LASSI-L detects early cognitive changes in pre-motor manifest Huntington's disease: a replication and validation study.

Background And Objectives: Cognitive decline is an important early sign in pre-motor manifest Huntington's disease (preHD) and is characterized by deficits across multiple domains including executive function, psychomotor processing speed, and memory retrieval. Prior work suggested that the Loewenstein-Acevedo Scale for Semantic Interference and Learning (LASSI-L)-a verbal learning task that simultaneously targets these domains - could capture early cognitive changes in preHD. The current study aimed to replicate, validate and further analyze the LASSI-L in preHD using larger datasets.

Using the LASSI-L to Detect Robust Interference Effects in Premanifest Huntington Disease.

Background: Diagnosis of manifest Huntington disease (HD) is based primarily on motor symptoms, but premanifest HD (preHD) is often associated with subtle cognitive decline. The Loewenstein-Acevedo Scales for Semantic Interference and Learning (LASSI-L) is a validated verbal learning test that can be used to detect early cognitive decline.

Objective: To determine the utility of the LASSI-L for detecting early cognitive decline in individuals with preHD and to compare the results of the LASSI-L with those of commonly used neuropsychological tests in HD.

A Multi-center Genome-wide Association Study of Cervical Dystonia.

Background: Several monogenic causes for isolated dystonia have been identified, but they collectively account for only a small proportion of cases. Two genome-wide association studies have reported a few potential dystonia risk loci; but conclusions have been limited by small sample sizes, partial coverage of genetic variants, or poor reproducibility.

Objective: To identify robust genetic variants and loci in a large multicenter cervical dystonia cohort using a genome-wide approach.

Temporal course of the tourette syndrome clinical triad.

Background: Tourette syndrome (TS) is a disorder characterized by childhood onset of motor and phonic tics, often with improvement of tic symptoms by young adult years. The temporal course of tics and commonly comorbid behavioral symptoms is still not well characterized.

Methods: In order to clarify the time course of tics and comorbid attention deficit hyperactivity disorder (ADHD) or obsessive compulsive disorder (OCD) in TS, we administered a brief survey regarding the course of symptoms at a single point in time to 53 TS patients aged 13-31 years.

Botulinum toxin for painful spasms from focal seizures: theoretical considerations and case report.

Introduction: Botulinum toxin (BTX) has been used to treat muscle overactivity and can reduce pain from muscle spasm. There are no previous reports to our knowledge of using BTX to treat the pain from intractable focal motor seizures.

Case Report: We present a case of chronic, focal, muscle pain, and spasm secondary to intractable focal motor seizures treated with BTX.

Writing from the wards: advice for residents.

Introduction: Published articles share information and ideas across fields and can be a part of educational and career development. Academic and personal interests are the typical motivators, but many residents have trouble with the logistics of writing and the simple act of getting started.

Objective: The following article gives concrete advice, structural recommendations and addresses logistical concerns in writing manuscripts, focusing on case reports.

Comparison of enrollees and decliners of Parkinson disease sham surgery trials.

Concerns have been raised that persons with serious illnesses participating in high-risk research, such as PD patients in sham surgery trials, have unrealistic expectations and are vulnerable to exploitation. A comparison of enrollees and decliners of such research may provide insights about the adequacy of decision making by potential subjects. We compared 61 enrollees and 10 decliners of two phase II neurosurgical intervention (i.

An approach to evaluating the therapeutic misconception.

Subjects enrolled in studies testing high risk interventions for incurable or progressive brain diseases may be vulnerable to deficiencies in informed consent, such as the therapeutic misconception (TM). However, there is a continuing debate about the definition and measurement of TM, making assessments of TM controversial. In this qualitative pilot study of persons enrolled in a phase I test of gene transfer for Parkinson’s disease, we developed and tested an interview guide focusing on how the subjects made their decision to participate, with an emphasis on understanding the as the unit of interest, rather than focusing only on isolated statements.

Ethics of sham surgery: perspective of patients.

Sham surgery is used as a control condition in neurosurgical clinical trials in Parkinson's disease (PD) but remains controversial. This study aimed to assess the perspective of patients with PD and the general public on the use of sham surgery controls. We surveyed consecutive patients from a university-based neurology outpatient clinic and a community-based general internal medicine practice.

The neurology resident as teacher: evaluating and improving our role.

There is a need to improve the quality of teaching done by neurology residents because neurology residents are intimately involved in medical student education. This article reviews the available literature on techniques to improve the teaching ability of resident physicians. Eight randomized prospective studies were identified from health and education databases.

The challenges of residents teaching neurology.

Background: Teaching is integrated into the daily practice of residents, and it is a skill necessary for practice as well as academics. The settings in which teaching and learning take place are ubiquitous but include classrooms, small groups, bedside rounds, and grand rounds. Given the learning environment of residency, neurology residents should have working knowledge of basic principles of effective teaching to make learning successful.