CASE REPORT

The parent of a five-year-old female child with LGS was interviewed to obtain history for the study purpose. The child was diagnosed with Lennox Gastaut Syndrome around 3 years of age at paediatric medicine department of a tertiary care hospital. The child is a second - born of a non-consanguineous marriage with no history of seizure disorders or genetic diseases to parents.

The child was born by normal vaginal delivery at 38 weeks of gestation. At birth, the child had birth asphyxia and was shifted to NICU for effective management. The child’s APGAR score was not available and her birth weight was 2.9kg. She remained in NICU for a few days.

The child’s growth and development were reported to be delayed. There was a mild delay in physical and cognitive development. At 9 months of age, she had seizure episodes and developed infantile spasms. She was started with sodium valproate for control of seizure episodes. At the age of 3years, she developed flexor spasm awakening from sleep and was followed by loss of attained cognitive skills and language skills. She also had multiple types of generalized seizures, myoclonic jerks and absence seizures. Motor examination revealed increased muscle tone in right upper limb and lower limb, brisk deep tendon reflexes, and extensor plantar response. Magnetic Resonance Imaging (MRI) brain revealed subependymal zone tubers and cortex tubers with atrophy of left frontal lobe. EEG findings revealed slow spike and wave pattern-SSW(<2.5Hz) which was present when the child is awake and paroxysmal fast activity during sleep. She was diagnosed with Lennox Gastaut Syndrome and Tuberous Sclerosis and was started with multiple anti-epileptic drugs such as sodium valproate, clonazepam and levetiracetam. Even though resective surgeries are found to be effective in improving IQ and controlling seizure episodes, the parents of the index child were not discussed regarding surgical interventions.

INTRODUCTION:

Caring for a child with epilepsy involves facing multiple challenges simultaneously. Most of the time, it is much more than simply counting the number of seizures and adjusting the medications. A majority of these children will have associated learning and behaviour disorders leading to significant difficulties at school. Lennox-Gastaut syndrome (LGS) is a severe form of epilepsy that typically becomes apparent during infancy or early childhood. Affected children experience several different types of seizures most commonly atonic, tonic and atypical absence seizures. Children with Lennox-Gastaut syndrome may also develop cognitive dysfunction, delays in reaching developmental milestones and behavioral problems. Lennox-Gastaut syndrome can be caused by a variety of underlying conditions, but in some cases no cause can be identified. Lennox-Gastaut syndrome can be difficult to treat because it is resistant (refractory) to many kinds of antiseizure medications. Research is ongoing to identify and assess new therapies for Lennox-Gastaut syndrome.

Synonyms:

· Childhood Epileptic Encephalopathy

· West Syndrome

Incidence:

Lennox-Gastaut syndrome affects more in males than females. Lennox-Gastaut syndrome is estimated to occur in 1.28 people per 100,000 and is account for 1-4 percent of all cases of childhood epilepsy. The annual incidence in children is estimated to be 2 per 100,000 children.

Causes:

Approximately 70-80 percent of children with Lennox-Gastaut syndrome has an identifiable cause. Approximately 17-30 percent of individuals with Lennox-Gastaut syndrome have a previous history of West syndrome.

Mainly occurs due to the following causes:

1. Idiopathic causes

2. Cryptogenic causes

3. Symptomatic causes

Predisposing Factors:

· An abnormal development of the brain cortex or cortical dysplasia.

· Meningitis

· Tuberous sclerosis

· Cortical sclerosis

· Hypoxia ischemia injury

· Traumatic brain injury

· Infantile spasm

· Stroke

· Birth injuries.

· Reduced oxygen supply that occurs before birth (perinatal hypoxia)

· Infections of the central nervous system such as encephalitis and rubella.

· A rare, genetic disorder called tuberous sclerosis.

In general, these cases tend to be more severe. Lennox-Gastaut syndrome may also be classified as cryptogenic, in which the cause is unknown or cannot be determined after evaluation. Cryptogenic cases are presumed to result from an unidentified condition (secondary Lennox-Gastaut syndrome). Child with cryptogenic Lennox-Gastaut syndrome do not have a previous history of seizure activity, prior neurological problems or cognitive impairment before the development of the disorder. Cryptogenic cases generally have a later onset than symptomatic cases. Researchers have not discovered any genes that are associated with Lennox-Gastaut syndrome, although the disorder may have a genetic component that contributes to its development. More research is necessary to determine the specific factors, including any potential genetic factors that are involved in the development of Lennox-Gastaut syndrome.

