Segawa Syndrome, a Dramatic Response to Dopamine.

Segawa syndrome usually manifests as dystonia, disturbance of gait with fatigue, and may be confused with spasticity. Also known as dopamine-responsive dystonia (DRD), it should be considered in any child who presents with paroxysmal or progressive hypertonia of unknown etiology, which responds dramatically to levodopa. It is a clinical diagnosis, but the level of pterins in cerebrospinal fluid and guanosine triphosphate cyclohydrolase-1 (GTCH 1) gene mutation testing done by molecular genetic testing are confirmatory. Our case is a 45-year female with a family history of similar illness expressed as autosomal recessive inheritance pattern. She had symptoms onset at an early age of 13 years with features of dystonia of predominantly lower limbs, hence the inability to maintain posture and walk. Dramatic improvement with levodopa but sudden deterioration to dystonia due to noncompliance was evident in our patient with troublesome features of concomitant adjustment disorder during presentation.

Longitudinally extensive transverse myelitis, a disabling disorder with a good prognosis: a case series from Nepal.

Introduction: Longitudinally extensive transverse myelitis (LETM) is a rare spinal cord disorder with variable etiologies and presentations. It can present solely or as an association with other neurological disorders. Methodology: It was a series of cases of LETM in a tertiary care hospital. Clinical presentation and outcomes: The initial three cases presented with bilateral lower extremity weakness and were diagnosed as transverse myelitis while, the fourth case, already diagnosed as LETM presented with seizure followed by loss of consciousness. All four cases had a good prognosis to date with continued physiotherapy. Conclusion: The early diagnosis of the disease helps to guide the optimal management and decide the potential need for physiotherapy.

Implementing stroke care in a lower-middle-income country: results and recommendations based on an implementation study within the Nepal Stroke Project.

Background: Globally, the majority of strokes affect people residing in lower- and lower-middle-income countries (LMICs), but translating evidence-based knowledge into clinical practice in regions with limited healthcare resources remains challenging. As an LMIC in South Asia, stroke care has remained a healthcare problem previously unaddressed at a national scale in Nepal. The Nepal Stroke Project (NSP) aims to improve acute stroke care in the tertiary healthcare sector of Nepal. We hereby describe the methods applied and analyze the barriers and facilitators of the NSP after 18 months. Methods: The NSP follows a four-tier strategy: (1) quality improvement by training healthcare professionals in tertiary care centers; (2) implementation of in-hospital stroke surveillance and quality monitoring system; (3) raising public awareness of strokes; and (4) collaborating with political stakeholders to facilitate public funding for stroke care. We performed a qualitative, iterative analysis of observational data to analyze the output indicators and identify best practices. Results: Both offline and online initiatives were undertaken to address quality improvement and public awareness. More than 1,000 healthcare professionals across nine tertiary care hospitals attended 26 stroke-related workshops conducted by Nepalese and international stroke experts. Monthly webinars were organized, and chat groups were made for better networking and cross-institutional case sharing. Social media-based public awareness campaigns reached more than 3 million individuals. Moreover, live events and other mass media campaigns were instituted. For quality monitoring, the Registry of Stroke Care Quality (RES-Q) was introduced. Collaboration with stakeholders (both national and international) has been initiated. Discussion: We identified six actions that may support the development of tertiary care centers into essential stroke centers in a resource-limited setting. We believe that our experiences will contribute to the body of knowledge on translating evidence into practice in LMICs, although the impact of our results must be verified with process indicators of stroke care.

Stroke systems of care in South-East Asia Region (SEAR): commonalities and diversities.

The Southeast Asia Region (SEAR) accounts for nearly 50% of the developing world's stroke burden. With various commonalities across its countries concerning health services, user awareness, and healthcare-seeking behavior, SEAR still presents profound diversities in stroke-related services across the continuum of care. This review highlights the numerous systems and challenges in access to stroke care, acute stroke care services, and health care systems, including rehabilitation. The paper has also attempted to compile information on the availability of stroke specialized centers, Intravenous thrombolysis (IVT) ready centers, Endovascular therapy (EVT) ready centers, rehabilitation centers, and workforce against a backdrop of each country's population. Lastly, the efforts of WHO (SEARO)-CMCL (World Health Organization-South East Asia region, Christian Medical College & Hospital Ludhiana) collaboration towards improving stroke services and capacity among the SEAR have been described.

