This paper was prepared by  the Encephalitis Society with advice from Dr R. Appleton Consultant Paediatric Neurologist Alder Hey Children’s Hospital Liverpool

Subacute sclerosing panencephalitis (SSPE) is a serious viral encephalitis of children and young adults. It is a late manifestation of measles virus infection, developing usually 6 and 12 years after natural measles infection. Cases can also follow immunization with measles vaccine but at a lesser frequency than after natural measles.  The annual incidence is about 1 per million.  Males are more commonly affected, as are those living in rural areas.  Mortality is very high.  Those who  survive do so with considerable intellectual and also physical impairment.

The precise sequence of events that results in the development of this disease in a tiny proportion of the population is not known.  The great majority of children who develop SSPE are known to have had an attack of classical measles, usually many years before.  Over 50% of cases have had the disease at the unusually early age of under two years. 

The onset of the illness is insidious, the first signs are behavioural disturbances or a decline in school performance.   Not infrequently the family are told that the cause is “psychological”.  Progression of the illness will vary from child to child  but evidence that this is a more serious illness usually begins with loss of motor control and co-ordination.  This often starts with characteristic jerking movements which may at first appear as clumsiness.  These jerking movements are known as myoclonic seizures.  Grand mal (also called tonic-clonic) seizures may also occur.  As the condition worsens problems with swallowing and speech develop,  and vision may be impaired.

The duration of the illness can be as short as six weeks or as long as ten years or more.  Long-term survival has been reported and up to 5% may experience spontaneous long term improvement.  Treatment with inosoplex (isoprinisine) may be of benefit in slowly progressive cases. 

David’s Story - told by his parents

David had measles when he was four and although he was very poorly,  he recovered completely and continued to develop as a normal boy would.  Little did we know what was lurking in his brain. 

David loved sport and animals and was a loveable, happy, easy youngster.  He played table tennis in the 2nd division and took part in several tournaments.  He wanted to become a paramedic or youth worker and had recently started the Duke of Edinburgh award scheme.

In November 1998 he complained of a strange feeling passing through his body.  He visited the doctors but wasn’t taken seriously.  At Christmas we realized that something was really wrong.  David was falling over and having difficulty in eating and co-ordinating movements.  He was also having a spasm which affected his arm and face.  We now know it was caused by the brain messages getting scrambled.  These continued and caused all sorts of problems all through his illness.  David went back to the doctors at the beginning of January, still nothing was visibly wrong but several blood tests were taken.  Three weeks later we visited the doctors again.  He was sent to hospital for tests.  By now simple things, like tying shoe laces, were extremely difficult and his balance was poor.  David spent three weeks in hospital having all sorts of tests done, during which time we were guessing what was wrong.  The tests suggested Subacute sclerosing panecephalitis caused by the measles virus lying dormant in his brain, but it couldn’t be conclusive without a brain biopsy.  As there isn’t a cure anyway to put David through this sort of trauma seemed ludicrous. 

In just six months David went from a fit and healthy young man to a helpless individual who depended on us for everything, he couldn’t move and needed constant care and attention.  The most upsetting thing was when he could no longer communicate with us.  We managed through grim determination to care for him at home and he died there on the 20th April 1999.  He was only 18 years old.

The thing which most people remember about David was his smile.  We miss him terribly; but try to remember how he would have felt  if he knew how unhappy we are without him. 

David planted an oak tree when he was five at school:  the teacher had kept it in a pot all this time.  We have planted it in King’s Wood so part of him lives on.

David?

Where are you now? I do not know,
I call your name in vain.
Your room stands waiting your return
to end this ceaseless pain.
I know you will age no more,
as I grow old and grey.
Your time with us was far too short.
We wanted you to stay,
“Don’t worry Mum,” you breathed,
“There’s nothing we can do.”
Without a backward glance you sighed and went away,
Still, I see you from the hill,
you’re there, the air, the sea, the trees
but by my side you’ll never be.
My tears are falling like the rain,
they are no help to me.
In time my thoughts of you will fade,
and you’ll be a memory.

Christine Mitchell, David’s mother  1999

Last modified – 15/11/2006