Progressive multifocal leukoencephalopathy

Progressive multifocal leukoencephalopathy Microchapters
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Case #1
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This page is about clinical aspects of the disease.  For microbiologic aspects of the causative organism(s), see JC virus.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Progressive multifocal leucoencephalopathy, Multifocal leucoencephalopathy, Multifocal leukoencephalopathy, PML, PMLE

Natural History, Complications and Prognosis

Patients with Progressive Multifocal leukoencephalopathy(PML) have a long-term neurological sequelae and is often fatal. The mortality rate is 30%-50% ^[1] in early months of diagnosis. Those who are immunocompromised are at significant risk of reactivation.

COMPLICATIONS

PML may complicate into the following :

• Neurologic Dysfunction
• Dementia
• Blindness
• Seizures

PROGNOSIS

• The prognosis is variable and depends on the underlying severity of PML.
• Impairment of immune system (eg. HIV) or use of immunosuppressants may worsen prognosis and leads to rapid progression of the disease.^[1]
• Patients who survive have substantial morbidity and severe neurological disabilities.^[2]

Reference

1. Progressive Multifocal Leukoencephalopathy Information Page. NIH-National Institute of Neurological Disorders and Stroke. 29 July 2019. Retrieved 12 June 2021.
2. Castle, D., & Robertson, N. P. (2019). Treatment of progressive multifocal leukoencephalopathy. Journal of Neurology, 266(10), 2587–2589. https://doi.org/10.1007/s00415-019-09501-y

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | X Ray | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

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• Leukoencephalopathy with vanishing white matter