SIGNS AND SYMPTOMS:

Multiple types of seizures, which are basically electrical disturbances in the brain, affect children with Lennox-Gastaut syndrome. The most affected child experience multiple types of seizures with multiple times throughout the day. As the affected child grows types and frequency of seizure activity may change.

· The most common types of seizures associated with Lennox-Gastaut syndrome are tonic and atonic seizures. Tonic seizures cause increased muscle tone and muscle stiffness. They are characterized by sustained muscle contractions that can cause mild abnormalities such as slight bend of the body and interrupted breathing or more significant problems such as muscle spasms of the face and flexion or extension of the arms and legs. Affected children may extend their arms over their heads similar to a ballet dancer.

· Tonic seizures are usually brief (lasting between a few seconds and a minute) and are especially prevalent at night during sleep, but can also occur during the day. when awake can cause affected individuals to fall.

· A brief loss of consciousness.

· Atonic seizures cause a sudden loss of muscle tone and limpness, head to drop or nod, problems with posture or sudden falls. It can lead to injuries of the head and face because of sudden, unexpected falls. When sitting, affected child may collapse forward or backward at the waist. It may only partially affect consciousness and last only a few seconds.

· A third type of seizure commonly associated with Lennox-Gastaut syndrome is atypical absence seizures. This type of seizure is associated with unconsciousness usually marked by unresponsive staring, loss of memory. Absence seizures do not cause convulsions. They usually last only a couple of several seconds. If the child is developmentally delayed, the parents may only notice a subtle change in function or responsiveness.

Intelligence is not always affected in children with Lennox-Gastaut syndrome. Affected children may experience varying degrees of cognitive dysfunction and delays in reaching developmental milestones such as sitting, crawling or walking. Children with Lennox-Gastaut syndrome may develop normally before the onset of seizures, and then lose previously acquired skills (psychomotor regression). Because the seizures associated with Lennox-Gastaut syndrome are usually resistant to treatment, intellectual impairment and learning problems may worsen over time. Children with Lennox-Gastaut syndrome may also develop behavioral problems ranging from hyperactivity and irritability to autistic symptoms and psychosis.

Diagnosis:

1. Electroencephalography

2. Interictal EEG

3. MRI is preferred over CT scan

4. PET

Treatment:

The goal is to reduce the number of seizures with medication that causes the fewest side effects. Finding the right treatment for the child will probably take time and close coordination. Drugs used to treat seizures include:

· Cannabidiol (Epidiolex)

· Clobazam (Onfi)

· Felbamate (Felbatol)

· Iamotrigine (Lamictal)

· Rufinamide (Banzel)

· Topiramate (Topamax)

· Valproate, valproic acid (Depakene, Depakote)

Diets:

A special high-fat, low-carbohydrate diet, called the ketogenic diet, helps some people with epilepsy, including some children with LGS. It's a high-fat, low-protein, low-carb diet. It has to be started in a specific way and followed very strictly.

Surgery:

· Epilepsy surgery (typically a corpus callostomy, which involves severing the band of nerve fibers that connect the two halves of the brain to prevent seizures from spreading)

· Vagus nerve stimulation: The procedure involves implantation of a battery-operated device with connecting wires to the left vagus nerve, which is programmed to deliver a current at variable frequencies, pulse widths, and times

In Summary Lennox-Gastaut syndrome (LGS) is a severe form of epilepsy that typically becomes apparent during infancy or early childhood. Affected children experience several different types of seizures most commonly atonic, tonic and atypical absence seizures. Children with Lennox-Gastaut syndrome may also develop cognitive dysfunction, delays in reaching developmental milestones and behavioral problems. Lennox-Gastaut syndrome can be caused by a variety of underlying conditions, but in some cases no cause can be identified. A severe form of childhood epilepsy characterized by generalized multiple seizures, slowness of intellectual growth and specific EEG disturbance. The affected children commonly have intellectual disability, associated developmental delay and recurrent seizure episodes. Lennox-Gastaut syndrome can be difficult to treat because it is resistant (refractory) to many kinds of antiseizure medications. Research is ongoing to identify and assess new therapies for Lennox-Gastaut syndrome. A majority of children with LGS secondary to perinatal sequelae have poor seizure control and unfavorable neurological outcome.

REFERENCES:

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Received on 21.01.2024 Modified on 11.05.2024

Int. J. Nur. Edu. and Research. 2024; 12(3):212-214.

DOI: 10.52711/2454-2660.2024.00045