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PUBLISHER: DelveInsight | PRODUCT CODE: 1074048

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PUBLISHER: DelveInsight | PRODUCT CODE: 1074048

Developmental and Epileptic Encephalopathies (DEE) - Epidemiology Forecast - 2032

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DelveInsight's "Developmental and Epileptic Encephalopathy - Epidemiology Forecast to 2032" report delivers an in-depth understanding of the DEE, historical and forecasted epidemiology as well as the DEE market trends in the United States, EU5 (Germany, Spain, Italy, France, and United Kingdom) and Japan.

Geographies Covered

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

Study Period: 2019-2032.

Developmental and Epileptic Encephalopathy Disease Understanding and Treatment Algorithm

Developmental and Epileptic Encephalopathy (DEE) refers to a group of severe epilepsies that are characterized both by seizures, which are often drug-resistant, as well as encephalopathy, which is a term used to describe significant developmental delay or even loss of developmental skills. The syndromes of epileptic encephalopathies with onset in the neonatal period, infancy, and early childhood include early myoclonic encephalopathy, Ohtahara syndrome, West syndrome, Dravet syndrome (severe myoclonic epilepsy in infancy), Lennox-Gastaut syndrome (LGS), Landau-Kleffner syndrome (LKS), Epilepsy with continuous spike-and-waves during slow-wave sleep, Doose Syndrome (myoclonic atonic epilepsy), CDKL5 deficiency disorder (CDD), Tuberous sclerosis complex (TSC), SCN8A-DEE, and KCNQ2-DEE.

DEE Diagnosis

The diagnosis of these epileptic encephalopathies begins with an EEG which should include both the sleep and wake states. Moreover, Brain computed tomography (CT) scanning, and more specifically, magnetic resonance imaging (MRI) is also indicated. These should be performed before steroid treatment, which may lead to apparent atrophy on the CT or MRI scan.

Treatment

The approach to the treatment of epileptic encephalopathy has some of the general principles and approach to pediatric epilepsy treatment. The most appropriate anti-epileptic treatment is selected based on the type of epilepsy syndrome. Antiseizure medications includes clobazam, vigabatrin, zonisamide, phenobarbital, benzodiazapines, vigabatrin, and others are used for treatment of DEE. Steroids therapies, vagus nerve stimulation, ketogenic diet and epilepsy surgery are also used for management of DEE.

DEE Epidemiology

The DEE epidemiology division provides insights about historical and current DEE patient pool and forecasted trends for every seven major countries. It helps to recognize the causes of current and forecasted trends by exploring numerous studies and views of key opinion leaders. This part of the DelveInsight's report also provides the diagnosed patient pool and their trends along with assumptions undertaken.

Key Findings

The disease epidemiology covered in the report provides historical as well as forecasted epidemiology segmented by, diagnosed prevalent cases of DEE in the United States, EU5 (Germany, Spain, Italy, France, and United Kingdom), and Japan market from 2019 to 2032.

Country Wise- DEE Epidemiology

This section provides glimpse of the DEE epidemiology in the United States, EU5 (Germany, Spain, Italy, France, and United Kingdom), and Japan.

  • The total diagnosed prevalent cases of DEE in the 7MM were 302,897 in 2021, growing at a CAGR of 0.31% during the study period (2019-2032).
  • Epidemiology assessed for the condition showed that the US, in 2021, accounted for approximately 129,437 diagnosed prevalent cases of DEE.
  • Among the EU-5 countries in 2021, Germany had the highest diagnosed prevalent population of DEE with 21,041 cases, followed by France (17,258) and UK (16,381). In contrast, Spain had the lowest cases (11,743) in 2021.
  • Japan accounted for 23,798 diagnosed prevalent DEE cases in 2021.
  • LGS accounted for the highest number of diagnosed prevalent cases in 7MM. 79,396 cases, were observed in 2021 which are expected to increase by 2032.
  • In EU5 countries, the diagnosed prevalent cases of Tuberous sclerosis complex were highest in Germany with 4,651 in 2021.

