Pediatric Seizures: A Tale of Two Etiologies

Engine 7 and Medic 4 are dispatched to a 14-year-old female in active seizure on a warm, sunny morning. The caller reports the patient was playing basketball in her driveway when the child suddenly dropped to the ground and experienced a generalized tonic-clonic seizure. The caller tells the 9-1-1 call-taker she has administered an emergency glucagon pen into the patient’s right quadricep, as the patient has a history of type 1 diabetes.

Upon arrival EMS providers note the patient is postictal and semi-alert, lying on her left side on the concrete driveway. Responders learn the patient was visiting with a friend who lives at the house and is also a type 1 diabetic—they’d had a sleepover the night before.

Although seizures are a familiar call type for EMS systems, they can be stressful for both providers and bystanders. In cases of pediatric patients with known histories of type 1 diabetes, the assessment and management of this compounding condition adds to the complexity of the situation.

Basics of Seizure Management

Key steps for EMS providers to take with patients presenting in seizure include initial stabilization, administration of benzodiazepines, and consideration of the underlying etiology.¹  

Initial stabilization focuses on ensuring a clear and patent airway, adequate ventilation, and sufficient blood flow to safeguard cerebral perfusion and glucose levels.² This may include positioning of the head or patient in such a manner as to prevent aspiration of secretions, along with administration of supplemental oxygen. Airway adjuncts, such as a nasopharyngeal airway, or assisted ventilation using a bag-valve mask (BVM) should be considered. If the patient exhibits persistent hypoxia, bradycardia, hypotension, or other signs of poor perfusion—or if basic airway adjuncts or ventilation with a BVM do not sufficiently oxygenate them—an advanced airway may be indicated.

Benzodiazepines remain a first-line therapy for the treatment of prolonged convulsions or status epilepticus—a “repetitive seizure activity without recovery of consciousness in between episodes.”³ The preferred use of diazepam, lorazepam, or midazolam will be determined by local protocol, and administration options may include intravenous, intranasal, rectal, or oromucosal routes. As pediatric doses are often calculated based on weight, it is advantageous to have immediate access to a height-based tool to approximate the patient’s weight and avoid medication errors.⁴ 

When considering the underlying etiology, EMS providers should conduct a focused physical exam, obtain a blood glucose, and gather medical history information about the patient. The physical exam should assess for tremors or twitching in the face or extremities, lip smacking, gaze deviation, clenching of the jaw, and signs of poor perfusion.⁵ Treat low blood glucose—typically lower than 70 mg/dL⁶—per local protocol, often involving the administration of dextrose or glucagon. Finally, assessing for fever, trauma, drug use or withdrawal, medical or neurological disorders, or a history of seizures will inform rule-outs and guide additional assessments and treatments.

Type 1 Diabetes

Type 1 diabetes mellitus (T1D) represents about 10% of all diabetics in the U.S.⁷ This disease is not just diagnosed in children; it has also been detected in adults, prompting the medical community to no longer refer to it as juvenile diabetes. Still, T1D is an endocrine disorder that affects nearly one in 500 children in the U.S.⁸ and is believed to be caused by an autoimmune response that erroneously targets cells in the pancreas—specifically the islets of Langerhans, where insulin-secreting beta cells are found. This assault on the pancreas results in that organ ceasing to produce adequate insulin and the patient becoming dependent on external injections of insulin to manage blood glucose levels.⁹ Given the suspected cause, T1D patients are often screened for other autoimmune disorders, such as celiac disease, thyroid problems, and Addison’s disease.

Diabetic patients have an expanding portfolio of tools with which to monitor and manage blood glucose levels. While capillary blood glucose monitors are the mainstay of diabetes management, many T1D patients have the option to use continuous glucose monitors, replaceable sensors affixed to the body that measure interstitial glucose levels through a cannula inserted below the skin. These devices have advanced to where a transmitter can send sensor readings through Bluetooth technology to a smart device such as a cell phone. Updating every few minutes, this steady stream of biofeedback alerts the patient to nearly real-time glucose levels and trends. 

T1D patients work toward keeping glucose levels within a prescribed target range through external injections of insulin, which can be administered via a syringe, adjustable-dose pen, or pump. Insulin is available across a spectrum of fast- to long-acting duration of effect and works like a key, unlocking cells to help deliver sugar from the blood to cells. Every cell in the body has a lock on its cell wall, called a receptor. Insulin fits into that lock like a key, allowing sugar to enter the cells.

