An accurate diagnosis can make a real difference to your patients with Lennox-Gastaut syndrome (LGS) or Dravet syndrome^1-3

Patients with LGS or Dravet syndrome may reach adulthood without a specific diagnosis. At any age, an appropriate diagnosis can lead to more targeted care.

A specific diagnosis can help when setting expectations and planning for future care with patients and caregivers^1,2

Treatment plans and management can be personalized to their particular conditions^1,2

Without a specific diagnosis, it may be more difficult to access tailored treatment^1,3

Clinical features of LGS may delay or impede diagnosis

Presentation
is variable

At onset, not all patients present with the traditional signs and symptoms of LGS^2,4,5

LGS changes
over time

Patients may present with a number of different seizure types, which evolve as patients age²

Adolescent/adult
care complications

Details of medical history may be lost during transfer from pediatric to adult care, and characteristic EEG features may no longer be present by adulthood^3,6

The classic diagnostic triad is often used for initial diagnosis²:

Multiple seizure types
Cognitive impairment
Abnormal EEG

However, this triad cannot be solely relied on, as symptoms can change with age⁶

By adulthood

~50% - 75%

of patients diagnosed with LGS during childhood may no longer display all of the clinical and EEG features typically used to diagnose the syndrome⁶

$Refractory Epilepsy Screening Tool for LGS (REST-LGS)$

The Refractory Epilepsy Screening Tool for LGS (REST-LGS) was developed by a panel of experts to help lead to diagnosis for these patients.³

LEARN MORE

Signs and symptoms vary over time

IDENTIFYING LGS ACROSS AGE GROUPS

	YOUNG CHILDREN (symptoms emerge: 2-8 years; peak onset: 3-5 years^5,7)	ADOLESCENTS AND ADULTS
SEIZURE TYPES	Generalized tonic and atonic seizures, and atypical absences⁵ Tonic seizures during sleep^2,5 Generalized tonic-clonic and focal seizures may also occur⁵	Generalized tonic-clonic and atonic seizures, and atypical absences^2,6,7 Tonic seizures present during sleep and may occur during wakefulness^4,8 Increase in drop attacks leading to injury^6,9
SEIZURE FREQUENCY	Daily to several times per week^7,9,10	Changing frequency; fewer daytime seizures in some patients⁸
NEUROLOGICAL ISSUES	Occurs in early childhood^4,5 Up to one-third of patients may show normal functioning at or prior to seizure onset^4,5	Up to 95% of patients may have cognitive impairment⁵ Behavioral problems such as hyperactivity, aggression, and autistic traits are seen in 50% of patients with LGS^4,7,8 Motor impairment and gait deterioration may become more apparent^6,9
EEG	Pattern of slow spike-wave (SSW) complexes^2,5	Decrease in/disappearance of SSW complexes, but other EEG abnormalities such as paroxysmal fast rhythms may be present^8,9

Other common hallmarks of LGS:

Persistent seizures despite trial of 2 or more antiepileptic drugs³

Helmet/safety precautions to prevent seizure-related injuries⁶

If your patient is exhibiting even some of these symptoms, you may want to reevaluate their medical history and determine if they may have LGS²

The information provided is not intended to supersede independent clinical judgment or institutional protocols.

Diagnosing patients with Dravet syndrome can be challenging

Dravet syndrome often goes undiagnosed or can be misdiagnosed

Despite early presentation, Dravet syndrome may not always be diagnosed, as most patients present as normally developing infants with febrile seizures^1,11

At onset, patients with Dravet syndrome typically have a normal MRI and nonspecific EEG findings¹

Childhood history is not always available in older patients, which can further confound diagnosis¹

Though genetic testing is available, the presence of a sodium channel gene mutation is not sufficient for diagnosis, and absence of mutation does not exclude diagnosis^1,12

Signs and symptoms may vary over time

IDENTIFYING DRAVET SYNDROME ACROSS AGE GROUPS

	YOUNG CHILDREN (seizures develop: ≤1 year^1,11)	OLDER CHILDREN AND ADULTS
SEIZURE TRIGGERS	Typically hyperthermia¹ May include flashing lights, visual patterns, bathing, overexertion¹	Less frequently: hyperthermia¹ Exacerbated with the use of sodium channel agents¹
SEIZURE TYPE	Typically present as prolonged generalized or hemiclonic seizures and may include myoclonic seizures in the first 2 years of life^1,11 After age 2: myoclonic, focal, impaired awareness, and atypical absence seizures; obtundation status¹ Seizures often evolve into status epilepticus¹²	Persisting seizures: focal and/or generalized convulsive seizures; atypical absences; myoclonic and tonic seizures¹ Often occur nocturnally¹¹ Recurrent status epilepticus and obtundation status may become less frequent/may not occur¹
DEVELOPMENT & BEHAVIORAL DISORDERS	Normal cognitive development prior to seizure activity; cognitive deficits emerge between 18 months–5 years^1,12 Moderate to severe intellectual disability; slow language progression during second year of life^1,12	Behavioral disorders: attention deficits/hyperactivity; oppositional and personality disorders; autistic traits; psychotic features¹¹ Sleep disturbances¹
MOTOR SYMPTOMS	Most patients have normal gait for the first 5 years of life; some begin to develop crouched gait and hypotonia^1,13	Walking problems, such as crouched gait, ataxia, and balance problems^11,13,14 Intention tremor¹⁴ Extrapyramidal rigidity^11,14

Not all patients display all symptoms—even some of these characteristics can suggest Dravet syndrome¹¹

The information provided is not intended to supersede independent clinical judgment or institutional protocols.

Important Safety & Indications

CONTRAINDICATION: HYPERSENSITIVITY EPIDIOLEX (cannabidiol) oral solution is contraindicated in patients with a history of hypersensitivity to cannabidiol or any ingredients in the product. WARNINGS & PRECAUTIONS Hepatocellular Injury: EPIDIOLEX can cause dose-related transaminase elevations. Concomitant use of valproate and elevated transaminase levels at baseline increase this risk. Transaminase and bilirubin levels should be obtained prior to starting treatment, at one, three, and six months after initiation of treatment, and periodically thereafter, or as clinically indicated. Resolution of…

CONTRAINDICATION: HYPERSENSITIVITY EPIDIOLEX (cannabidiol) oral solution is contraindicated in patients with a history of hypersensitivity to cannabidiol or any ingredients in the product.

An accurate diagnosis can make a real difference to your patients with Lennox-Gastaut syndrome (LGS) or Dravet syndrome^1-3

Patients with LGS or Dravet syndrome may reach adulthood without a specific diagnosis. At any age, an appropriate diagnosis can lead to more targeted care.