Pehrsonburke7196

A total of 23 of 37 (62%) "entacapone failures" reported adverse events of which 16 stopped the drug. Among 36 of 57 (63%) patients who continued to use opicapone at 6 months, there was an improvement in OFF time of ~2 hours per day as measured by interview.

We conclude that opicapone can be an effective additional treatment for wearing off in Parkinson's disease (PD) in a subgroup of patients. The use of opicapone in our cohort with prior entacapone exposure, however, was associated with higher rates of adverse effects and treatment discontinuation than reported in published trial data of COMT inhibitor naïve patients.

We conclude that opicapone can be an effective additional treatment for wearing off in Parkinson's disease (PD) in a subgroup of patients. The use of opicapone in our cohort with prior entacapone exposure, however, was associated with higher rates of adverse effects and treatment discontinuation than reported in published trial data of COMT inhibitor naïve patients.

Apraxia of eyelid opening is a movement disorder characterized by an inability to raise the eyelids without any overt contractions of the orbicularis oculi muscle. There is currently no clinical scale to rate the severity of this condition.

To develop and validate a novel scale that considers phenomenological aspects relevant to the severity of the condition.

The study sample included 20 patients with apraxia of eyelid opening, either isolated (9 patients) or associated with blepharospasm (11 patients). To validate the scale, selected features were checked for reliability, reliable items were combined to generate the scale, and clinimetric properties were evaluated.

The novel severity scale yielded acceptable reliability, scaling assumptions, internal consistency, and sensitivity to change; a lack of floor and ceiling effects; and no correlation with the blepharospasm severity rating scale.

We propose a severity scale that considers the most relevant apraxia of eyelid opening motor abnormalities based on objective criteria. This scale can be reliably administered by general neurologists after a brief training.

We propose a severity scale that considers the most relevant apraxia of eyelid opening motor abnormalities based on objective criteria. This scale can be reliably administered by general neurologists after a brief training.

Cerebellar atrophy is a nonspecific imaging finding observed in a number of neurological disorders. Genetic ataxias associated with cerebellar atrophy are a heterogeneous group of conditions, rendering the approach to diagnosis challenging.

To define the spectrum of genetic ataxias associated with cerebellar atrophy in a Canadian cohort and the diagnostic yield of exome sequencing for this group of conditions.

A total of 92 participants from 66 families with cerebellar atrophy were recruited for this multicenter prospective cohort study. Exome sequencing was performed for all participants between 2011 and 2017 as part of 1 of 2 national research programs, Finding of Rare Genetic Disease Genes or Enhanced Care for Rare Genetic Diseases in Canada.

A genetic diagnosis was established in 53% of families (35/66). Pathogenic variants were found in 21 known genes, providing a diagnosis for 31/35 families (89%), and in 4 novel genes, accounting for 4/35 families (11%). Of the families, 31/66 (47%) remained without a genetic diagnosis. The most common diagnoses were channelopathies, which were established in 9/35 families (26%). Additional clinical findings provided useful clues to specific diagnoses.

We report on the high frequency of channelopathies as a cause of genetic ataxias associated with cerebellar atrophy and the utility of exome sequencing for this group of conditions.

We report on the high frequency of channelopathies as a cause of genetic ataxias associated with cerebellar atrophy and the utility of exome sequencing for this group of conditions.

Levodopa-carbidopa intestinal gel (LCIG) treatment has shown variable effect on dyskinesia in Parkinson's disease (PD).

To identify PD patients who are likely to have troublesome dyskinesia under LCIG treatment and describe the pharmacokinetic-dynamic profile and dyskinesia phenomenology of those patients.

PD patients were assessed for clinical and therapeutic variables, before LCIG treatment (

) and at last outpatient visit (

). Sub-groups of patients with and without "troublesome dyskinesia" (UPDRS IV, item 33 ≥2), matched for disease and LCIG treatment duration, underwent a pharmacokinetic-dynamic assessment.

We included 53 PD patients. After a mean of 51.7 ± 34.1 months of LCIG treatment, "off-time" was significantly reduced, whereas, dyskinesia duration/disability did not change. The multivariate regression model, adjusted for LCIG treatment duration, showed that being female increases the risk of presenting troublesome dyskinesia at

(odds ratio [OR] = 9.2; 95% confidence interval [CI] = 2.4-37.4) that was also significantly associated to longer off periods at

(OR= 4.4; 95% CI = 1.1-14.3). Female patients showed a higher risk for a higher dyskinesia score at

(sum of the items 32 and 33

= 0.001). Patients with troublesome dyskinesia showed a tendency for a lower motor benefit and the appearance of more severe dyskinesia despite similar levodopa plasma concentration.

Dyskinesia should be carefully monitored in patients undergoing LCIG, with particular caution for female patients. Whether combined clinical and pharmacodynamic assessments could be helpful to manage patients with troublesome dyskinesia under LCIG treatment needs further evaluation in a larger group of patients.

Dyskinesia should be carefully monitored in patients undergoing LCIG, with particular caution for female patients. Whether combined clinical and pharmacodynamic assessments could be helpful to manage patients with troublesome dyskinesia under LCIG treatment needs further evaluation in a larger group of patients.

Functional motor disorders (FMDs) are abnormal movements that are significantly altered by distractive maneuvers and are incongruent with movement disorders seen in typical neurological diseases.

The objectives of this article are to (1) describe the clinical manifestations of FMDs, including nonmotor symptoms and occurrence of other functional neurological disorders (FND); and (2) to report the frequency of isolated and combined FMDs and their relationship with demographic and clinical variables.

For this multicenter, observational study, we enrolled consecutive outpatients with a definite diagnosis of FMDs attending 25 tertiary movement disorders centers in Italy. Each patient underwent a detailed clinical evaluation with a definition of the phenotype and number of FMDs (isolated, combined) and an assessment of associated neurological and psychiatric symptoms.

Of 410 FMDs (71% females; mean age, 47 ± 16.1 years) the most common phenotypes were weakness and tremor. find more People with FMDs had higher educational levels than the general population and frequent nonmotor symptoms, especially anxiety, fatigue, and pain.