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Prompt diagnosis and treatment of autoimmune encephalitis (AE) hinges on clinicians’ familiarity with the condition. While understanding of AE has grown in the last several years, its rarity — with a prevalence of approximately 1 per 100,000 people in U.S. population-based studies — can complicate diagnosis, as can the number of conditions that mimic AE.
“When we are evaluating patients acutely, we need to be very careful and judicious in the differential diagnosis and not overcall the diagnoses,” says Amy Kunchok, MD, a neurologist in Cleveland Clinic’s Mellen Center for Multiple Sclerosis Treatment and Research who has a specialty interest in AE. Tumors, mitochondrial disorders, neurodegenerative diseases, primary psychiatric disorders and other conditions can mimic AE.
In the latest episode of Cleveland Clinic’s Neuro Pathways podcast, Dr. Kunchok provides a practical approach to the diagnosis and management of AE. She explores:
- Clinical features that should prompt examination for AE
- Components of a comprehensive evaluation for suspected AE, including neural antibody testing
- Initial treatment for patients with suspected AE
- Assessment of treatment effectiveness
- Management of patients with clinical relapse
- New developments and ongoing research in AE
Click the podcast player above to listen to the 19-minute episode now, or read on for a short edited excerpt. Check out more Neuro Pathways episodes at clevelandclinic.org/neuropodcast or wherever you get your podcasts.
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Excerpt from the podcast
Podcast host Glen Stevens, DO, PhD: What does a comprehensive evaluation for suspected autoimmune encephalitis look like?
Dr. Kunchok: We would typically do a spinal fluid. In addition to doing routine analyses, we send it for neural antibody testing to look for antibody biomarkers for autoimmune encephalitis. We also send the spinal fluid for differential diagnoses, such as infective encephalitis. Other markers in the spinal fluid that are helpful are things like oligoclonal bands or IgG index, which can also point to intrathecal immune activation.
We send the serum for neural antibodies, and we check the serum for complete blood count, a metabolic panel and any other screening that may be relevant for an alternative diagnosis of encephalopathies. In some cases, that may mean toxicology testing, B12, folate, thyroid function tests, etc.
Other tests that we routinely do include an EEG if a patient has new-onset seizures. This can also be helpful to identify encephalopathy. And an MRI of the brain can be useful to look for the classical features of limbic encephalitis as well as other inflammatory changes.