Fast-acting insulin is classically administered at mealtimes or as needed to correct elevated glucose levels. Mealtime insulin dosing depends on a calculation of carbohydrates consumed plus any correction of premeal blood glucose levels. Patients must try to account for each carbohydrate consumed at each meal—no easy task if the food eaten is without labeling or when the portion size has to be estimated. Inadvertent over- and underdosing of insulin could lead to acute hypoglycemia or chronic hyperglycemia.

Over the course of 12–24 hours, T1D patients also administer insulin to maintain a steady basal rate to help keep glucose levels from rising between boluses. This can be accomplished through injection of a long-acting form of insulin—using a syringe or pen—or by programming an insulin pump to inject microboluses of fast-acting insulin at regular intervals. Even when insulin doses are meticulously calculated and administered, hypoglycemia may develop, exacerbated by increased physical activity, exercise, or stress. 

For cases of a rapid-onset hypoglycemia, when the patient experiences a decreased level of consciousness or a seizure and is unable to bolster their blood glucose by consuming carbohydrates, T1D patients are often equipped with emergency glucagon kits. Glucagon causes the liver to convert stored glycogen into glucose and is effective in treating hypoglycemia only if sufficient liver glycogen is present. Because glucagon is of little or no help in states of starvation, adrenal insufficiency, or chronic hypoglycemia, administration of glucagon may not be effective.

Household members, teachers, sports coaches, and caregivers who have regular interactions with T1D patients can be trained to render aid with this device, now prepared as both an intramuscular injection and intranasal spray. EMS providers may encounter scenes where glucagon has been administered when rapid-onset hypoglycemia was presumed to have precipitated a seizure.

In the case introduced earlier, it is possible the patient was experiencing a profound case of hypoglycemia unawareness, and the sudden drop in blood glucose levels resulted in a seizure. However, EMS providers noted the patient had a continuous glucose monitor (CGM) affixed to her left upper abdomen. Assessment revealed a capillary blood glucose of 364 mg/dL that closely matched the reading transmitted to an app on the patient’s phone. Upon further investigation it was noted that at the time of the glucagon administration, the CGM measured the patient’s blood sugar in the range of 170 mg/dL and rising. This suggests the underlying etiology of the seizure was likely not acute hypoglycemia or prolonged hyperglycemia. 

Juvenile Myoclonic Epilepsy

Juvenile myoclonic epilepsy (JME) is a form of epilepsy that is not well understood but is often diagnosed in patients between 10–20 years of age.¹⁰ JME is an epilepsy disorder characterized by myoclonic, generalized tonic-clonic, and sometimes absence seizures.¹¹ Such seizures associated with JME frequently occur in the morning and are often triggered by profound fatigue, inadequate rest or sleep, stress, or the consumption of alcohol.

Described as quick jerks or violent twitching of the extremities, myoclonic seizures are a hallmark feature of JME. However, the Genetic and Rare Diseases Information Center estimates that myoclonic seizures “may be the only symptom in about 17% of the cases; in about 20% of the cases, the seizures occur in clusters, affecting only one side (unilateral) of the body, and start before a tonic-clonic seizure.”¹² 

Classically termed grand mal, the archetypal epileptic seizure is now referred to as a tonic-clonic seizure. The tonic phase begins with patients losing consciousness and falling, followed by a clonic phase of rhythmic jerking of the extremities.¹³ Once seizure activity subsides (sometimes after 60–90 seconds), the patient typically experiences a postictal phase. This phase is characterized by a gradual increase in mental status from stupor and confusion, along with disordered control of breathing.

Absence seizures were formerly known as petit mal seizures and refer to a “brief episode of loss of awareness, occurring without warning, lasting for a few seconds, and ending with immediate resumption of consciousness.”¹⁴ Such seizure activity in children may often be disregarded by the patient, and caregivers may characterize this behavior as daydreaming or “spacing out,” but these episodes could have serious consequences if they disrupt focus during activities such as driving or playing sports.

Routine medical therapy for JME patients typically includes the prescription of antiepileptic medications with the goal of reducing or even eliminating the occurrence of seizures. Valproic acid, lamotrigine, and levetiracetam are commonly prescribed medicines; the first two work by slowing brain excitation associated with seizures, while the latter slows the release of neurotransmitters. These treatments might be terminated if the patient is without seizure activity for a period of years.

