Dravet syndrome is a rare and complex early-onset epileptic encephalopathy that begins in the first year of life with prolonged seizures often linked to fever and progresses with an increasing burden of neurological and behavioral problems.
The seizures soon become drug-resistant. Between 1 and 4 years of age, myoclonus, atypical absences, and other types of seizures may appear. As age advances, the following also occur:

●      cognitive difficulties,

●      language disorders,

●      sleep disturbances,

●      postural instability,

●      ataxia.

The clinical picture is heterogeneous, but the chronic course and impact on quality of life are constant, requiring long-term specialist care.

What is Dravet Syndrome

Dravet syndrome is a form of developmental epilepsy, today defined as a “developmental and epileptic encephalopathy,” in which both epilepsy and the genetic substrate contribute to developmental delay. Its estimated incidence of about 1 in 15,700 births places it among low-prevalence conditions. For this reason, when Dravet syndrome is described as a rare disease, it refers to the need for dedicated care pathways and centers with specific expertise.

The initial symptoms of Dravet syndrome are febrile and afebrile clonic seizures, often prolonged. Over time, other seizure types and frequent nonconvulsive status epilepticus appear. Photosensitivity and thermolability are common triggers.
On the neurodevelopmental level, attention difficulties, behavioral disorders, ataxia, and gait problems may emerge, framing Dravet syndrome also as a possible motor disability.

As for the causes of Dravet syndrome, in 70–90% of cases a pathogenic variant is found in the SCN1A gene, which alters neuronal sodium channels (NaV1.1) and leads to hypofunction of GABAergic interneurons with network hyperexcitability. Genetic Dravet syndrome is therefore well documented; the variant is often de novo, but familial inheritance has also been described. In practical terms, the question “Is Dravet syndrome hereditary?” has a nuanced answer: rarely in the classical Mendelian sense, but genetic counseling—useful to help the patient and family understand medical information—is recommended to estimate reproductive risk.

The diagnosis of Dravet syndrome is clinical and instrumental: a history of onset in infancy with prolonged and drug-resistant seizures, supported by EEG and neuroimaging, and confirmed with a genetic test when indicated. Early recognition allows the implementation of more appropriate therapeutic strategies and emergency plans.

From a prognostic standpoint, the condition carries an increased risk of premature mortality, with SUDEP (sudden unexpected death in epilepsy) as the most frequent cause. Cohort studies report SUDEP rates around 9.3 per 1,000 person-years, with deaths concentrated in childhood, though risk persists later on. Discussing life expectancy in Dravet syndrome therefore means considering prevention, seizure control, and nighttime safety measures as essential parts of management.

Traditional Treatments for Dravet Syndrome

The cornerstone of treating Dravet syndrome is a combined, personalized, and evolving antiepileptic therapy. International guidelines and consensus statements indicate valproate as the first-line drug. Clobazam and stiripentol are frequently added, and in recent years fenfluramine has emerged as an effective add-on option.

Attention must be paid to avoiding sodium channel blockers that can worsen the condition (for example, carbamazepine or lamotrigine) and to managing status epilepticus through predefined action plans. Evidence from clinical trials and real-world studies confirms clinically significant reductions in seizure frequency with these regimens, even within a generally drug-resistant context.

Alongside medications, dietary therapies—such as the classic ketogenic diet and its variants (MAD, LGIT)—show effectiveness especially in the short to medium term, with response rates around 60–70% in the first months and variable retention over time. These strategies require dedicated teams (neurologist, dietitian, nurse) to ensure adherence, nutritional monitoring, and management of side effects.

Role of Cannabis in Dravet Syndrome

In the clinical context of Dravet syndrome, the term “cannabis” refers exclusively to cannabidiol (CBD) in a highly purified pharmaceutical form, prescribed as an add-on therapy by specialist centers when seizures remain difficult to control. It is not equivalent to generic hemp products and does not concern recreational use.

In Europe, its use is regulated: it is indicated for seizures associated with Dravet syndrome from 2 years of age, usually in combination with clobazam, with dosage, monitoring, and potential interactions managed by the neurologist. To explore non-medical aspects related to hemp oils intended for general well-being, one can consult the Eusphera CBD oil collection.

Mechanism of Action of CBD in the Nervous System

CBD has a multimodal profile: it indirectly modulates the endocannabinoid system (with allosteric effects on CB1), interacts with TRPV channels, acts as an antagonist/neuromodulator of GPR55, and influences serotonergic circuits (5-HT1A). The combined effect tends to reduce network hyperexcitability, a critical factor in genetic epileptic encephalopathies such as Dravet syndrome. Although some mechanisms remain under investigation, the body of preclinical evidence and recent reviews supports this pathophysiological framework.

Benefits of CBD for Dravet Syndrome

In a randomized trial published in the New England Journal of Medicine, the addition of CBD to existing therapy led to a significant reduction in convulsive seizures compared to placebo in children and adolescents with Dravet syndrome. The effect was observed both in terms of median percentage reduction and responder rates (above 50%), confirming the role of CBD as an add-on option in drug-resistant cases.

The most frequent adverse events are drowsiness, diarrhea, and decreased appetite. Increases in liver enzymes may occur, particularly with co-administration of valproate and in the presence of clobazam, requiring monitoring and therapeutic adjustments. European product information and regulatory evaluations specify its use in combination with clobazam in Dravet patients over 2 years of age.

H2- Integration of CBD with Traditional Therapies for Dravet Syndrome

Integrating CBD into therapy for Dravet syndrome means incorporating it into an existing treatment plan, with realistic objectives and careful evaluation of comorbidities, interactions, and monitoring. Recommendations suggest considering its use only after failure or intolerance of the standard combinations (valproate, clobazam, stiripentol) or in association when seizure control remains unsatisfactory.

Collaboration among the reference center, family, and caregivers is crucial to establish gradual titration, periodic liver function monitoring, review of emergency plans, and preventive measures against SUDEP (such as proper sleep hygiene and nighttime supervision when indicated). This approach is consistent with European regulatory guidelines and with the most recent studies on patient management.

Patient and Family Testimonials

Experiences shared by families and caregivers highlight how school management requires formal tools and structured communication. In many cases, an individualized education plan is implemented so that the school team is prepared to recognize and manage seizures quickly and coordinate with parents and clinicians. This approach is promoted by the Dravet Syndrome Foundation, with dedicated resources for schools and caregivers.

A team-based care organization, where specialists in neurology, developmental psychology/psychiatry, and other services work with a shared plan, is described as particularly useful even in pediatric multidisciplinary centers, where systematic information sharing among professionals contributes to continuity of care. The transition from pediatric to adult services is often a complex process that requires early preparation, goal setting, and ongoing advocacy by the family. Stories collected by Dravet Syndrome UK and their practical guides suggest beginning this planning around age 16, with clear timelines, responsibilities, and points of contact. In daily life, caregivers emphasize the importance of identifying and mitigating triggers (such as heat and physical exertion) and maintaining strict sleep hygiene, with continuous attention to coordination with the clinical team. These priorities emerge both from first-person accounts and from joint analyses of caregivers and clinicians published in the literature. At the same time, many families highlight the emotional and organizational burden of the condition and the need for support networks, training, and peer communities.