7. Balthasar K. iiber die Beteihgung des Globus pallidus bei Athetose und ParabaIIismus. Dtsch. Zschr. Neural. 1939; 148: 243-261.

8. Bertrand I, Christophe J. Etude anatomo-cbnique dun cas díhemibaIIisme. Hemoragie Iineaire du corps de Luys avec extensionau locus niger. Ren. Neural. 1942; 74: 140-141.

9. Dierssen G, Gioino GG, Cooper IS. Participation of ipsilateral hemisphere lesions in the pathology of hemichorea and hemiballismus. Neurology 1961; 11: 894-898.

10. Brion S, Guiot G, Derome P, Comoy C. HCmibaUismes postop Cratoires au tours de la chirurgie stereotaxique. A propos de

12 observations dont 2 anatomo-chniques dans une s&e de 850 interventions. Rev. Neural. 1965; 112: 410-443.

11. Tognetti F, Donati R. Hemiballism of ischemic origin: report of a case with unusual anatomical lesion. Actu Neural. 1986; 8: 150-153.

12. Reyes MG, Stevens ET. Hemiballismus from cerebellar metastases. 1. Neural. Neurosurg. Psychiatry 1989; 52: 684.

13. Gilon D, Constantini 5, Reches A. HemibaBism as a presentation of a meningioma. Eur. Neural. 1990; 30: 277-278.

14. Borremans JJ, Krauss JK, Fanardjian RV, Seeger W. Hemichoreahemiballism associated with an ipsilateral intraventricular cyst after resection of a meningioma. Parkinsonism Relat Disord. 1996;2(3):155-159.

15. Borgohain R, Singh AK, Thadani R, Anjaneyulu A, Mohandas S. Hemiballismus due to an ipsilateral striatal hemorrhage: an unusual localization. J Neurol Sci 1995;130:22-24.

16. Krauss JK, Pohle T, Borremans JJ. Hemichorea and hemiballism associated with contralateral hemiparesis and ipsilateral basal ganglia lesions. Mov Disord Off J Mov Disord Soc. 1999;14(3):497-501.

17. Xian WB, Zhang XS, Shi XC, Luo GH, Yi C, Pei Z. Corticostriatal Hypermetabolism in Moyamoya Disease-Induced Hemichorea: Two Case Reports and a Literature Review. Front Neurol. 2021;12:649014. doi: 10.3389/fneur.2021.649014. PMID: 34248815; PMCID: PMC8266195.

  References Top

Baik JS, Lee MS Movement disorders associated with moyamoya disease: A report of 4 new cases and a review of literatures. Mov Disord 2010;25:1482-6.  Back to cited text no. 1
Roig M, Montserrat L, Gallart A Carbamazepine: An alternative drug for the treatment of nonhereditary chorea. Pediatrics 1988;82:492-5.  Back to cited text no. 2
Peña J, Mora E, Cardozo J, Molina O, Montiel C Comparison of the efficacy of carbamazepine, haloperidol and valproic acid in the treatment of children with Sydenham’s chorea: Clinical follow-up of 18 patients. Arq Neuropsiquiatr 2002;60:374-7.  Back to cited text no. 3
Ahn ES, Scott RM, Robertson RL, Smith ER Chorea in the clinical presentation of moyamoya disease: Results of surgical revascularization and a proposed clinicopathological correlation. J Neurosurg Pediatr 2013;11:313-9.  Back to cited text no. 4
Zheng W, Wanibuchi M, Onda T, Liu H, Koyanagi I, Fujimori K, et al. A case of moyamoya disease presenting with chorea. Childs Nerv Syst 2006;22:274-8.  Back to cited text no. 5
Hao M, Qin X, Gao H A case of hemichorea-hemiballism induced by acute infarction of bilateral corona radiata and cortex. Cell Biochem Biophys 2015;73:171-4.  Back to cited text no. 6
Hwang KJ, Hong IK, Ahn T-B, Yi SH, Lee D, Kim DY Cortical hemichorea–hemiballism. J Neurol 2013;260:2986-92.  Back to cited text no. 7
Dietrich RB, Bradley WGJr Iron accumulation in the basal ganglia following severe ischemic-anoxic insults in children. Radiology 1988;168:203-6.  Back to cited text no. 8
Krauss JK, Pohle T, Borremans JJ Hemichorea and hemiballism associated with contralateral hemiparesis and ipsilateral basal ganglia lesions. Mov Disord Off J Mov Disord Soc 1999;14:497-501.  Back to cited text no. 9
Yamamoto H, Bogousslavsky J Ipsilateral hemiparesis and pyramidal tract abnormalities. Neurology 1998;50:316-6.  Back to cited text no. 10
Kannepalli NRV, Yadav R, Vazhayil V, Somanna S, Pal PK Ipsilateral hemichorea-hemiballism in a case of postoperative stroke. Tremor Hyperkinetic Mov 2016;6:359. doi: 10.7916/D8C53KQR.  Back to cited text no. 11

Correspondence Address:
Hima Pendharkar,
Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences, Bengaluru - 560 029, Karnataka
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Source of Support: None, Conflict of Interest: None


  [Figure 1], [Figure 2], [Figure 3]

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