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Progressive multifocal leukoencephalopathy developing in advanced pulmonal sarcoidosis

Hans-Ullrich Völker1, Klaus Kraft, Eva Arnold

  • 1Institute of Pathology, Military Hospital Ulm, Germany. ullrich.voelker@mail.uni-wuerzburg.de

Clinical Neurology and Neurosurgery
|July 3, 2007
PubMed

Insights

This case report details a rare instance of progressive multifocal leukoencephalopathy (PML) in a patient with advanced pulmonary sarcoidosis. The study highlights diagnostic challenges in patients with sarcoidosis and neurological symptoms.

Area of Science:

  • Neurology
  • Pulmonology
  • Infectious Diseases

Background:

  • Pulmonary sarcoidosis is an inflammatory disease that can affect multiple organs.
  • Progressive multifocal leukoencephalopathy (PML) is a rare, demyelinating disease of the central nervous system caused by the JC virus.
  • The co-occurrence of pulmonary sarcoidosis and PML is exceptionally rare, posing significant diagnostic challenges.

Observation:

  • A 49-year-old male with advanced pulmonary sarcoidosis presented with infratentorial neurological impairment.
  • Initial symptoms were misattributed to neurosarcoidosis due to the patient's known sarcoidosis.
  • Magnetic resonance tomography (MRI) revealed cerebellar demyelination, inconsistent with neurosarcoidosis, leading to consideration of acute disseminated encephalomyelitis (ADEM).

Findings:

  • Cerebrospinal fluid (CSF) and brain biopsy samples tested positive for JC virus DNA via PCR.
  • The diagnosis of PML was confirmed, despite initial diagnostic uncertainty.
  • Treatment with corticosteroids for suspected ADEM and later cidofovir for PML did not halt disease progression.

Implications:

  • This case underscores the critical need for a broad differential diagnosis in sarcoidosis patients with neurological symptoms.
  • Early and accurate diagnosis of PML is crucial, even in the presence of other confounding conditions like sarcoidosis.
  • The report highlights the diagnostic difficulties and the limited treatment options for PML, especially when co-occurring with other severe diseases.

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