Dravet Syndrome (DS) – Market Outlook, Epidemiology, Competitive Landscape, and Market Forecast Report – 2023 To 2033
Dravet syndrome, also known as Severe Myoclonic Epilepsy of Infancy (SMEI), is a rare and fatal form of epilepsy that typically starts during childhood. Initial seizures are often prolonged, and other types develop in the second year of life. It is characterized by frequent, prolonged seizures that are often triggered by high body temperature (hyperthermia). Additionally, it presents with developmental delay, sleep disturbances, ataxia, hypotonia, speech problems, and other health issues. Approximately 85% of cases result from a mutation or deletion in the SCN1A gene (located at 2q24.3), which encodes a voltage-gated sodium channel. Most of these mutations are not inherited but may be part of a familial spectrum of genetic epilepsy known as febrile seizures-plus (GEFS+) in 5-10% of cases. About 5% of female cases might be attributed to mutations in the PCDH19 gene (located at Xq22.1). The cause is unknown in around 10% of cases. In about 3% of initially negative cases, somatic mosaic deletions or mutations in SCN1A can be found. Diagnosis relies on clinical and electroencephalographic (EEG) findings. Initially, the EEG is usually normal, but later, it shows spikes or poly-spike waves with a background slowing and multifocal discharges. Brain magnetic resonance imaging typically appears normal. The presence of SCN1A pathogenic variants can confirm the diagnosis of Dravet syndrome. It's important to differentiate Dravet syndrome from myoclonic atonic epilepsy. The primary goal of treatment is to reduce seizure frequency and prevent status epilepticus (SE). Early in the disease, valproate, clobazam, stiripentol, and bromide may help control febrile seizures. Stiripentol, when used in combination with valproate and clobazam, may effectively reduce seizure duration and frequency. Adjunctive therapies like the ketogenic diet, topiramate, levetiracetam, and cannabidiol in combination with clobazam may also provide significant efficacy. Seizure frequency may decrease in adulthood, but seizures often remain resistant to treatment. Cognitive impairment is common, ranging from moderate to severe, and intractable epilepsy persists into adulthood. Children with Dravet syndrome experience progressive cognitive decline after initial language and visual function impairments. Approximately 86% of patients have intellectual deficiency, and 31% may develop autism spectrum disorder. Preventing convulsive status epilepticus in children may improve long-term outcomes.
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Dravet syndrome is a rare genetic disorder
affecting approximately 1 in every 20,000–40,000 births in the US.
Approximately 3% to 8% of children who experience their first seizure before 12
months may develop Dravet syndrome.
Thelansis’s
“Dravet Syndrome (DS) Market Outlook, Epidemiology, Competitive Landscape, and
Market Forecast Report – 2023 To 2033" covers disease overview,
epidemiology, drug utilization, prescription share analysis, competitive
landscape, clinical practice, regulatory landscape, patient share, market
uptake, market forecast, and key market insights under the potential Dravet
Syndrome (DS) treatment modalities options for eight major markets (USA,
Germany, France, Italy, Spain, UK, Japan, and China).
KOLs insights of Dravet Syndrome (DS)
across 8 MM market from the centre of Excellence/ Public/ Private hospitals
participated in the study. Insights around current treatment landscape,
epidemiology, clinical characteristics, future treatment paradigm, and Unmet
needs.
Dravet
Syndrome (DS) Market Forecast Patient Based Forecast Model (MS. Excel Based
Automated Dashboard), which Data Inputs with sourcing, Market Event, and
Product Event, Country specific Forecast Model, Market uptake and patient share
uptake, Attribute Analysis, Analog Analysis, Disease burden, and pricing
scenario, Summary, and Insights.
Thelansis Competitive Intelligence (CI) practice
has been established based on a deep understanding of the pharma/biotech
business environment to provide an optimized support system to all levels of
the decision-making process. It enables business leaders in forward-thinking
and proactive decision-making. Thelansis supports scientific and commercial
teams in seamless CI support by creating an AI/ ML-based technology-driven
platform that manages the data flow from primary and secondary sources.

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