Prognosis in Infantile Spasms – #EpilepsyNYC Infantile Spasms Awareness Week

Summary:

– The overall intellectual and neurodevelopmental outcome in children with infantile spasms is generally poor
– The long-term prognosis is directly related to the underlying cause of the seizures
– Better developmental outcomes are noted in patients without an identified cause (cryptogenic/idiopathic)
– Although the spasms tend to resolve by early to mid childhood, a majority of the children develop other types of seizures and epilepsies.
– There is a better outcome noted in those patients treated earlier in the course of their seizures, and in those that have an early and sustained response to treatment.

Infantile Spasms

The long-term prognosis of patients with infantile spasms has been studied extensively over the years. Many studies have examined the outcome of infantile spasms including neurodevelopmental and intellectual disabilities, prognostic significance of the EEG, and the development of chronic epilepsy.
The consensus of all of these studies is that the overall outcome, with regards to chronic epilepsy and neurodevelopmental disability is generally poor in patients with infantile spasms.

Chronic Epilepsy

Epileptic spasms usually resolve, latest by mid-childhood, in the majority of patients. Infrequently, spasms may persist in adulthood. Even if the spasms resolve, it has been found that 50-70% of the patients will develop other seizure types. Approximately 50% of the patients with a history of infantile spasms go on to develop chronic intractable epilepsy. There is a strong relationship seen between a history of infantile spasms (IS) and development of Lennox- Gastaut syndrome, with 18-50% of IS patients developing Lennox-Gastaut syndrome.

EEG

While features of hypsarrhythmia define the EEG of patients with infantile spasms, evidence of this abnormality on the first EEG was seen not to influence the response to treatment. However, if the hypsarrhythmia persisted despite treatment, or was atypical and/or asymmetrical, this was seen to adversely influence the long-term outcome.

Neurodevelopmental disability

The most important factor in predicting the cognitive and developmental outcome of patients is etiology.

Mental retardation occurs in about 70 % of patients with infantile spasms, usually involving severe to profound retardation and often associated with other psychiatric problems. Other neurological deficits, such as cerebral palsy, may be seen in about 30-50% of patients.
Most children with infantile spasms experience slowing, plateauing, or regression of their developmental progress.

Studies have consistently shown that infants with symptomatic infantile spasms are at much higher risk (86-90%) of developing mental retardation, autism and other behavioral and cognitive disabilities, compared to infants with idiopathic infantile spasms. Symptomatic infantile spasms, in particular, are associated with autism spectrum disorders (ASD) in up to 35% of cases.
10-20% of patients with normal development prior to the start of their spasms will have normal mental function or only be mildly impaired long-term. 28-50% of patients with idiopathic infantile spasms will go on to have normal or borderline cognitive development.

Mortality

The premature death rate for infantile spasm ranges from 5-31%. Most of the deaths (61%) occurred at or before age 3 years, while only 10% occurred after age 20 years.

In most cases, the mortality is seen to be a result of the underlying neurological cause of the infantile spasms.

Factors for a favorable outcome in Infantile Spasms

• Cryptogenic or idiopathic cause
• Age of onset of spasms > 4 months
• No atypical or partial seizures
• Typical EEG abnormalities – hypsarrhythmia
• Early onset of treatment
• Early and good response to treatment

Q&A with Patricia McGoldrick, NP, MPA, MSN

NEXT UP: Our 1st Community Spotlight featuring Team Gabriella & The Epilepsy Road Warriors

 

The team at EFMNY would like to thank you for your questions! After each post, we’ll post answers from our experts to the most frequently asked questions we receive.  Please note that these Q&A post, like our provider articles, should not be taken as medical advice.  Each patient is unique.  For medical advice regarding your specific condition, please consult your doctor.

 

Q&A with Patricia McGoldrick, NP, MPA, MSN:

1. I just found out that one of the medications I’ve taken for years is available in generic form. If it’s only one of the two medications I take, is there less risk in trying the generic?

This is difficult to answer without knowing which medications you are taking. Some of the medications in generic form are made by the same companies that make the brand name medications and so are consistent. The answer depends on the medications, the doses, how long you have been seizure free and a host of other issues. Please ask your provider, as he or she knows you best!

2. My daughter had surgery earlier this year. We were hoping that she would be medication free. However, she is still on meds. Is this typical for kids who have had epilepsy surgery?

 The type of surgery, the medications, the length of time after the surgery and the cause of the epilepsy must all be taken into consideration, as well as the EEG findings and the post-operative MRI findings. Typically, medications are considered for some period of time after the surgery. Again, this is a great question for your provider! (more…)

Switching Seizure Medications: Part II

How soon should medications be changed?

The typical rule of thumb is that medications should be switched if they are ineffective.  Once a person has been on the appropriate medication for their type of seizures, and has reached the appropriate or maximum dose and is still having seizures, the medication should be changed.  Usually a second medication is added and then “titrated up” (that is adjusted in a step-wise progression) until seizure control is reached or the maximum dose of the second medication is achieved. If seizures are under control, consideration should be given to weaning the first medication.  Having said that, there are often instances when seizure control is reached with two medications used in combination and the person with epilepsy remains on two medications.  The risk of seizures and injury must be weighed against the risk of side effects.

Should medications be changed if there are side effects?

