There is very little known about self-limiting and pharmacoresponsive focal epilepsy, which means that it is currently unknown how common it is, only that out of every 100 children whose epilepsy starts below the ages of 2 years, between 5 and 10 will have this particular syndrome. What is known is that it appears to affect males and females equally and that the syndrome has been found to affect generations in the same family.
In past years, self-limiting and pharmacoresponsive focal epilepsy has been known by other names, including benign partial epilepsy and benign focal epilepsy.
With self-limiting and pharmacoresponsive focal epilepsy seizures often start between 4 months and 9 month of age, but can start between 2 and up to 18. The seizures are typically focal and often occur in clusters of 5 to 10 at a time. During a focal seizure, the child will suddenly cease what they are doing and stare straight ahead or to the side. Their head may also tilt to one side and occasionally they may present with a twitching in one side of the face.
Focal seizures are often followed by a tonic-clonic seizure, which is when the body becomes rigid and the child may fall if standing.
In order to diagnose self-limiting and pharmacoresponsive focal epilepsy, a detailed history or description must be given from someone who has witnessed the seizures. A diagnosis of this type is often only made after several other epilepsy types have been ruled out, which means it can be a lengthy process. Self-limiting and pharmacoresponsive focal epilepsy is often confused for other syndromes, including Dravet syndrome, epilepsy with migrating focal seizures in infancy and Panayiotopoulos syndome.
Common tests include an electroencephalogram (EEG) and a magnetic resonance imaging (MRI) scan. Occasionally a blood test may be ordered to ensure that the child with the syndrome is not experiencing low glucose or calcium levels which may have been a cause for seizure activity.
Children who have been diagnosed with self-limiting and pharmacoresponsive focal epilepsy and are experiencing frequent or clusters of seizures may be prescribed an anti-epileptic medication (AED). Common medications include carbamazepine, levetiracetam, lamotrigine and sodium valproate.
With such a limited understanding of self-limiting and pharmacoresponsive focal epilepsy, it is impossible to say with any certainty that seizures are controlled in most children. However, it is thought that in most cases, the seizures will cease between a few months to a year after starting, which is known as ‘spontaneous remission’.
In the majority of cases, children with self-limiting and pharmacoresponsive focal epilepsy who are taking medication may be gradually phased off 12 to 18 months after their first prescription.
Development and behavioural issues are not thought to affect children with this syndrome, however, with so little known it is difficult to understand the long term outlook.
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