Longitudinal Study of Neurogenetic Disorders

NCT ID: NCT03492060

Last Updated: 2025-10-27

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Total Enrollment

1000 participants

Study Classification

OBSERVATIONAL

Study Start Date

2018-06-13

Study Completion Date

2030-12-31

Brief Summary

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The purpose of this study is to analyze patterns in individuals with hnRNP (and other) genetic variants, including their neurological comorbidities, other medical problems and any treatment. The investigators will maintain an ongoing database of medical data that is otherwise being collected for routine medical care. The investigators will also collect data prospectively in the form of questionnaires, neuropsychological assessments, motor assessments, and electroencephalography to examine the landscape of deleterious variants in these genes.

Detailed Description

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Neurodevelopmental disorders are a group of disorders in which the development of the central nervous system is disturbed. The genetic basis for many neurodevelopmental disorders has continued to expand and a recent gene called HNRNPH2 (Heterogeneous Nuclear Ribonucleoprotein H2, encoded by HNRNPH2) is one such gene that is associated with a common neurodevelopmental disorder characterized by developmental delay, intellectual disability, autism and autistic features, and tone abnormalities, among other multisystem problems.

The investigators will expand the genetic cohort to include any individual with a confirmed variant in any gene presenting with neurodevelopmental abnormalities. This is non-interventional study that examines both data previously used in clinical practice and prospective data collection in the form of questionnaires and assessments. The investigators will examine patterns of initial presentation, patterns in neurological evaluations; neurological testing including brain MRI and electroencephalography, and outcomes in individuals with genetic variants.

Genes of Focus:

hnRNPA1 hnRNPA2 hnRNPB1 hnRNPB2 hnRNPC2 hnRNPD hnRNPE1 hnRNPE2 hnRNPE3 hnRNPE4 hnRNPG hnRNPH1 hnRNP H2 hnRNPI hnRNPK hnRNPL hnRNPM hnRNPP hnRNPQ1 hnRNPQ2 hnRNPQ3 hnRNPR hnRNPU

Conditions

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Neurodevelopmental Disorders Intellectual Disability Developmental Delay Autism Spectrum Disorder Seizures Hypertonia, Muscle Hypotonia

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

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Variant in a hnRNP gene

Individuals with a variant in any hnRNP gene who present with neurodevelopmental abnormalities are eligible for the study.

No interventions assigned to this group

Variant in other gene

Individuals with a confirmed variant in other genes who present with neurodevelopmental abnormalities are eligible for the study.

No interventions assigned to this group

Eligibility Criteria

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Inclusion Criteria

* Individuals must have had whole genome/exome sequencing and have a confirmed variant in any gene.

Exclusion Criteria

* Subjects who cannot provide genetic confirmation of a predicted deleterious variant in any gene.
Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Simons Foundation

OTHER

Sponsor Role collaborator

New York University

OTHER

Sponsor Role collaborator

Hackensack Meridian Health

OTHER

Sponsor Role collaborator

Universitätsklinikum Hamburg-Eppendorf

OTHER

Sponsor Role collaborator

Columbia University

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Jennifer M. Bain, MD, PhD

Role: PRINCIPAL_INVESTIGATOR

Columbia University

Locations

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Columbia University Irving Medical Center

New York, New York, United States

Site Status RECRUITING

Countries

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United States

Central Contacts

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Jennifer M. Bain, MD, PhD

Role: CONTACT

646-426-3876

Joanna Feng

Role: CONTACT

Facility Contacts

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Study Team

Role: primary

Joanna Feng

Role: backup

References

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Bain JM, Cho MT, Telegrafi A, Wilson A, Brooks S, Botti C, Gowans G, Autullo LA, Krishnamurthy V, Willing MC, Toler TL, Ben-Zev B, Elpeleg O, Shen Y, Retterer K, Monaghan KG, Chung WK. Variants in HNRNPH2 on the X Chromosome Are Associated with a Neurodevelopmental Disorder in Females. Am J Hum Genet. 2016 Sep 1;99(3):728-734. doi: 10.1016/j.ajhg.2016.06.028. Epub 2016 Aug 18.

Reference Type BACKGROUND
PMID: 27545675 (View on PubMed)

Bain JM, Thornburg O, Pan C, Rome-Martin D, Boyle L, Fan X, Devinsky O, Frye R, Hamp S, Keator CG, LaMarca NM, Maddocks ABR, Madruga-Garrido M, Niederhoffer KY, Novara F, Peron A, Poole-Di Salvo E, Salazar R, Skinner SA, Soares G, Goldman S, Chung WK. Detailed Clinical and Psychological Phenotype of the X-linked HNRNPH2-Related Neurodevelopmental Disorder. Neurol Genet. 2021 Jan 29;7(1):e551. doi: 10.1212/NXG.0000000000000551. eCollection 2021 Feb.

Reference Type RESULT
PMID: 33728377 (View on PubMed)

Davis TJ, Salazar R, Beenders S, Boehme A, LaMarca NM, Bain JM. A Prospective, Longitudinal Study of Caregiver-Reported Adaptive Skills and Function of Individuals with HNRNPH2-related Neurodevelopmental Disorder. Adv Neurodev Disord. 2024;8(3):445-456. doi: 10.1007/s41252-023-00346-1. Epub 2023 Aug 7.

Reference Type DERIVED
PMID: 39220267 (View on PubMed)

Related Links

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http://hnrnph2.com

Original HNRNPH2 genetic cohort website

Other Identifiers

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AAAR7203

Identifier Type: -

Identifier Source: org_study_id

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