Progressive multifocal leukoencephalopathy in the background of chlorambucil treated chronic lymphocytic leukemia: a case report from Nepal.

Introduction: Progressive multifocal leukoencephalopathy is a rare manifestation in itself. Although many immunosuppressive states are associated with the disease, its occurrence in the setting of chronic lymphocytic leukaemia treated with chemotherapy is seldom reported to date. Case presentation: A 67-year-old woman with known chronic lymphocytic leukaemia who was previously receiving chlorambucil treatment was identified as having progressive multifocal leukoencephalopathy; her prognosis is currently good. Clinical discussion: Although a rare disease in an immunocompromised setting, progressive multifocal leukoencephalopathy often leads to a grave outcome. However, the authors describe a case with a good prognosis to date. Conclusion: Progressive multifocal leukoencephalopathy should be in differentials in immunocompromised patients with dementia. Given that the later prognosis of the disease is unpredictable, an earlier diagnosis would be better for immunological reconstitution.

Stroke epidemiology and outcomes of stroke patients in Nepal: a systematic review and meta-analysis.

BACKGROUND: With an increasing burden of stroke, it is essential to minimize the incidence of stroke and improve stroke care by emphasizing areas that bring out the maximum impact. The care situation remains unclear in the absence of a national stroke care registry and a lack of structured hospital-based data monitoring. We conducted this systematic review and meta-analysis to assess the status of stroke care in Nepal and identify areas that need dedicated improvement in stroke care. METHODS: A systematic literature review was conducted to identify all studies on stroke epidemiology or stroke care published between 2000 and 2020 in Nepal. Data analysis was done with Statistical Package for Social Sciences (SPSS) and Comprehensive Meta-analysis (CMA-3). RESULTS: We identified 2533 studies after database searching, and 55 were included in quantitative and narrative synthesis. All analyses were done in tertiary care settings in densely populated central parts of Nepal. Ischemic stroke was more frequent (70.87%) than hemorrhagic (26.79%), and the mean age of stroke patients was 62,9 years. Mortality occurred in 16.9% (13-21.7%), thrombolysis was performed in 2.39% of patients, and no studies described thrombectomy or stroke unit care. CONCLUSION: The provision of stroke care in Nepal needs to catch up to international standards, and our systematic review demonstrated the need to improve access to quality stroke care. Dedicated studies on establishing stroke care units, prevention, rehabilitation, and studies on lower levels of care or remote regions are required.

Are digital social media campaigns the key to raise stroke awareness in low-and middle-income countries? A study of feasibility and cost-effectiveness in Nepal.

BACKGROUND: Stroke is a major global health problem and was the second leading cause of death worldwide in 2020. However, the lack of public stroke awareness especially in low- and middle-income countries (LMICs) such as Nepal severely hinders the effective provision of stroke care. Efficient and cost-effective strategies to raise stroke awareness in LMICs are still lacking. This study aims to (a) explore the feasibility of a social media-based stroke awareness campaign in Nepal using a cost-benefit analysis and (b) identify best practices for social media health education campaigns. METHODS: We performed a stroke awareness campaign over a period of 6 months as part of a Stroke Project in Nepal on four social media platforms (Facebook, Instagram, Twitter, TikTok) with organic traffic and paid advertisements. Adapted material based on the World Stroke Day Campaign and specifically created videos for TikTok were used. Performance of the campaign was analyzed with established quantitative social media metrics (impressions, reach, engagement, costs). RESULTS: Campaign posts were displayed 7.5 million times to users in Nepal. 2.5 million individual social media users in Nepal were exposed to the campaign on average three times, which equals 8.6% of Nepal's total population. Of those, 250,000 users actively engaged with the posts. Paid advertisement on Facebook and Instagram proved to be more effective in terms of reach and cost than organic traffic. The total campaign cost was low with a "Cost to reach 1,000 users" of 0.24 EUR and a "Cost Per Click" of 0.01 EUR. DISCUSSION: Social media-based campaigns using paid advertisement provide a feasible and, compared to classical mass medias, a very cost-effective approach to inform large parts of the population about stroke awareness in LMICs. Future research needs to further analyze the impact of social media campaigns on stroke knowledge.

