Treatment Trials

6 Clinical Trials for Various Conditions

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ENROLLING_BY_INVITATION
Phenylbutyrate for Monogenetic Developmental and Epileptic Encephalopathy
Description

This study is to evaluate the use of glycerol phenylbutyrate for monogenetic developmental epileptic encephalopathies (DEEs). DEEs are characterized by epilepsy and developmental delay in early life. Two examples of DEEs are STXBP1 and SLC6A1, though there are dozens of others. STXBP1 Encephalopathy is a severe disease that can cause seizures and developmental delays in infants and children. SLC6A1 neurodevelopmental disorder is characterized by developmental delay and often epilepsy. Both STXBP1 encephalopathy and SLC6A1 neurodevelopmental disorder cause symptoms because there are not enough working proteins made by these genes. It is possible that a medication called phenylbutyrate may help the the remaining proteins work better for STXBP1, SLC6A1, and/or other similar DEEs caused by single genes (i.e. "monogenetic"). This study is to test if glycerol phenylbutyrate is safe and well tolerated in children with monogenetic DEE.

RECRUITING
STXBP1 and SYNGAP1 Related Disorders Natural History Study
Description

The purpose of this study is to find out more about STXBP1 and SYNGAP1 related disorders. The information gathered by this study will be used to prepare for clinical treatment trials. The primary objective of the study is to better define and outline the clinical spectrum of STXBP1 and SYNGAP1 through detailed developmental, seizure, and quality of life assessments as an extension of routine clinical care.

RECRUITING
Online Study of People Who Have Genetic Changes and Features of Autism: Simons Searchlight
Description

Simons Searchlight is an observational, online, international research program for families with rare genetic variants that cause neurodevelopmental disorders and may be associated with autism. Simons Searchlight collects medical, behavioral, learning, and developmental information from people who have these rare genetic changes. The goal of this study is to improve the clinical care and treatment for these people. Simons Searchlight partners with families to collect data and distribute it to qualified researchers.

Conditions
16P11.2 Deletion Syndrome16p11.2 Duplications1Q21.1 Deletion1Q21.1 Microduplication Syndrome (Disorder)ACTL6BADNPAHDC1ANK2ANKRD11ARID1BASH1LBCL11ACHAMP1CHD2CHD8CSNK2A1CTBP1CTNNB1 Gene MutationCUL3DDX3XDNMT3ADSCAMDYRK1AFOXP1GRIN2AGRIN2BHIVEP2-Related Intellectual DisabilityHNRNPH2KATNAL2KDM5BKDM6BKMT2C Gene MutationKMT2EKMT5BMBD5MED13LPACS1PPP2R5D-Related Intellectual DisabilityPTCHD1RESTSCN2A EncephalopathySETBP1 Gene MutationSETD5SMARCA4 Gene MutationSMARCC2STXBP1 Encephalopathy With EpilepsySYNGAP1-Related Intellectual DisabilityTBR1ARHGEF9HNRNPUPPP3CAPPP2R1ASLC6A12p16.3 Deletions5q35 Deletions5q35 Duplications7q11.23 Duplications15Q13.3 Deletion Syndrome16p11.2 Triplications16P12.2 Microdeletion16P13.11 Microdeletion Syndrome (Disorder)17Q12 Microdeletion Syndrome (Disorder)17Q12 Duplication Syndrome17Q21.31 Deletion Syndrome17q21.3 DuplicationsACTBADSLAFF2ALDH5A1ANK3ARXATRX Gene MutationAUTS2 SyndromeBCKDKBRSK2CACNA1CCAPRIN1CASKCASZ1CHD3CICCNOT3CREBBP Gene MutationCSDE1CTCFDEAF1DHCR7DLG4EBF3EHMT1EP300 Gene MutationGIGYF1GRIN1GRIN2DIQSEC2-Related Syndromic Intellectual DisabilityIRF2BPLKANSL1KCNB1KDM3BNEXMIFKMT2AMBOAT7MEIS2MYT1LNAA15NBEANCKAP1NIPBLNLGN2NLGN3NLGN4XNR4A2NRXN1NRXN2NSD1 Gene MutationPHF21APHF3PHIPPOMGNT1PSMD12RELNRERERFX3RIMS1RORBSCN1ASETD2 Gene MutationSHANK2SIN3ASLC9A6SONSOX5SPASTSRCAPTAOK1TANC2TCF20TLK2TRIOTRIP12UPF3BUSP9XVPS13BWACWDFY3ZBTB20ZNF292ZNF4622Q37 Deletion Syndrome9q34 Duplications15q15 Deletions15Q24 DeletionNR3C2SYNCRIP2q34 Duplication2q37.3 Deletion6q16 Deletion15q11.2 BP1-BP2 Deletion16p13.3 Deletion17Q11.2 Microduplication Syndrome (Disorder)17p13.3Xq28 DuplicationCLCN4CSNK2BDYNC1H1EIF3FGNB1MED13MEF2CRALGAPBSCN1BYY1Xp11.22 DuplicationPACS2MAOAMAOBHNRNPCHNRNPDHNRNPKHNRNPRHNRNPUL25P Deletion SyndromeTCF7L2 Gene MutationHECW2
NOT_YET_RECRUITING
A Clinical Trial of CAP-002 Gene Therapy in Pediatric Patients With Syntaxin-Binding Protein 1 (STXBP1) Encephalopathy
Description

The goal of this clinical trial is to learn about the safety of CAP-002 gene therapy in children with Syntaxin-Binding Protein 1 (STXBP1) Encephalopathy. It will also provide information about whether CAP-002 demonstrates efficacy in treating children with STXBP1 with and without seizures. Participants will have a single infusion of CAP-002, visit the clinic regularly for 2 years for checkups and tests and have seizures recorded in a diary by their caregiver.

NOT_YET_RECRUITING
Registry and Natural History of Epilepsy-Dyskinesia Syndromes
Description

The Registry and Natural History of Epilepsy-Dyskinesia Syndromes is focused on gathering longitudinal clinical data as well as biological samples (blood, urine, and/or skin/tissue) from male and female patients, of all ages, who have a genetic diagnosis of epilepsy-dyskinesia syndromes. Through prospective review and molecular data analysis, the study aims to identify patterns and correlations between movement and seizure disorders, uncovering genotype-phenotype relationships. The initiative's goals are to enhance understanding of epilepsy-dyskinesia syndromes, inform precision medicine approaches, and foster international collaboration.

RECRUITING
A Retrospective Survey-based Multicenter Study to Delineate the Molecular and Phenotypic Spectrum of Epilepsy-dyskinesia Syndromes
Description

The Epilepsy-Dyskinesia Study aims to advance the understanding of the clinical and molecular spectrum of epilepsy-dyskinesia syndromes, monogenic diseases that cause both movement disorders and epilepsy. Addressing challenges in rare disease research -such as small, geographically dispersed patient populations and a lack of standardized protocols- the study employs a multinational retrospective survey endorsed by the International Parkinson and Movement Disorder Society. This survey seeks to collect comprehensive data on clinical features, disease progression, age of onset, genetic variants, and concurrent neurological conditions, standardizing data collection across countries to provide a unified understanding of these conditions. Through retrospective review and molecular data analysis, the study aims to identify patterns and correlations between movement and seizure disorders, uncovering genotype-phenotype relationships. The initiative\'s goals are to enhance understanding of epilepsy-dyskinesia syndromes, inform precision medicine approaches, and foster international collaboration.