CNNH Presents at ASET Neurotech Conference

Laura Szklarski, BS, R-EEG.T and Kimberly Catterall, R-EEG.T, Neurotechnologists at CNNH, presented a poster entitled “High Density Electroencephalography (HD-EEG) and Desensitization Techniques Improve Compliance Without Sedation or Restraint For Children and Adults with Behavioral Challenges,” at the American Society of Electroneurodiagnostic Technologists (ASET) 2016 Conference in Pittsburgh, PA last Thursday.

The poster educated the technologist community on High Density EEG and strategies to improve compliance for EEG with behaviorally challenged individuals without the need for sedation or restraint. The poster was extremely well-received and commended by the community, and it was evident that technologists are eager for strategies and assistance in avoiding the use of sedation and restraint for their patients. CNNH is a leader in providing quality neurodiagnostic testing for individuals who would otherwise be challenging and non-compliant in a typical hospital setting.

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The Epilepsy – Autism Connection

Under certain conditions, anyone can experience a seizure. However, individuals with neurodevelopmental disabilities and brain disorders are more prone to developing recurrent seizures, or epilepsy. Why?

What is Epilepsy?
Epilepsy is diagnosed when there are recurrent, unprovoked seizure events. Seizures occur when there is a sudden change in the electrical activity of the brain that disrupts normal brain functions. Seizures can take many forms. The more widely recognized types produce observable events, such as “convulsive” shaking, loss of body control, involuntary movements, and/or loss of awareness or consciousness. However, seizures can also be very subtle, for example disrupting a person’s thought patterns or memory but without noticeable external symptoms. There can also be unexpected “subclinical” electrical changes of the brain (particularly “spikes”), detected by electroencephalography (EEG, a device that records the electrical activity of the brain) monitoring, that are neither noticeable to the individual experiencing this phenomenon nor to observers. Unrecognized and untreated, epilepsy carries a risk of injury from seizures, including trauma, drowning, aspiration, and rarely, sudden unexpected death (SUDEP), as well as unfavorably affecting behavior, development, memory or learning. In those individuals with Autism Spectrum Disorder (ASD), having an associated diagnosis of epilepsy can increase the risk of mortality seven-fold.1

Is There an Autism-Epilepsy Connection?
It has been observed for many years that individuals diagnosed with ASD are at high risk of developing epilepsy. Various studies have placed the risk between 6% to 26% of children and adolescents, with a cumulative risk into the adult years reaching as high as 67%.2 Overall, the risk of epilepsy is higher in ASD if there are associated intellectual disabilities or Cerebral Palsy.3 Why is there this connection? Generally, any brain disorder will increase the predisposition and lower the threshold for generating seizures. Additionally, ASD and epilepsies appear to share common biological and anatomical mechanisms. Advances in biotechnologies have revealed that there are many types of genetic variations causing or contributing to both ASD and the generation of seizures. Innovations in neuroimaging tools and neuropathological studies have revealed “disconnectivity” of various brain circuits leading to both autism and epilepsies. Various prenatal, neonatal and childhood metabolic, infectious and inflammatory disorders have been implicated in both ASD and epilepsy. Thus, understanding the epilepsy-autism connection is providing clues to the causes of ASD.

How is Epilepsy Diagnosed in ASD?
The behavioral and developmental complications of ASD can obscure or mimic the clinical manifestations of seizures; thus, the recognition of seizures is not always apparent in this population. Oftentimes children and adults with ASD do not have classical seizure manifestations. An EEG is necessary to determine if there are clinical or subclinical seizures, or abnormal electrical discharges that might be interfering with brain function or present a high risk for recurrent seizures. Language regression can signify a severe form of an “epileptic encephalopathy” (“Landau-Kleffner” and other related syndromes). Such individuals have very abnormal EEGs, with abnormal electrical patterns worsening with sleep but usually without observable clinical events. Infantile seizures associated with fever can sometimes evolve to autism, and, like other epilepsy syndromes, can be associated with genetic defects of “ion transport” (ions include sodium, calcium, potassium, magnesium). Some individuals with ASD and epilepsy will have “drop attacks” (a sudden loss of motor/body control causing a person to drop their head or fall to the ground) and their EEG will have a specific electrical pattern (“Lennox-Gastaut Syndrome”). Other individuals might only have a staring spell, have unwitnessed seizure events during sleep, or may have classic convulsions.

