More than 250 individuals from Huntington's disease (HD) families and their friends participated in the Huntington Study Group (HSG) Symposium and Clinical Research Workshop held in Seattle on November 10, 2012. Attendance was higher in Seattle than any of the prior cities hosting the symposium over the previous 5 years. At this "one of a kind" meeting HD families sat at circular tables with clinical researchers from all over the world, sharing information, hearing about new research -- and most important -- learning from each other.

The day was divided into 3 parts: symposium, poster, and workshop sessions . . .

HSG-2012-Huntington’s Disease in Clinical Practice

Dates: 11/8/2012 – 11/10/2012

Tuition: Free for preregistration, $25.00 at the door. Location: Hyatt at Olive 8, 1635 8th Avenue, Seattle, WA

Course Director: LaVonne Veatch Goodman MD, in coordination with members of the Huntington Study Group Education Committee

Course Description: This course will coincide with the Huntington Study Group Annual Meeting and Research Symposium. Despite the availability of symptomatic therapies, Huntington’s disease patients and families are often under-treated. To address this problem, national and local Huntington’s experts will present practical information for community providers (physician generalists and subspecialists, nurses, and social workers) on the symptomatic treatment and care for Huntington’s disease patients.

Topics on the first two days will include current expert recommendations for genetic counseling, treatment of both motor and behavioral/psychiatric symptoms, use of ancillary speech and physical therapies, advocating for disability benefits, and palliative care for late and end-stage patients. On the final day, there will be a poster session and presentations by the experts on recent clinical research.

Thursday, November 8, 2012 (3 CME Hours)

2:00-3:00 pm


3:00-3:30 pm

Welcome and Introduction

Ira Shoulson, MD

LaVonne Veatch Goodman, MD

3:30-5:00 pm

Breakout Sessions

UHDRS Training

Physical, Occupational and Speech Therapy for HD

Ramon Rodriguez, MD

Karen Anderson, MD

Leigh Beglinger, PhD

Ashwini Rao, EdD, OTR

Deanna Britton, PhC, CCC-SLP

5:00-5:30 pm


5:30-6:30 pm

Evolution of Genetic Counseling for Huntington’s Disease

Clare Gibbons, MS, CCGC, CGC

Thomas Bird, MD

Susan Creighton, MS, CGC

Corrie Smith, MS. CGC

6:30-7:30 pm

Welcome Reception

Friday, November 9, 2012 (7 CME Hours)

7:00-7:45 am

Registration and Breakfast

7:45-8:00 am

Welcome and Introduction

LaVonne Veatch Goodman, MD

8:00-8:30 am

Clinical Genetics of HD

Thomas Bird, MD

8:30-9:00 am

Overview of Pre and Early HD

Blair Leavitt, MD

9:00-9:30 am

Overview of Middle Stages of HD

Ali Samii, M.D.

9:30-10:00 am


10:00-11:00 am

Successfully Advocating for HD Disability

Sam Frank, M’D.

Jane Kogan, LMSW

Bonnie L. Hennig, MSW, LCSW, QCSW, DCSW

11:00-12:00 am

Overview and Management of Late and End-stage HD

Martha Nance, MD

12:00-1:00 pm


1:00-3:00 pm

Behavioral Treatments for HD with Case Presentations

Mary Edmondson, MD.

Karen Anderson, MD

Mark Groves, MD

3:00-3:30 pm


3:30-4:30 pm

Identification and Management of Motor Symptoms of HD

Ralf Reilmann, MD


Support Services and Clinical Research Opportunities in the Northwest

Chris Wick, MSW, LICSW

LaVonne Veatch Goodman, MD

Saturday, November 10, 2012 (3 CME Hours)

Sixth Annual Huntington Disease Clinical Research Symposium: 8:00am -12:30pm

Symposium Poster Session from 8:00-9AM

The Symposium will feature keynote addresses beginning at 9:00am by:

  • Charles Sabine, HD Advocate and Mary Edmondson, MD, Duke University Medical Center
  • Ashwini Rao, EdD, OTR, Columbia University
  • Pierre Tariot, MD, Banner Alzheimer's Institute
  • Blair Leavitt, MD, University of British Columbia

In addition to these keynote addresses, clinical scientists will be presenting their original research on relevant topics of interest to the HD community today. The Symposium will conclude with an open panel discussion, which will present audience members with the opportunity to interact with the day's presenters.

