One Side of the Story; Clues to Etiology in Patients with Asymmetric Chorea

Abstract

Background: Chorea can be due to a large number of etiologies. Unilateral chorea is classically related to a contralateral structural lesion, e.g. of the putamen or subthalamic nucleus, however, based upon personal impressions, we have observed that systemic disease, in particular metabolic or autoimmune conditions, can also lead to a unilateral or markedly asymmetric presentations. We sought to investigate this impression by reviewing the literature. Methods: A PubMed search was conducted using the terms asymmetric” AND “chorea” OR “hemichorea” OR “unilateral” AND “chorea” OR “monochorea” OR “right greater than left” AND “chorea” OR “left greater than right” AND “chorea” OR “right more than left” AND “chorea” OR “left more than right” AND “chorea” as well as “hemiballismus” NOT “stroke” NOT “infarct” NOT “dyskinesia. A total of 243 sources were felt to meet criteria and were reviewed. Results: The most common etiology of reported hemi- or asymmetric chorea was diabetic non-ketotic hyperglycemic hemichorea/hemiballismus. Other common diagnoses were Sydenham’s disease, antiphospholipid syndrome and drug-induced chorea. The vast majority of patients with hemi- or asymmetric chorea had acquired rather than genetic, degenerative or congenital causes. Conclusion: Despite the potential limitations of our literature review, the evidence presented here supports the observation that the vast majority of asymmetric or unilateral chorea presentations are due to acquired causes, and in this situation an exhaustive search for reversible etiology should be undertaken. However, presentation with symmetric, generalized chorea does not exclude reversible causes, and investigations should address these in addition to genetic and neurodegenerative etiologies.

Recurrent hemichorea with different etiologies in one patient

“…Other causes including hypoglycemia, Sydenham’s disease, uremia and anti-phospholipid antibody syndrome have also been reported. These different etiologies of hemichorea result in different brain imaging appearance [ 1 ]. Typically, in hyperglycemic hemichorea, CT scans show hyperdense lesions at the contralateral basal ganglia and T1 MRI shows hyperintensity [ 6 , 7 ].…”

“…The unilateral movement disorder, hemichorea, commonly stems from acute ischemic or hemorrhagic stroke of contralateral cerebral lesions and is typically followed by hyperglycemia and other systemic diseases, such as hypoglycemia, uremia and anti-phospholipid antibody syndrome [ 1 ]. Several cases of recurrent hemichorea have been reported and associated with the same etiology [ 2–5 ], However, different etiologies could contribute to the recurrence of hemichorea in the same patient.…”

“…Differential diagnosis should be made by detailed history taking, laboratory examinations and image studies. Among them, brain magnetic resonance imaging (MRI) is a helpful tool [ 1 ]. In this work, we report a case of recurrent same-side hemichorea with different etiologies diagnosed by brain MRI images.…”

Clinical and Radiological Follow-Up of a Pfizer-BioNTech COVID-19 Vaccine-Induced Hemichorea-Hemiballismus; Insights Into Mechanisms of Basal Ganglia Dysfunction

“…Other inflammatory/auto-immune causes of hemichorea include antiphospholipid syndrome (APLS) with or without systemic lupus erythematosus (SLE) and autoimmune encephalitides such as anti-N-methyl-D-aspartate (NMDA) receptor encephalitis [2]. Additionally, a history of inflammatory chorea appears to predispose toward recurrence later in life, as can be seen in women recovered from Sydenham's disease in childhood who go on to develop estrogen-induced chorea or chorea gravidarum in adulthood [2]. Despite these well-described entities, the mechanisms responsible for the appearance of chorea and the reasons for the asymmetry are not well understood [2].…”

“…Additionally, a history of inflammatory chorea appears to predispose toward recurrence later in life, as can be seen in women recovered from Sydenham's disease in childhood who go on to develop estrogen-induced chorea or chorea gravidarum in adulthood [2]. Despite these well-described entities, the mechanisms responsible for the appearance of chorea and the reasons for the asymmetry are not well understood [2].…”