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.
ReviewHemiballism: revisiting a classic disorder
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
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