Autism And Epilepsy: The Complex Relationship Between Cognition, Behavior And Seizure

There is an increased and variable association between autism and epilepsy. Autism is a complex neurodevelopmental disorder. When associated with epilepsy, it places the child and the family in a very demanding and stressful situation to cope with. In order to help these children and their families, it is important to understand the relationship between behavior patterns, cognitive processes, language development and occurrence of seizures. So far we are yet to fully understand this relationship. I have tried to present here a brief review of what we know so far, using a case example emphasizing the fact that behind the diseases there are the people. “It is much more important to know what sort of a patient has a disease than what sort of a disease a patient has. ” – William Osler THE STORY OF LITTLE C I met little C for the first time in the follow-up clinic with his mother. He was seven years old but looked much younger. He was well dressed and groomed with bright eyes, short fair hair and was shorter than the average child of that age. He walked into the room, making no eye contact. He took a blank sheet of paper that lay on the desk to a smaller table that stood in one corner of the room and started drawing. He had a sad and blank expression on his face with no reciprocal interaction and never spoke a word all through the session which lasted nearly two hours. He seemed to be in his own world engrossed in his drawing, oblivious to the happenings in the room. By the end of the session he had filled the page with figures of little trees in neat rows and columns that looked uncannily identical. His mother pointed out that this was pretty much the routine in every follow-up clinic for the last two years. C was born full term after a normal pregnancy and was delivered normally. As far as the mother could remember, his early motor developmental milestones were normally achieved but he was late to develop speech. He would have some restless nights when he tossed and turned in bed. He was a very easy child to look after as he was not at all demanding. He had no problems feeding and was adequately toilet trained. He was fully immunized. One day, when he was around two years of age while his mother was feeding him, C suddenly ‘went blank’ with a vacant stare and started making clicking noises with his tongue, which lasted for less than a minute. This did not recur and so the mother never consulted a doctor. As he grew older, there appeared clear changes in his behavior. He very much liked routines. The least change would bring on a tantrum, when he would become quite aggressive. These were frequent with violence mostly directed towards objects and sometimes towards mother and siblings. By contrast, he was unusually caring towards the pet cat and they seemed to like each others company. He could indicate his needs but was less verbal and was very literal in the use of language. He preferred playing alone for long hours and never mixed with his siblings, a brother 3 years older to him and a sister who was a year younger. His play was unimaginative and was repetitive. He had a fascination for trees. He liked to look at pictures of trees in books again and again. He liked drawing only pictures of trees. From the age of five years, very often and very suddenly, he would demonstrate behavior out of character. His eyes would suddenly appear glazed and his face would become pale. He would then make strange clicking noises with his tongue and repeated movements in the air as if trying to climb. This would last for a few seconds to several minutes. He would then sleep for several hours. A maternal uncle had learning disability. Mother had separated from his father when C was about one and a half Autism And Epilepsy: The Complex Relationship Between Cognition, Behavior And Seizure 2 of 6 years old and there was no contact with him. Father had a history of violent behavior and was seeing a psychiatrist, but more details were not available. There were no problems reported with the siblings. Mother, who had received high school education, was unemployed. The family received emotional support from mother’s mother and sister. C had been fully assessed by a child psychiatrist, a pediatrician, a child neurologist, speech and language therapist and a clinical psychologist. He had undergone general-physical and neurological examinations. His laboratory investigations included routine blood and urine analysis and genetic testing which did not reveal any abnormalities. Psychometric tests reported a below average intelligence in the borderline range. Routine and sleep EEGs showed paroxysmal abnormalities over the left temporal lobe. An MRI scan of the head was normal. From the history, C demonstrates a qualitative impairment in the development of reciprocal social interaction and communication. It appears that this was evident from infancy though in a subtle form. At two years of age he appears to have had an absence seizure. Whether this contributed to cause further impairments or, the progress was natural is hard to tell. He shows some restriction and repetition in his interests and activities. Speech was late to develop but he was able to use it though in a concrete way. Psychological testing revealed only borderline impairments in intelligence. These features are consistent with a diagnosis of autism. At the age of five he started having clear cut seizures. Whether the behavioral difficulties were subtle forms of seizures is hard to tell. EEG revealed abnormalities with a left temporal lobe focus. These are consistent with a diagnosis of complex partial seizures. C was receiving the antiepileptic medication valproic acid. He was identified as having special educational needs and was receiving specialized help in school. His mother was a very patient person. She showed a lot of interest in the problem. She never blamed C or anyone else for the situation. She was willing to learn and understand. She understood that C was suffering and made it her goal to make things better for him. The seizures were under control. He was showing very small and slow improvements in his communication. His behavior was much improved. His school work was showing a gradual progress. The questions that arose in my mind in this case were, were seizures a cause of autism in this child, or an association? Could there be other causes? Were the improvements shown, due to the treatment of epilepsy or due to other factors? What is the relationship between cognition, behavior and seizures? I decided to ‘dig’ into the literature and find out.