Adult-Onset Autoimmune Chorea (S08.003)

Abstract

Objective: To describe adults consecutively evaluated with autoimmune chorea (Mayo Clinic, 1997-2011). Background Data regarding adult-onset autoimmune chorea is limited. Design/Methods: Inclusion criteria: 1) chorea as the predominant complaint; 2) an autoimmune cause was suspected based on: presence of a paraneoplastic antibody or cancer (Paraneoplastic Group) or clinical/serological evidence of autoimmunity without cancer (Non-paraneoplastic Group). The Groups were statistically compared (Fisher or Wilcoxon tests). Results: Chorea-onset was acute/subacute in 36/38 patients (median-age 68 years; range, 18-87 [women, 22]) and affected whole-body (28 patients), hemi-body (5), or extremities only (5; upper, 4; lower, 1); median follow-up, 13 months (range, 1-228). Paraneoplastic Group: 17/38 patients; 16 had ≥1 cancer (small-cell lung, 4; hematological, 4; adenocarcinoma [2 each of breast, colon, prostate]; other, 3) and 5 had ≥1 paraneoplastic antibody (CRMP-5 IgG, 4; ANNA-1, 2; other, 2). Male sex (p=0.02), older age (p=0.001), severe chorea (p=0.04), and weight loss (p=0.04) were more common. Non-paraneoplastic Group: 21/38 patients; antiphospholipid syndrome, SLE (6 patients each) and spontaneous remissions were more common (p=0.02). Both groups were similar in respect of: coexisting neurological disorders (cognitive/personality change, 11; neuropathy, 9; other, 4), MRI findings (irrelevant to chorea), other coexisting autoimmune diseases (thyroid, 5; Sjogren disease, 4; other, 12), other antibody findings (antinuclear antibody, 20; phospholipid antibody, 16; SS-A/SS-B, 7; other, 9) and physician-reported treatment responses (p>0.05). Immunotherapy-responsiveness was documented in 18/21 patients (median treatment-duration, 1.5 months [range 0.25-228]): corticosteroids, 18; plasmapheresis, cyclophosphamide, chemotherapy, 2 each. Complementary steroid-sparing strategies employed included: hydroxychloroquine, azathioprine, 5 each; cyclophosphamide, mycophenolate, 1 each. Effective symptomatic therapies (13/24 patients) were: risperidone, 6; benzodiazepines, 3; fluphenazine, reserpine, divalproex, gabapentin, 2 each. Final outcomes (30 patients) were: remission, 14 (including 4 untreated patients); improved, 11; unchanged, 3; worsened, 2. Conclusions: Autoimmune chorea is usually rapid-onset and frequently remits. Male sex, older age and severe symptoms should strengthen the suspicion for cancer. Disclosure: Dr. O9Toole has nothing to disclose. Dr. Ahlskog has nothing to disclose. Dr. Matsumoto has nothing to disclose. Dr. Pittock has received research support from Alexion Pharmaceuticals, Inc. Dr. Bower has received personal compensation for activities with Teva Neuroscience and Merck & Co., Inc. as a consultant. Dr. Lennon has nothing to disclose. Dr. Lachance has nothing to disclose. Dr. Fealey has nothing to disclose. Dr. McKeon has nothing to disclose.