From suspected Creutzfeldt-Jakob disease to confirmed histoplasma meningitis

Vivek Batra1, Armen Khararjian2, Joseph Wheat

  • 1Department of Community Physicians, Johns Hopkins, Columbia, Maryland, USA.

BMJ Case Reports
|July 9, 2016
PubMed

Insights

A man with chronic obstructive lung disease developed severe neurological symptoms, initially suspected as Creutzfeldt-Jakob disease. Autopsy revealed acute histoplasma meningitis with cerebritis as the cause of death.

Area of Science:

  • Neurology
  • Infectious Diseases
  • Pathology

Background:

  • A 77-year-old male patient with chronic obstructive lung disease (COPD) on steroid therapy presented with subacute headaches and ataxia following a fall.
  • The patient's condition rapidly worsened, exhibiting myoclonic jerks, fever, and severe encephalopathy.

Observation:

  • Diagnostic workup included electroencephalogram (EEG), brain magnetic resonance imaging (MRI), and lumbar puncture, suggesting Creutzfeldt-Jakob disease (CJD).
  • Despite extensive investigations, the patient's neurological status deteriorated, leading to death 12 days after admission.

Findings:

  • A post-mortem brain examination unexpectedly revealed acute histoplasma meningitis with patchy superficial cerebritis.
  • The findings indicate that a fungal infection, not a prion disease, was the underlying cause of the patient's fatal neurological decline.

Implications:

  • This case highlights the importance of considering opportunistic infections, such as fungal meningitis, in immunocompromised patients presenting with severe neurological symptoms.
  • It underscores the diagnostic challenges in differentiating infectious encephalopathies from neurodegenerative diseases like CJD, especially in steroid-treated individuals.
  • The study emphasizes the critical role of autopsy in establishing definitive diagnoses and understanding the etiology of complex neurological conditions.

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