Clinical Utility of Neurophysiologic Classification (and Declassification) of Myoclonus.

Background: Movement clinical neurophysiology studies can distinguish myoclonus, tremor, and other jerky movements; however, there has been limited demonstration of their real-world clinical impact.

Objective: The aim was to investigate movement study utility in clarifying movement classification and guiding patient management.

Method: A retrospective study of myoclonus-related movement studies was performed.

Directed stimulation of the dentato-rubro-thalamic tract for deep brain stimulation in essential tremor: a blinded clinical trial.

Objective: Observational studies utilising diffusion tractography have suggested a common mechanism for tremor alleviation in deep brain stimulation for essential tremor: the decussating portion of the dentato-rubro-thalamic tract. We hypothesised that directional stimulation of the dentato-rubro-thalamic tract would result in greater tremor improvement compared to sham programming, as well as comparable improvement as more tedious standard-of-care programming.

Methods: A prospective, blinded crossover trial was performed to assess the feasibility, safety and outcomes of programming based solely on dentato-rubro-thalamic tract anatomy.

Urine levels of the polyglutamine ataxin-3 protein are elevated in patients with spinocerebellar ataxia type 3.

Introduction: Accumulation of polyglutamine (polyQ) ataxin-3 (ATXN3) contributes to the pathobiology of spinocerebellar ataxia type 3 (SCA3). Recently, we showed that polyQ ATXN3 is elevated in the plasma and cerebrospinal fluid (CSF) of SCA3 patients, and has the potential to serve as a biological marker for this disease [1]. Based on these findings, we investigated whether polyQ ATXN3 can also be detected in urine samples from SCA3 patients.

Physiology-Based Treatment of Myoclonus.

Myoclonus can cause significant disability for patients. Myoclonus has a strikingly diverse array of underlying etiologies, clinical presentations, and pathophysiological mechanisms. Treatment of myoclonus is vital to improving the quality of life of patients with these disorders.

Botulinum toxin for cervical dystonia: addressing treatment failure and improving outcomes.

Introduction: Cervical dystonia is a form of focal dystonia characterised by tilting and turning of the head and neck. This can cause significant disability in affected patients. Botulinum toxin injections are the mainstay of therapy.