Overall, since epilepsy is a diagnosable condition with important “actionable” clinical implications, clinical evaluations of individuals with ASD should include screening and assessment of seizure risk and susceptibility. In addition to a comprehensive medical and neurological history and examination, there are other neurodiagnostic tests that a specialist may consider or recommend, including:

EEG: This device measures the activity of brain cells (“neurons”) in real-time. Conventional EEG can be difficult to obtain in ASD since the application of the approximately 20 electrodes with glues/pastes/scalp abrasion can be traumatic in those with behavioral challenges. Newer “dense-array” EEG technologies allow for application of many more electrodes (128 sponge electrodes) using a specially designed “sensor net” that is applied in minutes without discomfort or risk of infection.

Neuroimaging: Magnetic Resonance Imaging (MRI) is the study of choice to assess for significant structural abnormalities or malformations of the brain. Other neuroimaging technologies (positron emission tomography [PET], single-photon emission computerized tomography [SPECT], MR Spectroscopy [MRS], diffusion tensor imaging [DTI]) can be useful in select cases.

Laboratory Evaluations: There are wide arrays of relevant laboratory tests of the blood, urine, and on occasion cerebrospinal fluid (CSF) or skin/muscle tissue, which can help identify a biological cause of epilepsy and/or ASD.

Genetics: There has been an explosion of the availability of advanced genetic testing technologies, which are transforming the understanding, diagnosis and treatment of epilepsy and ASD. In many cases, the sequencing of thousands of “genes of interest” can be performed on a saliva sample. Genetic testing can also provide information concerning risk, susceptibility and prevention of complications of epilepsy and ASD, sometimes with implications for other family members.

Neuropsychological Evaluation: Both epilepsy and ASD can have profound effects on neuropsychological function. Neuropsychological evaluations include a series of empirically validated, norm-referenced, and evidence-based tests that quantitatively and qualitatively measure brain function, processing and output.

How is Epilepsy Treated?
Treatment must be individualized and personalized, and include programs to encourage healthy lifestyles (good sleep patterns, nutrition, physical activity, and stress reduction). Therapeutic strategies should target identifiable biological mechanisms and triggers of epilepsy, and simultaneously attempt to relieve seizure burden. Therapies should attend to medical and neurological/neuropsychological complications of epilepsy and/or medications, involve preventative strategies for risk reduction and SUDEP, and provide for a “rescue” plan in cases of acute seizure events. The mainstay for limiting seizure recurrence and promoting spike suppression are antiepileptic drugs (AEDs). In cases of identifiable metabolic and genetic disorders, nutritional supplements or dietary alterations may be indicated. Other options include specific diets such as the ketogenic and related diets (high protein and fat, and minimal carbohydrates), neurostimulation (devices that deliver periodic electrical currents, such as the vagal nerve stimulator [VNS] and other modalities) and “seizure surgery” (the localization and removal of brain tissue identified as the source of seizure generation). Research into the common biological sources of epilepsy and autism may lead to new and novel therapies that propose to alter the course of the disease.

In most cases, epilepsy is a treatable condition. Through proper screening and diagnosis, and the utilization of pragmatic therapies and interventions, the quality of life for children, adolescents and adults who may be suffering from epilepsy and ASD can be improved.

References

Pickett J, Xiu E, Tuchman R, et al. Mortality in Individuals with Autism, with and without Epilepsy. J Child Neurol 2011; 26:932-939.