Scope of the Report:

  • The Developmental and Epileptic Encephalopathies (DEE) report covers a detailed overview explaining its causes, symptoms, classification, pathophysiology, diagnosis and treatment patterns
  • The Developmental and Epileptic Encephalopathies (DEE) Epidemiology Report and Model provide an overview of the global trends of Developmental and Epileptic Encephalopathies (DEE) in the seven major markets (7MM: US, France, Germany, Italy, Spain, UK, and Japan)
  • The report provides insight into the historical and forecasted patient pool of Developmental and Epileptic Encephalopathies (DEE) in seven major markets covering the United States, EU5 (Germany, Spain, France, Italy, UK), and Japan
  • The report helps recognize the growth opportunities in the 7MM for the patient population
  • The report assesses the disease risk and burden and highlights the unmet needs of Developmental and Epileptic Encephalopathies (DEE)
  • The report provides the segmentation of the Developmental and Epileptic Encephalopathies (DEE) epidemiology

Report Highlights:

  • 11-year Forecast of Developmental and Epileptic Encephalopathies (DEE) epidemiology
  • 7MM Coverage
  • Prevalent and Diagnosed Cases of Developmental and Epileptic Encephalopathies (DEE)
  • Cases of Developmental and Epileptic Encephalopathies (DEE) by Mutation Types
  • Developmental and Epileptic Encephalopathies (DEE) Cases associated with Clinical Manifestations

KOL views

We interview, KOLs and SME's opinion through primary research to fill the data gaps and validate our secondary research. The opinion helps understand the total patient population and current treatment pattern. This will support the clients in potential upcoming novel treatment by identifying the overall scenario of the indications.

Key Questions Answered

  • What will be the growth opportunities in the 7MM with respect to the patient population pertaining to Developmental and Epileptic Encephalopathies (DEE)?
  • What are the key findings pertaining to the Developmental and Epileptic Encephalopathies (DEE) epidemiology across 7MM and which country will have the highest number of patients during the forecast period (2019-2032)?
  • What would be the total number of patients of Developmental and Epileptic Encephalopathies (DEE) across the 7MM during the forecast period (2019-2032)?
  • Among the EU5 countries, which country will have the highest number of patients during the forecast period (2019-2032)?
  • At what CAGR the patient population is expected to grow in 7MM during the forecast period (2019-2032)?
  • What is the disease risk, burden and unmet needs of Developmental and Epileptic Encephalopathies (DEE)?
  • What are the currently available treatments of Developmental and Epileptic Encephalopathies (DEE)?

Reasons to buy:

The Developmental and Epileptic Encephalopathies (DEE) Epidemiology report will allow the user to -

  • Develop business strategies by understanding the trends shaping and driving the global Developmental and Epileptic Encephalopathies (DEE) market
  • Quantify patient populations in the global Developmental and Epileptic Encephalopathies (DEE) market to improve product design, pricing, and launch plans
  • Organize sales and marketing efforts by identifying the age groups and sex that present the best opportunities for Developmental and Epileptic Encephalopathies (DEE) therapeutics in each of the markets covered
  • Understand the magnitude of Developmental and Epileptic Encephalopathies (DEE) population by its epidemiology
  • The Developmental and Epileptic Encephalopathies (DEE) Epidemiology Model developed by DelveInsight is easy to navigate, interactive with dashboards, and epidemiology based with transparent and consistent methodologies. Moreover, the model supports data presented in the report and showcases disease trends over 11-year forecast period using reputable sources

Key Assessments

  • Patient Segmentation
  • Disease Risk & Burden
  • Risk of disease by the segmentation
  • Factors driving growth in a specific patient population
Product Code: DIEI0561

Table of Contents

1. Key Insights

2. Report Introduction

3. Executive Summary

4. Key Events

5. SWOT Analysis

6. Epidemiology and Market Methodology

7. Developmental and Epileptic Encephalopathy (DEE) Market Overview at a Glance

  • 7.1. Market Share (%) Distribution by Therapies in 2021
  • 7.2. Market Share (%) Distribution by Therapies in 2032

