Review
Movement disorders and the osmotic demyelination syndrome

https://doi.org/10.1016/j.parkreldis.2013.04.005Get rights and content

Abstract

With the advent of MRI, osmotic demyelination syndromes (ODS) are increasingly recognised to affect varied sites in the brain in addition to the classical central pontine lesion. Striatal involvement is seen in a large proportion of cases and results in a wide variety of movement disorders. Movement disorders and cognitive problems resulting from ODS affecting the basal ganglia may occur early in the course of the illness, or may present as delayed manifestations after the patient survives the acute phase. Such delayed symptoms may evolve over time, and may even progress despite treatment. Improved survival of patients in the last few decades due to better intensive care has led to an increase in the incidence of such delayed manifestations of ODS. While the outcome of ODS is not as dismal as hitherto believed – with the acute akinetic-rigid syndrome associated with striatal myelinolysis often responding to dopaminergic therapy – the delayed symptoms often prove refractory to medical therapy. This article presents a review of the epidemiology, pathophysiology, clinical features, imaging, and therapy of movement disorders associated with involvement of the basal ganglia in ODS. A comprehensive review of 54 previously published cases of movement disorders due to ODS, and a video recording depicting the spectrum of delayed movement disorders seen after recovery from ODS are also presented.

Section snippets

Epidemiology and pathophysiology

Hitherto thought to be uncommon, ODS is increasingly reported today, and has accounted for 0.4–0.56% of admissions to neurology services at tertiary-care referral centres and 0.06% of all admissions to the medical service of a general hospital [4], [5], [6]. Clinically recognised ODS may be on the rise possibly due to the inability of some patients to tolerate rapid increase in sodium levels [2]. Magnetic resonance imaging (MRI) has enabled ante-mortem diagnosis of ODS, and has expanded its

Movement disorders due to ODS

Clinical or radiologic evidence of neurologic damage due to ODS begins 0.5–7 days after osmotic shifts occur, but may be delayed by as long as 16 days [19], [23], [31], [32]. Symptoms may be mild and a high degree of suspicion is necessary to make the diagnosis [11]. Patients generally – but not in all cases – exhibit a biphasic course in which the first set of symptoms are due to a nonlocalising encephalopathy due to hyponatraemia, and a period of relative improvement (the “lucent interval”)

Role of MRI

By 1972, only 2 of 100 reported cases of ODS had been diagnosed ante-mortem [10]. The modern scenario is very different, thanks to the advent of neuroimaging. MRI is decisive in making a diagnosis of ODS, helping to uncover new cases, delineate the extent of and trace evolution of the lesion, and correlate progression or regression with clinical features [8]. It is useful to detect asymptomatic or mild ODS cases [2], [10], [16]. Although clinical features of ODS usually precede MRI changes –

Diagnosis

ODS is easily suspected in the typical clinical setting where a patient with recent or ongoing electrolyte disturbance, malnutrition, alcohol abuse, recent liver transplant, severe systemic illness or a combination of these develops acute quadriparesis, parkinsonism or coma. However the protean manifestations listed above as well as the extensive list of conditions known to predispose to ODS necessitate a high index of suspicion, particularly when an individual fails to recover as expected or

Management and prognosis

Prevention of ODS is of paramount importance. CPM has been associated with low, high or normal sodium levels [29] but hyperosmolarity or rapid osmotic shifts are more important in the pathogenesis of myelinolysis than the absolute sodium level. Concomitant malnutrition, alcohol or drug abuse, Addison's disease, hypoxia, immunosuppression, hypoglycaemia, hypokalaemia or azotaemia all increase the likelihood of developing ODS after rapid sodium correction [22], [23], [52]. Patients with very low

Conclusion

The widespread use of MRI has resulted in increasing recognition of the association of ODS – in particular EPM – with movement disorders. Parkinsonism and less commonly dystonia are often noted in the initial evaluation of patients with EPM, while those surviving the acute illness may manifest delayed dystonia, parkinsonism, choreoathetosis and other movement disorders. Such delayed movement disorders are likely to become more common as improvements in intensive care techniques lead to

Ethical approval

The patient depicted in the video provided written informed consent to being filmed and for the recording to be used for teaching and research. The hospital ethics committee of Goa Medical College approved the article.

Funding

None.

Conflict of interest

None.

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