Autoimmune chorea in adults

Orna O'Toole, Vanda A Lennon, J. Eric Ahlskog, Joseph Y. Matsumoto, Sean J Pittock, James Howard Bower, Robert Fealey, Daniel H Lachance, Andrew B McKeon

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Abstract

Objectives: To determine the characteristics of adult-onset autoimmune chorea, and compare paraneoplastic and idiopathic subgroups. Methods: Thirty-six adults with autoimmune chorea were identified at Mayo Clinic (Rochester, MN) from 1997 to 2012. Medical record and laboratory data were recorded. Nonparaneoplastic (n = 22) and paraneoplastic cases (n = 14) were compared. Results: Women accounted for 21 patients (58%). Median age at symptom onset was 67 years (range 18-87 years). We estimated the incidence for Olmsted County was 1.5 per million person-years. Symptom onset was subacute in all. Chorea was focal (20 patients) or generalized (16 patients). Although chorea predominated, other neurologic disorders frequently coexisted (29 patients); abnormal eye movements were uncommon (4 patients). No patient had NMDA receptor antibody or any immunoglobulin (Ig)G yielding a detectable immunofluorescence binding pattern restricted to basal ganglia. Two had synaptic IgG antibodies novel to the context of chorea (GAD65,1; CASPR2,1). In the paraneoplastic group, 14 patients had evidence of cancer. Of 13 with a histopathologically confirmed neoplasm, small-cell carcinoma and adenocarcinoma were most common; 6 patients had a cancer-predictive paraneoplastic autoantibody, with CRMP-5-IgG and ANNA-1 being most common. In the idiopathic group, 19 of the 22 patients had a coexisting autoimmune disorder (most frequently systemic lupus erythematosus and antiphospholipid syndrome); autoantibodies were detected in 21 patients, most frequently lupus and phospholipid specificities (19 patients). The paraneoplastic group was older (p = 0.001), more frequently male (p = 0.006), had more frequent weight loss (p = 0.02), and frequently had peripheral neuropathy (p = 0.008). Conclusions: Autoimmune chorea is a rare disorder with rapid onset. Male sex, older age, severe chorea, coexisting peripheral neuropathy, and weight loss increase the likelihood of cancer.

Original languageEnglish (US)
Pages (from-to)1133-1144
Number of pages12
JournalNeurology
Volume80
Issue number12
DOIs
StatePublished - Mar 19 2013

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Chorea
Immunoglobulin G
Peripheral Nervous System Diseases
Autoantibodies
Weight Loss
Neoplasms
Small Cell Carcinoma
Antiphospholipid Syndrome
Antibodies
Dyskinesias
Eye Movements
Basal Ganglia
Nervous System Diseases
N-Methyl-D-Aspartate Receptors
Age of Onset
Systemic Lupus Erythematosus
Medical Records
Fluorescent Antibody Technique
Phospholipids
Adenocarcinoma

ASJC Scopus subject areas

  • Clinical Neurology
  • Arts and Humanities (miscellaneous)

Cite this

Autoimmune chorea in adults. / O'Toole, Orna; Lennon, Vanda A; Ahlskog, J. Eric; Matsumoto, Joseph Y.; Pittock, Sean J; Bower, James Howard; Fealey, Robert; Lachance, Daniel H; McKeon, Andrew B.

In: Neurology, Vol. 80, No. 12, 19.03.2013, p. 1133-1144.

Research output: Contribution to journalArticle

O'Toole O, Lennon VA, Ahlskog JE, Matsumoto JY, Pittock SJ, Bower JH et al. Autoimmune chorea in adults. Neurology. 2013 Mar 19;80(12):1133-1144. https://doi.org/10.1212/WNL.0b013e3182886991
O'Toole, Orna ; Lennon, Vanda A ; Ahlskog, J. Eric ; Matsumoto, Joseph Y. ; Pittock, Sean J ; Bower, James Howard ; Fealey, Robert ; Lachance, Daniel H ; McKeon, Andrew B. / Autoimmune chorea in adults. In: Neurology. 2013 ; Vol. 80, No. 12. pp. 1133-1144.
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AU - Ahlskog, J. Eric

AU - Matsumoto, Joseph Y.

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AU - Bower, James Howard

AU - Fealey, Robert

AU - Lachance, Daniel H

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