Elsevier

The Lancet Neurology

Volume 2, Issue 11, November 2003, Pages 661-668
The Lancet Neurology

Review
Hemiballism: revisiting a classic disorder

https://doi.org/10.1016/S1474-4422(03)00554-4Get rights and content

Summary

Hemiballism is a rare movement disorder that presents with unilateral flinging movements of the limbs. In traditional teaching, it has been characterised as almost pathognomonic of a lesion in the subthalamic nucleus (STN). The prognosis was described as grave, with severe disability and death in many cases. However, review of more recent reports shows that the STN is directly involved in only a minority of cases. The prognosis is benign in most cases, with almost all patients responding well to treatment and many having spontaneous remission, although long-term prognosis of cerebrovascular disease may not be so good. There have also been recent insights into the pathophysiology of hemiballism, which have emphasised the importance of altered firing patterns in basal-ganglia structures. Recent studies have pointed to previously unrecognised causes, particularly non-ketotic hyperosmolar hyperglycaemia and complications of HIV infection, that may account for a substantial proportion of cases of hemiballism.

Section snippets

Clinical description

Patients typically present with an acute or subacute onset of flinging movements of one side of the body.1, 2, 3, 4 Movements are involuntary, irregular, of wide amplitude, and poorly patterned. They commonly involve the arm and leg together, in many cases proximally, with facial involvement in about half of cases. Movements are increased with action, decreased with relaxation, and absent during sleep. The amplitude can be variable. Some patients have severe movements that can cause physical

Anatomical localisation of hemiballism

On the basis mainly of experiments with animals and early case-reports, hemiballism was thought to be a disorder primarily of the STN. Martin, 5 in a 1934 review on hemiballism concluded, “Since attention was directed to the importance of the subthalamic lesion, no case of 'hemiballismus' has been fully described in which the corpus Luysii has not been damaged … Reliable evidence that focal damage to any other structure in the brain results in hemichorea is extremely scanty”. Whittier4 reviewed

Differential diagnosis for hemiballism: new causes

Stroke remains, without question, the commonest single cause of hemiballism. However, series and case-reports have called attention to many other causes. The differential diagnosis includes any focal lesion located generally (but not exclusively) in basal-ganglia structures. Reported focal lesions have included neoplasms,9, 4, 29, 30, 31 vascular malformations,19, 32, 33 tuberculomas,4, 14, 34 demyelinating plaques,35 and lesions associated with tuberous sclerosis.10 Disorders that predispose

Hyperglycaemic hemiballism

In 1960, Bedwell34 reported a case of hemiballism in a patient with severe hyperglycaemia that resolved as the blood-glucose abnormality was corrected, only to recur episodically as she experienced lapses in diabetic control. Hyperglycaemia as a cause of hemiballism was reported only sporadically until several recent reports from eastern Asia gave a much more complete description of this disorder.13, 49, 50, 51, 52, 53, 54, 55 With more than 60 reported cases, this is now the second commonest

Hemiballism and HIV

There have been many case-reports and series of hemiballism occurring in the context of AIDS.1, 2, 9, 59, 60, 61, 62, 63 In some cases, hemiballism has been the presenting feature of the disease. Typically, it is associated with a secondary opportunistic infection, and by far the commonest reported is cerebral toxoplasmosis.24, 59, 60, 63 As would be expected, most of the toxoplasmosis lesions that present with hemiballism are in the contralateral basal ganglia. Hemiballism associated with HIV

Formulation of the classic model

Much of our understanding of the pathogenesis of hemiballism originated with the work of Whittier, Mettler, and Carpenter in 1949 and 1950.64, 65 In this series of experiments, multiple lesions of basal-ganglia structures were made in rhesus monkeys, and the behavioural effect of the lesions was monitored. In most locations, creation of a lesion did not elicit movement abnormalities. However, lesions that destroyed at least 20% of the STN resulted in hemiballism of the contralateral limb.

New findings challenge the classic model

There are, however, many limitations of this simplified model for the understanding of hemiballism. First, there are many other interconnections between structures in the basal ganglia that are not shown in figure 3, and their role in the pathogenesis of hemiballism has yet to be elucidated.71 Second, there seems little doubt that many, if not most, cases of hemiballism in human beings are caused by lesions outside the STN, and the model as presented does little to explain these. Third, the

Prognosis

As could perhaps be expected from the predominance of autopsy-based studies, early reports described hemiballism as a grave disorder that was unremitting and fatal.1, 4, 5 However, with the increased recognition of milder phenotypes and reversible causes, and owing to the development of effective symptomatic drug therapy, the prognosis of hemiballism is clearly much more benign than previously believed. Hyland and Foreman78 were the first to describe a benign natural course of hemiballism. Of

Treatment

For non-symptomatic management, the first priority is to look for causes that require aetiology-specific treatment. The commonest of these include hyperglycaemia, infections, and neoplastic lesions. Patients who have severe ballism may injure the affected limbs by striking walls and bedrails, and padding of the limb may be important to prevent injury. Severely affected patients may experience medical complications of the excessive movement such as dehydration or rhabdomyolysis, which can be

Conclusion

Since the original descriptions of hemiballism, much has changed in our understanding of this condition. Contrary to the common view that the disorder is caused by lesions of the STN, lesions affecting various areas can cause hemiballism, and STN lesions account for only a minority of cases. Nonketotic hyperglycaemia and HIV-related infection are important considerations in the differential diagnosis of hemiballism. The prognosis of this disorder is better than previously appreciated, and most

Search strategy and selection criteria

Data for this review were identified by searches of MEDLINE with the search terms “hemiballism”, “hemichorea”, and “hemiballismus”. References were also identified from relevant articles. Abstracts from meetings were included only when one of us had been present at the meeting. Only papers published in English were reviewed.

