RecruitingNCT03981276

Phenotypes, Biomarkers and Pathophysiology in Hereditary Spastic Paraplegias and Related Disorders


Sponsor

Dr. Rebecca Schule

Enrollment

2,000 participants

Start Date

Oct 14, 2019

Study Type

OBSERVATIONAL

Conditions

Summary

The aim of this study is to determine the clinical spectrum and natural progression of Hereditary Spastic Paraplegias (HSP) and related disorders in a prospective multicenter natural history study, identify digital, imaging and molecular biomarkers that can assist in diagnosis and therapy development and study the genetic etiology and molecular mechanisms of these diseases.


Eligibility

Inclusion Criteria8

  • One of the following:
  • Primary participant: Clinical or genetic diagnosis of HSP or a related disorder
  • Secondary participant: Unaffected family member (1st or 2nd degree relative) of primary participant (with the above-mentioned restrictions for special populations) able to give informed consent
  • Unrelated healthy control able to give informed consent
  • AND
  • Written informed consent
  • AND
  • \- Participants are willing and able to comply with study procedures

Exclusion Criteria2

  • Missing informed consent of primary or secondary participant/ healthy control/ legal representatives
  • For controls: evidence of a neurodegenerative disease or movement disorders; inability to give informed consent

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Interventions

OTHERClinical rating scale to measure disease severity and progression

A 13-item scale to rate functional impairment occurring in pure forms of spastic paraplegia (SP). Additional symptoms constituting a complicated form of SP are recorded in an inventory.

DIAGNOSTIC_TESTNext-Gen Sequencing (NGS)

Whole Genome Sequencing, Whole Exome Sequencing, Transcriptomics, Proteomics, Metabolomics


Locations(13)

University Innsbruck

Innsbruck, Austria

German Center for Neurodegenerative Diseases (DZNE) Bonn

Bonn, Germany

University of Erlangen

Erlangen, Germany

University Medicine Essen

Essen, Germany

University Göttingen

Göttingen, Germany

University Heidelberg

Heidelberg, Germany

University of Lübeck

Lübeck, Germany

German Center for Neurogedenerative Diseases (DZNE) Magdeburg

Magdeburg, Germany

German Center for Neurodegenerative Diseases (DZNE) München

München, Germany

University of Regensburg

Regensburg, Germany

German Center for Neurodegenerative Diseases (DZNE) Rostock

Rostock, Germany

University of Tübingen and German Center for Neurodegenerative Diseases (DZNE) Tübingen

Tübingen, Germany

IRCCS Medea Scientific Institute, Conegliano-PIeve di Soligo Research Centre

Pieve di Soligo, Italy

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NCT03981276


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