Other medical conditions with epilepsy as a significant feature

Landau-Kleffner syndrome
This syndrome typically begins with partial or generalised seizures (including atypical absence seizures) and language comprehension and speech are lost after two or more years of normal development. The range of impairments includes behavioural disorders and global cognitive, motor and social regression with many autistic features. Anti-epileptic drugs (AEDs) often have no effect on the cognitive deficit but high doses of sodium valproate, often in combination with lamotrigine, sometimes help.

Tuberous sclerosis

Approximately 80% of children with tuberous sclerosis (TS) have some form of epileptic seizures and mental retardation is seen in 60%. The epileptic disorder is often severe and resistant to the treatments available, with a very low remission rate. Various epileptic disorders occur in children with TS but infantile spasms are the most common presentation. As these children age, the epileptic disorder may change; in some children, Lennox-Gastaut syndrome emerges.

Surgery with removal of the epileptogenic tuber should be considered if there is poor response to treatment. The surgical outcomes vary but there is marked improvement in a substantial number of children.

Autism

Approximately 20-30% of children and adolescents with autism develop some form of epileptic disorder. The seizures are observed more frequently in patients with more severe cognitive impairment.

Rett syndrome

Rett syndrome is a major cause of severe mental retardation associated with seizures in girls. It is characterized by a progressive mental and growth impairment that starts in infancy.

They develop an autism-like syndrome with stereotyped movements, some of which, especially those in the hands, are considered very typical of the disease. Epileptic seizures are seen in 25-30% of cases, mostly generalized and complex partial.

Cerebral palsy

Epileptic seizures occur in 25-50% of children with cerebral palsy (CP). The incidence is related to the severity of the brain damage. It is higher in those children with quadriplegia, lower in those with congenital hemiplegia and much lower in children with diplegia and the dyskinetic form of CP.

The first epileptic seizures typically are seen during infancy. The seizure disorder is the consequence of the brain abnormalities associated with the CP. Clinical studies indicate that early seizures are associated with more cognitive deficiencies.

Practically every type of epileptic seizure has been described in individuals with CP. Generalized tonic and tonic-clonic seizures and partial complex seizures are most frequent. Some syndromes, such as infantile spasms and Lennox-Gastaut syndrome are particularly frequent in children with CP.