Analysis of clinical data of anti-dipeptidyl peptidase-like protein 6 encephalitis
Journal Title: Chinese Journal of Nervous and Mental Diseases - Year 2024, Vol 50, Issue 3
Abstract
[Objective] We analyzed clinical characteristics of dipeptidyl-peptidase-like protein 6 (DPPX) antibody-associated encephalitis and evaluated the effect of immunotherapy on anti-DPPX encephalitis. [Methods] A total of 9 patients with anti-DPPX encephalitis were retrospectively analyzed, including clinical manifestations, laboratory examinations, imaging features, treatment and prognosis. [Results] Nine patients were identified (medium age 45 years old, 5 were men). Neurologic disorders were multifocal: cognitive dysfunction (3 cases), psychosis (anxiety, irritability, auditory hallucination, abnormal behavior) (3 cases), CNS hyperexcitability (6 cases), brainstem (5 cases), cerebellar dysfunction (4 cases), sleep disorders (3 cases), dysautonomia (1 case), allodynia (1 case). In additional, there were 4 patients complicated with weight loss and/or gastrointestinal symptoms. CSF data showed that white cell counts were elevated in 2 patients and protein levels were elevated in 4 patients. EEG revealed epileptiform discharges in 3 patients and delta wave in 1 patient. Abnormalities were found in the brain MRIs of 4 patients including T2/FLAIR lesions in the hippocampus, insula. Seven patients received corticosteroids and six patients received plasma exchange. Seven patients subsequently treated with Mycophenolate Mofetil. Among 7 patients with adequate follow-up data, all patients had recovered to some degree and 3 of them completely remitted after 4 months to 2 years of follow-up. [Conclusion] The clinical symptoms of anti-DPPX encephalitis were diverse, the classic triad of clinical symptoms were weight loss and/or gastrointestinal disorders, CNS hyperexcitability and cognitive disorders. Long-term oral Mycophenolate Mofetil may be helpful in improving prognosis and reducing recurrence.
Authors and Affiliations
Genxia HE, Junchao. QIAN
Keywords
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- EP ID EP750445
- DOI 10.3969/j.issn.1002-0152.2024.03.004
- Views 44
- Downloads 0
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