Cited by 501Autoimmune Neurological Disorders and TreatmentsLung Cancer Research StudiesSystemic Lupus Erythematosus Research

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Paraneoplastic and oncologic profiles of patients seropositive for type 1 antineuronal nuclear autoantibodies

Lucchinetti, Claudia F., Kimmel, David W., Lennon, Vanda A.·1998·Neurology

DOI 10.1212/wnl.50.3.652

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Abstract

Type 1 antineuronal nuclear autoantibody (ANNA-1, also known as "anti-Hu") is a marker of neurologic autoimmunity that is highly associated with small-cell lung carcinoma (SCLC). To determine the spectrum of symptoms and signs as well as the frequency of cancer in adult patients who are seropositive for ANNA-1, we reviewed 162 sequential patients (67% female) identified as ANNA-1-positive in a comprehensive immunofluorescence screening test. In 21% of these patients, the antibody test requested by the physician was not ANNA-1. By the end of the follow-up period, cancer had been found in 142 patients (88%). Ten of these lacked evidence of SCLC (4 had prostate carcinoma, 3 breast carcinoma, 1 both prostate carcinoma and melanoma, 1 lymphoma, and 1 squamous-cell lung carcinoma). Of the 132 patients (81%) with proven SCLC, 17 had one or more coexisting malignant neoplasms (6 had renal carcinoma, 4 another lung primary carcinoma, 3 prostate carcinoma, 3 breast carcinoma, and 4 assorted neoplasms). The diagnosis of SCLC in 128 patients (97%) followed the onset of paraneoplastic symptoms. SCLC was identified in 10 patients by chest MRI after an equivocal chest radiograph or CT; in 28 by bronchoscopy, mediastinoscopy, or thoracotomy; and in 7 at autopsy. Neurologic signs in decreasing frequency were neuropathy (sensory > mixed somatic > autonomic > cranial [especially cranial nerve VIII] > motor), cerebellar ataxia, limbic encephalitis, polyradiculopathy, associated Lambert-Eaton myasthenic syndrome, myopathy, myelopathy, opsoclonus/myoclonus, motor neuronopathy, brachial plexopathy, and aphasia. Nineteen patients had a solely gastrointestinal initial presentation, including gastroparesis, pseudo-obstruction, esophageal achalasia, or other dysmotility. We conclude that seropositivity for ANNA-1 can expedite the diagnosis and treatment of otherwise occult cancer in patients, especially tobacco abusers, with varied neurologic and gastroenterologic presentations. The search for SCLC should not end on discovering a different neoplasm.

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Authors: Lucchinetti, C. F., Kimmel, D. W., & Lennon, V. A.
Affiliations: Mayo Clinic (US)
ORCID: Lucchinetti, Claudia F.
Lennon, Vanda A.
Venue: Neurology
Publisher: Ovid Technologies (Wolters Kluwer Health)
Published: 1998-03
Volume: 50
Issue: 3
Pages: 652-657
ISSN: 0028-3878 (ISSN-L)
1526-632X
DOI: 10.1212/wnl.50.3.652
OpenAlex: W2130696458
Cited by: 499
References: 50
Citations by year: 2026: 10 · 2025: 1 · 2024: 19 · 2023: 13 · 2022: 11 · 2021: 25 · 2020: 20 · 2019: 19 · 2018: 11 · 2017: 18 · 2016: 22 · 2015: 16 · 2014: 12 · 2013: 23 · 2012: 22
FWCI: 10.26
Type: journal article
Language: en
Open access: closed
Locations: Neurology (publishedVersion)
PubMed (publishedVersion)
Funders: National Institutes of Health (5r01ca037343-11)
National Cancer Institute (CA-37343)
Topics: Health Sciences › Medicine › Clinical Neurology › Autoimmune Neurological Disorders and Treatments
Health Sciences › Medicine › Oncology › Lung Cancer Research Studies
Health Sciences › Medicine › Rheumatology › Systemic Lupus Erythematosus Research
Keywords: anti-Hu antibodies, small cell lung cancer, paraneoplastic syndrome, multiple primary neoplasms, gastrointestinal motility disorders, Lambert-Eaton myasthenic syndrome, limbic encephalitis, opsoclonus-myoclonus syndrome, cancer screening, tobacco use, achalasia, gastroparesis
MeSH terms: Adult, Aged, Autoantibodies, Cerebrospinal Fluid Proteins, Female, Gastrointestinal Diseases, Gastrointestinal Motility, Humans, Lung Neoplasms, Male, Middle Aged, Neoplasms, Nerve Tissue Proteins, Nervous System Diseases, Paraneoplastic Syndromes, Incidence, RNA-Binding Proteins, Carcinoma, Small Cell, ELAV Proteins

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Paraneoplastic and oncologic profiles of patients seropositive for type 1 antineuronal nuclear autoantibodies

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