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Anti‐neuronal nuclear autoantibody type 2: Paraneoplastic accompaniments
Author(s) -
Pittock Sean J.,
Lucchinetti Claudia F.,
Len Vanda A.
Publication year - 2003
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.10518
Subject(s) - medicine , autoantibody , opsoclonus , limbic encephalitis , dystonia , pathology , peripheral neuropathy , encephalopathy , myoclonus , myelopathy , neurological examination , cancer , antibody , immunology , surgery , anesthesia , endocrinology , psychiatry , biology , spinal cord , genetics , diabetes mellitus , cell culture , neuroblastoma
We identified the IgG autoantibody ANNA‐2 (“anti‐Ri”) in 34 patients in a 12‐year period by immunofluorescence screening of sera from approximately 75,000 patients with subacute neurological disorders that were suspected to be paraneoplastic. Detailed clinical information was available for 28 patients (10 men, 18 women). Cancer was diagnosed in 24 patients (86%); 21 had histologically proven carcinoma (10 lung, 9 breast, 1 cervical, 1 bladder), and 3 had an intrathoracic imaging abnormality. Cancer anteceded neurological symptoms in 4 of 28 patients. Cancer detection frequency increased with continued surveillance. Neurological disorders, in decreasing frequency, were brainstem syndrome (including opsoclonus, myoclonus, or both), cerebellar syndrome, myelopathy, peripheral neuropathy, cranial neuropathy, movement disorder, encephalopathy, Lambert–Eaton syndrome, and seizures. Four patients had laryngospasm and four had jaw opening dystonia (two with neck dystonia). Nine (32%) were wheelchair‐bound 1 month after neurological symptom onset. Most improved neurologically after immunomodulatory or tumor‐directed therapy. Accompanying autoantibodies, found in 73% of sera, included ANNA‐1, ANNA‐3, CRMP‐5‐IgG, P/Q‐type and N‐type Ca 2+ channel antibodies, and muscle‐type acetylcholine receptor antibody. Some neurological accompaniments of ANNA‐2 may reflect potentially pathogenic humoral or cell‐mediated responses to coimmunogenic tumor antigens, for example, Lambert–Eaton syndrome (P/Q‐type Ca 2+ channel antibody) and peripheral neuropathy (ANNA‐1 effector T cells). Ann Neurol 2003