References (101)

  • GrandasF

    Ballism

  • ShannonK

    Ballism

  • JR Whittier

    Ballism and subthalamic nucleus (nucleus hypothalamicus; corpus Luysi): review of the literature and study of thirty cases

    Arch Neurol Psychiatry

    (1947)
  • JP Martin

    Hemichorea associated with a lesion of the corpus luysii

    Brain

    (1934)
  • JP Martin

    Hemichorea (hemiballismus) without lesions in the corpus luysii

    Brain

    (1957)
  • D Goldblatt et al.

    Recurrent hemichorea following striatal lesions

    Arch Neurol

    (1974)
  • DeweyRB et al.

    Hemiballism-hemichorea: clinical and pharmacologic findings in 21 patients

    Arch Neurol

    (1989)
  • A Vidakovic et al.

    Hemiballism: report of 25 cases

    J Neurol Neurosurg Psychiatry

    (1994)
  • A Ristic et al.

    Long-term prognosis of vascular hemiballismus

    Stroke

    (2002)
  • Chung SJ, Im JH, Kim JS, Myoung CL. Hemichorea after stroke: clinico-radiologic correlation with magnetic resonance...
  • MH Chang et al.

    Putaminal petechial haemorrhage as the cause of chorea: a neuroimaging study

    J Neurol Neurosurg Psychiatry

    (1997)
  • M Arai

    Long-standing, nonprogressive hemichorea associated with hemiatrophy of the contralateral striatum and pallidum

    J Neurol

    (1999)
  • JK Krauss et al.

    Hemichorea and hemiballism associated with contralateral hemiparesis and ipsilateral basal ganglia lesions

    Mov Disord

    (1999)
  • J Guridi et al.

    The subthalamic nucleus, hemiballismus and Parkinson's disease: reappraisal of a neurosurgical dogma

    Brain

    (2001)
  • T Tsubokawa et al.

    Control of persistent hemiballismus by chronic thalamic stimulation: report of two cases

    J Neurosurg

    (1995)
  • A Tamaoka et al.

    Hemichoreahemiballism caused by arteriovenous malformations in the putamen

    J Neurol

    (1987)
  • AO Rossetti et al.

    Neurogenic pain and abnormal movements contralateral to an anterior parietal artery stroke

    Arch Neurol

    (2003)
  • S Crozier et al.

    Transient hemiballism/hemichorea due to an ipsilateral subthalamic nucleus infarction

    Neurology

    (1996)
  • JK Krauss et al.

    Functional stereotactic surgery for hemiballism

    J Neurosurg

    (1996)
  • L Milandre et al.

    Transient involuntary movement disorders and thalamic infarction

    Rev Neurol (Paris)

    (1993)
  • P Pestre et al.

    Hemichorea in acquired immunodeficiency syndrome: toxoplasmosis abscess in the striatum

    Rev Neurol (Paris)

    (1991)
  • F Carella et al.

    Hemichorea due to a cavernous angioma of the caudate: case report of an aged patient

    Ital J Neurol Sci

    (1992)
  • JM Bejar et al.

    Hemiballism and tremor due to ependymal cyst

    Mov Disord

    (1992)
  • LG Lazzarino et al.

    Hemichorea-hemiballism and anosognosia following a contralateral infarction of the caudate nucleus and anterior limb of the internal capsule

    Riv Neurol

    (1991)
  • AR Crossman et al.

    Experimental hemichorea/hemiballismus in the monkey: studies on the intracerebral site of action in a drug-induced dyskinesia

    Brain

    (1984)
  • DJ Bronster et al.

    Hemiballism secondary to a metastatic neoplasm of the subthalamic nucleus as demonstrated by CT scan

    Mt Sinai J Med

    (1983)
  • JP Glass et al.

    Hemiballism and metastatic brain tumor

    Neurology

    (1984)
  • MS Lee et al.

    Movement disorders following lesions of the thalamus or subthalamic region

    Mov Disord

    (1994)
  • H Kurita et al.

    Relief of hemiballism from a basal ganglia arteriovenous malformation after radiosurgery

    Neurology

    (1999)
  • SF Bedwell

    Some observations on hemiballismus

    Neurology

    (1960)
  • D Riley et al.

    Hemiballism in multiple sclerosis

    Mov Disord

    (1988)
  • LS Tam et al.

    Hemiballismus in systemic lupus erythematosus: possible association with antiphospholipid antibodies

    Lupus

    (1995)
  • A Kirk et al.

    Cardioembolic caudate infarction as a cause of hemichorea in lupus anticoagulant syndrome

    Can J Neurol Sci

    (1993)
  • L Alvarez et al.

    Dorsal subthalamotomy for Parkinson's disease

    Mov Disord

    (2001)
  • CC Chen et al.

    Hemiballism after subthalamotomy in patients with Parkinson's disease: report of 2 cases

    Mov Disord

    (2002)
  • PC Su et al.

    Subthalamotomy for advanced Parkinson disease

    J Neurosurg

    (2002)
  • NK Patel et al.

    Unilateral subthalamotomy in the treatment of Parkinson's disease

    Brain

    (2003)
  • CL Opida et al.

    Bilateral ballismus in phenytoin intoxication

    Ann Neurol

    (1978)
  • PA Nausieda et al.

    Chorea induced by oral contraceptives

    Neurology

    (1979)
  • MF Levesque et al.

    Ventral intermediate thalamotomy for posttraumatic hemiballismus

    Stereotact Funct Neurosurg

    (1992)
  • Cited by (0)

    View full text