Symptoms
The condition typically develops gradually over weeks to months, with patients experiencing:
- Severe diarrhoea and significant weight loss before neurological symptoms emerge
- Progressive cognitive decline
- Memory problems
- Sleep disorders
- Tremors and muscle spasms
- Balance problems and difficulty walking
- Psychiatric symptoms like agitation, anxiety, confusion or depression
- Seizures in some cases
The combination of gastrointestinal and neurological symptoms is particularly characteristic of DPPX encephalitis.
How is it diagnosed?
Diagnosis of DPPX encephalitis requires:
- Detection of DPPX antibodies in blood or cerebrospinal fluid (CSF)
- MRI brain imaging (though results may be normal)
- Lumbar puncture to analyse CSF
- EEG to assess brain activity patterns
- Screening for underlying cancers that may trigger the condition
Is it treatable?
Yes, DPPX encephalitis is treatable with immunotherapy. Treatment options include:
- High-dose corticosteroids
- Intravenous immunoglobulin (IVIG)
- Plasma exchange
- Rituximab
- Cyclophosphamide
Treatment often requires hospitalisation, especially in the acute phase of the illness. Some patients may need intensive care support.
What is the course of the illness?
The course varies between patients. Many patients respond well to immunotherapy, though recovery can be slow. Early treatment typically leads to better outcomes. Without treatment, the condition can be severe and potentially life-threatening. Relapses may occur, requiring ongoing or repeated treatment. It is important to follow up in the long-term to monitor for relapses.
Can it be prevented?
Currently there is no known way to prevent DPPX encephalitis. Research is ongoing to better understand the triggers of this condition and potential preventative strategies.
By Prav Prathapan, Encephalitis International (awaiting peer review)
FS079V1 DPPX encephalitis
Date created: December 2024 / Last updated: December 2024 / Review date: December 2027
Disclaimer: We try to ensure that the information is easy to understand, accurate and up to date as possible. If you would like more information on the source material and references the author used to write this document, please contact Encephalitis International. None of the authors of the above document has declared any conflict of interest, which may arise from being named as an author of this document.
