DelveInsight’s ‘West Syndrome - Market Insights, Epidemiology and Market Forecast– 2030’ report delivers an in-depth understanding of the West Syndrome, historical and forecasted epidemiology as well as the West Syndrome market trends in the United States, EU5 (Germany, Spain, Italy, France, and United Kingdom) and Japan.
The West Syndrome market report provides current treatment practices, emerging drugs, and market share of the individual therapies, current and forecasted 7MM West Syndrome market size from 2017 to 2030. The report also covers current West Syndrome treatment practice/algorithm, market drivers, market barriers and unmet medical needs to curate the best of the opportunities and assesses the underlying potential of the market.
• The United States
• EU5 (Germany, France, Italy, Spain, and the United Kingdom)
Study Period: 2017–2030
West Syndrome Disease Understanding and Treatment Algorithm
West Syndrome Overview
West syndrome is an age-related specific epileptic encephalopathy due to multiple and diverse causes. It is characterized by a unique type of seizure called epileptic (infantile) spasms and gross EEG abnormalities of hypsarrhythmia. Usually, West syndrome consists of a characteristic triad: infantile spasms, the arrest of psychomotor development, and hypsarrhythmia, although one element may be missing. Spasms may be flexor, extensor, lightning, or nods, but most commonly they are mixed. Onset peaks are observed between the ages of 4–7 months and always occur before the age of 1 year. Also, boys are more commonly affected, and the prognosis is generally poor.
West syndrome may be separated into two groups: the symptomatic group, which is characterized by previous existence of brain damage signs (psychomotor retardation, neurologic signs, radiologic signs, or other types of seizures) or by a known etiology, and the smaller, cryptogenic group that is characterized by a lack of previous signs of brain damage and known etiology. The prognosis appears to be partly based on early therapy with adrenocorticotropic hormone or oral steroids. Besides, the ILAE new diagnostic scheme classifies West syndrome amongst ‘epileptic encephalopathies’ and prefers ‘epileptic spasms’ rather than ‘infantile spasms’.
It is a constellation of symptoms which are characterized by epileptic or infantile spasms, abnormal pattern of brain waves called hypsarrhythmia, and mental disability. The spasms that occur may range from violent jack-knife or “salaam” movements where the entire body bends in half, or they may change as little as a slight twitching of the shoulder or eye. These spasms usually start in the early months after birth, and medication can sometimes be used to help. They can also occur in elderly patients; they are called “epileptic spasms” rather than infantile spasms if this occurs.
West Syndrome Diagnosis
The diagnosis of infantile spasms is made by a detailed account of the seizures or history of someone who has seen them. Ideally, this will also include being able to watch some seizure videos. The video is especially helpful when the child is experiencing spasm clusters; this is because a variety of nonepileptic disorders (as well as colic) arise in infancy and can be confused with childhood spasms. West syndrome diagnosis is based on a combination of the clinical features and a typical EEG. The EEG’s pattern is rather disorganized, called hypsarrhythmia. EEG is often irregular in children with West syndrome, but the abnormality is often only seen during sleep. Infantile spasms and West syndrome have several different causes similar to many other epilepsy syndromes. In 7–8 out of every 10 children with West syndrome, one specific cause will be found.
Both children who have childhood spasms will require an EEG. Two EEGs may sometimes be needed. The first EEG is often done when the baby is awake, but if this does not show the pattern of hypsarrhythmia, then another EEG will be done when the baby sleeps. A magnetic resonance imaging (MRI) brain scan is also required for all children with infantile spasms and West syndrome. Also, they may need several additional tests, which involve blood checks, urine tests, and even other tests to try to determine the root cause of such a fluid stream from the spine.
West Syndrome Treatment
Therapy targets for children with childhood spasms are the highest quality of life (without seizures), fewest adverse effects from therapy, and the lowest number of drugs. Treatment may require a team of specialists to coordinate the efforts. Pediatrists, neurologists, surgeons and/or other health care professionals may need to plan treatment for an affected child systematically and comprehensively.
