Human study shows that inflammation in the esophagus (swallowing tube) and stomach is common and occurs more frequently in Huntington's disease (HD) as it progresses. Study also shows that individuals often do not complain. Lower GI function has not been investigated in individuals with HD, but if mouse models of HD are predictive, we can expect problems there too. But the biggest contributor to gut distress in HD is likely due to medication side effects, less intake of water and more sedentary lifestyle as the disease progresses.

Pathological laughter or crying (shortened to PLC) refers to laughter or crying that is deemed socially inappropriate for the situation. Often the individual will laugh or cry spontaneously for no apparent reason. The abnormal behavior is one of impaired emotional expression. Can this be a symptom of Huntington's disease; and if so how can it be treated?

Dysphagia, or difficulty swallowing, is a big problem in Huntington's disease (HD), but surprisingly little is known about it. It is not known how early it begins, or how symptoms and swallowing dysfunction progress over the stages of HD. Further, even less is known about techniques that might prevent, alleviate or treat it. As such, we welcome the recent attention given this symptom by investigators from Europe.

A recent press release from Auspex Pharmaceuticals reported on an interim analysis from their ongoing ARC-HD trial that switching from tetrabenazine (Xenazine) to SD-109, resulted in equal control of chorea in Huntington's disease. SD-109 is a chemically modified form of tetrabenazine that allows for a lower dosing regimen. It is hoped that lower dosing of SD-109 will result in fewer side effects than the present form tetrabenazine.

For more than a decade, couples at risk for Huntington's disease have had the option to conceive an unaffected child by utilizing the reproductive process of preimplantation genetic diagnosis (PGD). If PGD became common practice in HD, it could eliminate the majority of disease for the next generation. So if PGD has the potential to mostly "wipe out" HD for the next generation, why is it so rarely utilized?

Caregiving for a person with later stage Huntington's disease is a loving long-term commitment that can bring rewarding fulfillment but sometimes may be hazardous to your health. Though some later studies do not confirm, an early often quoted study suggested that long-term caregivers who report emotional strain are at greater risk for medical illness and earlier death than those who are not caregivers. What are the health risks of caregiving? What can be done to give some relief for caregiver strain?

In a paper published this week in the prestigious journal Nature, Dr. Solomon Snyder and colleagues reported promising results in cell and mouse models of Huntington's disease (HD). They showed that these models were deficient in both (1) cysteine, one of the amino acid building blocks in proteins, and (2) an enzyme molecule involved in making cysteine. And more exciting, they showed that adding cysteine was protective in these models.

Might cysteine help people with Huntington's disease? Preliminary results reported in Raptor Pharmaceutical's recent press release suggests it may.

Raptor Pharmaceutical Corp. announced some positive (though preliminary) results from the 18-month review of their CYST-HD Phase 2/3 clinical trial testing a cysteamine drug for Huntington's disease (HD). They reported slower progression of motor impairment in those participants who had received drug compared to those who had received placebo. If these positive results hold up on further analysis and completion of the remaining 18 months of trial, this author believes we have very good reasons to be optimistic about this drug for HD.

What is this Raptor drug? Why try this drug in an HD clinical trial? And -- why might we be optimistic now?

Antipsychotic drugs are frequently prescribed for several symptoms of Huntington's disease (HD). Does it make any difference which one is used? While there is no clinical trial evidence for HD, early clinical trials in schizophrenia suggested that one is not that much better than another. However more recent reviews and studies make the important point that older ones -- like Haldol -- appear to have more unwanted side effects than the newer ones, side effects that may further impair gait, balance and cognition in HD.

First-HD is a clinical trial testing SD-109 ER, a new extended-release form of tetrabenazine (Xenazine). Finding the 90 participants, who must have moderately severe untreated chorea, has been difficult and slower than expected. Only 4 or 5 participants are needed for each of the 20 centers doing this trial. Why should we bother with this trial? Because we will never have better medical treatments if we don't join this, and other clinical trials.