8. Disease Background and Overview

  • 8.1. West Syndrome
    • 8.1.1. Clinical Manifestations
    • 8.1.2. Etiology
    • 8.1.3. Pathophysiology
    • 8.1.4. Diagnosis
    • 8.1.5. Differential Diagnosis
  • 8.2. Dravet Syndrome
    • 8.2.1. First Period of Mainly Febrile Convulsions
    • 8.2.2. Second Relentlessly Aggressive Period
    • 8.2.3. Third Static Period of Regression
    • 8.2.4. Etiology
    • 8.2.5. Diagnosis
    • 8.2.6. Differential Diagnosis
  • 8.3. Lennox-Gastaut Syndrome
    • 8.3.1. Etiology
    • 8.3.2. Pathophysiology
    • 8.3.3. Diagnosis
    • 8.3.4. Differential Diagnosis
  • 8.4. Landau-Kleffner Syndrome (LKS) (Acquired Epileptic Aphasia)
    • 8.4.1. Etiology
    • 8.4.2. Pathophysiology
    • 8.4.3. Diagnosis
    • 8.4.4. Differential Diagnosis
  • 8.5. Epilepsy with Continuous Spike-and-Waves during Slow-Wave Sleep
    • 8.5.1. Etiology
    • 8.5.2. Pathophysiology
    • 8.5.3. Diagnostic Procedures
    • 8.5.4. Differential Diagnosis
  • 8.6. Doose Syndrome (myoclonic atonic epilepsy)
    • 8.6.1. Causes
    • 8.6.2. Clinical features
    • 8.6.3. Diagnosis
    • 8.6.4. Differential diagnosis
  • 8.7. CDKL5 deficiency disorder (CDD)
    • 8.7.1. Signs and Symptoms
    • 8.7.2. Causes
    • 8.7.3. Diagnosis
    • 8.7.4. Differential Diagnosis
  • 8.8. Tuberous sclerosis complex (TSC)
    • 8.8.1. Sign and Symptoms
    • 8.8.2. Cause
    • 8.8.3. Diagnosis
  • 8.9. Genetic Epilepsy
    • 8.9.1. SCN8A-DEE
    • 8.9.2. KCNQ2-DEE
  • 8.10. Biomarkers of epileptic encephalopathies
    • 8.10.1. Neuroinflammation and Oxidative Stress
    • 8.10.2. MicroRNA as the Novel Diagnostic Tools for Epilepsy
    • 8.10.3. The Role of the Complement System in Epilepsy
    • 8.10.4. Role of Cytokines in Epileptogenesis
    • 8.10.5. Proteins and Amino Acids Role in Epilepsy

9. Treatment of DEE

  • 9.1. Antiseizure medications
    • 9.1.1. Clobazam (CLB)
    • 9.1.2. Vigabatrin
    • 9.1.3. Topiramate
    • 9.1.4. Levetiracetam
    • 9.1.5. Zonisamide
    • 9.1.6. Phenobarbital
    • 9.1.7. Cannabidiol
    • 9.1.8. Stiripentol
    • 9.1.9. Ethosuximide
    • 9.1.10. Carbamazepine
    • 9.1.11. Lamotrigine
    • 9.1.12. Benzodiazapines (BZDs)
    • 9.1.13. Succinimides
    • 9.1.14. Valproic acid
    • 9.1.15. Rufinamide
    • 9.1.16. Vigabatrin
    • 9.1.17. Felbamate
  • 9.2. Steroid therapy
    • 9.2.1. Prednisolone
    • 9.2.2. ACTH (Adrenocorticotropic Hormone)
  • 9.3. Other Therapies
    • 9.3.1. Vagus Nerve Stimulation
    • 9.3.2. Ketogenic diet
  • 9.4. Epilepsy surgery
  • 9.5. Treatment Algorithm

10. Treatment Guidelines

  • 10.1. Guidelines for Management of Developmental and Epileptic Encephalopathy (National Institute for Health and Care Excellence, 2021)
  • 10.2. Practice Guideline Update: Efficacy and Tolerability of new AEDs I: Treatment of new-onset epilepsy
  • 10.3. Practice Guideline Update: Efficacy and Tolerability of the New AEDs II: Treatment-resistant Epilepsy
  • 10.4. Recommendations for the management of infantile seizures: Task Force Report for the ILAE Commission of Pediatrics