In some children, treatment with anticonvulsant drugs may help to reduce or control various types of West syndrome-related seizure activity. Adrenocorticotropic hormone (ACTH), prednisone, vigabatrin, and pyridoxine are among the most common medications used to treat epileptic spasms. The benefits of the drug must be compared with the risks of each treatment causing side effects. ACTH, prednisone and other steroids, for example, are known to cause immunosuppression problems, hypertension, inflammation, gastric problems, anxiety and irritability. Vigabatrin can cause deep structures in the MRI to cause permanent visual field defects, irritability and transient hyperintensities. There is no standard protocol for using ACTH or any other treatment with steroids. Whether high dose ACTH or low dose ACTH is effective is unknown, or whether prednisone use is more effective than ACTH.
West Syndrome Epidemiology
The disease epidemiology covered in the report provides historical as well as forecasted epidemiology segmented by Prevalent Population of West Syndrome, Diagnosed Prevalent Population of West Syndrome and Gender-specific Diagnosed Prevalence of West Syndrome in the 7MM market covering the United States, EU5 countries (Germany, France, Italy, Spain, and United Kingdom) and Japan from 2017 to 2030.
This section provides glimpse of the West Syndrome epidemiology in the 7MM.
• The total diagnosed prevalent population of West Syndrome in the 7 major markets was found to be 8,057 in 2017.
• In the United States, the diagnosed prevalent cases of West Syndrome was found to be 3,999 in 2017.
• Delvelnsight has also analyzed gender-specific data for West Syndrome, which clearly suggests that it is more prevalent among males than females. It was found that in the United States the number of cases of West Syndrome in males and females were 2,399 and 1,600 respectively in 2017.
• In the EU5 countries the diagnosed prevalence of West Syndrome was found to be maximum in France with 737 cases, followed by the United Kingdom with 684 cases in 2017. While, the least number of cases were found in Spain, with 445 cases in 2017.
• In Japan, the diagnosed prevalence of West Syndrome was found to be 1,090 in 2017.
Country Wise- West Syndrome Epidemiology
The epidemiology segment also provides the West Syndrome epidemiology data and findings across the United States, EU5 (Germany, France, Italy, Spain, and the United Kingdom) and Japan.
West Syndrome Drug Chapters
The drug chapter segment of the West Syndrome report encloses the detailed analysis of West Syndrome marketed drugs and mid and late stage pipeline drugs. It also helps to understand the West Syndrome clinical trial details, expressive pharmacological action, agreements and collaborations, approval and patent details of each included drug and the latest news and press releases.
West Syndrome Marketed Drugs
Sabril (Vigabatrin): Lundbeck
Vigabatrin is an analog of gamma-aminobutyric acid (GABA), the main inhibitory neurotransmitter in the central nervous system, used in the treatment of refractory seizures and infantile spasms. It irreversibly inhibits the enzyme responsible for GABA metabolism, thereby increasing levels of circulating GABA. Although the drug is administered as a racemic mixture, only the S(+) enantiomer is pharmacologically active. It is an antiepileptic agent chemically unrelated to other anticonvulsants. It prevents the metabolism of GABA by irreversibly inhibiting GABA transaminase (GABA-T). Since, the drug is an irreversible inhibitor of gamma-aminobutyric acid transaminase (GABA-T), its duration of effect is thought to be dependent on the rate of GABA-T re-synthesis rather than on the rate of drug elimination. It is also indicated as monotherapy in the treatment of infantile spasms in patients between 1 month and 2 years of age for whom the potential benefits outweigh the risk of vision loss.
Product details in the report…
H.P. Acthar Gel: Questcor Pharmaceuticals/Mallinckrodt Pharmaceuticals
H.P. Acthar Gel is an adrenocorticotropic hormone (ACTH) analogue indicated as monotherapy for the treatment of infantile spasms in infants and children under 2 years of age and also indicated for the treatment of exacerbations of multiple sclerosis in adults. This gel was approved by the FDA in 1952 and has been used to treat IS for more than 50 years. It is recommended by the American Academy of Neurology and the Child Neurology Society as a treatment for IS. It was previously approved for various indications, including acute multiple sclerosis exacerbations and nephrotic syndrome. It is a purified preparation of adrenocorticotropic hormone (ACTH), obtained from the pituitary glands of pigs.
