Related Experiment Video
Updated: Oct 2, 2026

Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
Published on: July 4, 2007
Progressive Multifocal Leukoencephalopathy Misdiagnosed as Neuropsychiatric Systemic Lupus Erythematosus With a
Hussein Algahtani1,2,3, Bader Shirah2, Leen Othman2,3
1King Abdulaziz Medical City.
Introduction:
Progressive multifocal leukoencephalopathy (PML) is a rare infection of the central nervous system due to the reactivation of the John Cunningham polyomavirus. It is commonly a progressive fatal disease with worldwide distribution.
Case Report:
We describe a rare case of PML, which was misdiagnosed as neuropsychiatric systemic lupus erythematosus (SLE) with a catastrophic outcome due to delay in diagnosis with superadded cyclophosphamide therapy.
Conclusion:
There are several lessons taught from our case. Firstly, in patients with autoimmune disorders who are strongly immunosuppressed, the new onset of cognitive impairment and seizures should alert the treating physician to look carefully for PML. Secondly, in cases of SLE where the diagnosis of the cause of cognitive impairment and seizures is not clear, we suggest that immunosuppression should not be intensified until PML has clearly been ruled out. Lastly, multidisciplinary care in patients with suspected neuropsychiatric SLE including a neurologist, an infectious diseases consultant, a neuroradiologist, and a rheumatologist is needed.
Insights
Progressive multifocal leukoencephalopathy (PML), a rare brain infection, can be mistaken for neuropsychiatric lupus. Early recognition of PML in immunosuppressed patients is crucial to prevent fatal outcomes.
Area of Science:
- Neuroimmunology
- Infectious Neurology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare, often fatal, central nervous system infection caused by John Cunningham polyomavirus reactivation.
- PML affects immunosuppressed individuals and has a global prevalence.
Observation:
- A rare case of PML was misdiagnosed as neuropsychiatric systemic lupus erythematosus (SLE).
- Delayed diagnosis and subsequent cyclophosphamide therapy contributed to a catastrophic outcome.
Findings:
- New-onset cognitive impairment and seizures in immunosuppressed patients with autoimmune disorders warrant investigation for PML.
- Intensifying immunosuppression in suspected neuropsychiatric SLE cases should be deferred until PML is ruled out.
Implications:
- Highlights the importance of considering PML in differential diagnoses for neurological changes in immunosuppressed patients.
- Emphasizes the need for multidisciplinary collaboration in managing suspected neuropsychiatric SLE to ensure accurate diagnosis and timely treatment.
Related Concept Videos
Rocky Mountain Spotted Fever
Encephalitis l: Introduction
Encephalitis ll: Pathophysiology
Multiple Sclerosis l: Introduction
Hepatic Encephalopathy

