A 12-month prospective real-life study of opicapone efficacy and tolerability in Emirati and non-White subjects with Parkinson's disease based in United Arab Emirates.

Parkinson's disease (PD) is the second most common neurodegenerative disorder, and the condition is complicated by the emergence of wearing off/motor fluctuations with levodopa treatment after a variable period. COMT inhibitors when used as adjunct therapy to levodopa tend to smoothen out these wearing off fluctuations by enhancing delivery of levodopa and increasing its bioavailability to the brain. The study was conducted to investigate the motor and nonmotor effect, safety and tolerability of the third generation once-daily COMT inhibitor (opicapone), as add-on, adjuvant therapy to levodopa and at 6 and 12 months follow-up in a real-life cohort of consecutive Emirati and non-White PD patients. A real-life observational analysis using tolerability parameters as used previously by Rizos et al. and Shulman et al. based on clinical database of cases rat Kings College Hospital Dubai Parkinson care database. This was a prospective, single-arm follow-up clinical evaluation study that evaluated the effectiveness of opicapone 50 mg once-daily regime in 50 patients diagnosed with idiopathic neurodegenerative disorder. All patients were assessed with scales used in clinical pathway and include motor Unified Parkinson's Disease Rating Scale (UPDRS), nonmotor symptom scale (NMSS), quality of life (PDQ8) Parkinson's fatigue scale (PFS16) and King's Parkinson's Pain Scale (KIPS). Out of 50 patients treated with opicapone (72% male, mean age 66.9 years (SD 9.9, range 41-82 years) and mean duration of disease 5.7 years (SD 2.5 range (2-11), there was significant statistical improvements shown in motor function-UPDRS part 3: baseline 40.64 ± 2.7, at 6 months 32.12 ± 3.14 and after 12 months 33.72 ± 3.76. Nonmotor burden NMSS: 107.00 ± 21.86, at 6 months 100.78 ± 17.28 and 12 months 96.88 ± 16.11. Reduction in dyskinesias (UPDRS part 4): baseline 8.78 ± 1.07, at 6 months 7.4 ± 0.81 and 12 months 6.82 ± 0.75. Opicapone provides beneficial motor and nonmotor effects in Emirati and other non-White Parkinson's patients, resident in UAE, proving its efficacy across different racial groups as COMT activity may vary between races.

Safety and tolerability of long-term apomorphine infusion in advanced Parkinson's disease: an Indian multi-center (APO-IND) experience.

Advanced Parkinson's Disease (APD) is complicated by the emergence of motor and non-motor fluctuations, which are initially predictable and eventually become unpredictable, in part due to erratic gastric absorption and short half of oral levodopa. Attempts to manage such fluctuations with oral dopaminergic drugs often lead to disabling dyskinesias. Continuous Subcutaneous Apomorphine Infusion (CSAI), despite being approved for the treatment of APD since 1993, was approved in India only in 2019. We studied the safety, tolerability and efficacy of CSAI in Indian patients with APD in a registry design to raise local awareness of this important treatment. We conducted a prospective registry-based observational audit at 10 centers across different states of India. Patients with APD, not responding to or with significant side effects from oral dopaminergic therapy, were assessed at baseline and at month 6 and 12 following CSAI infusion. Fifty-one patients completed the study, CSAI significantly reduced the functional impact of dyskinesia (p < 0.01 at 6 months and p < 0.001 at 12 months). There was a significant improvement in the OFF-state from baseline (p < 0.01 at 6 months and p < 0.001 at 12 months) No discernible side effects were observed apart from mild site reaction (n = 7), nausea (n = 7) skin nodules (n = 2). CSAI demonstrated safety, efficacy, tolerability and improved quality of life in patients with APD, as shown in previous studies. Our study highlighted current existing inequalities in treatment availability, lack of awareness, knowledge gap, affordability and cost remains a concern regarding apomorphine use in Indian PD population.

Wilson's Disease Update: An Indian Perspective.

