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Paraneoplastic and autoimmune encephalitis

Josep Dalmau, MD, PhD
Myrna R Rosenfeld, MD, PhD
Section Editor
Lisa M DeAngelis, MD, FAAN, FANA
Deputy Editor
April F Eichler, MD, MPH


Encephalitis is an inflammatory condition of the brain with many etiologies. There are several types of encephalitis that are immune-mediated, including the classic paraneoplastic encephalitis syndromes and the encephalitis syndromes associated with antibodies against neuronal cell surface/synaptic proteins. The latter are often referred to as autoimmune encephalitis.

While the paraneoplastic encephalitis syndromes are invariably cancer-related, the autoimmune encephalitis syndromes may occur in the presence or absence of cancer. Thus, nomenclature can be confusing, as the paraneoplastic encephalitis syndromes are autoimmune, and autoimmune encephalitis may be paraneoplastic. In this topic, the term autoimmune encephalitis refers specifically to those syndromes that are associated with antibodies to neuronal cell surface/synaptic proteins.  

An overview of paraneoplastic syndromes and other paraneoplastic disorders are discussed separately. (See "Overview of paraneoplastic syndromes of the nervous system" and "Paraneoplastic syndromes affecting peripheral nerve and muscle" and "Paraneoplastic syndromes affecting the spinal cord and dorsal root ganglia" and "Paraneoplastic cerebellar degeneration" and "Opsoclonus myoclonus syndrome".)


Clinical features — Paraneoplastic encephalitis may manifest as limbic or brainstem encephalitis or be part of widespread involvement of the neuraxis, as in paraneoplastic encephalomyelitis. In the majority of cases, symptoms have an acute to subacute onset, and evaluation of the cerebrospinal fluid (CSF) often shows abnormalities such as pleocytosis, increased protein concentration, oligoclonal bands, and elevated immunoglobulin G (IgG) index, suggesting an inflammatory process. In more than half of patients, the neurologic syndrome develops before the cancer diagnosis is known.

Limbic encephalitis — Limbic encephalitis refers to an inflammatory process localized to structures of the limbic system (eg, hippocampus, amygdala, hypothalamus, cingulate gyrus, limbic cortex), although the pathologic, clinical and radiological findings are often not confined to these areas. Limbic encephalitis is considered a classical paraneoplastic syndrome, defined as a disorder that is commonly due to a paraneoplastic process. However, as noted below, limbic encephalitis is the main manifestation of several of the autoimmune encephalitis syndromes that may occur without a cancer association. (See 'Autoimmune encephalitis' below.)    

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Literature review current through: Nov 2017. | This topic last updated: Jul 26, 2017.
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