Progressive multifocal leukoencephalopathy as first manifestation of sarcoidosis

Sylvie De Raedt1, Patrick Lacor, Alex Michotte

  • 1Department of Neurology UZ Brussel, Brussels, Belgium. Sylvie.deraedt@uzbrussel.be

Insights

Progressive multifocal leukoencephalopathy (PML), a brain disorder, was the first sign of sarcoidosis in a healthy man. Treatment with cidofovir stabilized his condition, a rare occurrence in such cases.

Area of Science:

  • Neuroimmunology
  • Viral Neurology

Background:

  • Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease.
  • PML is typically seen in immunocompromised individuals.
  • JC virus is the causative agent of PML.

Observation:

  • A previously healthy male presented with PML as the initial symptom of sarcoidosis.
  • No prior immunosuppressive treatment was noted in the patient.
  • This presentation is uncommon, with few documented cases.

Findings:

  • The patient received cidofovir treatment for PML.
  • Neurological and radiological stabilization was achieved post-treatment.
  • This is the first reported case of PML and sarcoidosis treated with cidofovir.

Implications:

  • Highlights a rare association between PML and sarcoidosis.
  • Suggests potential therapeutic role for cidofovir in this specific PML-sarcoidosis context.
  • Underscores the importance of considering sarcoidosis in unexplained PML cases.

Related Concept Videos

Multiple Sclerosis l: Introduction01:19

Multiple Sclerosis l: Introduction

Multiple sclerosis is a chronic autoimmune disease of the central nervous system (CNS) that affects the brain, spinal cord, and optic nerves. It is an inflammatory demyelinating disorder and a leading cause of neurological disability in young adults.EpidemiologyMS commonly begins between 20 and 40 years of age and is twice as common in women. Its exact cause remains unclear, but genetic susceptibility contributes, with higher risk in first-degree relatives and identical twins. A greater...
Encephalitis l: Introduction01:19

Encephalitis l: Introduction

Encephalitis is inflammation of the brain parenchyma, most often due to infections or autoimmune processes. It presents with neuropsychiatric features such as fever, altered mental status, behavioral changes, cognitive dysfunction, seizures, focal deficits, and sometimes autonomic instability. In some cases, the meninges are also involved, resulting in meningoencephalitis.Infectious CausesInfectious encephalitis is most commonly viral but can also result from bacterial, fungal, or parasitic...
Encephalitis ll: Pathophysiology01:26

Encephalitis ll: Pathophysiology

Encephalitis is inflammation of the brain parenchyma caused by direct viral invasion or immune-mediated mechanisms triggered by infections or tumors. Both processes lead to neuronal injury, disrupted neurotransmission, and diverse neurological symptoms, often with overlapping clinical and pathological features.Autoimmune EncephalitisIn autoimmune encephalitis, antibodies target neuronal antigens on cell surfaces, synapses, or within neurons. A key example is anti-NMDAR encephalitis, which can...
Rocky Mountain Spotted Fever01:26

Rocky Mountain Spotted Fever

Rocky Mountain Spotted Fever (RMSF) is a severe tick-borne illness caused by Rickettsia rickettsii, a Gram-negative, coccobacillary bacterium. This pathogen is an obligate intracellular parasite, requiring a host cell for replication. Transmission occurs through the bite of an infected tick. In the United States, the most important vectors are Dermacentor variabilis (American dog tick) and Dermacentor andersoni (Rocky Mountain wood tick), though other tick species may also serve as vectors.
Cryptococcal Meningitis01:27

Cryptococcal Meningitis

Cryptococcal meningitis is a life-threatening opportunistic infection predominantly associated with HIV/AIDS, accounting for over 100,000 deaths annually worldwide. However, it also affects individuals with other forms of immunosuppression, including those undergoing immunosuppressive therapy, organ transplant recipients, patients with innate immunodeficiencies, and individuals with hematological disorders. The infection is caused mainly by Cryptococcus neoformans and Cryptococcus gattii,...
Rabies01:28

Rabies

Rabies is a lethal zoonotic disease caused by a single-stranded, negative-sense RNA virus of the Lyssavirus genus, within the family Rhabdoviridae. Its primary mode of transmission to humans is through bites or saliva-contaminated scratches from infected mammals such as dogs, bats, raccoons, or foxes. Transmission can also occur if infectious saliva contacts abraded skin or intact mucous membranes, including the conjunctiva.Viral Entry and Early ReplicationOnce introduced at the bite or scratch...