Syndrome and outcome of antibody-negative limbic encephalitis
Language English Country Great Britain, England Media print-electronic
Document type Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't
Grant support
R01 NS077851
NINDS NIH HHS - United States
PubMed
29667271
PubMed Central
PMC6037545
DOI
10.1111/ene.13661
Knihovny.cz E-resources
- Keywords
- antibodies, autoimmune, limbic encephalitis, paraneoplastic,
- MeSH
- Autoantigens immunology MeSH
- Autoantibodies analysis MeSH
- Adult MeSH
- Immunohistochemistry MeSH
- Immunotherapy MeSH
- Memory, Short-Term MeSH
- Rats MeSH
- Cells, Cultured MeSH
- Leukocytosis MeSH
- Leukocytes immunology MeSH
- Middle Aged MeSH
- Humans MeSH
- Limbic Encephalitis immunology psychology therapy MeSH
- Magnetic Resonance Imaging MeSH
- Neoplasms complications MeSH
- Neurons immunology MeSH
- Memory Disorders etiology psychology MeSH
- Aged MeSH
- Treatment Outcome MeSH
- Animals MeSH
- Check Tag
- Adult MeSH
- Rats MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Animals MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
- Research Support, N.I.H., Extramural MeSH
- Names of Substances
- Autoantigens MeSH
- Autoantibodies MeSH
BACKGROUND AND PURPOSE: The aim was to report the clinical characteristics of 12 patients with limbic encephalitis (LE) who were antibody-negative after a comprehensive immunological study. METHODS: The clinical records of 163 patients with LE were reviewed. Immunohistochemistry on rat brain, cultured neurons and cell-based assays were used to identify neuronal autoantibodies. Patients were included if (i) there was adequate clinical, cerebrospinal fluid (CSF) and magnetic resonance imaging information to classify the syndrome as LE, (ii) magnetic resonance images were accessible for central review and (iii) serum and CSF were available and were confirmed negative for neuronal antibodies. RESULTS: Twelve (7%) of 163 LE patients [median age 62 years; range 40-79; 9 (75%) male] without neuronal autoantibodies were identified. The most frequent initial complaints were deficits in short-term memory leading to hospital admission in a few weeks (median time 2 weeks; range 0.5-12). In four patients the short-term memory dysfunction remained as an isolated symptom during the entire course of the disease. Seizures, drowsiness and psychiatric problems were unusual. Four patients had solid tumors (one lung, one esophagus, two metastatic cervical adenopathies of unknown primary tumor) and one chronic lymphocytic leukemia. CSF showed pleocytosis in seven (58%) with a median of 13 white blood cells/mm3 (range 9-25). Immunotherapy included corticosteroids, intravenous immunoglobulins and combinations of both drugs or with rituximab. Clinical improvement occurred in six (54%) of 11 assessable patients. CONCLUSIONS: Despite the discovery of new antibodies, 7% of LE patients remain seronegative. Antibody-negative LE is more frequent in older males and usually develops with predominant or isolated short-term memory loss. Despite the absence of antibodies, patients may have an underlying cancer and respond to immunotherapy.
Department of Neurology Masaryk University and University Hospital Brno Czech Republic
Department of Neurology University of Pennsylvania Philadelphia PA USA
Department of Radiology Hospital Clinic Barcelona Spain
Institució Catalana de Recerca i Estudis Avançats Barcelona Spain
Service of Neurology Hospital 12 de Octubre Madrid Spain
Service of Neurology Hospital Clinic Barcelona Spain
Service of Neurology Hospital Nuestra Señora del Rosario Madrid Spain
Service of Neurology Hospital Son Espases Palma de Mallorca Spain
Service of Neurology Hospital Son Llatzer Palma de Mallorca Spain
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Antibody-Negative Autoimmune Encephalitis: A Single-Center Retrospective Analysis
