Anti-Ma2-associated limbic encephalitis with coexisting chronic inflammatory demyelinating polyneuropathy in a patient with non-Hodgkin lymphoma: A case report

Medicine (Baltimore). 2017 Oct;96(40):e8228. doi: 10.1097/MD.0000000000008228.

Abstract

Rationale: We report the rare case of a 74-year-old man with anti-Ma2-associated paraneoplastic neurologic syndrome (PNS), and review and analyze the clinical manifestations, diagnosis, and treatment of the disease.

Patient concerns: The patient presented with a 5-month history of muscle weakness, progressive body aches, and weakness and numbness in both lower extremities. Before his hospitalization, he had experienced cognitive function decline; ptosis, inward gaze, and vertical gaze palsy in the right eye; and occasional visual hallucinations. Brain and spinal cord magnetic resonance imaging (MRI) yielded normal results. Anti-Ma2 antibodies were detected in both serum and cerebrospinal fluid. A 4-hour electroencephalogram showed irregular sharp slow waves and δ waves in the temporal region. Electromyography showed peripheral nerve demyelination. Positron-emission tomography/computed tomography (PET-CT) examination revealed hypermetabolism in the lymph nodes of the whole body. Biopsy of the lymph nodes showed non-Hodgkin lymphoma.

Diagnosis: A clinical diagnosis of lymphoma and PNS was made.

Interventions: The patient was treated with intravenous dexamethasone (15 mg/day) for 3 days.

Lessons: We have presented a rare case of a PNS involving both the central and peripheral nervous systems. The clinical features of this case indicated anti-Ma2-associated encephalitis and chronic inflammatory demyelinating polyneuropathy. PET-CT played a critical role in enabling early diagnosis and prompt treatment in this case.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Antibodies / blood
  • Antibodies / cerebrospinal fluid
  • Antigens, Neoplasm / immunology
  • Humans
  • Limbic Encephalitis / complications
  • Limbic Encephalitis / immunology*
  • Lymphoma, Non-Hodgkin / complications*
  • Lymphoma, Non-Hodgkin / immunology
  • Male
  • Nerve Tissue Proteins / immunology
  • Paraneoplastic Syndromes, Nervous System / complications*
  • Paraneoplastic Syndromes, Nervous System / immunology
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / complications
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / immunology*

Substances

  • Antibodies
  • Antigens, Neoplasm
  • Ma2 antigen
  • Nerve Tissue Proteins