Wilson's disease (WD) is an autosomal recessive disorder due to ATP7B gene mutation, resulting in defective copper metabolism, with liver and brain being primarily affected. Being a treatable disorder, early diagnosis and proper management of WD may result in near complete recovery. It has received significant attention over the past 50 years, with several Indian contributions. This study collates published Indian studies on WD in Pubmed and Embase databases and puts them in perspective. Several Indian case series suggest that WD may be more prevalent than thought. Commonly detected ATP7B mutation in India is p.C271X. Although initial Indian series reported significant osseomuscular presentation, neuropsychiatric and hepatic manifestations dominated the later reports. A significant male predominance is observed in Indian series. Pure hepatic presentation starts earlier than neurological or osseomuscular WD. A positive family history may be seen in nearly 50% of Indian WD cases with a high rate of consanguinity. Up to two-third of Indian cases may be initially misdiagnosed, with a mean diagnostic delay of up to 2 years. Abnormalities in serum ceruloplasmin and 24-hour urinary copper has been reported in more than four-fifth cases. Brain MRI is abnormal in nearly all neurological WD cases. Copper chelation remains the mainstay of therapy, with D-penicillamine being the most widely used chelator in India. Global Assessment Scale for WD is a comprehensive tool for clinical monitoring. Hepatic presentation carries a five-time higher mortality risk than neurological, with up to 90% Indian neurological WD cases recovering to pre-morbid functionality with adequate therapy.

Cross-sectional analysis of the Parkinson's disease Non-motor International Longitudinal Study baseline non-motor characteristics, geographical distribution and impact on quality of life.

Growing evidence suggests that non-motor symptoms (NMS) in Parkinson's disease (PD) have differential progression patterns that have a different natural history from motor progression and may be geographically influenced. We conducted a cross-sectional analysis of 1607 PD patients of whom 1327 were from Europe, 208 from the Americas, and 72 from Asia. The primary objective was to assess baseline non-motor burden, defined by Non-Motor Symptoms Scale (NMSS) total scores. Other aims included identifying the factors predicting quality of life, differences in non-motor burden between drug-naïve and non-drug-naïve treated patients, and non-motor phenotypes across different geographical locations. Mean age was 65.9 ± 10.8 years, mean disease duration 6.3 ± 5.6 years, median Hoehn and Yahr stage was 2 (2-3), and 64.2% were male. In this cohort, mean NMSS scores were 46.7 ± 37.2. Differences in non-motor burden and patterns differed significantly between drug-naïve participants, those with a disease duration of less than five years, and those with a duration of five years or over (p ≤ 0.018). Significant differences were observed in geographical distribution (NMSS Europe: 46.4 ± 36.3; Americas: 55.3 ± 42.8; Asia: 26.6 ± 25.1; p < 0.001), with differences in sleep/fatigue, urinary, sexual, and miscellaneous domains (p ≤ 0.020). The best predictor of quality of life was the mood/apathy domain (ß = 0.308, p < 0.001). This global study reveals that while non-motor symptoms are globally present with severe NMS burden impacting quality of life in PD, there appear to be differences depending on disease duration and geographical distribution.

Impact of ethnicity on the natural history of Parkinson disease.

Parkinson disease (PD) affects people of all races and ethnicity worldwide. PD is a multineurotransmitter and multisystem disorder and our current concept of the natural history of PD has changed considerably over the past decades. Many aspects of this heterogeneous condition still remain unexplained; one aspect that is poorly studied is the role of ethnicity and manifest motor and non-motor PD. Some preliminary data suggest that the prodromal risk of developing PD, clinical symptom expression and the experience of living with the condition may vary between different ethnic groups. Several factors might play a role in the influence of ethnicity on PD, such as pharmacogenetics, sociocultural aspects and environmental exposures. Increased knowledge on the role of ethnicity in PD may help shed light on the symptom expression and treatment response of PD, address inequalities in health care delivery worldwide and improve the delivery of personalised medicine.

Restless legs syndrome.