Product details in the report…
West Syndrome Emerging Drugs
Cannabidiol (CBD) oral solution: INSYS Therapeutics
Cannabidiol (CBD) oral solution is being developed by INSYS Therapeutics. It is a phytocannabinoid that belongs to the class of 113 cannabinoids in cannabis plants, accounting for up to 40% of the plants extract. CBD is found to act on cannabinoid (CB) receptors of the endocannabinoid system, which are found in numerous areas of the body, including the peripheral and central nervous systems, including the brain. The endocannabinoid system regulates many physiological responses of the body, including pain, memory, appetite, and mood. More specifically, CB1 receptors can be found within the pain pathways of the brain and spinal cord where they may affect CBD-induced analgesia and anxiolysis, and CB2 receptors have an effect on immune cells, where they may affect CBD-induced anti-inflammatory processes.
Product details in the report…
Epidiolex (GWP42003-P): GW Pharmaceuticals
Epidiolex (GWP42003-P), whose active product ingredient is cannabidiol, is an orally administered cannabinoid CB1 receptor antagonist for being investigated for the treatment of infantile spasms by GW Pharmaceuticals along with its US subsidiary Greenwich Biosciences. Epidiolex is GW pharmaceutical’s lead product and is a highly purified plant-derived (marijuana) cannabidiol.
CBD has been shown to have analgesic, anticonvulsant, muscle relaxant, anxiolytic, neuroprotective, anti-oxidant, and anti-psychotic activity. This wide variety of effects is likely due to its complex pharmacological mechanisms. In addition to binding to CB1 and CB2 receptors of the endocannabinoid system, there is evidence that CBD activates 5-HT1A serotonergic and TRPV1–2 vanilloid receptors, antagonizes alpha-1 adrenergic and µ-opioid receptors, inhibits synaptosomal uptake of noradrenaline, dopamine, serotonin and g-aminobutyric acid and cellular uptake of anandamide, acts on mitochondria Ca2 stores, blocks low-voltage-activated (T-type) Ca2 channels, stimulates the activity of the inhibitory glycine-receptor, and inhibits the activity of fatty amide hydrolase (FAAH).
Product details in the report…
JBPOS0101: BioPharm Solutions
JBPOS0101 is an antiepileptic drug candidate, which possesses highly potent and broad-spectrum antiepileptic activity as demonstrated in testing done by BioPharm Solutions and NIH NINDS Anticonvulsant Screening Program. It is an antagonist of metabotropic glutamate receptors 1 and 7. Additionally, JBPOS0101 may have a strong functional role in blood–brain barrier related neuroprotection against lithium-pilocarpine induced status epilepticus, collagenase induced hemorrhage and tPA induced cell death.
JBPOS0101 controls the occurrence of spasms directly and indirectly, and it works on the mGluR family receptor, including other unknown mechanisms. Unlike other existing drugs, it directly works on the ion channel related to the mode of action; as a result, it has a low probability of developing tolerance and has more extensive therapeutic coverage. The candidate has also been awarded orphan drug status for the West Syndrome.
Product details in the report…
West Syndrome Market Outlook
This section of the report includes the dynamics of market scenario for West Syndrome in the 7MM, i.e., the United States, EU5 (Germany, France, Italy, Spain, and the United Kingdom), and Japan.
The National Organization for Rare Disorders (NORD) defines West syndrome as a rare neurological syndrome with a constellation of symptoms characterized by epileptic/infantile spasms, abnormal brain wave patterns called hypsarrhythmia and intellectual disability. Any disorder that can lead to brain damage can be an underlying cause of West syndrome including trauma, brain malformations such as hemimegalencephaly or cortical dysplasia, infections, chromosomal abnormalities such as Down syndrome, or neurocutaneous disorders such as tuberous sclerosis complex (TSC). Since there are so many potential causes of West syndrome, treatment is based on the cause. It has been estimated that 0.31/1000 live births in the United States are affected by this syndrome.
