Overview

Overview

The Huntington’s disease Center of Excellence (HD COE) research team has a dedicated focus on clinical care, research, and innovation. The uniqueness of our Center is that it was developed from the ground up as a multidisciplinary center for HD care and treatment. Therefore, integrated and seamless medical management, neuropsychiatric support, and PT/OT/speech interventions are at the heart of our approach. Our HD COE has dedicated movement disorder research coordinators, psychiatrists, neurosurgeons, neuropsychologists, and the support of basic scientists at the Cleveland Clinic Lerner Research Institute for faster project start-up and better overall compliance and communication during clinical trials.

We are a distinctive center in which the clinical and research operations are merged. All physicians participate in research and work together in developing ideas and proposals and recruiting for clinical trials. Our HD COE has participated in numerous clinical trials focusing on predicting the onset and progression of pre-manifest and manifest Huntington disease. As an example, we were one of the nationally-recognized centers that participated in functional MRI imaging for HD in the PREDICT clinical trial. We were also part of the large CREST-E multicenter trial that evaluated the safety, tolerability, and efficacy of creatine in HD. The aims of our Center are 1) the development of biomarkers for the progression of Huntington disease and to understand their relationship to disease progression; 2) the prediction of pre-manifest Huntington's disease by using clinical and imaging measures; and 3) merging information from neuroimaging data, serial clinical information, and genetic information to predict accurately the onset of disease and the efficacy of novel drug intervention on disease progression.

Cleveland Clinic has been a certified site for Huntington's disease study group (consortium of academic research institutions). We currently are enrolling patients for a study called ENROLL-HD, which is the world's largest observational study for Huntington's disease families.

Contact Information

Adam Margolius, MD
margola@ccf.org

Publications

Publications

Network topology and functional connectivity disturbances precede the onset of Huntington’s disease. Brain. 2015 Aug; 138(8): 2332–2346.Deborah L. Harrington, Mikail Rubinov,Sally Durgerian, Lyla Mourany, Christine Reece, Katherine Koenig, Ed Bullmore, Jeffrey D. Long, Jane S. Paulsen  for the PREDICT-HD investigators of the Huntington Study Group and Stephen M. Rao.

Disruption of response inhibition circuits in prodromal Huntington disease.   Cortex 2014 Sep;58:72-85. A. Rao a, Deborah L. Harrington b,c, Sally Durgerian d,Christine Reece e, Lyla Mourany e, Katherine Koenig f, Mark J. Lowe f,Vincent A. Magnotta g, Jeffrey D. Long g, Hans J. Johnson g, Jane S. Paulsen g, and Stephen M. Rao. doi: 10.1016/j.cortex.2014.04.018. Epub 2014 Jun 2. 

Diffusion Weighted Imaging of Prefrontal Cortex in Prodromal Huntington’s Disease. Human Brain Mapping 2014 35:1562–1573 (2014)Joy T. Matsui, Jatin G. Vaidya, Hans J. Johnson, Vincent A. Magnotta,Jeffrey D. Long,James A. Mills,1 Mark J. Lowe, Ken E. Sakaie,Stephen M. Rao, Megan M. Smith,and Jane S. Paulsen.

Prediction of manifest Huntington's disease with clinical and imaging measures: a prospective observational study. PREDICT-HD Investigators and Coordinators of the Huntngton Study Group. Lancet Neurol. 2014 Dec;13(12):1193-201. doi: 10.1016/S1474-4422(14)70238-8. Epub 2014 Nov 3.

CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion... PREDICT-HD study of the Huntington Study Group (HSG) collaborator. Neurology. 2012 Mar 6;78(10):690-5. doi: 10.1212/WNL.0b013e318249f683. Epub 2012 Feb 8.

Members & Collaborations

Members & Collaborations

Cleveland Clinic