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Dipeptidyl-peptidase-like protein 6 encephalitis treated with immunotherapy
Lan Ye1, Margret Schnegelsberg1, Mark Obermann1
1Department of Neurology, Asklepios Hospitals Schildautal, Seesen, Germany.
Abstract:
This case describes a middle-aged man with anti-dipeptidyl-peptidase-like protein 6 (DPPX) encephalitis who exhibited the triad of memory loss, diarrhea, and tremor. The progression of his disease resembled neurodegenerative disease, and his first presentation at our department was 2 years after the first onset of symptoms. Antibodies against DPPX were positive in both serum and cerebrospinal fluid. No related tumor was found. The patient was initially treated with corticosteroid therapy and plasmapheresis. Despite moderate response to this treatment, corticosteroids were ceased because of adverse effects such as Cushing syndrome, deep vein thrombosis, and osteoporosis. After five cycles of treatment with rituximab, the patient experienced no further progression of neurologic symptoms and no adverse effects. The case adds to the understanding of the diagnosis, treatment, and potential prognosis of anti-DPPX encephalitis.
Insights
This case study details anti-dipeptidyl-peptidase-like protein 6 (DPPX) encephalitis, a rare condition presenting with memory loss, diarrhea, and tremor. Rituximab treatment halted disease progression without adverse effects.
Area of Science:
- Neuroimmunology
- Neurology
Background:
- Anti-dipeptidyl-peptidase-like protein 6 (DPPX) encephalitis is a rare autoimmune disorder.
- DPPX antibodies are implicated in autoimmune encephalitis, often mimicking neurodegenerative conditions.
Observation:
- A middle-aged male patient presented with a 2-year history of memory loss, diarrhea, and tremor.
- DPPX antibodies were detected in serum and cerebrospinal fluid; no associated tumor was identified.
Findings:
- Initial treatment with corticosteroids and plasmapheresis yielded a moderate response but led to significant adverse effects.
- Five cycles of rituximab therapy resulted in stabilization of neurological symptoms and no further adverse events.
Implications:
- This case highlights rituximab as a potentially effective treatment for anti-DPPX encephalitis.
- Early diagnosis and alternative treatment strategies are crucial for managing this condition and improving patient prognosis.
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