The hallmark of hemiballsimus is the development of large-amplitude, involuntary, irregular and violent movement of the limbs on one side of the body [1]. The proximal aspects of the arm and leg are most commonly affected, whereas facial involvement is seen in about 50% of cases [1]. These large-amplitude movements can cause severe exhaustion and dehydration, but also local injury and even death in extreme cases [7].

• Cerebral Palsy

  Intrathecal baclofen (ITB) therapy is widely used in the management of spastic hypertonia and dystonia resulting from a multitude of conditions that present with upper motor neuron syndrome signs, such as cerebral palsy, stroke, brain and spinal injuries [ncbi.nlm.nih.gov]

  Intrathecal baclofen (ITB) therapy has been in use for treating different types of movement disorders, such as multiple sclerosis and cerebral palsy. ITB therapy can also help in treating hemiballismus. [epainassist.com]

• Asymptomatic

  He remained asymptomatic after euglycemia was achieved and was discharged on premixed insulin 30:70 subcutaneously, which he tolerated well for a few months. [ijem.in]

  In contrast, acute basal ganglia changes on MRI are noted in some cases of asymptomatic non-ketotic hyperglycemia [ 9 ]. [pubs.sciepub.com]

• Vomiting

  CASE REPORT: A 45-year-old woman presented with choreoathetoid movements of the right upper limb, persistent vomiting and generalized body weakness. [lookfordiagnosis.com]

• Vascular Disease

  It results from cerebral vascular disease and is usually due to infarction of one subthalamic nucleus (corpus Luysii) or of the pathway connecting this nucleus to the mid-brain. 1, record 1, English, - hemiballismus Record number: 1, Textual support number [btb.termiumplus.gc.ca]

• Suggestibility

  These results are consistent with the hyperkinetic model, which suggests that hemiballismus results from decreased inhibition of the pallidal relay nucleus of the thalamus by the GPi. [ncbi.nlm.nih.gov]

• Tremor

  TREMOR Tremor is a universal phenomenon. The description of tremors often relies on an assessment of the tremor frequency: this is hard to judge with the naked eye. [academic.sun.ac.za]

  We report two cases of post-stroke hemiballismus and contralateral tremor. Both patients presented acute hemiballismus. In one of the patients, the tremor started with acute symptomatology and the other was delayed. [revecuatneurol.com]

  No paciente com tremor de Holmes havia lesões no mesencéfalo e no cerebelo, secundárias à co-infecção pelo bacilo da tuberculose e pelo Toxoplasma gondii. [scienceopen.com]

  Also Published In Title Tremor and Other Hyperkinetic Movements [academiccommons.columbia.edu]

• Excitement

  The pathophysiology of the hemiballismus in this case may be loss of tonic inhibition of the lateral globus pallidus from the putamen, leading in turn to greater inhibition of the subthalamic nucleus, less excitation of the medial globus pallidus, and [ncbi.nlm.nih.gov]

  The destruction of the STN causes a decrease of this inhibitory function and increases thalamic excitation of the motor and premotor cortex, resulting in involuntary movement. 12 This mechanism is supported by findings during deep brain stimulation of [dx.doi.org]

  The subthalamic nucleus essentially provides the excitement needed to drive the globus pallidus. Injury to this area or its efferent or afferent connections can induce this disorder contralateral to the side of the lesion. [en.wikipedia.org]

• Choreoathetoid Movements

  A 68-year-old woman with poorly controlled diabetes mellitus presented to the emergency department with choreoathetoid movements affecting the upper and lower left limbs. Computed tomography of the brain did not show any intracranial abnormalities. [ncbi.nlm.nih.gov]

  CASE REPORT: A 45-year-old woman presented with choreoathetoid movements of the right upper limb, persistent vomiting and generalized body weakness. [lookfordiagnosis.com]

The diagnosis can be made during physical examination, as this hyperkinetic disorder is easily distinguished from other movement disorders. If stroke is presumed to be the cause, either CT or MRI of the endocranium may be performed to confirm the diagnosis.

• Hypoglycemia

  Hypoglycemia, hypernatremia, hyponatremia, hypomagnesemia, and hypocalcemia also represent some of metabolic portion of acquired etiologies [ 2 ]. It can also be a manifestation of hypoglycemia [ 3 ]. [pubs.sciepub.com]

  As part of the complications arising from HIV infections, Hemiballismus may arise in patients due to hypoglycemia stemming from their use of pentamidine. [medicalook.com]

  HIV infection complications, such as hypoglycemia from pentamidine use, in patients who have AIDS also can cause hemiballismus. There are some cases where hemiballismus is the only apparent symptom of AIDS. [epainassist.com]

  After three months, he experienced recurrent episodes of hypoglycemia, and was shifted to glimepride 1 mg twice per day, which is continuing at time of writing. [ijem.in]

• Gliosis

  Amyotrophic Lateral Sclerosis causes loss of neurons and gliosis including the subthalamic nucleus and other regions of the brain. Any type of disorder which causes loss of neurons and gliosis has the tendency to cause hemiballismus. [epainassist.com]

