Serum carnitine levels and levocarnitine supplementation in institutionalized Huntington's disease patients.

Along with antioxidant properties, carnitine is an important regulator of lipid metabolism in humans. While beneficial effects of carnitine have been demonstrated in animal models of Huntington's disease (HD), metabolism of carnitine has not been studied in humans with this illness. In this retrospective database review from 23 patients admitted to a HD-specialized nursing home unit, we found a relatively high prevalence of hypocarnitinemia (6 cases, 26%). Our review suggests that catabolism and chronic valproate use predisposed our patients to develop hypocarnitinemia. The patients with low serum carnitine levels who received levocarnitine supplementation, during a mean period of 7.3 months, showed improvement in motor, cognitive and behavioral measures. We hypothesize that observed improvement related to the resolution of reversible metabolic encephalopathy and myopathy associated with secondary carnitine deficiency. In conclusion, notwithstanding its limitations, this is the first study to report measurements of carnitine levels in HD patients, revealing relatively high prevalence of hypocarnitinemia in our population. Our findings suggest that HD patients with hypocarnitinemia may benefit from low-dose levocarnitine supplementation. Further studies of carnitine metabolism and supplementation in HD patients are warranted.

Training mental healthcare providers to reduce at-risk patients' access to lethal means of suicide: evaluation of the CALM Project.

The objective of this study was to evaluate the "CALM" (Counseling on Access to Lethal Means) training, in which community-based mental health care providers were trained to work with at-risk clients and their families to assess and reduce access to lethal means of suicide, including firearms. In 2006, CALM trainers conducted workshops in 7 community-based mental health care centers in New Hampshire towns. Participants completed a post-test immediately after the workshop and a follow-up questionnaire approximately 6 weeks later. At follow-up, 65% reported that they had counseled clients' parents about access to lethal means (n = 111). Findings also indicate that the workshop influenced participants' attitudes, beliefs, and skills regarding conducting lethal means counseling.

Sleep patterns in patients with Huntington's disease and their unaffected first-degree relatives: a brief report.

Polysomnographic sleep patterns in Huntington's disease (HD) have been studied sporadically in small groups of patients, providing variable results. In this study, by comparing the polysomnographic sleep patterns of HD patients and their unaffected relatives, identifying sleep traits more specifically related to the HD gene was attempted. The results corroborated previously reported findings of prolonged sleep latency and the virtual absence of nocturnal respiratory disturbances in early HD. Sleep latency in the HD patients positively correlated with the results of a screening test for frontal lobe dysfunction. Larger, more standardized studies will be needed to correlate genetic markers and sleep patterns in HD.