Anti-dipeptidyl-peptidase-like protein-6 antibody-associated encephalitis masquerading as weight loss and confusion: a case report
Abstract Background Anti-Dipeptidyl-peptidase-like protein-6 (DPPX) antibody-associated encephalitis is a rare autoimmune condition that affects the central and enteric nervous systems. It often presents with neuropsychiatric symptoms, cerebellar ataxia, and gastrointestinal dysmotility, though clin...
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BMC
2025-07-01
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| Series: | BMC Neurology |
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| Online Access: | https://doi.org/10.1186/s12883-025-04337-8 |
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| author | Victoria Sefcsik Paulette Rodriguez Monica Makar Magnus Chun Rayeheh Bahar Supriya Kairamkonda Mutsumi Kioka |
| author_facet | Victoria Sefcsik Paulette Rodriguez Monica Makar Magnus Chun Rayeheh Bahar Supriya Kairamkonda Mutsumi Kioka |
| author_sort | Victoria Sefcsik |
| collection | DOAJ |
| description | Abstract Background Anti-Dipeptidyl-peptidase-like protein-6 (DPPX) antibody-associated encephalitis is a rare autoimmune condition that affects the central and enteric nervous systems. It often presents with neuropsychiatric symptoms, cerebellar ataxia, and gastrointestinal dysmotility, though clinical manifestations can vary widely. Case presentation We report the case of a 38-year-old woman with psychiatric symptoms who developed severe cognitive impairment, seizures, and a profound weight loss of 100 pounds over one year. Her symptoms initially led to multiple psychiatric diagnoses and treatments, delaying recognition of the underlying autoimmune process. Ultimately, cerebrospinal fluid analysis revealed high-titer anti-DPPX antibodies (1:1000). Despite aggressive immunotherapy—including corticosteroids, plasmapheresis, IVIG, and rituximab—the patient’s condition deteriorated, and she died under hospice care. Discussion This case expands the known clinical spectrum of anti-DPPX encephalitis by documenting the most extreme reported case of weight loss associated with the disease. The prodromal presentation with isolated weight loss underscores the diagnostic challenges when neuropsychiatric symptoms emerge late. Our case highlights the importance of maintaining clinical suspicion for autoimmune encephalitis in patients with unexplained weight loss and neurocognitive decline. Conclusion Profound, unexplained weight loss may be an early and under-recognized manifestation of anti-DPPX encephalitis. Early recognition and immunotherapy are critical, but the potential for rapid progression and relapse demands heightened clinical vigilance and further research into optimal treatment strategies. |
| format | Article |
| id | doaj-art-7e397b58c6324fabb77a830f053e7170 |
| institution | Kabale University |
| issn | 1471-2377 |
| language | English |
| publishDate | 2025-07-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Neurology |
| spelling | doaj-art-7e397b58c6324fabb77a830f053e71702025-08-20T03:45:51ZengBMCBMC Neurology1471-23772025-07-012511810.1186/s12883-025-04337-8Anti-dipeptidyl-peptidase-like protein-6 antibody-associated encephalitis masquerading as weight loss and confusion: a case reportVictoria Sefcsik0Paulette Rodriguez1Monica Makar2Magnus Chun3Rayeheh Bahar4Supriya Kairamkonda5Mutsumi Kioka6Family Medicine Residency, 99th Medical Group, Nellis Air Force BaseKirk Kerkorian School of Medicine, University of NevadaSpring Valley Hospital Medical CenterKirk Kerkorian School of Medicine, University of NevadaSpring Valley Hospital Medical CenterDivision of Neurology, Kirk Kerkorian School of Medicine, University of NevadaDivision of Pulmonary and Critical Care Medicine, Kirk Kerkorian School of Medicine, University of NevadaAbstract Background Anti-Dipeptidyl-peptidase-like protein-6 (DPPX) antibody-associated encephalitis is a rare autoimmune condition that affects the central and enteric nervous systems. It often presents with neuropsychiatric symptoms, cerebellar ataxia, and gastrointestinal dysmotility, though clinical manifestations can vary widely. Case presentation We report the case of a 38-year-old woman with psychiatric symptoms who developed severe cognitive impairment, seizures, and a profound weight loss of 100 pounds over one year. Her symptoms initially led to multiple psychiatric diagnoses and treatments, delaying recognition of the underlying autoimmune process. Ultimately, cerebrospinal fluid analysis revealed high-titer anti-DPPX antibodies (1:1000). Despite aggressive immunotherapy—including corticosteroids, plasmapheresis, IVIG, and rituximab—the patient’s condition deteriorated, and she died under hospice care. Discussion This case expands the known clinical spectrum of anti-DPPX encephalitis by documenting the most extreme reported case of weight loss associated with the disease. The prodromal presentation with isolated weight loss underscores the diagnostic challenges when neuropsychiatric symptoms emerge late. Our case highlights the importance of maintaining clinical suspicion for autoimmune encephalitis in patients with unexplained weight loss and neurocognitive decline. Conclusion Profound, unexplained weight loss may be an early and under-recognized manifestation of anti-DPPX encephalitis. Early recognition and immunotherapy are critical, but the potential for rapid progression and relapse demands heightened clinical vigilance and further research into optimal treatment strategies.https://doi.org/10.1186/s12883-025-04337-8Anti-DPPXEncephalitisLumbar punctureHallucinationsUnintentional weight loss |
| spellingShingle | Victoria Sefcsik Paulette Rodriguez Monica Makar Magnus Chun Rayeheh Bahar Supriya Kairamkonda Mutsumi Kioka Anti-dipeptidyl-peptidase-like protein-6 antibody-associated encephalitis masquerading as weight loss and confusion: a case report BMC Neurology Anti-DPPX Encephalitis Lumbar puncture Hallucinations Unintentional weight loss |
| title | Anti-dipeptidyl-peptidase-like protein-6 antibody-associated encephalitis masquerading as weight loss and confusion: a case report |
| title_full | Anti-dipeptidyl-peptidase-like protein-6 antibody-associated encephalitis masquerading as weight loss and confusion: a case report |
| title_fullStr | Anti-dipeptidyl-peptidase-like protein-6 antibody-associated encephalitis masquerading as weight loss and confusion: a case report |
| title_full_unstemmed | Anti-dipeptidyl-peptidase-like protein-6 antibody-associated encephalitis masquerading as weight loss and confusion: a case report |
| title_short | Anti-dipeptidyl-peptidase-like protein-6 antibody-associated encephalitis masquerading as weight loss and confusion: a case report |
| title_sort | anti dipeptidyl peptidase like protein 6 antibody associated encephalitis masquerading as weight loss and confusion a case report |
| topic | Anti-DPPX Encephalitis Lumbar puncture Hallucinations Unintentional weight loss |
| url | https://doi.org/10.1186/s12883-025-04337-8 |
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