Reply to: Hemichorea Associated with CASPR2 Antibody

This letter was written in reply to this letter to the editor: Vynogradova I, Savitski V, Heckmann JG. Hemichorea associated with CASPR2 antibody. Tremor Other Hyperkinet Mov. 2014; 4. doi: 10.7916/D8VM49C5 The above letter to the editor was written in response to this article: Ramdhani RA, Frucht SJ. Isolated Chorea Associated with LGI1 Antibody. Tremor Other Hyperkinet Mov. 2014; 4. doi: 10.7916/D8MG7MF

Optimizing Clinical Assessments in Parkinson's Disease Through the Use of Wearable Sensors and Data Driven Modeling

The emergence of motion sensors as a tool that provides objective motor performance data on individuals afflicted with Parkinson's disease offers an opportunity to expand the horizon of clinical care for this neurodegenerative condition. Subjective clinical scales and patient based motor diaries have limited clinometric properties and produce a glimpse rather than continuous real time perspective into motor disability. Furthermore, the expansion of machine learn algorithms is yielding novel classification and probabilistic clinical models that stand to change existing treatment paradigms, refine the application of advance therapeutics, and may facilitate the development and testing of disease modifying agents for this disease. We review the use of inertial sensors and machine learning algorithms in Parkinson's disease

Neural correlates of abnormal sensory discrimination in laryngeal dystonia

AbstractAberrant sensory processing plays a fundamental role in the pathophysiology of dystonia; however, its underpinning neural mechanisms in relation to dystonia phenotype and genotype remain unclear. We examined temporal and spatial discrimination thresholds in patients with isolated laryngeal form of dystonia (LD), who exhibited different clinical phenotypes (adductor vs. abductor forms) and potentially different genotypes (sporadic vs. familial forms). We correlated our behavioral findings with the brain gray matter volume and functional activity during resting and symptomatic speech production. We found that temporal but not spatial discrimination was significantly altered across all forms of LD, with higher frequency of abnormalities seen in familial than sporadic patients. Common neural correlates of abnormal temporal discrimination across all forms were found with structural and functional changes in the middle frontal and primary somatosensory cortices. In addition, patients with familial LD had greater cerebellar involvement in processing of altered temporal discrimination, whereas sporadic LD patients had greater recruitment of the putamen and sensorimotor cortex. Based on the clinical phenotype, adductor form-specific correlations between abnormal discrimination and brain changes were found in the frontal cortex, whereas abductor form-specific correlations were observed in the cerebellum and putamen. Our behavioral and neuroimaging findings outline the relationship of abnormal sensory discrimination with the phenotype and genotype of isolated LD, suggesting the presence of potentially divergent pathophysiological pathways underlying different manifestations of this disorder

Adult-onset Idiopathic Focal Lower Extremity Dystonia: A Rare Task-Specific Dystonia

Background:Adult-onset focal lower extremity (LE) dystonia is rare, but there have recently been a number of case series that have reported an idiopathic variant triggered during ambulation.Methods:We describe nine patients with idiopathic, focal task-specific LE dystonia. We conducted a comparative analysis that included our cohort and several recently published case series to further characterize the disorder.Results:A total of 48 patients (37 female, 11 male) were compared. The average age of onset was 48 years; 36 patients had distal extremity involvement (75%), 5 proximal (10%), and 7 both proximal and distal (15%). Among 33 patients in which the dystonic side was known, 20 were affected on the left (61%). Inversion of the foot with flexion of one or more toes was the most prevalent pattern in those with distal extremity involvement. Discussion:This is a novel task-specific dystonia triggered during ambulation that is often misdiagnosed as an orthopedic or psychogenic issue.</p

Acute Hemorrhagic Necrotizing Encephalopathy Initially Presenting as Bilateral Sixth Nerve Palsy; A Case Report

Acute hemorrhagic necrotizing encephalopathy is described as acute encephalopathy with neurologic deficits. It usually occurs after a viral illness. While it classically presents in the form of an acute encephalopathy with subsequent other neurologic deficits, to our knowledge, bilateral sixth nerve palsy has not been described as an initial presenting feature

Primary Dystonia: Conceptualizing the Disorder through a Structural Brain Imaging Lens

Background: Dystonia is a hyperkinetic movement disorder of involuntary, twisting repetitive movements. The anatomical structures and pathways implicated in its pathogenesis as well as their relationship to the neurophysiological paradigm of abnormal surround inhibition, maladaptive plasticity and impaired sensorimotor integration remain not well delineated. Objective: We review the use of high-resolution structural brain imaging using voxel-based morphometry (VBM) and diffusion tensor imaging (DTI) techniques for evaluation of brain changes in primary torsion dystonia and their relationships to the pathophysiology of this disorder. Methods: A search in PubMed was conducted to identify the relevant literature. Discussion: Structural imaging has enhanced our understanding of the pathophysiological mechanisms of dystonia. In particular, VBM and DTI data have revealed microstructural disturbances in the basal ganglia, sensorimotor cortices and cerebellum along with aberrations in the cortico-striato-pallido-thalamic and cerebello-thalamo-cortical pathways.&nbsp; When combined with functional brain imaging and neurophysiological modalities, a structure-function relationship can be established in the dystonia brain network at the sensorimotor, plasticity, cortical disinhibition and cerebellar outflow connectivity levels. Structural imaging highlighted new anatomical substrates and, with a combined structural-functional approach, has offered new opportunities for investigation of the neurodevelopmental, environmental and/or genetic interplay in the brain networks of dystonia patients.&nbsp;</p

Opsoclonus-Myoclonus-Ataxia Syndrome Related to the Novel Coronavirus (COVID-19)

Supplemental Digital Content is Available in the Text

Isolated Chorea Associated with LGI1 Antibody

Background:&nbsp;Leucine‐rich glioma inactivated 1 (LGI1) antibody produces a syndrome of limbic encephalitis, hyponatremia, and facio‐brachial dystonic seizures that is non‐paraneoplastic and responsive to corticosteroids. Parkinsonism, tremor, and generalized chorea are rare manifestations of LGI1, but, when present, commonly accompany other signs of limbic encephalitis.Case Report:&nbsp;We present a case of LGI1‐related isolated chorea in a 53‐year‐old Japanese male. His chorea responded to high‐dose steroids, suggesting a potential role for this synaptic antibody in triggering chorea.Discussion:&nbsp;This case highlights a new treatable etiology of chorea.This article prompted a response letter:Vynogradova I, Savitski V, Heckmann JG. Hemichorea associated with CASPR2 antibody. Tremor Other Hyperkinet Mov. 2014; 4. doi:&nbsp;10.7916/D8VM49C5 The authors' response to the letter: Ramdhani RA, Frucht SJ. Reply to: hemichorea associated with CASPR2 antibody. Tremor Other Hyperkinet Mov. 2014; 4. doi: 10.7916/D8QV3JM0</p

Opsoclonus-Myoclonus-Ataxia Syndrome Related to the Novel Coronavirus (COVID-19)

Supplemental Digital Content is Available in the Text

Reply to: Hemichorea Associated with CASPR2 Antibody

This letter was written in reply to this letter to the editor:Vynogradova I, Savitski V, Heckmann JG. Hemichorea associated with CASPR2 antibody. Tremor Other Hyperkinet Mov. 2014; 4. doi: 10.7916/D8VM49C5The above letter to the editor was written in response to this article:Ramdhani RA, Frucht SJ. Isolated Chorea Associated with LGI1 Antibody. Tremor Other Hyperkinet Mov. 2014; 4. doi: 10.7916/D8MG7MFC</p