Syndrome and outcome of antibody‐negative limbic encephalitis
2018
Background and purposeThe aim was to report the clinical
characteristics of 12 patients with limbic encephalitis (LE)
who were antibody-negative after a comprehensive immunological
study. MethodsThe 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. ResultsTwelve (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/mm(3) (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. ConclusionsDespite 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. Click for the corresponding questions to this
CME article.
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