IAPRD new consensus classification of myoclonus.

Introduction: Recent new advances in myoclonus characterization and etiology justify an update of the 40-year-old respected classification of myoclonus proposed by Marsden, Hallett, and Fahn. New advances include genetic studies and clinical neurophysiology characterization.

Methods: The IAPRD appointed an expert panel to develop a new myoclonus classification.

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.

Conjugal Synucleinopathies: A Clinicopathologic Study.

Background: While preclinical studies have shown that alpha-synuclein can spread through cell-to-cell transmission whether it can be transmitted between humans is unknown.

Objectives: The aim was to assess the presence of a synucleinopathy in autopsied conjugal couples.

Methods: Neuropathological findings in conjugal couples were categorized as Parkinson's disease (PD), dementia with Lewy bodies (DLB), Alzheimer's disease with Lewy bodies (ADLB), incidental Lewy body disease (ILBD), or no Lewy bodies.

Oil-based compounding flavors more accepted by feline patients.

Objective: To evaluate the voluntary acceptance of 10 commercially available compounding flavors in cats.

Animals: 46 healthy cats between 1 and 12 years of age.

Procedures: Each cat underwent a 14-day study period consisting of a 4-day acclimation period followed by a 10-day trial period in which each cat was randomly offered 10 different compounding flavors.

Myoclonus and other jerky movement disorders.

Myoclonus and other jerky movements form a large heterogeneous group of disorders. Clinical neurophysiology studies can have an important contribution to support diagnosis but also to gain insight in the pathophysiology of different kind of jerks. This review focuses on myoclonus, tics, startle disorders, restless legs syndrome, and periodic leg movements during sleep.

A Preliminary Report of Network Electroencephalographic Measures in Primary Progressive Apraxia of Speech and Aphasia.

The objective of this study was to characterize network-level changes in nonfluent/agrammatic Primary Progressive Aphasia (agPPA) and Primary Progressive Apraxia of Speech (PPAOS) with graph theory (GT) measures derived from scalp electroencephalography (EEG) recordings. EEGs of 15 agPPA and 7 PPAOS patients were collected during relaxed wakefulness with eyes closed (21 electrodes, 10-20 positions, 256 Hz sampling rate, 1-200 Hz bandpass filter). Eight artifact-free, non-overlapping 1024-point epochs were selected.

Clinical Diagnostic Accuracy of Early/Advanced Parkinson Disease: An Updated Clinicopathologic Study.

Objective: To update data for diagnostic accuracy of a clinical diagnosis of Parkinson disease (PD) using neuropathologic diagnosis as the gold standard.

Methods: Data from the Arizona Study of Aging and Neurodegenerative Disorders (AZSAND) were used to determine the predictive value of a clinical PD diagnosis. Two clinical diagnostic confidence levels were used, possible PD (PossPD, never treated or not responsive) and probable PD (ProbPD, 2/3 cardinal clinical signs and responsive to dopaminergic medications).

Toward allele-specific targeting therapy and pharmacodynamic marker for spinocerebellar ataxia type 3.

Spinocerebellar ataxia type 3 (SCA3), caused by a CAG repeat expansion in the ataxin-3 gene (), is characterized by neuronal polyglutamine (polyQ) ATXN3 protein aggregates. Although there is no cure for SCA3, gene-silencing approaches to reduce toxic polyQ ATXN3 showed promise in preclinical models. However, a major limitation in translating putative treatments for this rare disease to the clinic is the lack of pharmacodynamic markers for use in clinical trials.

Dystonic Golfer's cramp: Pilot study of propranolol and looking at the hole.

Introduction: There is limited data in the scientific literature using quantitative methods to assess response of golfer's cramp to intervention. The objective of this pilot study was to use quantitative measures to study the effect of propranolol and looking at the hole when putting.

Methods: 14 golfers completed 50 10' putts (10 each x 5 conditions): two-handed looking at the ball, right hand only looking at the ball, two-handed looking at the hole, then following a single 10 mg oral dose of propranolol two-handed and right hand only putts looking at the ball.

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.

Addressing Sexual Harassment in the #MeToo Era: An Institutional Approach.

Sexual harassment is a particularly pernicious form of harassment that can result in long-lasting psychological damage to victims. In health care, it has deleterious effects on teamwork and communication and may affect patient care. Although concerns regarding sexual harassment in the workplace, including within health care, are not new, increased attention has been focused on this topic since late 2017 as a result of the #MeToo movement.

Neuropathological Findings in Parkinson's Disease With Mild Cognitive Impairment.

Objective: There are few neuropathological studies on Parkinson's disease with mild cognitive impairment (PD-MCI). Those published reveal coexisting Lewy body and Alzheimer's disease pathology. Our objective is to determine the pathology that underlies PD-MCI.

Slow Orthostatic Tremor: Review of the Current Evidence.

Background: Orthostatic tremor (OT) is defined as tremor in the legs and trunk evoked during standing. While the classical description is tremor of ≥13 Hz, slower frequencies are recognized. There is disagreement as to whether the latter represents a slow variant of classical OT, or different tremor disorder(s) given frequent coexistent neurological disease.

Electroencephalography in Primary Progressive Aphasia and Apraxia of Speech.

