Circadian Rhythm, Cognition, and Mood Disorders in Huntington's Disease.

BACKGROUND: Sleep disturbances are an early and prominent feature of Huntington's disease (HD). OBJECTIVE: The current study investigated the relation between sleep quality impairment and cognitive and psychiatric symptoms in patients with HD. METHODS: Sleep quality, daytime sleepiness, and neurocognitive symptoms were assessed in 38 mutation carriers (23 premanifest and 15 early stage) and 38 age-and sex-matched controls using standardized questionnaires (the Pittsburgh Sleep Quality Index, Epworth Sleepiness Scale, the cognitive section of the Unified Huntington's Disease Rating Scale, the Hospital Anxiety and Depression Scale, and the Irritability Scale). RESULTS: Compared to controls, HD patients had worse sleep quality (p = 0.016), which was associated with more severe cognitive impairment and higher anxiety, depression and irritability scores. These findings suggest that HD patients may have a delayed sleep phase, as indicated by the increased sleep onset latency (p = 0.019) and later wake-up time (0.013), which was associated with worse cognitive performance and greater depressive and anxiety symptoms. CONCLUSIONS: Our data provide further evidence for an association between sleep quality in HD and cognitive performance and psychiatric symptoms.

Treatment of Parkinson disease, time and dosage: "does simple dosage facilitate compliance and therapeutic goals?".

Current available treatment for Parkinson disease has many drawbacks: single action on the nigrostriatal pathway, no halt of disease progression, pulsatile dopaminergic stimulation, complex treatment regimens, and motor complications. Compliance with treatment may be irregular in a variable number of patients. Factors such as age, education, complexity of posology, stage of disease, disease comprehension, cognitive function, or family support significantly influence compliance either in a positive or negative way. The consequences of noncompliance include withdrawal symptoms, increase in number of admissions, or, in severe cases, hyperthermia syndrome secondary to levodopa deficit in the case of infradose. In situations of overdose, dyskinesia or psychiatric complications may arise. The ideal treatment should have the potency of levodopa but not its side effects, act on striatal D2 receptors in a continuous way with a single dose and have low potential of addiction. Thus far, simple regimens are only applicable in early stages.

Evolution of dose and response to botulinum toxin A in cervical dystonia: a multicenter study.

Botulinum neurotoxin (BoNT) is an effective treatment for cervical dystonia (CD). Long-term changes of several variables, including the dose of BoNT, in these patients is largely unknown. We reviewed the clinical charts of 275 patients with CD treated with BoNT type A (BoNT-A) for at least 5 years since 1989 at ten tertiary centers. The mean dose of BoNT-A per session during the first 5 years of treatment was calculated and the appearance of resistance was noted. The dose of BoNT-A for the whole group showed a significant trend to increase over time (year 1: 180 ± 65 U; year 5: 203 ± 63 U; ANOVA: p < 0.0001). However, when we studied the evolution of the dose of BoNT-A for those patients (n = 49) first injected after 2000 (introduction of current BOTOX preparation in our country), there was no significant increase in dose (year 1: 181.8 ± 75 U; year 5: 181.7 ± 75 U; ANOVA p: ns). A total of 19 patients became secondary nonresponders; all but one of these patients began BoNT-A treatment before 2000. In summary, there is a statistically significant increase of mean dose of BoNT-A per session over time, and this could be explained by the appearance of secondary nonresponders. On the other hand, those patients initially treated after 2000 did not show any statistically significant increase in dose for 5 years. This could be explained by better experience and techniques, fewer immunogenic problems with the current BoNT-A, and also less variability of the dose per vial.