Psychogenic non-epileptic seizures in early Huntington's disease

Huntington's disease (HD) is a neurodegenerative condition characterised by motor dysfunction with involuntary movements and loss of voluntary control, cognitive deterioration and psychiatric problems. We report a 51-year-old man with early HD who experienced stereotyped episodes of repetitive, purposeless complex movements and unresponsiveness. His neurological examination was compatible with HD as were all investigations. We diagnosed psychogenic non-epileptic seizures. While seizures are common in juvenile-onset HD, they are no more prevalent in adult-onset HD than in the general population. However, neuropsychiatric symptoms are common in HD and can involve a number of different complaints. Patients may experience dissociative attacks soon after manifesting a HD diagnosis. Such episodes can be managed with patient and carer education, cognitive behavioural therapy and anxiolytic selective serotonin reuptake inhibitors

Autosomal dominant polycystic kidney disease and coronary artery dissection or aneurysm: A systematic review

Importance Autosomal dominant polycystic kidney disease (ADPKD) has been associated with cardiovascular abnormalities such as intracranial and aortic aneurysms. Objective To systematically review the case reports and case series of ADPKD patients with coronary artery dissection or aneurysm. Evidence review Systematic review registration number: CRD42015015723. Data sources: MEDLINE, Web of Science and OpenGrey, reference lists of studies. Study selection: Published case reports and case series. Data extraction: Two parties analyzed the studies. Disagreements were solved by consensus or by a third party. Funding: none. Findings The reports of 23 patients (22 from 17 studies-six with coronary artery dissection and 16 with coronary artery aneurysm-and one with coronary dissection) were analyzed and reported here. Most patients were symptomatic. Coronary dissection showed female and left descending anterior artery predominance, features similar to non-ADPKD patients, but a median diagnostic age below expected (41 vs. 50 years old). Coronary aneurysms had male and right coronary artery predominance but lower median diagnostic age (44 years old) and higher rate of multiple vessel affection than reported for non-ADPKD patients. Conclusion and relevance Clinical disparities may suggest a different mechanism of aneurysm formation compared to the population without ADPKD. Nevertheless, lack of access to data of one patient and text of one article limited our conclusions. Coronary aneurysms and dissections represent a source of coronary syndromes and death in ADPKD. Mutation of ADPKD-related genes may predispose to coronary abnormalities, especially aneurysms. Further analysis regarding this association is necessary

Adverse events with botulinum toxin treatment in cervical dystonia: How much should we blame placebo?

INTRODUCTION: Botulinum toxin (BoNT) is the first line therapy for cervical dystonia (CD), with most patients receiving many treatment sessions, and so come to recognize and expect the benefits and harms of BoNT, making it difficult to separate which adverse events (AEs) are driven by BoNT and which come from patients' expectations. METHODS: Using the results of three Cochrane systematic reviews of randomized controlled trials (RCTs) we pooled results to calculate the risk of general and specific AEs associated with BoNT, and the proportion of AEs that cannot be pharmacologically attributed to BoNT. RESULTS: Fifteen RCTs, enrolling 1604 patients, were included. BoNT was associated with an increased risk of AEs, but 79% of this increased risk cannot be pharmacologically attributed to BoNT. CONCLUSIONS: Patients with CD attach a considerable expectation of harm due to BoNT, reflected in the large proportion of non-pharmacologically-mediated AEs

Meta-research metrics matter: letter regarding article “indirect tolerability comparison of Deutetrabenazine and Tetrabenazine for Huntington disease”

Here we discuss the report by Claassen and colleagues describing an indirect treatment comparison between tetrabenazine and deutetrabenazine for chorea in Huntington’s disease using individual patient data. We note the potential for discrepancies in apparently statistically significant findings, due to the rank reversal phenomenon. We provide some cautionary observations and suggestions concerning the limitations of indirect comparisons and the low likelihood that good quality evidence will become available to guide clinical decision comparing these two agents

Deep brain stimulation for dystonia

This is a protocol for a Cochrane Review (Intervention). The objectives are as follows: To compare the efficacy, safety and tolerability of deep brain stimulation versus placebo, sham intervention, or best medical care, including botulinum neurotoxin and resective/lesional surgery, in people with dystonia

Tetrabenazine versus deutetrabenazine for Huntington's disease: twins or distant cousins?

BACKGROUND: Tetrabenazine is the only US Food and Drug Administration-approved drug for Huntington's disease, and deutetrabenazine was recently tested against placebo. A switching-trial from tetrabenazine to deutetrabenazine is underway, but no head-to-head, blinded, randomized controlled trial is planned. Using meta-analytical methodology, the authors compared these molecules. METHODS: RCTs comparing tetrabenazine or deutetrabenazine with placebo in Huntington's disease were searched. The authors assessed the Cochrane risk-of-bias tool, calculated indirect treatment comparisons, and applied the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) framework. RESULTS: The evidence network for this report comprised 1 tetrabenazine trial and 1 deutetrabenazine trial, both against placebo. Risk of bias was moderate in both. Participants in the tetrabenazine and deutetrabenazine trials did not differ significantly on motor scores or adverse events. Depression and somnolence scales significantly favored deutetrabenazine. CONCLUSION: There is low-quality evidence that tetrabenazine and deutetrabenazine do not differ in efficacy or safety. It is important to note that these results are likely to remain the only head-to-head comparison between these 2 compounds in Huntington's disease