Background: Past research has demonstrated that electroencephalography (EEG) is sensitive to what we now know as Primary Progressive Aphasia (PPA); however, the EEG profiles of patients with Primary Progressive Apraxia of Speech (PPAOS) and PPA, in the context of current consensus criteria, have not been studied.

Aims: The primary goal of this study was to explore the EEG profiles of patients of the nonfluent/ agrammatic variant of PPA (agPPA) and PPAOS.

Methods And Procedures: Three patients with agPPA and five patients with PPAOS (two with aphasia) completed a head MRI scan and clinical EEG recording.

Nomenclature of Genetically Determined Myoclonus Syndromes: Recommendations of the International Parkinson and Movement Disorder Society Task Force.

Genetically determined myoclonus disorders are a result of a large number of genes. They have wide clinical variation and no systematic nomenclature. With next-generation sequencing, genetic diagnostics require stringent criteria to associate genes and phenotype.

Unified Staging System for Lewy Body Disorders: Clinicopathologic Correlations and Comparison to Braak Staging.

This study was designed to correlate clinical findings with the extent of pathologic a-synuclein (aSyn) in the brain using the Unified Staging System for Lewy Body disorders (USSLB). Data from 280 cases from the Arizona Study of Aging and Neurodegenerative Disorders are presented. Each case had a complete USSLB staging and at least 1 full research clinical assessment, including subspecialty neurologist-administered movement and cognitive evaluation.

Myoclonus.

Purpose Of Review: This article offers clinicians a strategic approach for making sense of a symptom complex that contains myoclonus. The article presents an evaluation strategy that highly leverages the two major classification schemes of myoclonus. The goal of this article is to link evaluation strategy with diagnosis and treatment of myoclonus.

Faster cognitive decline in dementia due to Alzheimer disease with clinically undiagnosed Lewy body disease.

Background: Neuropathology has demonstrated a high rate of comorbid pathology in dementia due to Alzheimer's disease (ADD). The most common major comorbidity is Lewy body disease (LBD), either as dementia with Lewy bodies (AD-DLB) or Alzheimer's disease with Lewy bodies (AD-LB), the latter representing subjects with ADD and LBD not meeting neuropathological distribution and density thresholds for DLB. Although it has been established that ADD subjects with undifferentiated LBD have a more rapid cognitive decline than those with ADD alone, it is still unknown whether AD-LB subjects, who represent the majority of LBD and approximately one-third of all those with ADD, have a different clinical course.

Tau immunoreactivity in peripheral tissues of human aging and select tauopathies.

Many studies have been directed at understanding mechanisms of tau aggregation and therapeutics, nearly all focusing on the brain. It is critical to understand the presence of tau in peripheral tissues since this may provide new insights into disease progression and selective vulnerability. The current study sought to determine the presence of select tau species in peripheral tissues in elderly individuals and across an array of tauopathies.

Lewy body pathology in Alzheimer's disease: A clinicopathological prospective study.

Objective: Identify clinical features predictive of Lewy body pathology in Alzheimer's disease (AD) patients in an ongoing longitudinal clinicopathologic study.

Material And Methods: We queried the Arizona Study of Aging and Neurodegenerative Disorders (AZSAND) database for dementia cases with AD pathology (1997-2015). Subjects received longitudinal comprehensive clinical evaluations including motor/neuropsychological assessment and Apo-E4 genotyping.

The Yips: Methods to Identify Golfers with a Dystonic Etiology/Golfer's Cramp.

Purpose: To determine whether quantitative methods could separate golfers with a possible dystonic cause of the "yips" from those that appear to be nondystonic.

Methods: Twenty-seven golfers completed 10 two-handed and 10 right hand-only putts. Surface EMG assessed forearm muscle co-contraction and motion detectors monitored wrist and putter movements.

Essential tremor and depression.

Introduction: Depression and neuropsychiatric disorders in individuals with essential tremor (ET) are not well characterized in the literature.

Methods: We compared 104 ET subjects with 481 non-ET controls involved in the Arizona Study of Aging and Neurodegenerative Disorders. An analysis of baseline depression scales and neuropsychiatric inventory (NPI) was done between the two groups.

Predicting alpha-synuclein pathology by REM sleep behavior disorder diagnosis.

Inability to accurately diagnose Lewy type alpha-synucleinopathy (LTS) pre-mortem has been a major obstacle to clinical care and research. Probable REM sleep behavior disorder (PRBD) diagnosed with support of instruments such as the Mayo Sleep Questionnaire (MSQ) may provide a cost effective means of predicting LTS. Since 2007, 602 subjects in the Arizona Study of Aging and Neurodegenerative Disorders had clinician assessment for PRBD (298 with, 304 without support of the MSQ), completed cognitive and movement examinations, and had neuropathological assessment.

Sandfly fever in Afghanistan-a sometimes overlooked disease of military importance: a case series and review of the literature.

Sandfly fever, sometimes known as pappataci fever or Phlebotomus fever, is a vector transmitted viral illness with a history of affecting naïve military formations that travel through or fight in areas in which the infection is endemic. We present a series of 4 hospitalized cases of sandfly fever (2 presumptive, 2 laboratory confirmed) that were admitted to a Role 3 hospital in Afghanistan for evaluation and treatment following medical evacuation from a forward area for marked fevers and malaise. Laboratory evaluation of these cases was significant for leukopenia and thrombocytopenia, consistent with historical descriptions of sandfly fever.