[A child with choreic movement, generalized convulsion and severe neurological deterioration responded to

Yoichiro Nakae1, Mana Kurihara, Toshitaka Kohagizawa

  • 1Department of Pediatrics, Kanagawa Rehabilitation Hospital, Atsugi, Kanagawa. nakae@msj.biglobe.ne.jp

Insights

A pediatric autoimmune disorder caused severe neurological symptoms in a young girl. Prompt cyclophosphamide treatment led to significant recovery, suggesting an antiphospholipid syndrome-like mechanism.

Area of Science:

  • Pediatric Neurology
  • Autoimmunology
  • Rheumatology

Background:

  • Autoimmune disorders can present with severe neurological manifestations in children.
  • Antiphospholipid syndrome (APS) is characterized by autoantibodies and thrombotic events, but pediatric cases with neurological symptoms are less understood.

Observation:

  • A 3-year-old girl experienced acute hemiplegia, seizures, chorea, and rapid motor decline, becoming bedridden.
  • The patient exhibited a severe, progressive neurological deficit suggestive of a systemic autoimmune process.

Findings:

  • Intravenous cyclophosphamide pulse therapy induced a remarkable clinical improvement.
  • The child recovered significantly, with only mild dysarthria remaining by age six.
  • The clinical presentation and response to treatment suggested an autoimmune pathogenesis, potentially similar to antiphospholipid syndrome.

Implications:

  • Cyclophosphamide therapy may be effective in treating pediatric neurological autoimmune conditions with APS-like features.
  • Early diagnosis and aggressive immunosuppressive treatment can lead to substantial recovery in affected children.
  • Further research into pediatric autoimmune neurological disorders and their management is warranted.

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