Abstract
Acute-onset movement disorders (MDs) are an increasingly recognized neurological emergency in both adults and children. The spectrum of possible causes is wide, and diagnostic work-up is challenging. In their acute presentation, MDs may represent the prominent symptom or an important diagnostic clue in a broader constellation of neurological and extraneurological signs. The diagnostic approach relies on the definition of the overall clinical syndrome and on the recognition of the prominent MD phenomenology. The recognition of the underlying disorder is crucial since many causes are treatable. In this review, we summarize common and uncommon causes of acute-onset movement disorders, focusing on clinical presentation and appropriate diagnostic investigations. Both acquired (immune-mediated, infectious, vascular, toxic, metabolic) and genetic disorders causing acute MDs are reviewed, in order to provide a useful clinician’s guide to this expanding field of pediatric neurology.
Highlights
Acute-onset movement disorders (MDs) are an increasingly recognized neurological emergency in both adults and children [1,2]
Epstein–Bar virus (EBV) encephalitis may cause parkinsonism [98,99], choreoathetosis may be seen in the acute phase of influenza A, EBV and HHV-6 encephalitis [100,101,102], and tics have been described in post-varicella encephalitis [103]
MDs are rarely associated with herpes simplex virus (HSV) encephalitis, and the numerous reports of post-HSV encephalitis relapses associated with chorea and other MDs are due to subsequent anti-NMDAr Autoimmune Encephalitis (AE) [41,42,107]
Summary
Acute-onset movement disorders (MDs) are an increasingly recognized neurological emergency in both adults and children [1,2]. No data are available for hypokinetic disorders, the rarest of pediatric MDs. Given the vulnerability of the basal ganglia to different noxae, a broad range of neurological and systemic diseases may result in acute-onset MDs. Three different clinical scenarios are possible: (1) the acute or subacute appearance of a novel-onset MD, (2) the acute or subacute exacerbation of a chronic, pre-existing MD; and (3) the occurrence of a paroxysmal MD—a disturbance that has a discrete timing of onset and cessation. Three different clinical scenarios are possible: (1) the acute or subacute appearance of a novel-onset MD, (2) the acute or subacute exacerbation of a chronic, pre-existing MD; and (3) the occurrence of a paroxysmal MD—a disturbance that has a discrete timing of onset and cessation In this latter case, patients frequently come to medical attention after the resolution of symptoms. Paroxysmal MD and status dystonicus are not treated, as they have been extensively reviewed elsewhere [6,7,8,9]
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