Practice Guideline: Epilepsy management in persons with mental
retardation
Target audience: PCPs, neurologists
References:
1) Hanzel TE, Bauernfeind JD, Kalachnik JE, Harder SR. 2000. Results of barbiturate
antiepileptic drug discontinuation on antipsychotic medication dose in individuals with
intellectual disability. Journal of Intellectual Disability Research Vol. 44 Part 2 pp 155-163.
2) Coulter DL. 1997. Comprehensive Management of Epilepsy in Persons with Mental
Retardation. Epilepsia 38(Suppl. 4): S24-S31.
3) Pellock JM, Morton LD. 2000. Treatment of Epilepsy in the Multiply Handicapped.
Mental Retardation and Developmental Disabilities Research Reviews 6: 309-323
4) Browne TR, Holmes GL. 2001. Epilepsy. N Engl J Med, Vol. 344, No. 15.
5) Alvarez, Norberto. 2001. Epilepsy in Adults with Mental Retardation. EMedicine
Journal, Volume 2, Number 1.
Key points:
1. Quality of life is an important primary consideration when making epilepsy
treatment decisions in individuals with mental retardation. 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.
2. 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 2/3 of the time, a specific epilepsy syndrome cannot be identified
though the seizures can usually be classified as partial or generalized.
3. Studies show that the vast majority of patients with MR and epilepsy can be
managed with monotherapy and that reduction and discontinuation of multiple
AEDs does not necessarily lead to deterioration in seizure control.
4. 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.
5796 Shelby Oaks Drive, Suite 11
Memphis, TN 38134
901-266-7276 888-276-9299 Fax: 901-266-3913
www.csnwt.org Email: inquiry@csnwt.org
5. AEDs are often used for treatment of maladaptive behaviors in individuals with
mental retardation. In the patient with concomitant epilepsy, care must be taken to
assure dosage adjustment that does not adversely affect one disorder or the other.
6. 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.
7. Respiratory problems are frequent in this population and may trigger increase in
seizure frequency. Raising AED doses may actually worsen seizures in this setting.
Discussion:
General
Epilepsy management in persons with mental retardation 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.
Assessing and responding to changes in seizure frequency also poses unique challenges in
this group of patients. Adults with mental retardation 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 neuralgic
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 MR and epilepsy. Drugs, which act as
CNS depressants may further, compromise swallowing function and the patient’s 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 a high degree of suspicion of possible occult infection when seizure
frequency increases with no change in AE drug or drug 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 MR
commonly take a number of routine medications many of which may interfere with or
potentiate the actions and/or side effects of AEDs. Multiple providers making avoidance of
complications even more challenging may prescribe these medications.
Finally, since 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 ADLs, should be
maintained.
5796 Shelby Oaks Drive, Suite 11
Memphis, TN 38134
901-266-7276 888-276-9299 Fax: 901-266-3913
www.csnwt.org Email: inquiry@csnwt.org
Approach:
1. 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, medications use
history, a listing of other medical problems, hospitalizations, etc. Direct care staffs
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.
2. Exams: For evaluation of therapeutic efficacy, an examination is often not
necessary. Furthermore, for CSN patients, reimbursement is not conditioned on the
patient being present. If an examination is required and difficulties are anticipated,
e.g. behavioral issues are present, request that familiar staff accompany the patient
and assist with obtaining compliance.
3. 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
interference with ADLs that have been experienced. Changes in sleep patterns,
difficulty eating, falls, or even respiratory infections may signal adverse effects of
treatment.
4. Medication management: Monotherapy is an important goal for a number of
reasons. First, these patients are often on multiple medications many of which can
interact with AEDs (changing blood levels of one or the other, additive side effects,
etc.). Second, these patients often cannot verbally report side effects. Third, many
of the common side effects of AEDs can lead to worsening of already compromised
ADLs. 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.
5. Behavioral issues: Since a significant percent of these patients 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.
**These practice guidelines have been developed by CSN to assist physicians and other healthcare professionals. Practitioners are encouraged to use the information
provided in the guidelines. It is not the intention of CSN to provide specific medical advice for particular patients and the recommendations contained in the
guidelines may not be appropriate for use in all circumstances. Any decision to adopt a particular recommendation must be made by the practitioner based upon
available facts and circumstances presented by individual patients.
It is not the intention of CSN in promulgating these guidelines to interfere with the provider/patient relationship, nor are these guidelines intended to represent the
standard of care in any given circumstance. These guidelines are recommendations to be used in the sole discretion of the provider and are not meant to dictate the
manner or style of clinical practice employed in rendering services to a particular patient.
5796 Shelby Oaks Drive, Suite 11
Memphis, TN 38134
901-266-7276 888-276-9299 Fax: 901-266-3913
www.csnwt.org Email: inquiry@csnwt.org
Questions for practice guideline on Epilepsy management in persons
with MR
1. The primary goal in treatment of epilepsy should be reduction to the greatest degree
possible in the number of seizures. True or false.
2. The best way to get information on seizure frequency and severity is to ask the staff
accompanying the patient at the time of the appointment. True or false.
3. Name two other chronic problems that are common in this population and which
may impact seizure frequency and/or seizure treatment decisions____________
________________________________________________________________
4. Name three other motor phenomena that occur frequently in this population and
which may mimic seizure activity_____________________________________
5. What are two ways that infections can affect seizure frequency______________
________________________________________________________________
6. What class of AED should be avoided due to adverse behavioral effects_______
________________________________________________________________
5796 Shelby Oaks Drive, Suite 11
Memphis, TN 38134
901-266-7276 888-276-9299 Fax: 901-266-3913
www.csnwt.org Email: inquiry@csnwt.org