Incorporating tech in a comprehensive LGS treatment plan

By Naveed Saleh, MD, MS | Medically reviewed by Moody Kassem, MD, MBA
Published September 27, 2023

Key Takeaways

  • Advances in technology can help with the monitoring and the treatment of high-risk seizures in patients with Lennox-Gastaut syndrome (LGS).

  • Seizure monitoring devices connect to smartphones to allow parents or other caregivers to help their loved ones in real time, as well as provide important information to HCPs.

  • Remote monitoring can help guide clinical decision-making, and the benefits and drawbacks of potential technological interventions should be carefully weighed with the parents of your patients with LGS.

Both children and adults with LGS are at high seizure risk and exhibit frequent seizures. These individuals may also lack the capability of sensing danger or can wander from their homes/schools, thus placing them in further danger if not properly supervised. 

Technological options are now available to not only assist with seizure control but also ensure safety in patients who experience seizures, according to authors writing for the LGS Foundation.[] Here is an overview of some of these options. 

Video monitoring

Owing to the advancements in smartphone and internet technology, video surveillance offers the capability for parents and caregivers to engage in live video monitoring with the potential to detect hazards.

The advantages of employing this technology are particularly notable when family members are preoccupied, for instance, during meal preparation or while tending to domestic tasks in various areas of the home.

Seizure monitoring

Seizure monitoring devices offer caregivers a valuable means to promptly detect seizures as they occur in real time. These devices operate by sensing motion, providing a crucial layer of vigilance for patients living with LGS. Several types of seizure monitoring devices are currently available in the market, including mattress-based systems, camera devices, and smartwatches. However, it's essential to acknowledge the limitations of these devices.

Seizure monitoring technology primarily focuses on detecting seizures characterized by significant movements. Seizures without pronounced motor activity, such as absence or partial/focal seizures, may not trigger these devices.

Moreover, it's critical to understand that no seizure device has been developed to prevent seizures or SUDEP (Sudden Unexpected Death in Epilepsy). The efficacy of seizure alert devices still requires more rigorous scientific evaluation. While some studies have examined these devices systematically, their real-world performance and their status with regard to FDA approval remain uncertain.

In addition to electronic monitoring, some families opt for alternative methods, including pulse oximeters or specially trained seizure dogs, to keep a watchful eye on their loved ones with LGS. Pediatric neurologists play a pivotal role in guiding families through the selection of monitoring technologies, carefully weighing the pros and cons of each option and providing tailored advice based on individual patient needs.

Related: Research update: The latest news in LGS treatment

One notable wearable device in this domain is SAMi, The Sleep Activity Monitor. SAMi offers a practical solution for parents, caregivers, and individuals who need to monitor potentially hazardous movements during sleep or rest. This wearable device is compatible with Apple products and allows for live streaming of patient data. It also permits the customization of alarm thresholds across multiple devices, enabling caregivers to respond promptly to any concerning events.[]

Healthcare providers should encourage patients to share automatically recorded information from these monitoring devices.

This collaborative effort helps physicians gain insights into nighttime motion patterns, identify emerging behaviors, assess changes over time, and establish the frequency and duration of seizure events. Such data-sharing can significantly aid in refining treatment plans and optimizing care for individuals living with LGS.

Vagus nerve stimulation

Vagus nerve stimulation (VNS) is an established and effective therapeutic approach for managing drug-resistant epilepsy, particularly in patients who are not responsive to other treatment options. This medical intervention involves the implantation of a device that stimulates the vagus nerve—a cranial nerve that plays a critical role in regulating various bodily functions, including seizures.

Numerous clinical studies and real-world evidence have shown that VNS can significantly reduce seizure frequency and improve seizure control in a substantial number of patients with epilepsy.[]

The authors of an article in Seizure, reporting on the use of vagus nerve stimulation (VNS), give some background on this therapy.[]

The first vagus nerve stimulator was approved in 1997 and indicated for drug-resistant epilepsy. This form of epilepsy is defined as persistent seizures within a 12-month period during which the patient with epilepsy receives treatment with at least two indicated and adequately dosed and tolerated antiseizure medications. 

In 1997, 1999, and 2013, the American Academy of Neurology reviewed VNS therapy. Overall, up to 82% of epileptic seizures are linked to increases in heart rate, thus providing the rationale for auto-stimulation models for VNS. Auto-stimulation models have detect-and-respond modes that respond to the detection of ictal tachycardia by delivering extra stimulation to the vagus nerve. 

“Like the older VNS models, in addition to the auto-stimulation paradigm, these devices include a standard mode that allows care takers to stimulate with a magnet over the device, triggering a pulse of slightly higher current intensity over the baseline pulses,” the authors wrote. “This additional pulse is designed to disrupt the epileptic discharge, thereby ending the clinical seizure.” 

The FDA has greenlighted the utilization of VNS as an adjunctive therapy in adults and children greater than 4 years old with partial onset seizures that are refractory to medications.

Two auto-stimulation VNS models are currently on the market: the FDA-approved Aspire SR VNS model and the SenTiva model. In clinical trials, VNS stimulation in the context of LGS has proven effective, with newer models of the Aspire SR distinguishing themselves, and a majority of patients experiencing >50% seizure improvement.

The study of VNS published in Seizure was a retrospective chart review of 71 children with LGS who received treatment with either the Aspire SR  or the SenTiva model of auto-stimulation VNS. The results showed that  >50% seizure reduction was achieved by 55% of the patients at 6 months, by 67.7% at 12 months, and by 65% at 24 months.

Moreover, at 12 months, 11% were seizure free, with this number rising to 17% at 24 months. At 2 years post-implantation, most families noted at least a 50% amelioration of one or more quality-of-life measures. 

VNS therapy, while generally safe and effective, carries a range of potential adverse events associated with both the surgical implantation process and the ongoing electrical stimulation. Surgical complications, such as infection, bleeding, or injury to adjacent structures, are infrequent but possible, as noted by neurologist and MDLinx medical advisory board member Moody Kassem, MD, MBA.

Hoarseness or voice changes are among the most common side effects due to the proximity of the vagus nerve to the larynx. Coughing, throat discomfort, and shortness of breath can also occur, typically during stimulation. Over time, neck pain and discomfort may arise, especially after device adjustments. Device-related complications, including lead migration, fractures, or device malfunction, pose long-term risks. Infections at the surgical site, while rare, demand attention, as do potential allergic reactions to device materials.

What this means for you

LGS patients are at high risk for dangerous seizures. Technology can help lessen this risk and improve seizure control and quality of life—popular tech today includes remote monitoring and VNS devices. Data from monitoring can help guide clinical decision-making. The benefits and drawbacks of potential technological interventions should be carefully weighed with patients.

Read Next: Can Lennox-Gastaut syndrome mimic other disorders?

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