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Hemiballismus

Hemiballismus is a hyperkinetic movement disorder most commonly arising after stroke and large-amplitude flinging movements of contralateral limb or limbs is pathognomonic for this condition. Spontaneous remission is frequent within several months, but D2 blockers, neuroleptics and surgery are used when necessary.


Presentation

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].

Marcus Gunn Pupil
  • Abstract The authors report a case of a 15-month-old infant who presented a left hemiballismus associated with left Marcus-Gunn pupil, and retrobulbar optic neuritis, which disappeared spontaneously in 7 days.[ncbi.nlm.nih.gov]
Tremor
  • Also Published In Title Tremor and Other Hyperkinetic Movements[academiccommons.columbia.edu]
  • It is evident that these early writers recognized a difference between the movement patterns of tremor chorea and athetosis.[jamanetwork.com]
Choreoathetoid Movements
  • Abstract 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.[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]
Homonymous Hemianopsia
  • After experiencing an initial coma for several days, the patient was found to have a right-sided homonymous hemianopsia and a right hemiparesis, which was more marked at the shoulder and was accompanied by preservation of finger movement.[ncbi.nlm.nih.gov]

Workup

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.

Ketonuria
  • She was initially admitted with hyperglycaemia but was negative for ketonuria or metabolic acidosis. Neuroimaging showed bilateral lentiform nuclei and left caudate hyperdensities on CT and T1-weighted hyperintensity on MRI.[ncbi.nlm.nih.gov]

Treatment

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].

Prognosis

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

Etiology

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].

Epidemiology

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].

Sex distribution
Age distribution

Pathophysiology

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].

Prevention

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).

Summary

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].

Patient Information

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.

References

Article

  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.

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Last updated: 2018-06-22 12:23