  Essentially any disorder that causes some form of neuronal loss or gliosis in the basal ganglia has the potential to cause hemiballismus. [en.wikipedia.org]

  As well as the typical neuropathological findings of ALS (marked upper and lower motor neuron loss), post‐mortem examination showed prominent neuronal loss and gliosis in the subthalamus, and in the internal globus pallidus, substantia nigra pars compacta [doi.org]

The goal of treatment is to resolve the underlying cause [1], which may not be confirmed in all cases. Dopamine-2 receptor blockers and neuroleptics (haloperidol, perphenazine) are first-line pharmacologic agents used for patient in whom spontaneous remission of symptoms does not occur [1] [2]. Catecholamine-depleting agents such as reserpine and tetrabenazine, on the other hand, are used in more resistant cases, whereas surgery is considered to be the last resort of care [1]. Stereotactic pallidotomy may be performed in patients who suffer from persistent hemiballismus that is refractory to therapy [6].

The prognosis is generally good, as spontaneous improvement or remission occurs in many patients [2].

The most common cause of hemiballismus is stroke, while other notable conditions include nonketotic hyperglycemia and complications of advanced human immunodeficiency virus (HIV) infection [5].

Approximately 1% of individuals who suffer from acute stroke develop hemiballismus and symptoms appear around 4 days after the initial incident, but in a small fraction of patients, the onset may be within 24 hours [1].

Presumably, the conditions that induce this hyperkinetic disorder interfere with the basal ganglia and the pathogenesis is thought to involve abnormal neuronal signaling in the globus pallidus, most likely caused by focal lesions in the contralateral basal ganglia and the subthalamic nucleus [1] [3].

Fortunately, the disorder is self-limiting and can resolve either spontaneously or through the use of drugs, as prevention strategies can only be aimed at preventing its occurrence as a part of the underlying disease (maintaining general health through exercise and diet to reduce the risk of stroke, control of blood sugar and management of HIV by regular use of antiretroviral drugs).

Hemiballismus is a rare disorder of hyperkinetic movement characterized by involuntary, irregular and violent large-amplitude movements of contralateral limbs, most frequently the proximal aspects of the arm and leg [1]. Stroke is the most common cause, affecting approximately 1% of patients, and the mechanism of disease is thought to involve impairment of neuronal signaling in the basal ganglia, most notably in the area of globus pallidus, which is one of the most important regions that control body movement [2] [3]. The diagnosis is made based on clinical findings, while magnetic resonance imaging (MRI) or computed tomography (CT) of the brain may be performed to exclude stroke as the underlying cause. The prognosis is generally good, as spontaneous remission is observed within months, but because symptoms may sometimes be severe and even life-threatening for the patient (exhaustion, dehydration and injury), drugs such as dopamine-2 receptor blockers, neuroleptics and catecholamine-depleting agents are used in therapy [3]. If hemiballismus is refractory to therapy, pallidotomy may be performed [4] [5].

Hemiballismus is a rare condition of abnormal body movement most commonly encountered in patients after suffering from stroke. Involuntary, irregular and large-amplitude movements of either one or two limbs on the same side (for example left arm and/or left leg are affected) are main features of this disorder and the diagnosis should be made during physical examination. Treatment is sometimes not necessary, as the condition resolves on its own in many patients in a period of months, but some individuals experience extreme fatigue and can suffer from life-threatening trauma. For this reason, drugs that interfere with dopamine and other neurotransmitters that regulate body movement are given, most commonly haloperidol, perphenazine, reserpine and tetrabenazine. If symptoms persist despite therapy, surgical removal of globus pallidus, one of the most important structures involved in movement regulation, is recommended, as it is almost always damaged in these patients.

1. Shrestha P, Adhikari J, Poudel D, Pathak R, Karmacharya P. Cortical Hemiballism: A Case of Hemiballismus Associated with Parietal Lobe Infarct. N Am J Med Sci. 2015;7(12):572-574.
2. Grandas F. Hemiballismus. Handb Clin Neurol. 2011;100:249-260.
3. Hawley JS, Weiner WJ. Hemiballismus: current concepts and review. Parkinsonism Relat Disord. 2012;18(2):125-129.
4. Postuma RB, Lang AE. Hemiballism: revisiting a classic disorder. Lancet Neurol. 2003 Nov;2(11):661-668.
5. Guay DR. Tetrabenazine, a monoamine-depleting drug used in the treatment of hyperkinetic movement disorders. Am J Geriatr Pharmacother. 2010;8(4):331-373.
6. Slavin KV, Baumann TK, Burchiel KJ. Treatment of hemiballismus with stereotactic pallidotomy. Case report and review of the literature. Neurosurg Focus. 2004 Jul 15;17(1):E7.
7. Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson J, Loscalzo J. eds. Harrison's Principles of Internal Medicine, 18e. New York, NY: McGraw-Hill; 2012.