Braypitts9261
Although infusion therapies optimize dopaminergic drug delivery in fluctuating DBS-treated patients, DBS added on LCIG treatment has an additive symptomatic effect that allows levodopa dose reduction in patients with drug-induced side effects.
Selected PD patients on 1 DAT may experience additional benefit from a second DAT, for several reasons along the course of their disease. Although infusion therapies optimize dopaminergic drug delivery in fluctuating DBS-treated patients, DBS added on LCIG treatment has an additive symptomatic effect that allows levodopa dose reduction in patients with drug-induced side effects.
Cervical dystonia (CD) is the most common focal isolated dystonia. Preclinical studies report that AMPA-selective glutamate receptor antagonists improve dystonia. Perampanel is a clinically available, AMPA receptor antagonist that has shown efficacy and safety in epilepsy.
To determine safety and tolerability of perampanel in CD.
We performed a phase 2a, open-label, multicenter study to evaluate tolerability and safety of perampanel in CD. Included subjects had primary CD; those on botulinum toxin were 8 weeks post last injection. All subjects received perampanel 2 mg/day, titrated 2 mg weekly over 6 weeks, to maximum 12 mg/day; maintenance phase was 4 weeks, ending at week 10. Primary endpoints included tolerability, defined as ability to remain on perampanel for the maintenance period, at any dose level and safety, determined from adverse events (AEs) collected at each visit. Secondary exploratory endpoints included Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS), quality of life (cervical dystonia impact profile [CIDP]-58) and Clinical Global Impression of change (CGI).
CD participants (n=25) were recruited. Eight subjects withdrew; 4 because of AEs, 3 for other reasons and 1 lost to follow up. One subject tolerated 12 mg/day. Eight subjects (30.8%) tolerated 2 mg, whereas 19.2% tolerated 4 mg/day, and 15.4% tolerated 6 mg or 8 mg/day. All subjects experienced AEs. The most common AEs were dizziness, imbalance, and irritability. Liproxstatin-1 solubility dmso Exploratory endpoints of TWSTRS showed some improved pain scores and CIDP-58 improved sleep.
Tolerability to perampanel was variable in CD subjects. Lower doses would be considered for future studies in this population.
Tolerability to perampanel was variable in CD subjects. Lower doses would be considered for future studies in this population.
The Montreal Cognitive Assessment (MoCA) and the Dementia Rating Scale-2 (DRS-2) are recommended screeners for Parkinson's disease mild cognitive impairment (PD-MCI). Cross-cultural studies examining their diagnostic precision have not addressed cultural bias in a multicultural setting.
To compare DRS-2 and MoCA performance between patients born in Canada, the USA, and the UK (Anglosphere group) and immigrant patients born elsewhere (International group). To identify sources of cultural bias by comparing group characteristics, and by assessing the relationships between performance and immigration and socio-development variables. link2 To examine the diagnostic precision of both tools in detecting PD-MCI in each group.
We conducted a clinical chart review of advanced PD patients who completed cognitive screeners (MoCA n=288, 30% International group; DRS-2 n=426, 31% International group). All completed a comprehensive neuropsychological assessment to apply Level II PD-MCI diagnostic criteria.
The International group performed worse than the Anglosphere group on the MoCA and DRS-2, and the only variable that accounted for some of the group difference was the Historical Index of Human Development, a societal variable, which fully mediated the group effect on the DRS-2. Diagnostic precision of the MoCA was at chance level in the International group, and was poorer than that of the DRS-II in this group and that of the MoCA in the Anglosphere group, although these were considered poor.
Our results support the recommendation to exert caution in using cognitive screeners to capture PD-MCI in all patients and particularly with first generation immigrants.
Our results support the recommendation to exert caution in using cognitive screeners to capture PD-MCI in all patients and particularly with first generation immigrants.
Functional movement disorders (FMD) are characterized by abnormal movements and motor symptoms incongruent with a known structural neurologic cause. While psychological stressors have long been considered an important risk factor for developing FMD, little is known about the impact of psychiatric comorbidities on disease manifestations or complexity.
To compare characteristics of FMD patients with co-occurring mood and trauma-related psychiatric conditions to FMD patients without psychiatric conditions.
We performed a retrospective cohort study of patients seen in the University of Colorado Health system between January 1, 2015 and December 31, 2019. Patients were included if they had a diagnosis of FMD, determined by ICD-10 coding and ≥1 phenomenology-related diagnostic code (tremor, gait disturbances, ataxia, spasms, and weakness), and at least one encounter with a neurology specialist. Fisher's exact and unpaired t-tests were used to compare demographics, healthcare utilization, and phenomenologies os without comorbid psychiatric conditions who may benefit from targeted physiotherapy alone.
Perry syndrome is a rare genetic parkinsonian disorder with TAR DNA binding protein 43 (TDP-43) pathology clinically presenting with parkinsonism, neuropsychiatric features, weight loss, and central hypoventilation. As respiratory complications are often the cause of death, studies likely show the early stage of the neurodegenerative process. Because of the rarity of this condition, few studies exist, and each case provides insight into pathological findings in this neurodegenerative condition.
