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From suspected Creutzfeldt-Jakob disease to confirmed histoplasma meningitis
Vivek Batra1, Armen Khararjian2, Joseph Wheat
1Department of Community Physicians, Johns Hopkins, Columbia, Maryland, USA.
Abstract:
A 77-year-old man with chronic obstructive lung disease who was on steroids, presented to the hospital after a fall with subacute headaches and ataxia. During the patient's hospital course, his clinical condition deteriorated with myoclonic jerks, fevers and severe encephalopathy. An extensive workup, including EEG, brain MRI and lumbar puncture, revealed possible Creutzfeldt-Jakob disease. Unfortunately, the patient failed to improve and died 12 days after admission. A brain-only autopsy revealed he had acute histoplasma meningitis with patchy superficial cerebritis.
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.