Adrenocorticotropic hormone (ACTH), prednisone, vigabatrin, and pyridoxine are among the most common medications used to treat epileptic spasms. Nitrazepam, sodium valproate (Epilim) and zonisamide (Zonegran) may also be helpful in treatment. Apart from ACTH and prednisone, other types of corticosteroids such as prednisolone and hydrocortisone are also used. Other therapies, including the ketogenic diet and other anti-pileptics medications, may also prove useful in the treatment of infantile spasms.
The current market of West Syndrome possesses only two approved products, namely Sabril (vigabatrin) and Acthar Gel (corticotropin injection) to treat infantile spasms.
As per our analysis, the major pipeline drugs that are expected to enter the market of West Syndrome comprises of two cannabinoid therapies in phase III of clinical development, namely Cannabidiol (CBD) oral solution (INSYS Therapeutics) and Epidiolex (GW Pharmaceuticals). Another drug JBPOS0101 (Bio-Pharm Solutions) is an antiepileptic agent, which is being explored for the treatment of infantile spasms in phase II clinical trial in the US.
This section includes a glimpse of the West Syndrome 7MM market.
• The market size of West Syndrome in the seven major markets was estimated to be USD 171.10 Million in 2017.
• The current treatment of West Syndrome is mainly dominated by the use of Hormonal Therapy (Acthar Gel Injection; Corticotropin), Vigabatrin – Anti-epileptic drugs (AEDs), Corticosteroids and Other off-label therapies.
• The United States accounts for the largest market size of West Syndrome, in comparison to EU5 (the United Kingdom, Germany, Italy, France, and Spain) and Japan.
• Among the EU5 countries, France had the highest market size with USD 9.76 Million in 2017, while Spain had the lowest market size of West Syndrome with USD 5.89 Million in 2017.
• The Japanese West Syndrome market accounted for USD 14.43 Million in 2017.
The United States Market Outlook
This section provides the total West Syndrome market size and market size by therapies in the United States.
EU-5 Market Outlook
The total aspregillosis market size and market size by therapies in Germany, France, Italy, Spain, and the United Kingdom are provided in this section.
Japan Market Outlook
The total West Syndrome market size and market size by therapies in Japan are provided.
West Syndrome Drugs Uptake
This section focusses on the rate of uptake of the potential drugs recently launched in the West Syndrome market or expected to get launched in the market during the study period 2017–2030. The analysis covers West Syndrome market uptake by drugs; patient uptake by therapies; and sales of each drug.
This helps in understanding the drugs with the most rapid uptake, reasons behind the maximal use of new drugs and allow the comparison of the drugs on the basis of market share and size which again will be useful in investigating factors important in market uptake and in making financial and regulatory decisions.
West Syndrome Development Activities
The report provides insights into different therapeutic candidates in phase II, and phase III stage. It also analyzes key players involved in developing targeted therapeutics.
Pipeline Development Activities
The report covers the detailed information of collaborations, acquisition and merger, licensing and patent details for West Syndrome emerging therapies.
Competitive Intelligence Analysis
We perform competitive and market Intelligence analysis of the West Syndrome market by using various competitive intelligence tools that include–SWOT analysis, PESTLE analysis, Porter’s five forces, BCG Matrix, Market entry strategies, etc. The inclusion of the analysis entirely depends upon the data availability.
Scope of the Report
• The report covers the descriptive overview of West Syndrome, explaining its causes, signs and symptoms, pathogenesis and currently available therapies.
• Comprehensive insight has been provided into the West Syndrome epidemiology and treatment.
• Additionally, an all-inclusive account of both the current and emerging therapies for West Syndrome are provided, along with the assessment of new therapies, which will have an impact on the current treatment landscape.
• A detailed review of West Syndrome market; historical and forecasted is included in the report, covering the 7MM drug outreach.
• The report provides an edge while developing business strategies, by understanding trends shaping and driving the 7MM West Syndrome market.
• In the coming years, West Syndrome market is set to change due to the rising awareness of the disease, and incremental healthcare spending across the world; which would expand the size of the market to enable the drug manufacturers to penetrate more into the market.
