HDSA Center of Excellence at Hennepin County Medical Center

Research Studies

End of Life Care in Huntington’s Disease: Study of Terminal Stage Characteristics

and Patterns of Use of Hospice in Patients staying in a long-term Facility (2005)

This is a retrospective chart review to characterize symptoms observed and

examine the role of hospice in end of life care in 61 patients with HD who died

at the Huntington’s Unit, University Good Samaritan Center, Minneapolis,

Minnesota, between January 1998 and July 2005. Additionally, the extent of

patient and family involvement in determining the nature of treatment in the

terminal stage will also be evaluated.

Qualitative Interviews for the PHAROS study

This was a study consisting of audio taped in-depth personal interviews about the experience of being at risk for Huntington disease (HD), and how these experiences affected the hopes, feelings, and beliefs about research and participation in research. The purpose of this study was to give both researchers interested in HD, and the

HD community as a whole, a better understanding of the experience of being at risk

for HD.

Prevalence of Gluten Sensitivity in HD study

The purpose of this study was to determine how common it is for patients with Huntington’s disease to have a sensitivity to wheat protein (gluten). Allergies to

wheat protein may cause cerebellar degeneration and ataxia (a failure of coordination resulting in “clumsiness” and unsteadiness of posture and walking) in some patients. Since the inheritance of Huntington’s disease is similar in many ways to that of ataxia, we checked patients with HD for wheat allergies by measuring certain antibodies against gluten in the blood, by means of a simple blood sample that was then assayed in the lab. These findings may help plan further projects to study the effect of a gluten-free diet in patients with HD who are allergic to wheat protein.

HD-MAPS (Huntington’s Disease – Modifiers in Age at Onset in Pairs of Siblings)

The purpose of this study was to examine changes in DNA, genetic material, in blood cells that are associated with Huntington’s disease. Nationally, 1000 blood samples were drawn from HD-affected siblings and were sent to a laboratory, where the

blood samples were tested for identification of specific markers on the genes. From

the identification of these specific markers, researchers hope to be able to determine information about onset age of HD. The markers may also provide important clues in delaying disease onset and in developing treatments for HD

Natural History of Huntington’s Disease (An HSG Study)

This study was undertaken to understand more about symptoms that HD patients have, and how the disease progresses over time. Patients were asked to do four neurological tests that were not part of the usual neurological examination. The four tests were: the word association test, the Stroop test, the Symbol Digit Modality Test, and the Luria test.