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Microscopic polyangiitis presenting with hemorrhagic stroke

Estíbaliz Iglesias1, Despina Eleftheriou2, Kshitij Mankad3

  • 1Unit of Pediatric Rheumatology, Department of Pediatrics, Hospital Sant Joan de Déu, Barcelona, Spain eiglesias@hsjdbcn.org.

Journal of Child Neurology
|May 22, 2013
PubMed

Insights

Microscopic polyangiitis rarely impacts the central nervous system. A 12-year-old girl with myeloperoxidase-associated microscopic polyangiitis experienced only brain hematomas, recovering fully with immunosuppression.

Area of Science:

  • Neurology
  • Rheumatology
  • Pediatrics

Background:

  • Microscopic polyangiitis (MPA) is a rare systemic vasculitis.
  • Antineutrophil cytoplasmic antibodies (ANCA) against myeloperoxidase (MPO) are associated with MPA.
  • Central nervous system (CNS) involvement in MPO-ANCA-positive MPA is uncommon, especially without other organ damage.

Observation:

  • A 12-year-old female presented with neurological symptoms.
  • The primary clinical manifestation was multiple acute parieto-occipital hematomas.
  • No other systemic organ involvement was initially detected.

Findings:

  • The patient's presentation was attributed to presumed microscopic polyangiitis.
  • The hematomas were the sole indicator of MPA in this case.
  • Diagnosis was supported by ANCA testing, though not explicitly stated in the abstract.

Implications:

  • This case highlights that CNS manifestations, such as intracranial hemorrhage, can be the sole presentation of MPA.
  • Early immunosuppressive therapy can lead to favorable outcomes in pediatric MPA with CNS involvement.
  • Highlights the importance of considering vasculitis in pediatric patients with unexplained neurological events.
Keywords:
hemorrhagic strokemicroscopic polyangiitismyeloperoxidase antineutrophil cytoplasmic antibodies

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