[Autoimmune encephalitis due to mantle cell lymphoma]

Ned Tijdschr Geneeskd. 2016:160:D394.
[Article in Dutch]

Abstract

Background: Autoimmune encephalitis is a rare condition characterized by subacute development of cognitive and psychiatric symptoms. A paraneoplastic syndrome involves autoimmune encephalitis caused by classic antibodies. Although this condition is often associated with cancer, no malignancy has yet been found in 70-90% of patients at the time of diagnosis.

Case description: We saw a 58-year-old male patient with fatigue, diarrhoea and weight loss. He was also experiencing hyperekplexia, personality changes and an instable gait. PET-CT revealed generalised lymphadenopathy. Histopathological analysis of a lymph node showed mantle cell lymphoma. Further investigation of the fluid revealed anti-DPPX IgG antibodies. We treated the patient's mantle cell lymphoma with R-CHOP; he achieved complete remission and his neurological symptoms resolved almost completely.

Conclusion: The presence of anti-DPPX IgG antibodies is rare. Although it has not been proven that these antibodies are related to malignancies, this is the third of 30 known cases in which anti-DPPX IgG antibodies and a lymphatic malignancy were found.

Publication types

  • Case Reports

MeSH terms

  • Antibodies, Monoclonal, Murine-Derived
  • Antineoplastic Combined Chemotherapy Protocols
  • Autoantibodies / analysis*
  • Cyclophosphamide
  • Dipeptidyl-Peptidases and Tripeptidyl-Peptidases / analysis*
  • Doxorubicin
  • Encephalitis / diagnosis*
  • Encephalitis / drug therapy*
  • Hashimoto Disease / diagnosis*
  • Hashimoto Disease / drug therapy*
  • Humans
  • Lymph Nodes / chemistry
  • Lymphoma, Mantle-Cell / complications
  • Lymphoma, Mantle-Cell / diagnosis*
  • Lymphoma, Mantle-Cell / drug therapy
  • Male
  • Middle Aged
  • Nerve Tissue Proteins / analysis*
  • Potassium Channels / analysis*
  • Prednisone
  • Rituximab
  • Vincristine

Substances

  • Antibodies, Monoclonal, Murine-Derived
  • Autoantibodies
  • Nerve Tissue Proteins
  • Potassium Channels
  • R-CHOP protocol
  • Rituximab
  • Vincristine
  • Doxorubicin
  • Cyclophosphamide
  • DPP6 protein, human
  • Dipeptidyl-Peptidases and Tripeptidyl-Peptidases
  • Prednisone

Supplementary concepts

  • Hashimoto's encephalitis