A case of primary central nervous system lymphoma presenting diffuse infiltrative leukoencephalopathy

Tatsuya Yamamoto1, Kazuho Kojima, Katsura Koibuchi

  • 1Department of Neurology, Graduate School of Medicine, Chiba University, Japan. tatsuya-yamamoto@mbc.nifty.com

Insights

Primary central nervous system lymphoma (PCNSL) can present subtly without mass formation. Early steroid response and brain biopsy are crucial for diagnosing this rare brain cancer.

Area of Science:

  • Neurology
  • Oncology
  • Neuroimaging

Background:

  • Primary central nervous system lymphoma (PCNSL) is a rare extranodal non-Hodgkin lymphoma.
  • Diagnosis can be challenging, especially in immunocompetent patients without a discernible mass on imaging.
  • Subtle neurological symptoms may precede definitive diagnosis.

Observation:

  • A 58-year-old immunocompetent male presented with progressive appetite loss, cognitive decline, gait issues, and personality changes over four months.
  • Brain MRI showed diffuse leukoencephalopathy without mass formation.
  • Initial high-dose steroid treatment led to rapid symptom improvement, with relapse upon withdrawal.

Findings:

  • Brain biopsy confirmed the diagnosis of primary central nervous system lymphoma (PCNSL).
  • The patient achieved successful treatment with high-dose methotrexate therapy.
  • Steroid responsiveness, despite lack of mass on MRI, was a key indicator.

Implications:

  • This case highlights the importance of considering PCNSL in immunocompetent individuals with unexplained neurological decline and diffuse leukoencephalopathy.
  • Early recognition of steroid responsiveness can guide diagnostic efforts.
  • Brain biopsy remains the gold standard for definitive PCNSL diagnosis when imaging is equivocal.

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