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Iatrogenic Creutzfeldt-Jakob disease following human growth hormone therapy: case report

Luís Otávio Sales Ferreira Caboclo1, Nancy Huang, Guilherme Alves Lepski

  • 1Department of Neurology, Medical School, University of São Paulo, São Paulo, SP, Brazil.

Arquivos De Neuro-Psiquiatria
|July 20, 2002
PubMed

Insights

A rare case of iatrogenic Creutzfeldt-Jakob disease (CJD) in Brazil was linked to human growth hormone (hGH) treatment. This case highlights a long incubation period and unusual sleep disturbances, emphasizing the risks of cadaver-derived treatments.

Area of Science:

  • Neurology
  • Infectious Diseases
  • Prion Diseases

Background:

  • Iatrogenic Creutzfeldt-Jakob disease (CJD) can result from medical procedures, including treatments using human growth hormone (hGH) derived from pituitary glands.
  • This report details a second case of iatrogenic CJD linked to hGH therapy in Brazil.

Observation:

  • A 41-year-old man developed iatrogenic CJD after receiving cadaver-derived hGH.
  • The incubation period was exceptionally long, spanning 28 years from the midpoint of treatment to symptom onset.
  • The patient presented with cerebellar and cognitive decline, accompanied by excessive somnolence and altered sleep architecture, confirmed by polysomnography.

Findings:

  • Cerebrospinal fluid (CSF) analysis detected 14-3-3 protein, a marker for neuronal damage.
  • Magnetic resonance imaging (MRI), particularly diffusion-weighted imaging (DW-MRI), showed increased signal intensity in the striatum.
  • These findings are consistent with prion disease pathology.

Implications:

  • This case underscores the potential long-term risks associated with prion contamination from biological therapies.
  • It highlights the importance of vigilant surveillance for iatrogenic CJD, especially in patients with a history of treatments using human-derived biologicals.
  • The distinct clinical presentation, including sleep disturbances, may aid in earlier diagnosis of iatrogenic CJD.

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