Amiet C, Gourfinkel-An I, Bouzamondo A, et al. Epilepsy in autism is associated with intellectual disability and gender: evidence from a meta-analysis. Biol Psychiatry 2008; 64:577-582.

McVicar KA, Ballaban-Gil K, Rapin I, et al. Epileptiform EEG abnormalities in children with language regression. Neurology 2005; 65:129-131.

Suren P, Bakken IJ, Aase H, et al. Autism spectrum disorder, ADHD, epilepsy, and cerebral palsy in Norwegian children. Pediatrics 2012; 130:e152-158.

Bolton PF, Carcani-Rathwell I, Hutton J, et al. Epilepsy in autism: features and correlates. Br J Psychiatry 2011; 198:289-294.

Viscidi EW, Triche EW, Pescosolido MF, et al. Clinical characteristics of children with autism spectrum disorder and co-occurring epilepsy. PLoS One 2013; 8(7):e67797.

Woolfenden S, Sarkozy V, Ridley G, et al. A systematic review of two outcomes in autism spectrum disorder – epilepsy and mortality. Dev Med Child Neurol 2012; 54:306-312.

By Mark Mintz

The Autism-Epilepsy Connection

Learn about the connections between Autism and Epilepsy in this webinar recording. Presented by Mark Mintz, MD, pediatric neurologist and president, ceo and founder of CNNH.

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Where Does Epilepsy Come From?

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Get a deeper understanding of epilepsy, its causes and treatments, in this webinar recording. Presented by Mark Mintz, MD, pediatric neurologist and president, CEO and founder of CNNH.

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All About HD – EEG Part 2

When people tell me that they’re considering going to a major medical center for a conventional EEG, I ask them, ‘Do they have High Density EEG?’ And when they respond, ‘What’s that?’, I proceed to tell them all the things that make High Density EEG (HD-EEG) so special for our patients…

  1. Ease of use: The electrodes in the HD-EEG system are easy to apply and do not involve any painful or potentially adverse procedures. There is no scrubbing of the scalp, no pastes used for electrode attachment to the scalp, nor is there a need to wash your hair after the exam is complete—all typical of traditional EEG. Our advanced HD-EEG system uses elastic nets to hold the electrodes in place, not paste, and it uses sponges, not metal disks, for electrical pickup.
  2. Lack of restraint: Even when dealing with non-compliant patients, adequate data can be obtained without the use of restraint or sedation. The HD-EEG electrodes go on something like putting on a swimming cap: gentle and painless. In just a couple of minutes they are on and ready to go. At CNNH, we have been successful obtaining HD-EEG’s from highly non-compliant children without ever needing to use sedation or restraint. During a conventional EEG exam, it can take 20 minutes or more to mark up your child’s head, scrub clean the sites and glue the electrodes to the scalp. Even compliant children sometimes need sedation just to get the electrodes placed, and it can take a full day for the effects of sedation to wear off.
  3. More electrodes: More electrodes mean more data and a better chance of detecting even highly localized brainwave “spikes”. Conventional EEG uses 18-24 electrodes. HD-EEG uses 128. This gives us six times as much data, data that we use to more finely examine brain activity and better determine the source of any abnormalities.
  4. Coverage: The HD-EEG net goes around the head and has some coverage of areas below the brain, areas that are not covered in conventional EEG systems. It is not only the number of electrodes that is important—it is where they are placed. Because of the way the cells of the brain are lined up, their activity results in an electric field. The field has a positive and a negative end.  This is referred to as a dipole. Because the surface of the brain is wrinkled, this dipole can be pointing in almost any direction, depending on exactly where in the brain it is located. As you might imagine, it is quite a difficult problem to figure out where the activity is occurring in the brain. Having electrodes down the sides of the head means we get a much better picture of both ends of the dipole. If you look at the placement of electrodes on a conventional EEG, you will see that they basically cover only the top of the head. Only seeing half the signal, they simply cannot give as good an answer as to where in the brain abnormal activity is occurring.

