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Hemiballismus

MedGen UID:
67443
Concept ID:
C0221169
Disease or Syndrome; Finding
Synonym: Hemiballism
SNOMED CT: Hemiballism (66637005); Hemiballismus (66637005); Body of Luys syndrome (66637005); Hemiballism (abnormal involuntary movements) (66637005); Hemiballismus-hemichorea syndrome (66637005)
 
HPO: HP:0100248

Definition

Hemiballismus is a rare movement disorder that is caused primarily by damage to various areas in the basal ganglia. Hemiballismus is usually characterized by involuntary flinging motions of the extremities. The movements are often violent and have wide amplitudes of motion. They are continuous and random and can involve proximal and/or distal muscles on one side of the body, while some cases even include the facial muscles. The more a patient is active, the more the movements increase. With relaxation comes a decrease in movements. [from HPO]

Conditions with this feature

Mitochondrial DNA depletion syndrome 17
MedGen UID:
1684823
Concept ID:
C5231412
Disease or Syndrome
Mitochondrial depletion syndrome-17 (MTDPS17) is an autosomal recessive dystonic or movement disorder (summary by Shafique et al., 2023). For a discussion of genetic heterogeneity of autosomal recessive mtDNA depletion syndromes, see MTDPS1 (603041).
Leukoencephalopathy, developmental delay, and episodic neurologic regression syndrome
MedGen UID:
1719567
Concept ID:
C5394367
Disease or Syndrome
Leukoencephalopathy, developmental delay, and episodic neurologic regression syndrome (LEUDEN) is characterized by global developmental delay apparent in early childhood, followed by episodic neurologic regression or decompensation associated with systemic stress, such as febrile infection. Affected individuals have hypotonia, gait difficulties or ataxia, poor or absent speech with dysarthria, and variable motor abnormalities, including spasticity, dystonia, extrapyramidal signs, and tremor. Many patients have seizures. Brain imaging shows diffuse white matter abnormalities, poor myelination, thin corpus callosum, and generalized cerebral atrophy with enlarged ventricles. The clinical features of the disorder and the abnormal brain imaging findings are progressive (summary by Mao et al., 2020).
Infantile-onset generalized dyskinesia with orofacial involvement
MedGen UID:
1798887
Concept ID:
C5567464
Disease or Syndrome
Infantile-onset limb and orofacial dyskinesia is an autosomal recessive neurologic disorder characterized by delayed motor development and onset of a hyperkinetic movement disorder in the first year of life. The disorder results in impaired walking and orofacial dyskinesia with difficulty talking; the severity is variable (summary by Diggle et al., 2016).

Professional guidelines

PubMed

Zager EL
Neurol Clin 1987 Nov;5(4):631-47. PMID: 3323880
Klawans HL, Moses H 3rd, Nausieda PA, Bergen D, Weiner WJ
N Engl J Med 1976 Dec 9;295(24):1348-50. doi: 10.1056/NEJM197612092952404. PMID: 980081
Swash M, Roberts AH, Zakko H, Heathfield KW
J Neurol Neurosurg Psychiatry 1972 Apr;35(2):186-91. doi: 10.1136/jnnp.35.2.186. PMID: 4260957Free PMC Article

Recent clinical studies

Etiology

Güzey Aras Y, Boncuk Ulaş S, Acar T, Acar BA
Acta Neurol Belg 2023 Apr;123(2):591-597. Epub 2023 Feb 7 doi: 10.1007/s13760-023-02206-0. PMID: 36749467Free PMC Article
Biswas S, Pendharkar HS, Murumkar VS
Neurol India 2022 Jul-Aug;70(4):1525-1533. doi: 10.4103/0028-3886.355127. PMID: 36076654
Nayak S, Sarangi AN, Sahoo SK, Mangla P, Tripathy M, Rao S, Gupta S, Paliwal VK, Sudhanshu S, Ravi C, Joshi K, Bhatia V, Bhatia E
Indian J Pediatr 2021 Aug;88(8):785-792. Epub 2021 Jan 7 doi: 10.1007/s12098-020-03567-7. PMID: 33409956
Suratos CTR, Benitez JAEL, Urquiza SC, Sacro CAL
BMJ Case Rep 2018 Apr 5;2018 doi: 10.1136/bcr-2017-223429. PMID: 29622707Free PMC Article
Laganiere S, Boes AD, Fox MD
Neurology 2016 Jun 7;86(23):2187-95. Epub 2016 May 11 doi: 10.1212/WNL.0000000000002741. PMID: 27170566Free PMC Article

