Movement disorders in paraneoplastic and autoimmune disease

Purpose of review: The most relevant advances in immune-mediated movement disorders are described, with emphasis on the clinical--immunological associations, novel antigens, and treatment.

Recent findings: Many movement disorders previously considered idiopathic or degenerative are now recognized as immune-mediated. Some disorders are paraneoplastic, such as anti-CRMP5-associated chorea, anti-Ma2 hypokinesis and rigidity, anti-Yo cerebellar ataxia and tremor, and anti-Hu ataxia and pesudoathetosis. Other disorders such as Sydenham's chorea, or chorea related to systemic lupus erythematosus and antiphospholipid syndrome occur in association with multiple Antibodies, are not paraneoplastic, and are triggered by molecular mimicry or unknown mechanisms. Recent studies have revealed a new category of disorders that can be paraneoplastic or not, and associate with Antibodies against cell-surface or synaptic proteins. They include anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis, which may cause dyskinesias, chorea, ballismus or dystonia (NMDAR Antibodies), the spectrum of Stiff-person syndrome/muscle rigidity (glutamic acid decarboxylase, amphiphysin, GABA(A)-receptor-associated protein, or glycine receptor Antibodies), neuromyotonia (Caspr2 Antibodies), and opsoclonus--myoclonus--ataxia (unknown antigens).

Summary: Neurologists should be aware that many movement disorders are immune-mediated. Recognition of these disorders is important because it may lead to the diagnosis of an occult Cancer, and a substantial number of patients, mainly those with Antibodies to cell-surface or synaptic proteins, respond to immunotherapy.