Hemiballismus

Authors:

Tasneem Kousar MBBS,

Ayesha Hameed MBBS,

Adeel Memon MD

Introduction

• Hemiballismus represents a continuum of involuntary hyperkinetic movement disorder with chorea and athetosis.

Epidemiology

• 1 to 2 per 1,000,000

Etiology

• The most common cause of Hemiballismus is Stroke.

• Non-ketotic hyperglycemia is the second most common cause

• Other causes are listed in below

Other Causes of Hemiballismus

• Intracranial hemorrhage

• Demyelinating disorders

• Neuroinfectious: Rare and more common in younger patients

• Vasculitis (e.g. systemic lupus erythematosus)

• Traumatic brain injury

• Metabolic disturbances (sodium, magnesium, calcium, hyperthyroid)

• Toxoplasmosis: complication of human immunodeficiency virus (HIV) infection

• Neoplasms

• ALS

• Drug use ( Levodopa, anticonvulsants, oral contraceptives and neuroleptics)

• Vasculitis (e.g. systemic lupus erythematosus)

• Toxic: alcohol, heavy metals exposure

Pathophysiology

• Damage in the basal ganglia structures leads to decreased excitatory transmission of the globus pallidus internus (GPi) resulting in disinhibition of the thalamus.

• This creates an overactivation of the corticospinal and corticobulbar tracts with random firing.

History and Clinical Examination

• Symptoms include: Motor involuntary, jerky, wide amplitude, irregular movements involving ipsilateral arms and legs.

• Associated symptoms include Cranial nerve signs such as anisocoria, ptosis, facial droop, dysarthria, headaches in patients with stroke or intracranial hemorrhage and Altered mental status with or without motor and sensory deficits may be present in patients with neoplasm.

• If constitutional symptoms occur then look for neuro-infectious and neuroinflammatory causes.

Etiologies

• Vascular

• Metabolic

• Traumatic

• Infections

Evaluation

• A thorough medical history and physical exam can pinpoint to the diagnosis and additional studies might not be required.

• Stroke work up if multiple vascular risk factors are present

• Serum labs depend on the suspected etiology

• Imaging studies may be used to rule out structural abnormalities, neoplasms or autoimmune disorders

• Lumbar puncture including flow cytology and cytometry should be obtained when neuroinfectious or neuroinflammatory/paraneoplastic etiology is suspected.

Serum Lab Studies

• Complete Metabolic Panel

• Serum osmolality

• ANA

• Ceruloplasmin

• CBC

• ESR

• ENA( extranuclear antibody)

• Lupus antibodies

• HbA1c

• CRP

• Vitamin panel( B1, B12)

• HIV antibody screen

Imaging Studies

• Contrast MRI

• Magnetic Resonance angiogram (MRA)

• Computed Tomography Angiogram (CTA)

• Digital Subtraction Angiogram (DSA)

Management

• Most cases are managed by treating the underlying cause and providing supportive treatment for comorbidities and complications.

• Medical Symptomatic Treatment (Table 1)
• Tetrabenazine can be used to treat severe hyperkinetic movements.

• Surgical Symptomatic Treatment is considered in refractory cases.A targeted stereotactic posteroventral pallidotomy is the procedure of choice.

Prognosis

• Good with resolution after treatment of the underlying cause

• Unresponsive cases need surgical intervention.

Patient Education

• Patients should be counselled that the cause of hemiballismus depends on the underlying etiology and symptoms resolve within reasonable time after treatment of the underlying conditions.

• However, consultation with the social worker and the psychologist can be helpful to mitigate the emotional effects of hemiballismus on patients as patients can feel distressed and handicapped because of these abnormal movements.

Further Reading

• Carrion, D. M., & Carrion, A. F. (2013). Non-ketotic hyperglycaemia hemichorea-hemiballismus and acute ischaemic stroke. BMJ case reports, 2013, bcr2012008359. https://doi.org/10.1136/bcr-2012-008359

• Noda, K., Hattori, N., & Okuma, Y. (2015). Hemiballism with leg predominance caused by contralateral subthalamic haemorrhage. BMJ case reports, 2015, bcr2014208525. https://doi.org/10.1136/bcr-2014-208525

Bibliography

• Cabrero, F. R. (2021, June 17). Hemiballismus. StatPearls [Internet]. https://www.ncbi.nlm.nih.gov/books/NBK559127/.

• Grandas F. Hemiballismus. Handb Clin Neurol. 2011;100:249-60. [PubMed]

• Laganiere S, Boes AD, Fox MD. Network localization of hemichorea-hemiballismus. Neurology. 2016 Jun 07;86(23):2187-95. [PMC free article] [PubMed]

• Noda K, Hattori N, Okuma Y. Hemiballism with leg predominance caused by contralateral subthalamic haemorrhage. BMJ Case Rep. 2015 Apr 09;2015 [PMC free article] [PubMed]

• Jaafar J, Rahman RA, Draman N, Yunus NA. Hemiballismus in Uncontrolled Diabetes Mellitus. Korean J Fam Med. 2018 May;39(3):200-203. [PMC free article] [PubMed]

• Wikimedia Foundation. (2021, August 6). Hyperkinesia. Wikipedia. https://en.wikipedia.org/wiki/Hyperkinesia.