All About HD – EEG Part 1

HD-EEG stands for High Density Electroencephalogram. ‘Electro’ is easy enough to understand, it refers to electricity.  ‘Encephalo’ refers to the brain.  As for ‘gram’, this ending means that it is drawn (before computers, the EEG was on paper) or recorded (as it is now, by being saved to a computer’s hard disk).  So by ‘EEG’ what we mean is ‘electric-brain-drawing’.  However, we are not really drawing a picture of the brain – we are drawing a picture of the activity of the brain.  Electroencephalogram and electroencephalography are long words and it’s easy to get caught up in trying to pronounce it, so we simply refer to it as EEG.

Now, the ‘HD’ stands for ‘High Density’. This refers to the fact that unlike a conventional EEG which covers the head with 18-24 electrodes, High Density EEG has 128 ‘electrodes’ for a high-resolution picture of brain activity. That ‘electrode’ part can be unsettling, but it ought not to be. At CNNH (and most everywhere else), no electricity is used in the equipment that gets placed on the head.  The sensing part of the equipment, the  ‘electrodes’, passively pick up the activity of your brain, much like a camera does with light when someone takes a picture of you.  You could call a camera a ‘light –camera’ because it senses light.  Similarly, you can think of an electrode as an ‘electric –camera’ because it senses electricity.  Our HD-EEG electrodes are simply a wire connected to a tiny plastic disc, with a wet sponge attached to the end. 

With HD-EEG, there is NO scraping of the scalp, NO shaving of hair or any of the discomforts experienced with conventional EEG testing. Even our most sensitive patients wear the HD-EEG nets without the need for sedation. The sponge simply rests on the head and is wetted with a mixture of slightly salty water and a little baby shampoo. We need to use water on the little sponges because water helps focus the brain signals so that they can be carried across the sponge to the wire. For best results from your HD-EEG, don’t use hair products before you come in as they could interfere with the signals from your brain. Also, as I mentioned, we use the wet sponge to focus the signal.  So, if you come in with your hair wet – it won’t work!  So no hair products, and make sure you hair is dry.  Then you are good to go!

Epilepsy and Daycare

From the Desk of Mark Mintz, MD

One of the burdens of having Epilepsy is the unpredictability of a sudden convulsive seizure (i.e. an event that involves loss and/or impairment of consciousness often associated with uncontrollable muscle stiffness or jerking/shaking movements). When convulsive seizures are prolonged, they can be damaging or life-threatening. Most convulsive seizure events can be stopped with the administration of specific short-acting medications (usually in the family of medications known as benzodiazepines). For many years, the only way to gain access to such medications was by emergency/ambulance personnel or in an emergency room setting. Major advances in the treatment of convulsive seizures occurred when a rectal diazepam gel (Diastat) was approved in 1997. The reason for rectal administration is that when someone is in the midst of a convulsive seizure, it is dangerous to administer anything by mouth because of the danger of choking and aspiration. Also, specially prepared medications administered rectally are absorbed into the bloodstream in a matter of minutes, which is an important aspect of medications given for seizure control. With proper training, rectal diazepam gel can be administered by lay people safely and easily. With the use of rectal diazepam gel, people with Epilepsy can avoid ambulances and emergency room visits, can travel without fear of inadequate access to emergency medical services, and often can reduce the risk of adverse effects of prolonged seizures.

However, despite such advances and freely available education and training about Epilepsy, individuals with Epilepsy (and their families) still encounter discrimination in many sectors of our society. Fortunately, the courts have enforced the protections afforded in the Americans with Disabilities Act (ADA), with a recent case illustrating this point. In “The United States of America v. Toddlers ‘N Tots, Inc”, a three year old was denied admission to a daycare program because of his Epilepsy and the need for daycare staff to be trained in administering rectal diazepam gel. The court decision found that the daycare’s decision was a violation of ADA, and they ordered the child to be admitted to the daycare program and that the daycare staff submit to proper training on epilepsy, seizures and the proper use/administration of diazepam rectal gel (the training to be done by the Epilepsy Foundation of New Jersey).

