Outcomes of stereotactic thalamotomy in patients of essential tremor: A systematic review.

BACKGROUND: Essential tremor is a neurological condition associated with movement disorder with more prevalence among adult group of population. The burden of essential tremor is peaking globally but with the advancement in the area of functional neurosurgery such as stereotactic thalamotomy, the quality of life of such patients can be improved drastically. METHODS: This systemic review was conducted in accordance to the guidance of preferred Reporting items for Systematic Review and Meta-Analysis(PRISMA). Databases of "PubMed", "Embase", "Web of Science", "Cinhal Plus", and "Scopus" from inception till 2023 was undertaken. A combination of keywords, Medical Subject Headings (MeSH), and search terms such as Search strategy for PubMed search was as follows: "stereotactic thalamotomy" AND "essential tremor". RESULTS: This systematic review analyzed 9 studies with a total of 274 patients of essential tremor patients. Unilateral thalamotomy was carried out among 268 patients and bilateral thalamotomy in rest of the patients. Vim and Vom nucleus were the site of thalamotmy with ventral intermedius nucleus being the major one. Ten different types of clinical tremor rating scales were used to assess pre operative and post operative improvement in the tremor scales of the individual patients. Dysarthria and limb weakness was noted post operative complication in majority of the cases. CONCLUSION: Our study revealed that stereotactic thalamotomy provided good functional outcome in patients of essential tremor who underwent unilateral thalamotomy compared to bilateral thalamotomy. The positive outcome outweighs the complications in such functional surgery.

Juvenile sialidosis: a rare case and review of the literature.

Background: Sialidosis is a rare variety of lysosomal storage disease that results in intracellular accumulation of sialic acid containing compounds. The authors report the first case of type II sialidosis, juvenile subtype in a 30-month-old male child from Nepal. Case presentation: Progressive hearing loss with coarse facies, hepatomegaly, kyphoscoliosis, dysostosis multiplex were the major features in a 30-month-old child born to healthy non-consanguineous parents. With the suspicion of lysosomal storage disease, urinary oligosaccharides were tested and were positive. Whole-exome sequencing revealed a mutation in the neuraminidase gene (NEU1) and established the diagnosis of sialidosis. Clinical discussion: Sialidosis is a rare autosomal recessive type of lysosomal storage disease resulting due to mutation of the neuraminidase gene leading to intracellular accumulation of sialic acid compounds. Based on the presence of visual symptoms, sialidosis is classified into type I and II varieties. Our case is of type II juvenile sialidosis. Conclusion: Despite rare, sialidosis is a life-threatening, and disabling disease. Exploring targeted therapy is the utmost to treat this condition.

Chronic kidney disease on the background of bardet-biedl syndrome: a case report and review of literature.

Introduction: Bardet-Biedl syndrome (BBS) is a rare autosomal recessive multisystem disorder characterized by retinal dystrophy, obesity, postaxial polydactyly, renal dysfunction, learning difficulties, and hypogonadism. In this case report, the authors present the clinical course and management of a patient with BBS who developed chronic kidney disease (CKD). Case presentation: An 18-year-old male presented to the emergency department with chief complaints of fever, cough, vomiting, and decreased urine output for 7 days. Parents complained that the child had a delay in development compared to other children of the same age group. On examination, the patient had tachypnea, periorbital and pedal edema, expiratory wheeze with bilateral basal crackles, polydactyly, central obesity, microtestes, and delayed developmental milestones. Ultrasonography revealed bilateral small kidneys with increased cortical echotexture and loss of corticomedullary differentiation. Based on clinical features, the patient was diagnosed with CKD in the background of BBS. Hemodialysis was initiated after the diagnosis. Discussion: The management of CKD in the background of BBS poses unique challenges due to the complex multisystem involvement of this genetic disorder. There should be early reorganization and management of this condition so that the patient can have a better quality of life. Moreover, in developing countries like Nepal, genetic testing and diagnosis should be made easily accessible for better patient outcome. Conclusion: Multidisciplinary approach involving nephrologists, ophthalmologists, endocrinologists, and geneticists is important to optimize the treatment and long-term management of Badet Biedel patients.

Non-adherence to anti-hypertensive medications in a low-resource country Nepal: a systematic review and meta-analysis.

