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Primary catastrophic antiphospholipid syndrome in children with midbrain infarction: a case report

Qinghua Dong1, Jianyun Yin1, Hang Su1

  • 1The Second Clinical Medical College of Lanzhou University, Lanzhou University, Lanzhou, China.

Frontiers in Pediatrics
|April 23, 2024
PubMed

Insights

Catastrophic antiphospholipid syndrome (CAPS) is a rare autoimmune disease causing extensive thrombosis. Prompt diagnosis and treatment of pediatric CAPS with midbrain infarction are crucial for survival and recovery.

Area of Science:

  • Autoimmune diseases
  • Pediatric rheumatology
  • Vascular medicine

Background:

  • Catastrophic antiphospholipid syndrome (CAPS) is a severe, multi-system autoimmune disorder characterized by widespread thrombosis.
  • Pediatric CAPS is exceptionally rare, with midbrain infarction significantly increasing mortality risk.
  • Early diagnosis and prompt intervention are critical for managing CAPS, especially when complicated by neurological events.

Observation:

  • A 14-year-old girl presented with neurological and gastrointestinal symptoms, initially misdiagnosed as intracranial infection.
  • Diagnostic workup revealed primary CAPS with concurrent midbrain infarction.
  • The patient received intensive care including anticoagulation, glucocorticoids, IVIG, and plasma exchange.

Findings:

  • The patient's condition improved significantly within 27 days of standardized treatment.
  • This case highlights the clinical presentation of pediatric CAPS with midbrain infarction.
  • Successful management involved a multi-faceted therapeutic approach.

Implications:

  • This case underscores the importance of considering CAPS in pediatric patients with unexplained neurological symptoms and thrombosis.
  • Timely and aggressive treatment can lead to favorable outcomes even in severe pediatric CAPS cases.
  • Raising awareness of pediatric CAPS is vital for improving diagnostic accuracy and patient prognosis.
Abstract

Keywords:
brain stem infarctioncatastrophic antiphospholipid syndromechoreapediatricthrombosis

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