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Background <p>Secondary causes of parkinsonism, such as tumors and autoimmune conditions, are rare. Cases of parkinsonism owing to antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis of the central nervous system (CNS) or lymphoma are scarce and often difficult to differentiate using neuroimaging. Herein, we present a rare and challenging case of secondary parkinsonism that showed subacute progression with ANCA positivity.</p> Case presentation <p>A 69-year-old healthy woman presented with subacute progressive parkinsonism. The patient showed proteinuria, hematuria, and positive proteinase 3-specific ANCA (46.0 U/mL; normal range &lt; 2 U/mL). Brain MRI revealed cerebral ischemic lesion and extensive white matter hyperintensities on T2-FLAIR indicative of CNS-ANCA vasculitis of the CNS. Although methylprednisolone pulse therapy improved her condition temporarily, it deteriorated 2 weeks later. Renal biopsy confirmed IgA nephropathy, and subsequent brain biopsy revealed diffuse large B-cell lymphoma (DLBCL). Despite the initiation of chemotherapy, her condition deteriorated further, and the patient died 1 month later.</p> Conclusion <p>We present a case of lymphoma with proteinuria, hematuria, positive ANCA, and brain MRI findings mimicking ANCA-associated vasculitis. DLBCL and IgA nephropathy can coexist, and differentiating between DLBCL and ANCA-associated vasculitis from MRI images can be challenging in some rare cases. This study highlights the importance of tissue diagnosis even when CNS vasculitis is suspected.</p>

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Subacute parkinsonism associated with proteinuria, hematuria, and PR3-ANCA positivity: a diagnostic challenge

  • 期刊论文

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  • 发表日期：

    2026年1月19日

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  • Akisato Nishigaki,
  • Hidehiro Ishikawa,
  • Asako Tamura,
  • Keita Matsuura,
  • Genshin Mouri,
  • Akihide Nakamura,
  • Kayo Tsujimoto,
  • Hiroyuki Kajikawa,
  • Yuichiro Ii,
  • Hiroshi Imai,
  • Masayuki Maeda,
  • Isao Tawara,
  • Akihiro Shindo

摘要

Background

Secondary causes of parkinsonism, such as tumors and autoimmune conditions, are rare. Cases of parkinsonism owing to antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis of the central nervous system (CNS) or lymphoma are scarce and often difficult to differentiate using neuroimaging. Herein, we present a rare and challenging case of secondary parkinsonism that showed subacute progression with ANCA positivity.

Case presentation

A 69-year-old healthy woman presented with subacute progressive parkinsonism. The patient showed proteinuria, hematuria, and positive proteinase 3-specific ANCA (46.0 U/mL; normal range < 2 U/mL). Brain MRI revealed cerebral ischemic lesion and extensive white matter hyperintensities on T2-FLAIR indicative of CNS-ANCA vasculitis of the CNS. Although methylprednisolone pulse therapy improved her condition temporarily, it deteriorated 2 weeks later. Renal biopsy confirmed IgA nephropathy, and subsequent brain biopsy revealed diffuse large B-cell lymphoma (DLBCL). Despite the initiation of chemotherapy, her condition deteriorated further, and the patient died 1 month later.

Conclusion

We present a case of lymphoma with proteinuria, hematuria, positive ANCA, and brain MRI findings mimicking ANCA-associated vasculitis. DLBCL and IgA nephropathy can coexist, and differentiating between DLBCL and ANCA-associated vasculitis from MRI images can be challenging in some rare cases. This study highlights the importance of tissue diagnosis even when CNS vasculitis is suspected.