From the CDC Public Health Image Library #132 via the Wikimedia commons.  Child with classic measles day-four rash.

From the CDC Public Health Image Library #132 via the Wikimedia commons. Child with classic measles day-four rash.

[Editor’s note: We have two posts today, this post by our regular contributor Dr. Clay Jones, and an excellent guest post by William London about a chiropractor’s dubious neuropathy treatment protocol. Enjoy today or over the weekend!]

As a pediatrician, even one who has spent the majority of his career caring only for hospitalized children, the death of a patient has been a rare occurrence. There are certainly some pediatric specialties, such as intensive care and oncology, that because of the nature of their patient population must develop a more intimate relationship with the end of life. But compared to the adult world, even their exposure pales in comparison. The most common form of pediatric cancer, acute lymphoblastic leukemia, has a cure rate that is over 90% for example – making the unnecessary death of Makayla Sault after some worthless “treatment” at the Hippocrates Health Institute all the more tragic.

A bit more common in pediatrics are the patients that require significant intervention, and who may come close to death, but recover thanks to advances in modern medicine. These patients, however, are dwarfed by the number of children who receive routine hospital care and recover fairly uneventfully. And most children emerge into adulthood having never had more than a few self-limited viral illnesses and maybe a cavity or two. This wasn’t always the case. In 1900, 10% of 1-year-old children would not make it to adulthood.

I remember every patient that has died while I took part in their care, the bulk of which occurred during my residency training over a decade ago. Their names are lost to time but not their faces, and certainly not the lasting emotional impression they made on me. My experiences with patients who did not survive have changed the way I think about and practice medicine, and significantly influenced the way I approach life in general.

During my first rotation as a resident in the pediatric intensive care unit, seven children died. This was a bad month even for a large academic facility. One young girl suffered a catastrophic injury to her brain because of a rare complication of infection caused by eating improperly cooked hamburger at a church picnic. And a newborn infant just a few days home from the hospital was accidentally smothered by her parents while sleeping between them in bed. I won’t list them all, but I would hope that it is clear why this month stands out in my mind as the worst of my career so far, and why to this day I have a deep and unwavering hatred for all-terrain vehicles.

Lately I have been thinking a lot about one of the children that died that month. I only knew him and his family for a short time but the course of his illness had been long and exceptionally tragic, and he had been brought to the hospital to die. Over the roughly two years prior to his death, this previously healthy and thriving teenager had slowly but steadily deteriorated, beginning with his parents noticing that his temperament had changed and that his grades were dropping. They thought that maybe he had started experimenting with drugs.

As the months passed, his condition worsened. His ability to think became more and more impaired and he began to have odd muscle spasms, eventually losing the ability to even walk or care for himself. He lost the ability to speak and then to swallow, becoming dependent on nutrition administered through a tube surgically inserted directly into his stomach. By the time of his last admission to a hospital, he was in a persistent vegetative state. His brain began to shut down, his drive to breathe became erratic.

The cause of his deterioration had been diagnosed by the time I knew him. It wasn’t related to a traumatic injury or stroke, and it wasn’t because of an infection in his brain, at least not a new one. It was actually the result of what some would consider a mild illness of childhood – no big deal even.

In reality this infection is one of the most common causes of death in children around the world and has only recently re-entered general public awareness in the United States thanks to widespread media coverage of a large cluster of cases centered at Disneyland. I’m of course talking about measles. The complication that ended my patient’s life in such a tortuous way is called subacute sclerosing panencephalitis or SSPE.

What is SSPE?

Measles can make children very sick, primarily when one or both of two organs are involved: the lungs and the brain. Pneumonia associated with measles, which can occur in as many as 1 out of every 20 young children who acquire the infection, is the most common culprit in fatal cases. But inflammation of the brain, called encephalitis, can kill during the initial illness or leave a child with permanent cognitive impairment or hearing loss. SSPE occurs when infection of the brain becomes clinically apparent years later, often after an otherwise mild and uncomplicated case.

Acute encephalitis occurs when the immune system reacts to measles virus that has found its way into the central nervous system. But the immune system doesn’t always respond aggressively. In some cases, a mutation occurs in the virus which renders it unable to produce certain proteins that would have served as a signal flare. In addition, particularly in children less than 2 years of age, an immature immune system simply fails to respond.

Regardless of the specific reason, measles virus is allowed to remain in neural tissue relatively unchecked and it slowly proliferates under the radar as the years go by. Eventually, usually in the ballpark of 6 to 8 years later, the progressive inflammatory process becomes clinically apparent. Death is almost certain and occurs within 1 to 3 years of diagnosis in most cases, although there are case reports of more rapid progression as well as the occasional patient who survives.

