[Progressive multifocal leukoencephalitis complicating polymyositis]

Mohamed Elloumi1, Nada Ayadi, Chokri Mhiri

  • 1Service de rhumatologie, Hôpital universitaire Hédi Chaker, Sfax, Tunisie. elloumu02@yahoo.fr

Presse Medicale (Paris, France : 1983)
|March 1, 2003
PubMed
Abstract

Insights

Progressive multifocal leukoencephalitis (PML) is a serious brain infection that can occur in patients with weakened immune systems, often due to treatments for systemic diseases. Early recognition is crucial for managing this neurological condition.

Area of Science:

  • Neurology
  • Immunology
  • Infectious Diseases

Background:

  • Progressive multifocal leukoencephalitis (PML) is a demyelinating disease of the central nervous system.
  • PML must be considered in patients with systemic diseases experiencing rapid neurological decline.

Observation:

  • A 17-year-old female with polymyositis, treated with corticosteroids, presented with neurological symptoms.
  • Cerebral damage, fever, and MRI findings suggestive of leukoencephalitis were observed two years post-diagnosis.

Findings:

  • PML is characterized by multifocal demyelination in the cerebral white matter, sparing grey matter.
  • While common in HIV-infected individuals, PML can also arise from other causes of immunodeficiency, including iatrogenic immunosuppression.

Implications:

  • This case highlights the importance of considering PML in immunocompromised patients with neurological deficits.
  • Corticosteroid treatment for systemic diseases can increase the risk of opportunistic infections like PML.
  • Prompt diagnosis and management are essential for patients presenting with symptoms suggestive of PML.

Related Concept Videos

Myasthenia Gravis: Overview and Treatment01:20

Myasthenia Gravis: Overview and Treatment

Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which leads...
Poliomyelitis01:17

Poliomyelitis

Poliomyelitis is caused by poliovirus, a small, non-enveloped, positive-sense RNA virus of the Picornaviridae family and Enterovirus genus. Transmission occurs primarily via the fecal-oral route, often through ingestion of contaminated water or food. The virus initially replicates in the oropharynx and intestinal mucosa, particularly in lymphoid tissues such as the tonsils, Peyer’s patches, and regional lymph nodes. Primary viremia follows, allowing dissemination throughout the body.In most...
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...
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...
Myasthenia Gravis ll: Pathophysiology01:22

Myasthenia Gravis ll: Pathophysiology

The disease process of myasthenia gravis begins at the neuromuscular junction, where antibodies attack key proteins needed for muscle activation. This immune reaction weakens signal transmission, leading to the characteristic muscle fatigue and weakness that define the condition.Immune-Mediated DamageIn most individuals, antibodies target acetylcholine receptors (AChRs) on the postsynaptic membrane of muscle cells. By blocking acetylcholine binding, these antibodies prevent the nerve signal...