Subacute Sclerosing Panencephalitis: An Almost Forgotten Disease
I’m entering shark infested waters without a lifeboat writing this. Yet, I still feel it is an important subject to discuss. I am willing to jump head first into these waters, if even one child is spared from this terrible disease. The disease is subacute sclerosing panencephalitis. If you have never heard of it, there is a reason for that. The reason is the MMR vaccine. Yes, I just took that big leap into that scary dark shark infested water. Now, that I have your attention and many of you are in fight or flight mode waiting to attack me for the research and science-based evidence I’m going to share here, let me explain, this is not meant to be pro-vaccine. It is simply meant to inform the public of a forgotten disease we may soon see reemerging.
Recently the pro-vaccine vs. anti-vaccine discussions have been everywhere. With the hearing on possibly mandating vaccines by the government things have really gotten fired up. I’ve read on numerous social media posts that parents aren’t scared of the measles and some are even hoping their children contract the virus to acquire “natural immunity”. Subacute sclerosing panencephalitis is one reason you should indeed fear the measles virus. It is also the reason you should not expose your unvaccinated children to the measles virus.
Subacute sclerosing panencephalitis (SSPE) signs and symptoms affect the central nervous system and often develop 7-10 years after a person recovers from measles. It is unknown why some people develop SSPE after they have seemingly recovered from the measles while others do not. Researchers suspect it is due to an irregular immune response or a mutant form of the measles virus. Early signs and symptoms include behavior changes and mild mental deterioration. Later symptoms include impaired motor function and myoclonic jerks, some people may develop seizures and become blind. In the end stages patients become comatose before succumbing to the disease. Currently there is no cure for SSPE, treatment is strictly supportive. Supportive treatment may include anticonvulsant and antispasmodic drugs. The prognosis for people with SSPE is poor. SSPE is always fatal. Let me repeat that it is ALWAYS fatal. Most affected people die within 1 to 3 years, lifespan following diagnosis can vary. Statistically SSPE is considered rare in developed countries with widespread immunization with the measles vaccine. Cases have declined by 90% in these countries. However, the risk is much higher in countries that do not have widespread measles vaccines.  With the recent upsurge of measles cases in the U.S. we may start seeing an upsurge in SSPE. Some studies have indicated SSPE is not as rare as it was once believed to be. Researchers looked at cases in the state of California from 1998-2015, they found 17 cases 12 of which had a history of the measles. In a sub analysis of the same study it was determined between the years 1988 – 1991 (during this time frame there was a measles resurgence in California) one in 1,367 individuals, under age five at the time of measles virus were later diagnosed with SSPE. One in 609 individuals diagnosed with measles under 1 year of age later developed SSPE.  There is a higher incidence rate in males and a higher rate of developing SSPE if the child is infected with the measles virus before two years of age.  Though the average age of onset is 12, SSPE has been diagnosed as late as age 36.
In one case study of a 36-year-old male, the patient presented with chorioretinitis (inflammation of the thin pigmented vascular coat of the eye and retina of the eye) two years prior to onset of neurological issues which was determined to be SSPE. The diagnosis of SSPE was confirmed by elevated measles antibody titers in cerebrospinal fluid and serum. This study suggests clinicians consider SSPE among the differential diagnoses in chorioretinitis. This seems to be particularly so if there is macular or peri macular involvement with concurrent involvement of the optic nerve in young patients.  Ocular disease affects 10-50% of patients with SSPE and leads to significant vision loss.  As interesting as this connection is, with the current prognosis of SSPE, I would think vision loss would be the last thing a parent or patient would be worried about. However, early diagnosis may help slow the progression with antiviral agents and Interferon Alpha. Immunoglobulins (a blood product that is given through the veins) and plasmapheresis (cleaning the blood of inflammatory chemicals) have also been reported to have some benefit. Despite initial improvements and slowing of progression, the disease eventually resumes its course, and as mentioned above there is no cure. 
More common complications with the measles virus are ear infections and diarrhea, both complications are mild, which I believe gives parents false securities about their children being infected with the measles virus. However, one in 10 children will have hearing loss, one out of every 20 children will get pneumonia, one out of every 1,000 will end up with encephalitis all due to the measles virus. For every 1,000 children that get the measles virus 1-2 will die. Pneumonia is the most common complication that ends in death.  Though SSPE is much less common than the previously mentioned complications, it remains the scariest to me. I would like to leave you with the following video, Sarah W’s story. I can’t imagine the pain this mother is going through watching her child deteriorate. If you choose not to vaccinate your children, please try not to expose them purposely. This could be detrimental to babies and immune compromised patients that your children may encounter while infected with the measles virus.
1. National Center for Advancing Translational Science. Retrieved from https://rarediseases.info.nih.gov/diseases/7708/subacute-sclerosing-panencephalitis
2. American Academy of Pediatrics. Study: Fatal Measles Complication Not as Rare as Previously Thought. Retrieved from https://www.aappublications.org/news/2016/10/28/Measles102816
3. Jeevagan V, Dissanayake A. Chorioretinitis: A Potential Clue to the Early Diagnosis of Subacute Sclerosing Panencephalitis. Pract Neurol 2017 Aug; 17(4): 293-296. Retrieved from https://www.ncbi.nlm.nih.gov/pubmed/28512208
4. Beato J, Falcao MS, Costa H, Figueira L, Santos-Silva R, Penas S, Brandao E, Carneiro A, Falcao-Reis F. Early Longitudinal Spectral Domain Optical Coherence Tomography Findings in Subacute Sclerosing Panencephalitis. Retina. 2017 May; 37(5): 45-47. Retrieved from https://www.ncbi.nlm.nih.gov/pubmed/28423386
5. Encephalitis Society. Retrieved from https://www.encephalitis.info/subacute-sclerosing-pan-encephalitis-sspe
6. Centers for Disease Control and Prevention. Retrieved from https://www.cdc.gov/measles/about/complications.html