• The companies and academics are working to assess challenges and seek opportunities that could influence West Syndrome R&D. The therapies under development are focused on novel approaches to treat/improve the disease condition.
• Report also covers Gender-specific diagnosed prevalence of West Syndrome, which clearly remarks a male preponderance and this finding maintains the hypothesis that male sex is a risk factor.
• Expected launch of a potential therapies, Cannabidiol (CBD) oral solution (INSYS Therapeutics) and Epidiolex (GW Pharmaceuticals), may increase the market size in the coming years, assisted by an increase in diagnosed prevalent population of West Syndrome.
West Syndrome Report Insights
• Patient Population
• Therapeutic Approaches
• West Syndrome Pipeline Analysis
• West Syndrome Market Size and Trends
• Market Opportunities
• Impact of upcoming Therapies
West Syndrome Report Key Strengths
• Eleven Years Forecast
• 7MM Coverage
• West Syndrome Epidemiology Segmentation
• Key Cross Competition
• Highly Analyzed Market
• Drugs Uptake
West Syndrome Report Assessment
• Current Treatment Practices
• Unmet Needs
• Pipeline Product Profiles
• Market Attractiveness
• Market Drivers and Barriers
• What was the West Syndrome market share (%) distribution in 2017 and how it would look like in 2030?
• What would be the West Syndrome total market size as well as market size by therapies across the 7MM during the forecast period (2020–2030)?
• What are the key findings pertaining to the market across the 7MM and which country will have the largest West Syndrome market size during the forecast period (2020–2030)?
• At what CAGR, the West Syndrome market is expected to grow at the 7MM level during the forecast period (2020–2030)?
• What would be the West Syndrome market outlook across the 7MM during the forecast period (2020–2030)?
• What would be the West Syndrome market growth till 2030 and what will be the resultant market size in the year 2030?
• How would the market drivers, barriers and future opportunities affect the market dynamics and subsequent analysis of the associated trends?
• What is the disease risk, burden and unmet needs of West Syndrome?
• What is the historical West Syndrome patient pool in the United States, EU5 (Germany, France, Italy, Spain, and the UK) and Japan?
• What would be the forecasted patient pool of West Syndrome at the 7MM level?
• What will be the growth opportunities across the 7MM with respect to the patient population pertaining to West Syndrome?
• Out of the above-mentioned countries, which country would have the highest prevalent population of West Syndrome during the forecast period (2020–2030)?
• At what CAGR the population is expected to grow across the 7MM during the forecast period (2020–2030)?
Current Treatment Scenario, Marketed Drugs and Emerging Therapies:
• What are the current options for the treatment of West Syndrome along with the approved therapy?
• What are the current treatment guidelines for the treatment of West Syndrome in the US and Europe?
• What are the West Syndrome marketed drugs and their MOA, regulatory milestones, product development activities, advantages, disadvantages, safety and efficacy, etc.?
• How many companies are developing therapies for the treatment of West Syndrome?
• How many therapies are developed by each company for the treatment of West Syndrome?
• How many emerging therapies are in the mid-stage and late stage of development for the treatment of West Syndrome?
• What are the key collaborations (Industry–Industry, Industry–Academia), Mergers and acquisitions, licensing activities related to the West Syndrome therapies?
• What are the recent novel therapies, targets, mechanisms of action and technologies developed to overcome the limitation of existing therapies?
• What are the clinical studies going on for West Syndrome and their status?
• What are the key designations that have been granted for the emerging therapies for West Syndrome?
• What are the 7MM historical and forecasted market of West Syndrome?
Reasons to buy
• The report will help in developing business strategies by understanding trends shaping and driving the West Syndrome.
• To understand the future market competition in the asprgillosis market and Insightful review of the key market drivers and barriers.
• Organize sales and marketing efforts by identifying the best opportunities for West Syndrome in the US, Europe (Germany, Spain, Italy, France, and the United Kingdom) and Japan.
• Identification of strong upcoming players in the market will help in devising strategies that will help in getting ahead of competitors.
• Organize sales and marketing efforts by identifying the best opportunities for West Syndrome market.
• To understand the future market competition in the West Syndrome market.
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