title: Autoimmune chorea in adults.
abstract: OBJECTIVE
  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.
authors: Department of Neurology, Mayo Clinic, College of Medicine, Rochester, MN, USA.
authors: Orna
authors: O
authors: O'Toole
authors: Vanda A
authors: VA
authors: Lennon
authors: J Eric
authors: JE
authors: Ahlskog
authors: Joseph Y
authors: JY
authors: Matsumoto
authors: Sean J
authors: SJ
authors: Pittock
authors: James
authors: J
authors: Bower
authors: Robert
authors: R
authors: Fealey
authors: Daniel H
authors: DH
authors: Lachance
authors: Andrew
authors: A
authors: McKeon
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000096:biosynthesis / Q000097:blood / Q000134:cerebrospinal fluid; D020274:Autoimmune Diseases of the Nervous System / Q000175:diagnosis* / Q000453:epidemiology* / Q000378:metabolism; D002819:Chorea / Q000175:diagnosis* / Q000453:epidemiology* / Q000378:metabolism; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000378:metabolism; D010523:Peripheral Nervous System Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000378:metabolism; D012307:Risk Factors; D055815:Young Adult
chemical_list: D001323:Autoantibodies