SSPE:
Subacute Sclerosing Panencephalitis


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A little about SSPE or Subacute Sclerosing Panencephalitis for you, esp. for the Peds Neuro people. SSPE is a fairly rapidly progressing fatal illness of the brain in children following persistent infection with measles virus (rubeola). Avg. age is 7-8 years and duration of illness is about 1-2 years. There are 4 stages:
1. Subtle behavioral change/declining school performance
2. Gen. or multifocal myoclonic jerking, seizures, akinesis, focal motor findings.
3. Coma with spasticity, decerebration or decortication.
4. Loss of cortical function and death.
The diagnosis is confirmed by burst supression EEG (sharp and slow wave complexes), CSF with incr. IgG, oligoclonal bands, and high titer measles virus antibody.
The disease is very rare because of good measles vaccination but does occur still (~2-5 cases/year). If interested in epidemiology, see Pediat. Neurol. 1989;5:339-41.
Treatments:
Immunosupression (not very good)
Immunostimulation (not very good)
Interferon induction (ok but toxic)
Antiviral agents (isoprinosine)
Intraventricular/intrathecal Interferon Injection (Alferon-N, fairly good success, dose is 1x10^6 IU twice weekly)

Hope this is somewhat helpful or interesting or both.
Many thanks to my friend Gurbani for the info.