11. Epidemiology and Patient Population of 7MM

  • 11.1. Key Findings
  • 11.2. Assumptions and Rationale
    • 11.2.1. Dravet Syndrome
    • 11.2.2. Lennox-Gastaut Syndrome
    • 11.2.3. Ohtahara Syndrome
    • 11.2.4. West Syndrome
    • 11.2.5. Landau-Kleffner Syndrome
    • 11.2.6. Tuberous Sclerosis Complex
    • 11.2.7. CDKL5 deficiency disorder
    • 11.2.8. Others
  • 11.3. Diagnosed Prevalent Population of DEE in 7MM
  • 11.4. The United States
    • 11.4.1. Diagnosed Prevalent Population of DEE in the United States
  • 11.5. EU5
    • 11.5.1. Diagnosed Prevalence of DEE in EU5
  • 11.6. Japan
    • 11.6.1. Diagnosed Prevalent Population of DEE in Japan

12. Appendix

  • 12.1. Bibliography
  • 12.2. Report Methodology

13. DelveInsight Capabilities

14. Disclaimer

15. About DelveInsight

Product Code: DIEI0561

List of Tables

  • Table 1: Summary of DEE Market, and Epidemiology (2019-2032)
  • Table 2: Key Events
  • Table 3: Main differences between atypical and typical absence seizures
  • Table 4: Nonepileptic and epileptic conditions to differentiate from LGS
  • Table 5: Landau-Kleffner syndrome versus ECSWS
  • Table 6: Molecular Genetic Testing Used in SCN8A-Related Encephalopathy with Epilepsy
  • Table 7: Summary of cytokines and their main role in epileptogenesis.
  • Table 8: Recommendations for monotherapy in adults with new-onset epilepsy with focal epilepsy or unclassified tonic- clonic seizures
  • Table 9: Monotherapy in children with new-onset epilepsy with either focal epilepsy or unclassified GTC seizures
  • Table 10: Monotherapy in adults and children with new-onset GE or unclassified GTC seizures
  • Table 11: Recommendation for childhood absence epilepsy
  • Table 12: For adult patients with TR focal epilepsy, are these AEDs effective as adjunctive therapy
  • Table 13: For adult patients with TR focal epilepsy, are these AEDS effective in reducing seizure f
  • Table 14: For adult and pediatric patients with TR generalized epilepsy (GE), are these AEDs effect
  • Table 15: For adult and pediatric patients with LGS, are these AEDs effective as adjunctive therapy
  • Table 16: For pediatric patients with TR focal epilepsy, are these AEDs effective as adjunctive the
  • Table 17: Summary of published studies addressing AED treatment for infants with seizures
  • Table 18: Total diagnosed Prevalent Population of DEE in the 7MM (2019-2032)
  • Table 19: Diagnosed Prevalent Population of DEE in the United States (2019-2032)
  • Table 20: Diagnosed Prevalence of DEE in EU5 (2019-2032)
  • Table 21: Diagnosed Prevalent Population of DEE in Japan (2019-2032)

List of Figures

  • Figure 1: Neonatal Seizure and epilepsy Classification
  • Figure 2: The spectrum of SCN1A Disorders
  • Figure 3: Oxidative stress and hypoxia as the key players of epileptogenesis
  • Figure 4: Potential mechanisms occurring during epileptogenesis and their correlations with one another.
  • Figure 5: Treatment algorithm
  • Figure 6: Total diagnosed Prevalent Population of DEE in the 7MM (2019-2032)
  • Figure 7: Diagnosed Prevalence of DEE in the United States (2019-2032)
  • Figure 8: Diagnosed Prevalence of DEE in EU5 (2019-2032)
  • Figure 9: Diagnosed Prevalence of DEE in Japan (2019-2032)
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Jeroen Van Heghe

Manager - EMEA

+32-2-535-7543

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Christine Sirois

Manager - Americas

+1-860-674-8796

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