As an emergency treatment for status epilepticus—a single, prolonged seizure or two or more consecutive seizures—a syringe of midazolam with a mucosal atomization device is often prescribed to the patient. Much like a glucagon pen, the intranasal (IN) administration of this medication can be performed by household members, teachers, coaches, and other trained individuals with whom the patient interacts. A number of studies have favored IN delivery of benzodiazepines over other routes given its ease of out-of-hospital use, rate of absorption, and speed in ceasing seizure activity.¹⁵  

For maximum effectiveness, “the nasal spray device must be inserted fully into the nasal vestibule with the atomizer tip placed at the nasal valve and then aimed laterally toward the turbinates.”¹⁶ Many factors influence the absorption of IN medication and its ability to terminate seizure activity (see sidebar).

Case Discussion

During the assessment and initial EMS treatment of the patient, the child’s parents were contacted by phone. Because the patient had regained consciousness and did not present with airway complications, traumatic injuries, or lasting neurological deficits, the EMS crew felt comfortable deferring transport and waiting for a parent to arrive on scene. When the patient’s father arrived, he was given a report of the EMS crew’s findings and ultimately declined ambulance transport for his daughter.

After she had fully recovered, the patient was referred by her pediatric endocrinologist to a pediatric neurologist, who obtained a detailed history and ordered an electroencephalogram. During the consultation the patient recalled occurrences of her arms “twitching” and uncontrollably dropping items she was holding. In addition, she reported fellow students would often tell her she appeared to be “spacing out” during class.

Unknown to the patient or her parents, the clustering of these myoclonic, absence, and tonic-clonic seizures was the hallmark of JME. The neurologist also noted the recent tonic-clonic seizure may have been potentiated by sleep deprivation at her friend’s house overnight.

With these two diagnoses, the patient, her parents, and other caregivers could all work together to help minimize her risk of seizures not only from hypoglycemia, but also from epilepsy by taking prescribed medications, limiting interruptions to sleep, minimizing stress, and avoiding other triggers—a tall order for an active and growing teenager living with both T1D and JME. 

References

1. Slovis CM. Guidelines for Treatment of Prolonged Seizures in Children and Adults. J Emerg Med Serv, 2017 Apr 10; www.jems.com/patient-care/guidelines-for-treatment-of-prolonged-seizures-in-children-and-adults/.

2. Sasidaran K, Singhi S, Singhi P. Management of acute seizure and status epilepticus in pediatric emergency. Indian J Pediatr, 2012 Apr; 79(4): 510–7.

3. Ibid.

4. Kaufmann J, Roth B, Engelhardt T, et al. Development and Prospective Federal State-Wide Evaluation of a Device for Height-Based Dose Recommendations in Prehospital Pediatric Emergencies: A Simple Tool to Prevent Most Severe Drug Errors. Prehosp Emerg Care, 2018 Mar–Apr; 22(2): 252–9.

5. Loza-Gomez A. Pediatric Seizures: Subtle and Often Difficult to Diagnose. J Emerg Med Serv, 2019 Jun 12; www.jems.com/patient-care/pediatric-seizures-subtle-and-often-difficult-to-diagnose/.

6. Berk J. #7: Become #1 in the Diagnosis and Management of T1DM. Cribsiders Pediatric Medicine, 2020 Sep 2; https://thecurbsiders.com/cribsiders/7.

7. Snyder SR. Case Studies in Hypoglycemia. EMS World, 2013 Aug; www.emsworld.com/article/10984817/case-studies-hypoglycemia.

8. Op. cit., Berk.

9. Gale. The Gale Encyclopedia of Children’s Health: Infancy through Adolescence. Gale, 2016.

10. Bergen D. Epilepsy. In: Weiner W, Goetz C, Shin R, Lewis S. Neurology for the Non-Neurologist. Philadelphia, PA: Lippincott Williams & Wilkins, 2010; pp. 143–155.

11. Genetic and Rare Diseases Information Center. Juvenile myoclonic epilepsy, https://rarediseases.info.nih.gov/diseases/6808/juvenile-myoclonic-epilepsy.

12. Ibid.

13. Op. cit., Bergen.

14. Op. cit., Bergen. 

15. Scansen K. Management of seizures in the emergency department. In: Weisleder P (ed.). Manual of Pediatric Neurology. Columbus, Ohio: World Scientific Publishing, 2012.

16. Wermeling DP. Intranasal delivery of antiepileptic medications for treatment of seizures. Neurotherapeutics, 2009 Apr; 6(2): 352–8.

Michael Heffner, MS, EMT-P, is assistant chief deputy for the Oregon Office of the State Fire Marshal.