Background: Nepal is a low resource country with cardiovascular diseases being the number one cause of mortality. Despite hypertension being the single most important risk factor for cardiovascular diseases, non-adherence to anti-hypertensive medications has not been assessed systematically. So, this systematic review and meta-analysis aims to analyze the prevalence of non-adherence to anti-hypertensive medications in Nepal. Methodology: This systematic review and meta-analysis was piloted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. Electronic databases of Embase, PubMed, Scopus, Web of Science, Cochrane Library, Cinhal Plus, and Google Scholar were searched from inception till 1 February 2023. The random-effects model with 95% confidence interval (CI) was used to calculate the non-adherence rate. Results: Altogether, 14 studies with a total of 3276 hypertensive patients were included in the meta-analysis. The pooled prevalence of non-adherence to anti-hypertensive medications was 49% (95% CI: 0.37-0.62, I2=98.41%, P<0.001). The non-adherence rate using Morisky Medication Adherence Scale (MMAS) was 55% (95% CI: 0.34-0.76, I2=99.14%, P<0.001), and using Hill-Bone Compliance Scale, the non-adherence rate was 45% (95% CI: 0.37-0.54, I2=84.36%, P<0.001). In subgroup analysis, the non-adherence was higher in rural areas 56% (95% CI: 0.51-0.61, I2=0.0%, P=0.46) compared to urban areas 42% (95% CI: 0.31-0.54, I2=96.90%, P<0.001). The trend of non-adherence was increasing after 2020. Additionally, forgetfulness, carelessness, cost of medications, number of comorbidities, and using an alternate form of medication were common factors associated with non-adherence. Conclusions: This meta-analysis showed that half of the hypertensive population of Nepal are non-adherent to their anti-hypertensive medications, thereby posing a significant long-term cardiovascular consequence among Nepali population.

Castleman disease of the pancreas mimicking pancreatic neuroendocrine tumor: A rare case report.

INTRODUCTION: Castleman's disease (CD) of the pancreas is a rare benign disease which can sometime resemble a pancreatic neuroendocrine tumor. PRESENTATION OF THE CASE: We report a case of a 46-years-old lady with an incidental pancreatic lesion. Preoperative diagnosis was a neuroendocrine tumor of the pancreas, for which anterior Radical Antegrade Modular Pancreatosplenectomy surgery (RAMPs) was performed. The postoperative histopathology showed features consistent with hyaline vascular type Castleman's disease. She had an uneventful surgical procedure and no serious postoperative complications. DISCUSSION: Pancreatic Castleman's disease is usually difficult to diagnose and there is no specific clinical manifestation to distinguish it from a neuroendocrine tumor. Even with radiological imaging it is very difficult to distinguish Castleman's disease from neuroendocrine tumors, and the preoperative imaging diagnosis may not match with the postoperative histopathological diagnosis. Treatment of CD is surgical resection and has a good prognosis. Partial resection and radiation therapy or observation alone is an alternative to complete surgical resection. CONCLUSION: Castleman disease of the pancreas sometimes mimics neuroendocrine tumors of the pancreas. Clinicians should consider Castleman's disease of the pancreas as a differential diagnosis for neuroendocrine like lesions of the pancreas.

Concerns over cardiovascular manifestations associated with monkeypox immunization: a literature review.

Following the recent surge in monkeypox cases worldwide, vaccines have gained popularity due to their protective role against the disease in the absence of specific pharmacological therapies for the disease. This article explores the possible challenges associated with the treatment and prevention of monkeypox with special emphasis on cardiovascular concerns relating to vaccines, warranting further investigation and research. Methods: Multiple electronic databases from inception till October 2022 were searched to find articles reporting the cardiovascular adverse events associated with monkeypox immunization. Results: Smallpox vaccines ACAM2000, JYNNEOS, and modified vaccinia Ankara have been used for monkeypox. ACAM2000 has been reported to have major cardiovascular adverse events such as myocarditis, dilated cardiomyopathy, and heart failure. Whereas JYNNEOS and modified vaccinia Ankara are associated with minor cardiovascular adverse events such as tachycardia, palpitation, electrocardiogram changes such as T wave inversion, and ST elevation. Conclusions: Despite having cardiovascular issues with the existing vaccines, clinicians and public health experts should measure the risk benefit aspect of smallpox vaccines to decide whether to go for mass immunization or not. Based on the lessons learned from the COVID-19 pandemic, immunization will be a better strategy to halt the monkeypox spread throughout the globe. However, further research is needed to determine the exact incidence and susceptibility to develop cardiovascular complications among monkeypox immunized individuals.