The important thing to remember about side effects is that they MAY occur, not that they will!  Medications are tested in clinical trials in large groups of persons.  Every event that occurs during the time of the trial is reported as a side effect, even if the event occurs only in one or two persons and is not conclusively determined to be a direct effect of the medication.

Often side effects occur when the medication is first initiated and may lessen or resolve over time as the person’s body “becomes used to the medication.”  A prime example is the side effect of drowsiness that occurs with oxcarbazepine and usually resolves within a week or two.  Another is the abdominal upset that can occur with ethosuximide and is managed by eating before the medication is taken.  On the other hand, intolerable side effects should result in changes of medications. (more…)

Q&A with Ruth Shinnar, RN, MSN

NEXT UP: Part I of “Switching Seizure Medications”

 by Dr. Fred Lado

 

The team at EFMNY would like to thank you for your questions! After each post, we’ll post answers from our experts to the most frequently asked questions we receive.  Please note that these Q&A post, like our provider articles, should not be taken as medical advice.  Each patient is unique.  For medical advice regarding your specific condition, please consult your doctor.

 

Q&A with Ruth Shinnar, RN, MSN:

 

1. I’ve heard that switching between brand drugs and generics can affect a change in seizure control and patterns. Is that true? Should I be concerned about using generics?

Switching between brand drugs and generics usually does not have an effect on seizure control. Unfortunately, there is no way to determine who will be affected by switching. The Federal Drug Administration (FDA) has set manufacturing guidelines for makers of generic drugs. The generic drug manufacturer must prove its drug is the same as (bioequivalent) the brand name drug. When a drug, generic or brand name, is mass-produced, very small variations in purity, size, strength, and other parameters are permitted. FDA limits how much variability is acceptable. Most people will not be affected by this variability but some are. If you are one of these people you can be prescribed the brand name and provide evidence of need to your insurance company with the help of your health care provider.

2. My daughter has experienced significant weight gain since she started taking seizure meds. While we recognize her concern, my husband and I are afraid that switching her medications will trigger more seizures. Do you have any recommendations for teens with epilepsy who have weight issues? 

Healthy eating habits and regular exercise are encouraged for everyone but medications can cause weight gain even when eating healthy. If the weight gain is felt to be from the antiepileptic medication a change may be beneficial. There are ways to safely switch over to another medication and you can discuss how to do this with your daughter’s health care provider.

3. Although my seizures are well controlled with my medication, I do get headaches more often that I used to. Is this typical? Should I talk to my doctor?

 Headaches can be a side effect from antiepileptic medications, although it is uncommon. Your neurologist can perform a headache evaluation and should be able to determine whether your medication should be changed. (more…)

When Seizure Types Change: Part II

Treatment

If in fact an evaluation has been made that confirms your seizure type has changed you may need to change medications. If you are now having seizures when they had previously been controlled it may mean that the medication you were taking does not work for the type of seizure you are now having.

A thorough evaluation is needed, as discussed in the previous blog, which will provide information about your new seizure type, why you are having a new seizure type, what this means in terms of controlling your epilepsy; and it will aid in choosing treatment.

Medications are usually the first line of treatment for seizures. They are chosen based upon seizure type. Your medication may need to be changed or a new medication may need to be added if your seizure type has changed.

Other things to think about when choosing a new medication are:

• previous response to medications of the same class,
• your age,
• gender,
• weight, allergies,
•  and possible interactions with other medications that you are taking.

  (more…)

Q&A with Dr. Shahin Nouri

NEXT UP: Part II of “When Seizure Types Change”  by Ruth Shinnar, RN, MSN

 

The team at EFMNY would like to thank you for your questions! After each post, we’ll post answers from our experts to the most frequently asked questions we receive.  Please note that these Q&A post, like our provider articles, should not be taken as medical advice.  Each patient is unique.  For medical advice regarding your specific condition, please consult your doctor.

 

Q&A with Dr. Shahin Nouri:

 

 1. My son had absence seizures for the better part of five years. Our doctor recently diagnosed him as seizure-free at the age of 13.  He is eager to go off of his medication, but we’re afraid it will lead to a return of his seizures. How is it determined that one is “seizure-free.”  Is this the same as being “epilepsy-free?”

Absolutely! Absence epilepsy is a condition of childhood and adolescence. The Majority of people with absence so to say “grow out it”.  Only rarely will absence seizures accompany a person into adulthood. So , depending on your child’s age, he can become seizure-free.

However, it is possible that someone has epilepsy, and a longer EEG shows abnormal electrical activity. In the case of absence seizures, these electrical events need to be up to 10 seconds long before a clinical manifestation, in other words a seizure comes to light. Therefore, as long as your child hasn’t had any clinical seizures, and a longer EEG is unremarkable, it is safe to assume he has grown out it, and therefore is epilepsy-free.

As a general rule, after two years of seizure freedom and a normal EEG and imaging (MRI)  it is possible in most patients to attempt weaning off anti-seizure medications.

2. To the best of my knowledge, my husband has been without seizures for a little over two years. He used to have convulsions.  One of his co-workers recently told me that he’s been “spacing out” a lot at work.  Could this be what you refer to as an absence seizure?

Episodes of staring in adults are most probably Complex Partial Seizures and not absence seizures. Absence is an uncommon variety of Primary Generalized Epilepsy and only very rarely accompanies a person into adulthood. More frequently, people have Localization Related Epilepsy that can cause partial seizures. When partial seizures cause change in alertness, they are called Complex Partial Seizures. (more…)

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