What is paraneoplastic limbic encephalitis?
Paraneoplastic limbic encephalitis (PLE) is a rare neurological syndrome associated with cancer, and selectively affects limbic system structures, including the hippocampus, hypothalamus, and amygdala. Patients often present with cognitive impairment, personality change, short-term memory loss, and seizures1.
How is paraneoplastic limbic encephalitis diagnosed?
Any patient who is suspected to have paraneoplastic limbic encephalitis should undergo the following tests: Blood test for monoclonal antibodies (anti-Hu, anti-Ta, anti-Ma, anti-GABA B receptor, and anti NMDA receptor) but the absence of this antibodies does not exclude the disease.
What causes paraneoplastic encephalitis?
Paraneoplastic syndromes are not caused by cancer cells directly disrupting nerve function, by the cancer spreading (metastasis), or by other complications such as infections or treatment side effects. Instead, they occur alongside the cancer as a result of the activation of your immune system.
Does autoimmune encephalitis show up on MRI?
In patients with anti-NMDAR encephalitis the brain MRI is normal in approximately 60% of the patients and shows nonspecific findings in the rest including, cortical-subcortical FLAIR changes in brain or posterior fossa, transient meningeal enhancement, or areas of demyelination.
What causes limbic encephalitis?
Cause. Limbic encephalitis is thought to be caused by a reaction of the immune system to various stimuli such as cancers , tumors , infections, and generalized autoimmune disorders. In many patients, limbic encephalitis is associated with a tumor or cancer. This is known as paraneoplastic limbic encephalitis (PLE).
Is limbic encephalitis genetic?
Study shows non-heritable but consistent genetic predisposition in some cases of AE – Autoimmune Encephalitis Alliance.
Paraneoplastic limbic encephalitis occurs due to involvement of the limbic system secondary to an autoimmune response to neurons of the brain provoked by the antibodies. Patients, thus, present with seizures, changes in mood, memory, and personality. Case Report:
What are the treatment options for limbic encephalitis?
These findings along with the serological confirmation of anti-HU-antibodies are consistent with the diagnosis of limbic encephalitis. Treatment consists of treating the underlying cancer with surgery, radiotherapy, hormonal medications or chemotherapy that removes the origin of the antibodies and may alleviate the symptoms.
What are paraneoplastic neurological disorders (PND)?
Paraneoplastic neurological disorders (PND) are defined as remote effects on the nervous system that are not caused directly by the tumor, its metastases, or metabolic disruptions. This syndrome occurs in less than 1 per 10,000 patients diagnosed with a malignancy.