Disseminated tuberculosis in an immunocompetent woman from the Himalayan region of Nepal: A case report.

Disseminated tuberculosis (TB) resulting from lymphohematogenous dissemination of Mycobacterium tuberculosis during primary infection or reactivation of latent disease is rare among young immunocompetent patients. Central nervous system TB (CNS TB) is one of the most challenging clinical diagnoses with high fatality. Here, we describe a young immunocompetent female with no known comorbidities initially presented with military pulmonary TB and later developed CNS TB. This presentation of disseminated TB in immunocompetent patient warrant early diagnosis and treatment.

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes syndrome: a case report from Nepal.

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like syndrome (MELAS) is a rare neurodegenerative inherited disorder that is characterized by stroke-like episodes, seizures, endocrine, and multiple system involvement. It is important to consider it as a differential diagnosis in a young patient with stroke-like episodes as it is progressive and has multiple complications. Case presentation: A 28-year-old male presented with slurring of speech and drowsiness for 7 h. He was a diagnosed case of type 2 diabetes mellitus, Wolf-Parkinson-White syndrome, and bilateral hearing loss. Clinical findings and investigations: The patient had expressive aphasia with impaired fluency, repetition, and naming. After being discharged, he represented with loss of consciousness and involuntary movements of the whole body. MRI and MRS showed extension of hyperintense lesions to parieto-occipital regions from temporal regions not limited by vascular territories. MELAS was considered, which was confirmed by molecular genetic analysis. Coenzyme Q10 was used for MELAS. Insulin, Linagliptin, and levetiracetam were used for diabetes and seizures. Regular follow-up was advised to the patient.MELAS is an important syndrome to consider in any young patient presenting with unexplained stroke disorders. A high index of suspicion is needed in an appropriate clinical setting to avoid misdiagnosis.

Initial Results of a Direct Aspiration First-Pass Technique to Treat Acute Ischemic Stroke Patients in Nepal.

Objective Endovascular therapy has become the mainstay of treatment of acute ischemic stroke (AIS) due to large vessel occlusion. A direct aspiration first-pass technique (ADAPT) using large bore aspiration catheters has been introduced as a rapid, simple method for achieving good revascularization and good clinical outcomes. The aim of this study was to assess the safety and efficacy of ADAPT in the treatment of AIS due to large-vessel occlusion in the Nepali patient population. Materials and Methods Retrospective data were collected for all consecutive patients treated for AIS with ADAPT from March 2019 through January 2021 at two hospitals. Outcomes were successful revascularization (modified thrombolysis in cerebral infarction score of 2b-3), time to revascularization, procedural complications, and good clinical outcome (modified Rankin Scale score of 0 to 2) and mortality at 90 days. Statistical Analysis Retrospective data were collected and descriptive statistics were calculated. Results Sixty-eight patients treated for AIS with ADAPT were included. The median National Institutes of Health Stroke Scale score at presentation was 13 (IQR 10-13.25). The median time from arterial puncture to revascularization was 40 minutes (IQR 30-45). Successful revascularization was achieved in 54 patients (79.4%). No cases of symptomatic intracranial hemorrhage occurred. At 90-day follow-up, good clinical outcome was achieved in 57 patients (83.8%), and 4 patients died (5.9%). Conclusion A direct aspiration first pass technique appears to be a fast, simple, safe, and effective method for the management of AIS in the Nepali patient population.

Selection of Young Animal Models of Autism over Adult: Benefits and Limitations.

Autism is a complex neurodevelopmental broad-spectrum disorder characterized by social interaction, and aberrant restrictive and repetitive behavior. The complex pathophysiology and unexplored drug targets make it difficult to standardize and validate the animal models of autism. The review was purposed for determining the benefits of younger animal models over adult models of autism. Similarly, animal models with respect to age, sex, body weight, number of animals used, along with autism inducing agents have been reviewed in this article. The differentiation of behavioral parameters has shown the benefits in the selection of younger animal models. Thus, we conclude that young and adolescence animal models of autism will be supporting for early detection and interventions with significant results.

