West Syndrome Market

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.

Geography Covered

The United States
EU5 (Germany, France, Italy, Spain, and the United Kingdom)
Japan

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.

Key Findings

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.