Urogenital myiasis in a post-menopausal rural woman: A case report.

BACKGROUND: Genital myiasis in females is a parasitic infection of the vulval region with the larva of various files species. Only a few cases of urogenital myiasis have been reported in the literature. CASE PRESENTATION: We present a case of 55 years postmenopausal, farmer female otherwise healthy presenting to the outpatient department with complaints of maggots and severe itching in the vulval region. Examination revealed erythema in the labia major and groin without lymphadenopathy. In the vaginal examination; inflammation and a large number of maggots were observed in the urethral meatus, labia minora, and vaginal canal, progressing to the cervix. With this, she was diagnosed to be a case of urogenital myiasis. She was managed with the extraction of maggots using turpentine oil, along with broad-spectrum antibiotics and Foleys catheterization for a week. Later during follow up, she was asymptomatic and examination revealed no maggots, and the lesions were healed. CLINICAL DISCUSSION: Extraction of maggots along with symptomatic management is the mainstay of treatment of myiasis. A significant number of the adult population in the rural areas of developing countries are illiterate and are not familiarized with education regarding genital hygiene. So, along with physicians, policy makers should also be involved in public awareness for genital hygiene. CONCLUSION: Despite being rare, urogenital myiasis is preventable and treatable condition. Efforts at increasing genital hygiene awareness in a low resource country are utmost for its prevention.

Complete small bowel obstruction due to ileal volvulus with autosomal dominant polycystic kidney disease: A rare case report.

INTRODUCTION AND IMPORTANCE: Volvulus is the twisting of the mesentery of the bowel along its axis. Ileal volvulus is a rare cause of small bowel obstruction. Ileal volvulus coexisting with autosomal dominant polycystic kidney disease has not been reported in the literature previously. CASE PRESENTATION: 65-year male with known history of autosomal dominant polycystic kidney disease (ADPKD) presented with pain abdomen for 5 days, obstipation for 3 days, and multiple episodes of bilious vomiting in the emergency department. Being a suspect of bowel obstruction, X-ray abdomen was done which showed features of small intestine obstruction. Further, to find the etiology of obstruction, contrast enhanced computed tomography (CECT) abdomen was done which showed swirling of the ileal loop and the ileal mesenteric vessels along with transition point in the ileal loop suggestive of ileal volvulus. Exploratory laparotomy with detorsion of the volvulus was done for management. CLINICAL DISCUSSION: Small bowel volvulus, more specifically ileal volvulus, is a rare cause of intestinal obstruction. Patients present with the cardinal features of bowel obstruction, i.e., abdominal pain, distension, vomiting, and constipation/obstipation. Our patient had coexisting ADPKD which further aggravated the clinical presentation. Definitive management of the volvulus includes exploratory laparotomy and detorsion along with resection of the bowel if found ischemic. In our case the bowel was healthy so only detorsion was done. CONCLUSION: Early diagnosis and meticulous exploratory laparotomy is utmost for the management of ileal volvulus. Besides, the secondary etiology (ADPKD in our case) should be managed to prevent future recurrences.

Factors affecting length of hospital stay in chronic obstructive pulmonary disease patients in a tertiary hospital of Nepal: A retrospective cross-sectional study.

Background: Despite the increasing burden of chronic obstructive pulmonary disease in Nepal, studies analyzing the factors associated with inpatient length of hospital stay are lacking. Methods: This is a retrospective, cross-sectional hospital-based study conducted between July 2020 and July 2021 on patients admitted to the inpatient ward of Pulmonary and Critical Care Medicine of Tribhuvan University Teaching Hospital with a primary diagnosis of acute exacerbation of chronic obstructive pulmonary disease. The sample size of our study was 90. Clinical and demographic factors, blood investigation parameters, and treatment received were analyzed via univariate and multivariate analysis to find the factors associated with length of stay. Results: The mean age of chronic obstructive pulmonary disease patients was 68.84 ± 10.22 years, with 42.2% of males and 43.3% of current smokers. The length of hospital stay ranged from 2 to 25 days, with an average stay of 6.69 ± 4.02 days. Factors associated with length of stay are the number of comorbidities (p = 0.007), blood eosinophils at admission (p = 0.022), and use of mechanical ventilatory support (p < 0.001). Conclusions: Proper management of comorbidities and eosinophilic exacerbations as well as careful use of mechanical ventilatory support are required to further reduce the duration of hospital stay in chronic obstructive pulmonary disease patients.