How does SSPE present?

The early stages of SSPE are subtle and often confused with other issues. As with my patient, it often begins with behavioral changes, such as irritability, and mild memory problems that mimic psychiatric conditions or drug use. As the condition progresses, central nervous system control of the muscles is affected. This leads to involuntary muscular activity in the form of spasms or rapid jerking movements. Seizures and impairment of vision are also common.

Eventually in most cases the ability to walk becomes impaired. Loss of motor control also tends to affect the ability to speak and swallow, which increases the risk of aspirating. Continued decline in cognitive capabilities occurs. Dementia, coma and a persistent vegetative state represent the final stages of the illness, with death resulting when the brain loses control of vital functions like breathing and cardiovascular regulation.

How is SSPE diagnosed and treated?

The diagnosis of SSPE requires clinical suspicion, especially in an area where measles rates are low. This currently still includes the United States but that may change if the ongoing increase in cases we are seeing continues. When a patient has signs and symptoms consistent with SSPE, the next step is usually imaging of the brain and obtaining an EEG. Results of these studies are often non-specific, although there are some EEG findings considered to be fairly consistent with the diagnosis. Testing the patient’s blood and spinal fluid for evidence of an immune response in the form of anti-measles antibodies (IgG) plays a role, as does PCR testing, which can reveal the presence of actual virus.

A variety of antiviral drugs have been tried over the years, as well as potent immune-suppressing medications, without significant success. If started very early in the course, preferably before there is significant cognitive impairment, there is a chance that treatment might slow the progression of symptoms. These therapies, when helpful, must be taken for the duration of the patient’s life. Unfortunately, there is no cure for SSPE.

Who gets SSPE and how common is it?

From the PMC 2640885, "Subacute sclerosing panencephalitis, a measles complication, in an internationally adopted child", via the Wikimedia commons.  Initial and three-month presentation of a thirteen-year-old adopted child with SSPE.

From the PMC 2640885, “Subacute sclerosing panencephalitis, a measles complication, in an internationally adopted child”, via the Wikimedia commons. Initial (A & B) and three-month (C & D) presentation of a thirteen-year-old adopted child with SSPE.  C & D show widespread shrinking of the brain tissue.

Where there is more measles, there is more SSPE. In regions where vaccine uptake is high, such as the United States, the incidence is extremely low with roughly ten cases diagnosed per year total. In developing regions where measles remains endemic and common, SSPE is diagnosed at a rate of 20 per million people. But this data likely doesn’t reflect the true risk considering that many cases will go undiagnosed.

The last large outbreak of measles in the United States occurred from 1989 to 1991 with over 55,000 cases reported. Most of the cases involved unimmunized preschool children. 11,000 patients were hospitalized and 123 died, which was consistent with the expected 1-2 deaths per 1,000 cases. Worse actually.

In 2005, CDC researchers combed through medical records and tested available brain tissue samples from patients diagnosed with SSPE. They wanted, using modern PCR technology, to determine the rate of SSPE specifically in patients who were infected with measles during the outbreak from 1989 to 1991. Their results revealed a risk of 1-2 per 10,000 cases, which was 10-fold higher than prior estimates.

Conclusion

Even considering the recent increase in measles cases in the United States, it is still a very rare condition. And SSPE is an uncommon sequelae, although perhaps not as uncommon as historical estimates would have us believe. But measles is not rare in many regions of the world, and our attempts at eliminating it have stalled. Worldwide measles deaths actually increased from 2012 to 2013 and the number of cases in some developed nations is increasing.

SSPE is a horrific potential complication of infection with measles that can arise years after even an uneventful case. No child should have to suffer through it and no parent should have to worry about their child developing it. And although there is no effective cure for the condition, there is a highly safe and effective vaccine which can prevent it.

 

 

Posted by Clay Jones

Clay Jones, M.D. is a pediatrician practicing at Newton-Wellesley Hospital in Newton, MA, and a regular contributor to the Science-Based Medicine blog. He primarily cares for healthy newborns and hospitalized children, and devotes his full time to educating pediatric residents and medical students. Dr. Jones first became aware of and interested in the incursion of pseudoscience into his chosen profession while completing his pediatric residency at Vanderbilt Children’s Hospital a decade ago. He has since focused his efforts on teaching the application of critical thinking and scientific skepticism to the practice of pediatric medicine. Dr. Jones has no conflicts of interest to disclose and no ties to the pharmaceutical industry. He can be found on Twitter as @skepticpedi and is the co-host of The Prism Podcast with fellow SBM contributor Grant Ritchey.

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