Risk factors and outcome of epilepsy in adults with cerebral palsy and/or intellectual disability

Several risk factors have been described for developing epilepsy in pediatric patients with Cerebral Palsy (CP): family history of epilepsy, low Apgar score, presence of neonatal seizures, presence of Intellectual Disability (ID) and motor impairment , presence of brain malformations or gray matter lesion in neuroimaging, and epileptiform activity in electroencephalogram. Many of these factors are also associated with increased drug resistance and risk of recurrence in pediatric CP with epilepsy.

Executive Summary
Adult patients with CP and ID will be recruited from outpatients who visited the ASPACE Foundation (Associació de Parálisi Cerebral), Barcelona (Spain), between July 2023 and July 2024. Study data will be obtained the review of the clinical history and the interview with the patient and/or relatives.

Context and Justification
Very few studies have focused on the study of neurological comorbidities (epilepsy, gait disorders, cognitive disorders) in adult patients with CP and/or ID. Similarly, a large percentage of these adult patients do not have a complete etiological study that includes neuroimaging and genetic testing.

General Objective
To review all etiological studies that have been conducted in a cohort of adult patients with CP and/or DI.

Specific Objectives
To evaluate those factors that increase the risk of epilepsy in patients with CP and/or DI.
To evaluate those factors that increase the risk of relapse or resistance to epilepsy drugs in patients with CP and/or DI.

Neurological evaluation
The etiology of the CP or ID, the complementary tests performed and the history of epilepsy will be reviewed. A neurologist examined the type (eg, spastic, ataxic, hypotonic) and degree of motor and speech impairment. Given the lack of validated clinical scales for adult CP patients, scales that are only validated for the pediatric population were used.

Gross Motor Function Classification System (GMFCS)
It describes the functional ability of the person with CP to sit and walk and the need for support products. Includes five levels. Level I people can do all the activities, although with some difficulty with speed, balance and coordination; Level V individuals have difficulty controlling their head and trunk posture in most positions and achieving any voluntary control of movement.

Classification of bimanual fine motor skills (BFMF)
It ranks fine motor skills according to the best ability (ability) to grasp, hold and manipulate objects for each hand separately, in eight levels. Level I includes people with minor limitations, and levels IV-V describe people with severe functional limitations.

Communication Function Classification System (CFCS)

It assesses everyday communication, including five levels. An individual who communicates at a CFCS level I is an “effective sender and receiver with unfamiliar and familiar partners,” while a person who is at CFCS level V is a “rarely effective sender or receiver, even with familiar partners.”

To define drug resistance, the International League Against Epilepsy (ILAE) definition is used: drug-resistant epilepsy is defined as the failure of adequate trials of two tolerated and appropriately chosen and used antiepileptic drugs (AEDs) ) (either in monotherapies or in combination) to achieve sustained seizure freedom.
Seizure recurrence is defined as the presence of seizures on tapering or discontinuing the AED after a seizure-free period (seizure-free for at least five years).

Psychiatric evaluation

The presence of psychiatric comorbidity was defined using DSM-IV diagnostic criteria. (American Psychiatric Association, 1994)

Neuropsychological assessment

The presence and severity of ID was assessed using the Wechsler Adult Intelligence Scale-IV (WAIS-IV) by a trained neuropsychologist.
Patients were classified into three subgroups based on IQ: mild ID (69-50), moderate ID (49-35) and severe ID (<34).