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July 9, 2015: Subacute Sclerosing Panencephalitis

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Brought to you by the Residents & Fellows Section of Neurology.

July 9, 2015

Subacute Sclerosing Panencephalitis

The most deadly neurological complication after an infection with the measles virus is a latent condition called subacute sclerosing panencephalitis. It has a low incidence in developed countries but remains high in countries with poor measles vaccination rates, such as in India with rates of 21 per million annually. The condition is thought to arise from a poor initial cellular immune response during an acute infection with the measles virus, thus allowing for the virus to fester intracellularly and remain dormant for as long as six to ten years. Eventually, the latent virus with cause significant inflammation with neuronal and oligodendrocyte death.

Symptoms gradually progress from personality changes to myoclonus and seizures and then to rigidity, coma, and autonomic instability. Diagnosis is made based on clinical history and elevated CSF measles titers and is supported by classic EEG and MRI findings. Treatment is non-curative but aimed at prolonging survival, and typically comprises interferon-alpha and isoprinosine. Prognosis is variable from 1 to 3 years survival in 95 percent of children.

References

  1. Gutierrez J, Issacson RS, Koppel BS. Subacute sclerosing panencephalitis: an update. Developmental Medicine and Child Neurology 2010; 52: 901-907.
  2. Measles (Rubeola) for Healthcare Professionals. In: Center for Disease Control, National Center for Immunization and Respiratory Diseases, Division of Viral Diseases [online]. Available at http://www.cdc.gov/measles/hcp/. Accessed November 2014.

Submitted by Sarah Wesley MD, Department of Neurology, Mount Sinai Beth Israel Medical Center.

Disclosures: Dr. Wesley is a member of the Residents & Fellows Section of Neurology.

For more clinical pearls and other articles of interest to neurology trainees, visit Neurology. Listen to this week's Neurology Podcast.

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