Journal of Clinical Movement Disorders最新文献

Background: Embarrassment can be a considerable problem for patients with essential tremor (ET) and is a major motivator for treatment. Depression is also a common feature of ET; as many as 35 % of patients report moderate to severe depressive symptoms. Our goal was to assess the associations between these motor and psychosocial factors (tremor, depression, embarrassment) in ET, with a particular interest in more fully assessing the possible association between depression and embarrassment.

Methods: Ninety one ET cases (age 70.4 ± 12.8 years) enrolled in a prospective, clinical-epidemiological study. Depressive symptoms were assessed with the Center for Epidemiological Studies Depression Scale (CESD-10, 0-30 [maximum]), embarrassment, with the Essential Tremor Embarrassment Assessment (ETEA, 0-70 [maximum]), and action tremor, with a detailed in-person neurological examination.

Results: Higher CESD-10 score was significantly associated with higher ETEA score (p = 0.005), but not with increasing tremor severity (p = 0.94). In stratified analyses, cases with no or minimal depressive symptoms had the lowest ETEA scores, cases with moderate depressive symptoms had intermediate ETEA scores, and cases with severe depressive symptoms had the highest ETEA scores (p = 0.01). Furthermore, at each level of tremor severity, cases with more depressive symptoms had more embarrassment.

Conclusions: Depressive symptoms seem to be more than a secondary response to the tremor in ET; they seem to amplify the level of embarrassment and, in addition to their own importance, seem to be a driver of other important clinical outcomes. Earlier treatment of depressive symptoms in ET patients could lessen the burden of secondary embarrassment.

A 45-year-old woman reported automatic behaviors and communication whilst she was being treated with pramipexole. These episodes vanished after the medication was tapered and she was started on levodopa/carbidopa. I hypothesize that the episodes were related to disordered awareness due to sleep disruption related to this medication.

Background: Embouchure dystonia is an unusual focal task-specific dystonia affecting the muscles that control the flow of air into the mouthpiece of a brass or woodwind instrument. The complexity of the embouchure and the relative rarity of the condition pose barriers for recognition and management of the disorder.

Methods: Case review and video survey.

Results: This paper presents four video compilations that illustrate the rich phenomenology of embouchure dystonia, in order to enhance recognition and diagnosis.

Conclusion: The phenomenology of embouchure dystonia is discussed.

Most Parkinson's disease (PD) patients present without known family history and without a diagnosed prodromal phase, underscoring the difficulty of employing primary (neuroprevention) and secondary (neuroprotection) preventions. In cases of monogenic forms, however, potential gene-carrying family members of a proband could engage in neuroprevention, such as exercise or diet modifications, to attenuate the risk of, or delay, disease development. However, a historical lack of recognized disease-modifying interventions has limited clinicians' ability to recommend reliable preventive measures in caring for at-risk populations. We briefly analyze the first retrospective study to examine caffeine consumption and PD risk in a LRRK2 R1628P cohort.

Background: The modulation of levodopa transport across the blood brain barrier by large neutral amino acids is well documented. Protein limitation and protein redistribution diets may improve motor fluctuations in patients with Parkinson's disease but the pharmacokinetics and pharmacodynamics of levodopa and amino acids are highly variable.

Methods: Clinical records of 1037 Parkinson's disease patients were analyzed to determine the proportion of patients with motor fluctuations related to protein interaction with levodopa. Motor fluctuations due to protein interaction with levodopa were defined as dietary protein being associated with (i) longer time to levodopa effectiveness, (ii) reduced benefit or duration of benefit, (iii) dose failures or (iv) earlier wearing off from a previously effective dose. Dose failures, sudden, painful or behavioral wearing-off periods, gait freezing, nausea, hallucinations, orthostasis, and dyskinesias were taken as markers of motor fluctuations, disease severity, and levodopa side effects potentially influenced by protein.

Results: 5.9 % of Parkinson's disease patients on levodopa, and 12.4 % with motor fluctuations on levodopa correlated their fluctuations with the relative timing of levodopa and protein intake. These patients were younger at disease onset, had worse motor fluctuations and had a higher incidence of family members with Parkinson's disease. Early wearing off or decreased dose efficacy were most commonly associated with protein interaction. 60 % of patients who modified their diets had weight loss.

Conclusions: This study suggests that clinically significant protein interaction with levodopa may occur mostly in a subset of Parkinson's disease patients with earlier disease onset and those with familial disease.