A novel TFG variant of uncertain significance in amyotrophic lateral sclerosis: A case report and review of literature.

Introduction: Amyotrophic lateral sclerosis is a neurodegenerative disease with wide variation of genetics associated with it. Among the different genes described, mutation in TFG is a rare finding in amyotrophic lateral sclerosis. Case presentation: A 35 years old right-handed male presenting with ipsilateral weakness was diagnosed with amyotrophic lateral sclerosis. He was found to have missense variant of TFG with uncertain significance on exome sequencing. Clinical discussion: The genetics involved in amyotrophic lateral sclerosis is ever-evolving. The identification of new TFG variant in this disease adds another evidence to the role of TFG in neurodegenerative disease. Conclusions: The finding of TFG variant of uncertain significance is a rare finding in amyotrophic lateral sclerosis. And with the identification of new TFG variant, it leads to further understanding of spectrum of TFG and its pathophysiology in amyotrophic lateral sclerosis.

Acute reversible monoparesis in multiple neurocysticercosis: A case report and review of literature.

Focal neurological deficit like monoparesis due to cortical lesions is a rare entity. In spite of the common presentations like seizures and headaches in neurocysticercosis, occurrence of reversible monoparesis is an atypical phenomenon. Even in the absence of infarct or hemorrhages, manifestation of neural deficit due to compressive effect only is an interesting finding. And on top of that, reversible nature of the deficit in space occupying lesion is a rare occurrence in the existing literature. Here, we describe a known case of neurocysticercosis with reversible acute monoparesis secondary to multiple neurocysticercosis. The variations with which neurocysticercosis can present broaden our understanding in its pathophysiology and management protocol.

D-penicillamine Induced Myasthenia Gravis in Wilson's Disease: A Case Report.

Myasthenia gravis is a neuromuscular junction disorder characterised by fluctuating muscle weakness, improved by using anti-cholinesterase drugs. In addition to the autoimmune aetiology, various factors such as infections, surgery, and drugs are known to precipitate the condition. We report a case of a 15-year-old boy with D-penicillamine-induced myasthenia gravis who presented with facial diplegia, dysphagia, and drooling of saliva, 6 years after the initiation of treatment for Wilson's disease. Therefore, clinicians should be more vigilant while prescribing patients with chelating drugs like D-penicillamine with regular monitoring of the new symptoms and keeping a very low threshold for the suspicion of myasthenia gravis. Keywords: d-penicillamine; myasthenia gravis; pyridostigmine; Wilson's disease.

Middle cerebral artery occlusion due to cardiac tissue following catheter ablation in Wolff-Parkinson-White syndrome.

Catheter ablation is a commonly performed procedure in patients with Wolff-Parkinson-White syndrome. A 56-year-old man developed an acute ischemic stroke immediately after undergoing radiofrequency catheter ablation of the left-sided accessory pathway. Neuroimaging revealed complete occlusion of the proximal middle cerebral artery. Mechanical thrombectomy (MT) was performed with successful retrieval of the thrombo-embolus. Histopathological examination of the thrombo-embolus confirmed organic cardiac tissue. The patient was later discharged from the hospital with no neurologic deficit. There is no report of successful MT in patients with large-vessel occlusion because of the embolization of cardiac tissue after catheter ablation. This report highlights the need to remain vigilant for signs of stroke after any cardiac intervention.

Transsellar trans-sphenoidal encephalocele with cleft lip, cleft palate and agenesis of corpus callosum.

Basal encephalocele is a rare congenital malformation. Among basal encephaloceles, the transsellar, trans-sphenoidal encephalocele is the least common subtype. We present the case of a newborn female, who presented to us with cleft lip and cleft palate. Diagnostic neuroimaging revealed the presence of transsellar trans-sphenoidal encephalocele along with agenesis of the corpus callosum. There are very few case reports of trans-sphenoidal encephalocele with corpus callosum agenesis in a patient with midline cleft lip and palate. In this report, we discuss the clinico-radiological findings of this extremely rare condition and present a brief review of the literature.

Management of Stroke During COVID-19 Pandemic: The Challenges in Nepal.

