Infantile spasms are a particularly harmful form of epilepsy, which can result in mental and physical retardation unless brought under control rapidly.
The primary distinguishing feature of infantile spasms are clusters of myoclonic spasms - the jerking of one or more limbs.
The age of onset of infantile spasms is usually between 4 and 7 months, and they usually disappear within a few months, often to be replaced by other forms of epilepsy.
The incidence of infantile spasms is between 1 in 2,000 and 1 in 4,000 of live births. This is about 25% to 30% of all childhood epilepsies.
Clinically, neurologists define infantile spasms by three characteristics:
Infantile spasms are often difficult to control with epileptic drugs - the drugs of choice appear to be ACTH and vigabatrin (sabril); however, infantile spasms do appear to be responsive to treatment by the ketogenic diet.
The prognosis for infantile spasms is poor. The spasms usually remit spontaneously after a few months, often to be replaced by other forms of epilepsy before the age of 5; only 15% of cases have complete recovery. 30% of cases progress to Lennox-Gastaut syndrome. 60% of cases have metal retardation at onset, rising to 80% subsequently.
How do I know if my child has infantile spasms?
Neurologists believe that it is important to identify infantile spasms as early as possible, in order to try to minimise the damage that they cause. The difficulty is that while the myoclonic spasms are very characteristic, they may initially be very subtle and difficult to recognise.
West syndrome and infantile spasms
Parents are often confused as to whether West syndrome (or West's syndrome) is the same as infantile spasms. Most neurologists use the terms interchangeably, preferring the term "infantile spasms". However, some neurologists attempt to distinguish West syndrome as a special case of infantile spasms.
Infantile spasms were first described by WJ West, an english doctor, in 1841 in a paper which appeared in the first volume of the Lancet (1, pp724-725). This described the condition of his own child, and gave a precise description of the syndrome and its development. The paper is also significant as the first major clinical study of paediatric neurology.
Infantile spasms is an epileptic syndrome characterised by the triad of myoclonic spasms, hypsarrhythmia and arrest of psychomotor development at onset. Infantile spasms seems particularly suitable for treatment by the ketogenic diet, because the frequency of fits makes it possible to monitor and fine tune the diet with precision.
Infantile spasms and immunisation
Many parents believe that their children's infantile spasms were caused by a DTP shot, and specifically by the pertussis (whooping cough) immunisation. This is an emotive issue, because compensation may be available if a causal effect can be shown. Parents are therefore motivated to believe that a DTP shot is the cause, whereas the authorities are motivated to dispute this.
(not available) Related epilepsy syndromes
Infantile spasms resources
There is very little useful information available on the web about infantile spasms. There is a mailing list and a number of BBS systems refer to infantile spasms; there is a UK support group, and many children on the ketogenic diet list have infantile spasms.
return to Infantile
spasms and other epilepsies
return to Generalised symptomatic epilepsies
(update 1.1: 8 November 2002)
(issue 1: 20 June 1998)