Restless legs syndrome (RLS), also known as Willis-Ekbom disease (WED), is a common movement disorder characterised by an uncontrollable urge to move because of uncomfortable, sometimes painful sensations in the legs with a diurnal variation and a release with movement. The pathophysiology is only partially known and a genetic component together with dopaminergic and brain iron dysregulation plays an important role. Secondary causes for RLS need to be excluded. Treatment depends on the severity and frequency of RLS symptoms, comprises non-pharmacological (eg lifestyle changes) and pharmacological interventions (eg dopaminergic medication, alpha-2-delta calcium channel ligands, opioids) and relieves symptoms only. Augmentation is the main complication of long-term dopaminergic treatment of RLS. This article will provide a clinically useful overview of RLS with provision of diagnostic criteria, differential diagnoses, possible investigations and different treatment strategies with their associated complications.

A study on axillary artery and its branching pattern among the population of West Bengal, India.

Variations in the branching pattern of the axillary artery have paramount importance among anatomists, surgeons and radiologists. A study was conducted on this topic in Kolkata, among the people of West Bengal, a state of India. The upper limbs of 70 cadavers were dissected bilaterally at the Department of Anatomy, Calcutta National Medical College, Kolkata, between 2008 and 2011. Among the study population, 52 cadavers (74.3%) were male and the rest were female, with average age 62.01 years (standard deviation = 6.58) and average height 1.59 meter (standard deviation = 0.096) respectively. The mean length of the axillary artery was 10.15 cm (standard deviation = 1.056). The superior thoracic, thoracoacromial and subscapular arteries were found to be constant branches of the axillary artery while the other branches showed considerable variations. Among those constant branches a high, significant correlation was found between the distance of origin of thoracoacromial artery from the outer border of the 1st rib and the length of the axillary artery, for the right and respectively the left upper limb of male cadavers. In females it showed a moderate, albeit significant correlation only. Similarly, the length of the axillary artery established a moderate correlation with the distance of origin of the superior thoracic and of the subscapular arteries on the right side of female cadavers. No other significant correlation was obtained.

Unilateral variation in the position of internal and external carotid arteries.

During the routine dissection of the neck region of a 77 years old male cadaver in the Department of Anatomy at Bankura Sammilani Medical College, a unilateral variation in the position and course of internal and external carotid arteries was noticed on the right side of neck. The internal carotid artery was anteromedial to the external carotid artery at the site of bifurcation of the common carotid artery. Knowledge of variation in the course and relation of internal and external carotid arteries is important for surgeons to perform neck surgery and also for the radiologists to interpret carotid system imaging.

Intestinal mantle cell lymphoma recurring at laparotomy scar region.

Mantle cell lymphoma (MCL) is a moderately aggressive variety of non-Hodgkins lymphoma. Extranodal presentation of MCL is well known, the intestine being a common site. The incidence of colorectal involvement is relatively rare. Moreover, the recurrence of the disease at laparotomy scar site is even more rare. We report an unusual case of incision line recurrence in a case of colonic MCL occuring three years after initial treatment.

Optimized point dose measurement: An effective tool for QA in intensity-modulated radiotherapy.

In some cases of Intensity-modulated radiotherapy (IMRT) point dose measurement, there exists significant deviation between calculated and measured dose at isocenter, sometimes greater than ±3%. This may be because IMRT fields generate complex profiles at the reference point. The deviation arises due to lack of lateral electronic equilibrium for small fields, and other factors such as leakage and scatter contribution. Measurements were done using 0.125-cc ion chamber and Universal IMRT phantom (both from PTW-Freiburg). The aim is to find a suitable point of measurement for the chamber to avoid discrepancy between calculated and measured dose. Various beam profiles were generated in the plane of the chamber for each field by implementing patient plan on the IMRT phantom. The profiles show that for the fields which are showing deviation, the ion chamber lies in the steep-gradient region. To rectify the problem, the TPS (Treatment Planning System) calculated dose is found out at various points in the measurement plane of the chamber at isocenter. The necessary displacement to the chamber, as noted from the TPS, was given to obtain the optimum result. Twenty cases were studied for optimization, whose percentage deviation was more than ±3%. The results were well within tolerance criteria of ±3% after optimization. The mean percentage deviation value for the 20 cases studied, with standard deviation of 2.33 under 95% confidence interval, was found out to be 2.10% ± 1.14. Those cases that have significant variation even after optimization are further studied with film dosimetry.