Practice Guideline:

Epilepsy Management in Adults with Intellectual Disabilities

Target Audience: PCPs, neurologists

Key Points:

Quality of life is an important primary consideration when making epilepsy treatment

decisions in individuals with intellectual disabilities. The goal is not to stop every
seizure from occurring but to optimize the balance between seizure control and the side
effects and other downsides of seizure treatment.
Other motor phenomena (e.g., tics, choreoathetosis, dystonia), behaviors, or syncope
are easily confused with seizure activity by lay and professional people alike making the
accurate tracking of seizure frequency a particular challenge. In addition, approximately
two-thirds of the time, a specific epilepsy syndrome cannot be identified though the
seizures can usually be classified as partial or generalized.
Studies show that the vast majority of patients with intellectual disabilities and epilepsy
can be managed with monotherapy and that reduction and discontinuation of multiple
anti-epilepsy drugs (AEDs) does not necessarily lead to deterioration in seizure control.
Monotherapy as a goal is also supported by the fact that most of these patients receive
multiple other medications presenting the potential for deleterious drug interactions, a
problem that is compounded when multiple AEDs are used.
AEDs are often used for treatment of maladaptive behaviors in individuals with
intellectual disabilities. In the patient with concomitant epilepsy, care must be taken to
assure dosage adjustment that does not adversely affect one disorder or the other.
Conversely, AEDs may have behavioral effects that are undesired and result in
otherwise unnecessary treatment with psychotropics. This is particularly true of the
barbiturate class.
Respiratory problems are frequent in this population and may trigger increase in seizure
frequency. Raising AED doses ay actually worsen seizures in this setting.

Discussion:

Epilepsy management in persons with intellectual disabilities has special significance for
a number of reasons. The prevalence of epilepsy in the mentally retarded is much
greater than in the general population. It ranges from approximately 20% to 50% with
cerebral palsy and greater degrees of retardation conferring higher risk. In addition,
many of these individuals have co-morbidities (such as dysphagia and associated risks
for dehydration and respiratory infections) that can hamper efforts to gain and/or
maintain seizure control.

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Assessing and responding to changes in seizure frequency also poses unique
challenges in this group of patients. Adults with intellectual disabilities often do not live
with family but reside in homes or congregate care settings where staff may be
relatively unfamiliar with seizures and epilepsy. Typically they will have known the
patient for only a short time. In addition, seizure recognition can be confounded by
other, commonly present neurologic symptomatology such as choreoathetosis.
Therefore your ability to obtain an accurate seizure history will depend on the extent to
which you can help educate direct care staff on seizure identification and train them on
seizure tracking. Case managers can also assist with this task.

Dysphagia is frequently present in individuals with intellectual disabilities and epilepsy.

Drugs which act as central nervous system depressants may further compromise
swallowing function and the patients ability to protect the airway. Respiratory infections
may be insidious in onset with an increase in seizure frequency, for example, being one
of the first symptoms. It is therefore very important to have high degree of suspicion of
possible occult infection when seizure frequency increases with no change in AEDs or
AED level. Likewise, care must be taken when there is a known infection since
antibiotics used or changes in serum albumin may alter AED levels.

Consistent with the high frequency of co-morbidities is the finding that adults with
intellectual disabilities commonly take a number of routine medications, many of which
may interfere with or potentiate the actions and/or side effects of AEDs. These
medications may be prescribed by multiple providers making avoidance of
complications even more challenging.

Finally, these individuals often suffer from a variety of functional impairments, which
interfere with their activities of daily living. Therefore, a focus on preventing additional
functional decline and avoiding treatments which may adversely affect activities of daily
living should be maintained.

Approach:

Getting the history: The history will typically be obtained from a direct care staff person
and/or the Case Manager. If a Case Manager is involved, they can assist with obtaining
records such as past EEG reports, seizure history, medication usage history, medical
problem list, hospitalization history, etc. Direct care staff are responsible for recording
recent events. If they are not using seizure tracking tools, providing and instructing
them in their use will assist with your management. If seizures are reported, ask staff to
provide details regarding the characteristics (time/circumstances of onset, movements
and other symptoms observed during and after, length, post-ictal condition, etc.). Also
inquire as to any history of missed drug doses, intercurrent illness, changes in other
medications, or other events that may effect seizure threshold or drug level.
Exams: For evaluation of therapeutic efficacy, an examination is often not necessary.
Furthermore, for CSN Members, reimbursement is not conditioned on the Member
being present. If an examination is required and difficulties are anticipated, e.g.,
behavioral issues are present, request that familiar staff accompany the Member and
assist in obtaining compliance.
Monitoring treatment efficacy: In addition to obtaining a reliable history on seizure
frequency and severity (see above) queries should be made as to any side effects or

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interference with activities of daily living that have been experienced. Changes in sleep
patterns, difficulty eating, falls, or even respiratory infections may signal adverse effects
of treatment.
Medication management: Monotherapy is an important goal for a number of reasons.
These individuals are often on multiple medications, many of which can interaction with
AEDs (changing blood levels of one or the other, additive side effects, etc.).
These individuals often cannot verbally report side effects.
Many of the common side effects of AEDs can lead to worsening of already
compromised activities of daily living.
Individuals who have been treated in institutional settings, many since childhood, often
are on multiple AEDs including those in the barbiturate class. Because of known
adverse behavioral effects, efforts should be made to avoid or, where possible
discontinue their use.
Behavioral issues: Since a significant percent of these individuals also receive
treatment for behavioral issues, it is important for there to be close communication
between different treating physicians as well as with psychologists, particularly when
medications are being used for both epilepsy and behaviors. It should be made clear to
all involved just what the intended use of each medication is and which provider will be
responsible for dosing decisions.

References:

Hanzel TE, Bauernfeind JD, Kalachnik JE, Harder SR. 2000. Results of barbiturate
antiepileptic drug discontinuation on anti-psychotic medication dose in individuals with
intellectual disability. Journal of Intellectual Disability Research. Vol. 44 Part 2 pp 155-
163.
Coulter DL. 1997. Comprehensive Management of Epilepsy in Persons with Mental
Retardation. Epilepsia. 38(Suppl. 4): S24-S31.
Pellock JM, Morton LD. 2000. Treatment of Epilepsy in the Multiply Handicapped.
Mental Retardation and Developmental Disabilities Research Reviews. 6: 309-323.
Browne TR, Holmes GL. 2001. Epilepsy. N Engl J Med. Vol. 344, No. 15.
Alvarez, Norberto. 2001. Epilepsy in Adults with Mental Retardation. EMedicine
Journal, Volume 2, Number 1.
Patsalos, P.N., Perucca, E. 2003
Clinically Important Drug Interactions in Epilepsy; Interactions Between Antiepileptic
Drugs and Other Drugs. The Lancet Neurology 2 (2): 473-481

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901-266-7276 888-276-9299 Fax: 901-266-3913
www.csnwt.org Email: inquiry@csnwt.org