On November 8-10, the Huntington Study Group (HSG) is bringing two exciting events to Seattle to coincide with their annual meeting. First, as they have done the last 5 years they will host a Clinical Research Symposium and Workshop for families in the Northwest communities. And next, a brand new event: "Huntington's Disease in Clinical Practice" a Continuing Education Course (up to 12.5 FREE hours) for doctors, nurses, social workers, and genetic counselors will be offered -- made possible by a grant from the Griffin Foundation.

Now is the time to sign up -- you and your family members for the symposium, and your doctor for the course..

Lithium is an old drug that was first used for manic depressive or bipolar disorder more than 60 years ago. Over the last decade this drug has also been considered as treatment for several neurodegenerative diseases including Huntington's disease (HD); but the problem is its high risk of toxicity, particularly for long term use. But now with the recent publication about NP03, a novel (or new) low-dose lithium reformulation showing neuroprotective benefit in the YAC128 mouse model of HD, it is back in the running as a promising potential HD therapeutic.

NP03 is a new drug from Medesis Pharma that delivers lithium in a water and oil microemulsion that allows for much lower (15X lower) therapeutic dosing. Barring future problems, this drug may be on its way to study in people.

The prestigious American Academy of Neurology (AAN) has published a new set of "evidence-based" guidelines for the treatment of chorea in Huntington's disease (HD). These guidelines are based on "best evidence", which uses only sets of data from clinical trials or studies testing drugs for chorea. But what if there isn't enough evidence to make sound medical recommendations -- which had been the conclusion drawn by the Cochrane Review and the survey of expert HD physician leaders from the European Huntington's Disease Network (EHDN) and the Huntington Study Group (HSG)?

Why be concerned about the AAN guidelines? At best they are inappropriate; at worst they have potential to harm.

At the recent Enroll-HD Global Investigator Meeting a week ago, we learned that this soon-to-launch study sponsored by CHDI is intended to be more than observing progression in Huntington's disease (HD). It will enable investigators to do "add-on" studies that focus on specific populations (like Juvenile onset), or on "cure" efforts like the search for biomarkers, or preparatory study of new drugs before launching large scale trials, and to identify participants for clinical trials, etc.

But we also learned about an exciting new focus: Enroll-HD data may be used to improve HD care at HD treatment centers. And in a presentation by Kathryn Sabadosa from the Cystic Fibrosis Foundation we learned how this might be done.

Over the past decade a number of over-the-counter (OTC) supplements and prescription drugs have been reported to slow down progression of impairment in animal models of Huntington's disease (HD). Whether Huntington's people "should" take supplements for potential human benefit is unknown. This author's opinion has evolved over the years and I am now more skeptical about using mouse model results than when we initiated the HDDW trials. What follows will be a supplement update.

But -- to keep this discussion in perspective -- it is much more likely that a healthy life style will give greater benefit than any present supplement.

Why is a healthy life style important in HD? A big part of the answer include factors that promote the process of neuroplasticity. The "plasticity" part of neuroplasticity refers to capacity to change, similar to how plastic is malleable and can be made into many shapes. Similarly, neuroplasticity describes the ability of nerve cells to change -- based on various forces -- by making new, or stronger connections to other nerve cells in the brain. Healthy life style factors promote these forces. And vitally important in brain diseases, healthy life style factors can help preserve brain function while damage occurs.

Lack of good sleep is a major problem in Huntington's disease (HD). Further, the degree of sleep disturbance correlates with levels of fatigue, depression, cognitive impairment, harmful metabolic or energy factors, and degree of damage seen on brain scans. Bad sleep is bad news. But the good news, at least in HD-mouse models, is that rigidly scheduled "environmental entrainment" can improve several of these factors. To put this in perspective: the level of benefit is far greater than that reported for any supplement or drug tested in mice.

And best news? HD individuals can do this too, and if the experts are correct we'll not only feel and think better, we may slow progression of the disease.

Huntington's disease (HD) is commonly characterized as a trilogy of overlapping disorders that include cognitive, motor, and psychiatric components. But this author suggests that we should add HD stigma to the list. The stigma part of the HD has great negative impact on many, but it is not well studied or recognized for the toxic part it plays through every stage of this disease.

Perhaps, if we thought of stigma as an official part of HD, it might get the attention it deserves.