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What is the Difference Between Dravet Syndrome and Lennox-Gastaut Syndrome

The key difference between Dravet syndrome and Lennox-Gastaut syndrome is that Dravet Syndrome is caused by a genetic mutation in the SCN1A gene, which is a sodium channel gene. Meanwhile, existing neurological abnormalities cause Lennox-Gastaut syndrome.

Epilepsy is a condition of the brain characterized by repeated seizures. In epilepsy, there is a sudden alteration of behavior due to temporary changes in the electrical function of the brain. It affects people of all ages, and approximately 50 million people worldwide have epilepsy. Dravet syndrome and Lennox-Gastaut syndrome are two medical conditions that are characterized by repeated seizures or epilepsy.

CONTENTS

1. Overview and Key Difference
2. What is Dravet Syndrome  
3. What is Lennox-Gastaut Syndrome
4. Similarities – Dravet Syndrome and Lennox-Gastaut Syndrome
5. Dravet Syndrome vs. Lennox-Gastaut Syndrome in Tabular Form
6. Summary – Dravet Syndrome vs. Lennox-Gastaut Syndrome

What is Dravet Syndrome?

Dravet syndrome is a rare type of epilepsy. It starts in the first year of the baby’s life. Dravet syndrome is an autosomal dominant disorder due to a genetic mutation of the SCN1A gene, which is a sodium channel gene. It can be due to inherited (10 %) or acquired (90 %) genetic mutation of the above gene. It is estimated that between 1 in 15,700 and 1 in 40,000 infants born in the United States have Dravet syndrome. Moreover, the signs and symptoms of Dravet syndrome may include seizures of many different types, sudden muscle jerking, frequent seizures, behavioral, intellectual, and developmental setbacks, anxiety, autistic-like behavior, delayed language development, and trouble speaking, difficulty with learning at school, behavioral and emotional dysregulations, growth and nutrition problems, difficulties in sleeping, trouble regulating body temperature, heart rate, and blood pressure.

Figure 01: Epilepsy

Dravet syndrome can be diagnosed through family history, physical examination, MRI scan, and electroencephalogram. Furthermore, treatment options for Dravet syndrome may include medications like stiripentol, cannabidiol, fenfluramine, ketogenic diet, vagus nerve stimulation, intravenous immunoglobulins, physical therapy, occupational therapy, speech and language therapy, and orthopedic surgery.

What is Lennox-Gastaut Syndrome?

Lennox-Gastaut syndrome is a type of severe epilepsy that occurs during childhood. This condition usually starts before age 10, and the diagnosis is more likely between 3 to 5 ages. Brain development problems before birth, traumatic brain injuries, infections like meningitis or encephalitis, inherited metabolic conditions, brain damage due to lack of oxygen, tuberous sclerosis, brain tumors, West syndrome, and de novo mutations can cause Lennox-Gastaut syndrome. Moreover, the signs and symptoms of this condition may include tonic seizures, atonic seizures, atypical seizures, myoclonic seizures, developmental delays, behavioral disturbances, and difficulty processing information.

Lennox-Gastaut syndrome can be diagnosed through physical examination, electroencephalogram (EEG), complete blood count (CBC), lumbar puncture toxicology screening, MRI scan, and CT scan. Furthermore, Lennox-Gastaut syndrome is treated through antiseizure medications like carbamazepine, oxcarbazepine, lamotrigine, phenytoin and valproic acid, vagus nerve stimulation, ketogenic diet, corpus callostomy, hemispherectomy, and resection.

What are the Similarities Between Dravet Syndrome and Lennox-Gastaut Syndrome?

What is the Difference Between Dravet Syndrome and Lennox-Gastaut Syndrome?

A genetic mutation in the SCN1A gene or sodium channel gene causes Dravet Syndrome. Meanwhile, existing neurological abnormalities cause Lennox-Gastaut syndrome. Thus, this is the key difference between Dravet syndrome and Lennox-Gastaut syndrome. Furthermore, Dravet syndrome occurs in the first year of the life of a baby. On the other hand, Lennox-Gastaut syndrome occurs before age 10, and the diagnosis is more likely between 3 and 5 ages.

The infographic below presents the differences between Dravet syndrome and Lennox-Gastaut syndrome in tabular form for side-by-side comparison.

Summary – Dravet Syndrome vs. Lennox-Gastaut Syndrome

Epilepsy is a chronic, noncommunicable disease of the brain. Dravet syndrome and Lennox-Gastaut syndrome are two medical conditions that are characterized by repeated seizures or epilepsy. Dravet Syndrome is caused by a genetic mutation in the SCN1A gene or sodium channel gene, while existing neurological abnormalities cause Lennox-Gastaut syndrome. So, this summarizes the difference between Dravet syndrome and Lennox-Gastaut syndrome.

Reference:

1. “Lennox-Gastaut Syndrome – Symptoms, Causes, Treatment.” National Organization for Rare Disorders.
2. “Dravet Syndrome: What It Is, Symptoms, Prognosis & Treatment.” Cleveland Clinic.

Image Courtesy:

1. “Spike-waves(CC BY-SA 2.0) via Commons Wikimedia