For many patients there is understandable apprehension about the use of diazepam rectal gels, particularly in public situations. Although not yet obtaining FDA approval, many clinicians will prescribe certain intranasal benzodiazepines, such as lorazepam or midazolam, for prolonged or cluster seizures. Intranasal midazolam is presently available through research protocols for cluster seizures. There have also been clinical trials for intramuscular auto-injectors of diazepam for acute repetitive seizures, but no study results have been posted and there is no FDA approval as yet.

By Mark Mintz

Good News for NJ Drivers with Epilepsy!

From the Desk of Mark Mintz, MD

People with Epilepsy are subjected to significant restrictions and regulations concerning their ability to obtain and maintain a driver’s license. The regulations and requirements vary state by state which is unfortunate since the manifestations of Epilepsy do not change across state lines. The intent of these driving laws is to protect the individual as well as passengers and other vehicles on the road. Nevertheless, certain states such as New Jersey have had very restrictive qualifications and administrative codes. When the laws are overly repressive they can adversely affect the quality of life for persons with Epilepsy by impairing independent travel and transportation, affecting employability, or inhibiting individuals from reporting seizure events that secondarily interfere with proper neurological care.

But there is Good News! After much lobbying and testimony, the New Jersey Motor Vehicle Commission (NJMCV) has taken a step in the right direction by easing a restrictive aspect of the administrative code. For many years, individuals with Epilepsy would have had their driving privileges suspended for one year after any seizure event. As of November 19, 2012, this waiting period has been reduced to a more sensible six months. This change will help to improve the quality of life for those enduring the vagaries of Epilepsy and other conditions that cause recurrent periods of impaired consciousness or impairment or loss of motor coordination.

N.J.A.C. 13:19-5.1

NEW JERSEY ADMINISTRATIVE CODE

Copyright (c) 2013 by the New Jersey Office of Administrative Law

*** New Jersey Register, Vol. 45, No. 2, January 22, 2013 ***

TITLE 13. LAW AND PUBLIC SAFETY
CHAPTER 19. COMPLIANCE AND SAFETY
SUBCHAPTER 5. CONVULSIVE SEIZURES
N.J.A.C. 13:19-5.1 (2012)

§ 13:19-5.1 Satisfaction of physical qualifications

Any person 16 years of age or older who suffers or who has suffered from recurrent convulsive seizures, recurrent periods of impaired consciousness, or from impairment or loss of motor coordination due to conditions such as, but not limited to, epilepsy, in any of its forms, shall, as a prerequisite to the issuance of a learner’s permit or driver’s license, renewal of a driver’s license, or retention of a driver’s license, establish to the satisfaction of the Chief Administrator that he or she has been free from recurrent convulsive seizures, recurrent periods of impaired consciousness, or from impairment or loss of motor coordination for a period of six months with or without medication and that he or she is physically qualified to operate a motor vehicle.

HISTORY:
As amended, R.1981 d.18, effective January 16, 1981.
See: 12 N.J.R. 606(a), 13 N.J.R. 150(b).
Freedom from impairments amended from two years to one.
Amended by R.2005 d.47, effective February 7, 2005.
See: 36 N.J.R. 4005(a), 37 N.J.R. 505(a).
Substituted “Chief Administrator” for “Director” and inserted “or she” following “he”.
Amended by R.2012 d.193, effective November 19, 2012.
See: 44 N.J.R. 1696(a), 44 N.J.R. 2957(a).
Inserted a comma following both occurrences of “consciousness”, “shall” and the last two occurrences of “driver’s license”, and substituted “six months” for “one year”.

Learn more about the New Jersey Administrative Code (N.J.A.C.) 13:19-5.1 (2012) here

By Mark Mintz