Diagnosis

Bologna M, Valls-Solè J, Kamble N, Pal PK, Conte A, Guerra A, Belvisi D, Berardelli A
Clin Neurophysiol 2022 Aug;140:110-125. Epub 2022 Jun 8 doi: 10.1016/j.clinph.2022.05.014. PMID: 35785630
Cincotta M, Walker RH
Tremor Other Hyperkinet Mov (N Y) 2022;12:3. Epub 2022 Jan 31 doi: 10.5334/tohm.675. PMID: 35136702Free PMC Article
Laganiere S, Boes AD, Fox MD
Neurology 2016 Jun 7;86(23):2187-95. Epub 2016 May 11 doi: 10.1212/WNL.0000000000002741. PMID: 27170566Free PMC Article
Hawley JS, Weiner WJ
Parkinsonism Relat Disord 2012 Feb;18(2):125-9. Epub 2011 Sep 17 doi: 10.1016/j.parkreldis.2011.08.015. PMID: 21930415
Grandas F
Handb Clin Neurol 2011;100:249-60. doi: 10.1016/B978-0-444-52014-2.00017-3. PMID: 21496584

Therapy

Ying C, Jingqing S, Tianli L, Jiahui H, Yuhan L, Liying Z, Bin LI, Lu L
J Tradit Chin Med 2023 Aug;43(4):829-833. doi: 10.19852/j.cnki.jtcm.20230308.004. PMID: 37454270Free PMC Article
Güzey Aras Y, Boncuk Ulaş S, Acar T, Acar BA
Acta Neurol Belg 2023 Apr;123(2):591-597. Epub 2023 Feb 7 doi: 10.1007/s13760-023-02206-0. PMID: 36749467Free PMC Article
Suratos CTR, Benitez JAEL, Urquiza SC, Sacro CAL
BMJ Case Rep 2018 Apr 5;2018 doi: 10.1136/bcr-2017-223429. PMID: 29622707Free PMC Article
Laganiere S, Boes AD, Fox MD
Neurology 2016 Jun 7;86(23):2187-95. Epub 2016 May 11 doi: 10.1212/WNL.0000000000002741. PMID: 27170566Free PMC Article
Guridi J, Obeso JA
Brain 2001 Jan;124(Pt 1):5-19. doi: 10.1093/brain/124.1.5. PMID: 11133783

Prognosis

Laganiere S, Boes AD, Fox MD
Neurology 2016 Jun 7;86(23):2187-95. Epub 2016 May 11 doi: 10.1212/WNL.0000000000002741. PMID: 27170566Free PMC Article
Hawley JS, Weiner WJ
Parkinsonism Relat Disord 2012 Feb;18(2):125-9. Epub 2011 Sep 17 doi: 10.1016/j.parkreldis.2011.08.015. PMID: 21930415
Grandas F
Handb Clin Neurol 2011;100:249-60. doi: 10.1016/B978-0-444-52014-2.00017-3. PMID: 21496584
Klawans HL, Moses H 3rd, Nausieda PA, Bergen D, Weiner WJ
N Engl J Med 1976 Dec 9;295(24):1348-50. doi: 10.1056/NEJM197612092952404. PMID: 980081
HYLAND HH, FORMAN DM
Neurology 1957 Jun;7(6):381-91. doi: 10.1212/wnl.7.6.381. PMID: 13430900

Clinical prediction guides

Mumoli L, Magro G, Bosco D
Neurol Sci 2024 Feb;45(2):825-826. Epub 2023 Oct 13 doi: 10.1007/s10072-023-07112-0. PMID: 37828390
Biswas S, Pendharkar HS, Murumkar VS
Neurol India 2022 Jul-Aug;70(4):1525-1533. doi: 10.4103/0028-3886.355127. PMID: 36076654
Bologna M, Valls-Solè J, Kamble N, Pal PK, Conte A, Guerra A, Belvisi D, Berardelli A
Clin Neurophysiol 2022 Aug;140:110-125. Epub 2022 Jun 8 doi: 10.1016/j.clinph.2022.05.014. PMID: 35785630
Cincotta M, Walker RH
Tremor Other Hyperkinet Mov (N Y) 2022;12:3. Epub 2022 Jan 31 doi: 10.5334/tohm.675. PMID: 35136702Free PMC Article
Laganiere S, Boes AD, Fox MD
Neurology 2016 Jun 7;86(23):2187-95. Epub 2016 May 11 doi: 10.1212/WNL.0000000000002741. PMID: 27170566Free PMC Article

Recent systematic reviews

Costa Hoffmeister M, Bonavides PSG, Maurer Wiercinski V, Alessio Baggio V, de Pádua Borges R, Francisco Xavier Junior G, Maraschin CK, Schaan BD
Arch Endocrinol Metab 2024 Mar 25;68:e220413. doi: 10.20945/2359-4292-2022-0413. PMID: 38530964Free PMC Article
Rosca EC, Bilavu R, Cornea A, Simu M
Int J Infect Dis 2023 Sep;134:256-260. Epub 2023 Jul 7 doi: 10.1016/j.ijid.2023.07.001. PMID: 37423421

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