While an estimated 1.8% of US adults have epilepsy, the prevalence is more than 22% among people with intellectual disabilities.1,2 Managing epilepsy in this patient population can be particularly challenging for a variety of reasons.
“This group will have a higher likelihood of communication problems and other issues that make it more difficult to be treated than patients with a higher IQ, and they tend to have more behavioral issues,” Scott Hirsch, MD, a neuropsychiatrist at NYU Langone Comprehensive Epilepsy Center and assistant professor in the departments of neurology, child and adolesecent psychiatry, and psychiatry at NYU Langone Medical Center, told Neurology Advisor. “You’re also likely to see more of every kind of mental illness in this group, including autism, ADHD, schizophrenia, and depression,” he said.
Generally, around 70% of patients with epilepsy respond to treatment and have good outcomes, while 30 to 40% are treatment resistant, according to Rohit Shankar, MRCPsych, a neuropsychiatrist and researcher with the Cornwall Partnership NHS Foundation Trust in the United Kingdom. Among patients with comorbid epilepsy and intellectual disabilities, “those who do not respond to treatment tend to have worse outcomes,” he told Neurology Advisor.
Adding to the clinical difficulty is the lack of guidelines pertaining to best practices for this group. In a review conducted by Dr Shankar and colleagues published in the European Journal of Neurology, the researchers found a dearth of reliable research to inform safe prescribing practices of antiepileptic drugs (AED) for people with intellectual disabilities.3
“It is quite surprising that no one has tried to understand this population and its diverse needs,” he said. Such diversity is an often overlooked point, he notes: “Patients in this population have been treated like a homogeneous subset,” when, in fact, there is a wide variety of presentation in this group.
On one end of the spectrum are patients with mild disability who are able to manage activities of daily living with little support. Approximately 10% to 12% of this group has epilepsy, and treatment is mainly focused on areas like compliance and risky behaviors, Dr Shankar explained. At the other extreme are patients with profound disability, whose needs are much more complex. They are more likely to have additional genetic conditions and structural brain damage, and they require constant care. Up to 50% of this group has epilepsy, and their treatment is more focused on factors such as the impact of medications, recognition of side effects, and informed consent. There may be an added layer of complexity in patients with epilepsy who also have a co-occurring intellectual disability and mental illness. “For instance, you may try to give medication to a patient with schizophrenia and intellectual disability, and they might think you’re trying to hurt them,” said Dr Hirsch.
Each person brings to the equation his or her “own level of understanding and ability to make informed choices around their care,” Dr Shankar said. He cautions practitioners to avoid lumping patients with intellectual disabilities together and emphasizes that a person-driven approach is key. With that considered, he said it is important to adhere to best practice guidelines for epilepsy as closely as possible when treating patients with both epilepsy and intellectual disabilities, though adjustments are sometimes necessary. “In certain situations, for example, it would be extremely difficult to get an MRI scan,” he said. In that case, the clinician would need to document the reason it was not possible and the steps that will be taken instead.
In the UK, there is legislation requiring healthcare and public service providers to make “reasonable adjustments” to ensure that people with disabilities have the “ability to participate and are given a level of dignity to get on board with their care as much as possible,” Dr Shankar said. This might include the availability of ready-made informational materials that are easy to understand – such as the use of larger letters or less complex language, or it could involve the use of a speech and language therapist to help facilitate communication. “Part of the challenge in diagnosis and treatment is that patients who can’t verbalize or recognize what they are feeling may be likely to get aggressive,” Dr Hirsch explained. “They can go from crying right to violence as a means of expressing this turmoil they feel inside.”
It is essential to first “get a good neuropsychiatric evaluation so you’ll have a good sense of the patient’s ability to communicate and comprehend” information relevant to their care, he said. If a patient does lack the mental capacity to make informed choices about their care, it becomes especially important for the clinician to partner with key stakeholders in the patient’s care.
“A lot of times clinicians will make unilateral decisions, which can lead to a loss of faith and confidence in them,” and it can also cause confusion about the patient’s clinical picture, Dr Shankar said. It is important to define seizure improvement for the patient, with agreement on the method of monitoring and reporting using consistent jargon. “It’s about having a clear structure and defining clear outcome.” For example, if a patient has 5 different types of seizures, it may be best to initially focus on the most frequent or severe type.
Providers often need to allow more clinic time for patients with comorbid epilepsy and intellectual disabilities: While 15–20 minutes might suffice for a typical patient encounter, a patient with an intellectual disability – and their family or caregiver – might require 30 to 45 minutes of consultation. Beware of the tendency of health care providers to over-prescribe pharmaceutical drugs to patients with intellectual disabilities, who are “more likely to be on higher doses and more medications overall,” Dr Hirsch warned. As long as appropriate safety measures are in place in the patient’s environment, Dr Shankar said, “We start very slow and very low” with medications. This pacing provides the opportunity to determine if the patient can tolerate the drug, and it helps earn the confidence of the patient and caregivers.
With patients who have intellectual disabilities, “clinicians tend to treat the epilepsy,” said Dr Shankar, who is currently helping to develop guidelines on AED prescribing in this patient group. “Our motto is ‘treat the person.”
- Epilepsy Fast Facts. Centers for Disease Control and Prevention. Updated February 2, 2016. Accessed May 12, 2016. http://www.cdc.gov/epilepsy/basics/fast-facts.htm
- Robertson J, Hatton C, Emerson E, Baines S. Prevalence of epilepsy among people with intellectual disabilities: A systematic review. Seizure. 2015; 29:46-62.
- Doran Z, Shankar R, Keezer MR, et al. Managing anti-epileptic drug treatment in adult patients with intellectual disability: a serious conundrum. Eur J Neurol. 2016; doi:10.1111/ene.13016
This article originally appeared on Neurology Advisor