The COVID-19 pandemic has significantly affected health care delivery globally. COVID-19 is associated with varied neurological manifestations including acute ischemic stroke. In densely populated South Asian nations like Nepal that have suboptimal baseline health care systems, we foresee unique challenges during this pandemic to ensure effective stroke management as well as the safety of health care workers involved in the management of stroke patients. Keywords: COVID-19; health care workers; safety; stroke management.

Prevalence of Stroke and Stroke Risk Factors in a South-Western Community of Nepal.

BACKGROUND: Stroke related studies in Nepal are primarily hospital-based and mainly from the capital city. OBJECTIVES: We aimed to estimate the prevalence of stroke and stroke risk factors in the South-Western community of Nepal. METHODS: A cross-sectional study was conducted from May to August 2018 among 549 randomly selected Nepalese participants from diverse ethnicity, aged ≥15 years, in a region with the availability of neurological support facilities. Data were collected using a stroke questionnaire designed for the purpose. Stroke was identified by enumerators using the Balance-Eyes-Face-Arms-Speech-Time (BEFAST) scale, and a senior neurologist confirmed it. We assessed the presence of major risk factors associated with stroke. RESULTS: The crude and age-standardised prevalence of stroke were 2368 and 2967 per 100,000 respectively. Of all the surveyed participants, 61% (n=335) reported consumption of full-fat dairy products >3 days per week, 87.6% (n=481) reported a high intake of salt (>5 g/day), 83.6% (n=459) with a low intake of fruits and vegetables (<400 g/day), 45.2% (n=248) with perceived stress related to work or home, 51.6% (n=283) with financial stress (283, 51.6%), 86.7% (n=457) with low high-density lipoprotein, 96.2% (n=507) with high blood urea nitrogen, 47.1% (n=356) were either overweight or obese 20.4% (n=112) with hypertension and 6.2% (n=34) with diabetes. CONCLUSIONS: The prevalence of stroke in the community of the South-Western part of Nepal is relatively higher than that estimated in South-Asia and global context. Our findings suggest an urgent community intervention, particularly with healthy lifestyles changes for future stroke prevention in the high-risk group.

Mechanical Thrombectomy for Basilar Artery Occlusion Stroke: A Case Report.

Posterior circulation strokes are potentially devastating events that carry a significant risk of morbidity and mortality. Acute basilar artery occlusion stroke is a rare posterior circulation stroke that needs emergent management. We report the case of a 67-year-old woman who developed an acute basilar artery occlusion. We achieved complete recanalization of the occluded basilar artery and its branches with endovascular mechanical thrombectomy. It is possible to achieve excellent results with mechanical thrombectomy in acute basilar artery occlusion if timely diagnosis and reperfusion can be done. We are not aware of any previous publication from Nepal describing this technique in acute basilar artery occlusion.

Endovascular treatment of complex vertebrobasilar junction aneurysms: A report of two cases.

INTRODUCTION: Vertebrobasilar junction (VBJ) aneurysms are uncommon posterior circulation aneurysms. The treatment of VBJ aneurysms is challenging and in most cases, endovascular management is preferred over neurosurgery. PRESENTATION OF CASE: We describe two patients with VBJ aneurysms who underwent successful neuro-interventional procedures. The first patient had concomitant basilar fenestration and was treated with balloon-assisted coiling. The second patient had difficult vascular anatomy and an anterior inferior cerebellar artery-posterior inferior cerebellar artery variant arising from the neck of the aneurysm. Braided stent-assisted coiling was done with transradial access. Both patients had a good neurologic recovery. DISCUSSION: Endovascular management of VBJ aneurysms is often complicated by anatomic difficulties like basilar fenestration, tortuosity of proximal vessels, atheromatous changes, and vascular stenosis. We achieved good post-procedure outcomes in both the patients. Optimal management of complex VBJ aneurysms often requires some modification to the usual interventional technique. CONCLUSION: VBJ aneurysms are generally treated with endovascular techniques. The transradial access, although rarely used by neurointerventionalists, has some distinct advantages over the transfemoral access, especially when dealing with right-sided VBJ aneurysms with marked tortuosity of proximal great vessels.