Epilepsy associated tuberous sclerosis; a case report from Bangladesh.

Tuberous sclerosis or Bourneville's disease is a rare autosomal dominant disease affecting many organs like the brain, heart, lungs, eyes, kidneys and skin. It is characterized by neurological manifestation like epilepsy, cutaneous changes and the formation of benign lesions in multiple organs. The symptoms are apparent only in late childhood, which limits the early diagnosis in infancy. Here, we report a case of a 15 year old female child with tuberous sclerosis.

Prevalence of rheumatic heart disease in South Asia: A systematic review and meta-analysis.

INTRODUCTION: Most countries in South Asia are endemic for rheumatic heart disease (RHD). We aimed to estimate the pooled prevalence of RHD in South Asia from population-based screening studies. METHODS: A systematic strategy was developed and used to search online databases for articles which described the prevalence of RHD in the South Asia region. Articles published in the English language between 1991 and 2021 were included in the review after fulfilling eligibility criteria. RESULTS: The pooled prevalence of RHD in South Asia was 2.79 per 1000 (95% Confidence interval (CI): 1.30-4.83) according to studies using auscultation followed by echocardiography of suspected cases and 18.28 per 1000 (95% CI: 11.59-26.44) for studies using echocardiography screening of all participants. The highest prevalence of 8.0 per 1000 (95% CI: 0.71-22.74) was observed in Pakistan, whereas the lowest prevalence of 0.32 per 1000 (95% CI: 0.18-0.48) was observed in Bangladesh. The prevalence of RHD has decreased to 2.30 per 1000 from 3.06 per 1000 by 24.84% in 30 years (1991-2021). The prevalence is more than twice higher using WHF criteria compared to WHO criteria. We observed no sex-related differences in prevalence (p = 0.16). CONCLUSIONS: The prevalence of RHD is still high in the South Asian population. Recent studies utilizing echocardiographic screening have identified subclinical cases of RHD, reflecting the significant burden of the disease in this region. More population-based echocardiography screening studies in every country in South Asia are required to estimate the accurate prevalence of the disease.

Increased expression of heat shock protein 70 and heat shock factor 1 in chronic dermal ulcer tissues treated with laser-aided therapy.

BACKGROUND: Chronic dermal ulcers are also referred to as refractory ulcers. This study was conducted to elucidate the therapeutic effect of laser on chronic dermal ulcers and the induced expression of heat shock factor 1 (HSF1) and heat shock protein 70 (HSP70) in wound tissues. METHODS: Sixty patients with 84 chronic dermal ulcers were randomly divided into traditional therapy and laser therapy groups. Laser treatment was performed in addition to traditional therapy in the laser therapy group. The treatment efficacy was evaluated after three weeks. Five tissue sections of healing wounds were randomly collected along with five normal skin sections as controls. HSP70-positive cells from HSP70 immunohistochemical staining were counted and the gray scale of positive cells was measured for statistical analysis. Reverse transcription-polymerase chain reaction (RT-PCR) and Western blotting were performed to determine the mRNA and protein expressions of HSF1 and HSP70. RESULTS: The cure rate of the wounds and the total efficacy in the laser therapy group were significantly higher than those in the traditional therapy group (P < 0.05, P < 0.01, respectively). Immunohistochemical staining revealed that the HSP70-positive cell count was significantly higher in laser therapy group than those in the traditional therapy group and controls (P < 0.01), and the gray scale of the cell signal was obviously lower than traditional therapy group and controls (P < 0.05). By contrast, the traditional therapy group and the control group were not significantly different. The RNA levels of HSF1 and HSP70 were higher in the laser therapy group by RT-PCR, but very low in normal skin and the traditional therapy group. The analysis on the gray scale of the Western blot bands indicated that the expression of HSF1 and HSP70 in the laser therapy group was significantly higher than in the traditional therapy group and the control group (P < 0.01), and the expression in the traditional therapy group was also higher than in the control group (P < 0.05). CONCLUSION: Laser-aided therapy of chronic dermal ulcers plays a facilitating role in healing due to the mechanism of laser-activated endogenous heat shock protection in cells in wound surfaces.