To study the clinical and pathological correlations of an autopsy case of Perry syndrome.
The patient was a woman in her 50s with Perry syndrome and a
gene mutation. Between October 2016 and July 2019, she underwent postmortem and pathological examination at University Hospital in London, Ontario, Canada. link3 Data were obtained through clinical pathological examination.
Microscopy showed significant neuronal loss with pigmentary incontinence and gliosis in the substantia nigra. There was no atrophy elsewhere, in to the clinical symptoms of hypoventilation and weight loss in Perry syndrome.
Some patients with Parkinson Disease (PD) report double vision, but its prevalence and determinants are unknown.
To determine the prevalence and risk factors for diplopia in PD.
Using data from 26,790 PD patients and 9257 controls in the Fox Insight Study, we compared the prevalence of diplopia using the Non-Movement Symptom Questionnaire. Associations with age, race, gender, disease duration, and scores on MDS-UPDRS part II, and Penn Parkinson's Daily Activity Questionnaire were assessed with generalized estimating equations.
The point prevalence of diplopia was higher in PD (18.1%) than controls (6.3%,
< 0.001) at baseline, and 28.2% of all PD patients reported diplopia at least once during the study (period prevalence). PD patients with diplopia were more likely to be older, non-white, have greater disease duration, and report greater motor, non-motor, and daily activity limitations.
Diplopia is common and associates with motor and non-motor severity in PD.
Diplopia is common and associates with motor and non-motor severity in PD.
During magnetic resonance-guided focused ultrasound for essential or parkinsonian tremor, adverse events (headache, nausea/vomiting, or anxiety) may alter the outcome of the procedure despite being mostly transient and mild.
Our aim was to analyze the relationship between demographic, procedural, and anesthetic characteristics with magnetic resonance/ultrasound-related events.
This was a retrospective study at the Clinica Universidad de Navarra of patients undergoing thalamotomy with magnetic resonance-guided focused ultrasound between September 2018 and October 2019. The anesthesia protocol included headache and nausea/vomiting prophylaxis and rescue therapy. Dexmedetomidine was used for anxiolysis in some patients after thorough multidisciplinary assessment.
A total of 123 patients were included. Headache was directly related to skull density ratio (
< 0.001) and skull thickness (
=0.02). Patients with a skull density ratio less than 0.48 had 3 times the odds of experiencing moderate or severeations. Its exact effect on tremor is still unclear.
Pimavanserin is a serotonin 2A receptor inverse agonist and antagonist used for the treatment of hallucinations and delusions in Parkinson's disease psychosis. Numerous studies support a modulatory role of serotonin in Tourette syndrome (TS).
To determine whether or not pimavanserin affects TS symptoms.
In this open-label study of TS adult patients, pimavanserin was titrated to 34 mg/day over 1 week and continued for an additional 7 weeks followed by a 2-week washout. Tic severity, the primary outcome measure, was assessed by the Yale Global Tic Severity Scale Total Tic Severity score (YGTSS-TTS). Secondary outcome measures included changes in the Yale-Brown Obsessive Compulsive Scale (Y-BOCS), the Tourette Syndrome Clinical Global Impression of Change (TS-CGIC), the Tourette Syndrome-Patient Global Impression of Impact (TS-PGII), and the Gilles de la Tourette Syndrome - Quality of Life scale (GTS-QOL).
We enrolled 12 patients, but 2 dropped out after week 2 due to non-serious side effects. In the 10 patients, mean (standard deviation (SD)) age 34 (12.9) who completed the study the mean (SD) baseline YGTSS-TTS was 34 (9.3). This decreased by 3.6 (4.9) points at week 8, a 12% reduction in tic severity (
= 0.03). This improvement is small and may not be clinically important. Significant improvement was noted in the TS-CGIC, TS-PGII and GTS-QO. No serious adverse events were reported.
The results of this study suggest that pimavanserin is safe and associated with improvement in motor and non-motor TS symptoms. These findings warrant further study by a larger, placebo-controlled, trial.
The results of this study suggest that pimavanserin is safe and associated with improvement in motor and non-motor TS symptoms. These findings warrant further study by a larger, placebo-controlled, trial.
The Friedreich Ataxia Rating Scale (FARS) and the Scale for the Assessment and Rating of Ataxia (SARA) are commonly used neurological rating scales in Friedreich ataxia (FRDA). The modified Friedreich Ataxia Rating Scale (mFARS) has been accepted as an appropriate outcome measure for clinical trials in FRDA.
The COVID-19 pandemic has resulted in limited face-to-face interactions with individuals involved in natural history studies and clinical trials. The aim of this study was to determine the validity of conducting the mFARS and SARA through video.
Individuals who had the mFARS administered face-to-face in the previous 6 months were invited to participate. Participants were sent instructions and asked to have a carer present to assist. The mFARS and SARA were then administered by video. Differences between face-to-face and video scores and the reliability between scores obtained face-to-face and by video were examined.
The mFARS and SARA were conducted by video with 19 individuals. Excellent test-retest reliability was seen in the mFARS lower limb coordination (ICC=0.
