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[A case of hypereosinophilic syndrome associated with paraplegia]

Chie Endo1, Susumu Miyake

  • 1Department of Pediatrics, Kagawa Prefectural Central Hospital, Takamatsu, Kagawa. c-endo@mail.central-hp.pref.kagawa.jp

No to Hattatsu = Brain and Development
|September 19, 2003
PubMed

Insights

Idiopathic hypereosinophilic syndrome in a child can cause severe neurological issues. Prompt treatment with corticosteroids effectively resolved symptoms and normalized eosinophil counts.

Area of Science:

  • Pediatric Hematology
  • Pediatric Neurology
  • Immunology

Background:

  • Idiopathic hypereosinophilic syndrome (HES) is a rare disorder characterized by persistent, marked eosinophilia.
  • Neurological manifestations in HES can be severe and debilitating, though less common in pediatric cases.

Observation:

  • A 3-year-old girl presented with fever, cough, significant eosinophilia (16,500/microliter), and markedly elevated serum IgE (114,685 u/ml).
  • She developed paraplegia, dysuria, and dyschezia despite normal cerebrospinal fluid, chest imaging, and nerve conduction studies.
  • Flaum's hematologic score was 4.

Findings:

  • Treatment with prednisolone led to rapid remission of neurological symptoms and normalization of eosinophil counts.
  • Eosinophilia recurred upon medication tapering, but neurological signs did not reappear.
  • Glucocorticoid therapy was successfully discontinued after 21 months.

Implications:

  • This case highlights the potential for severe neurological complications in pediatric idiopathic hypereosinophilic syndrome.
  • Early corticosteroid intervention can effectively manage both hematologic and neurologic aspects of HES in children.
  • Long-term monitoring is crucial for managing recurrent eosinophilia and preventing neurological relapse.

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