Amyotrophic lateral sclerosis with respiratory failure and dysautonomia: a case report.

Amyotrophic lateral sclerosis (ALS) is a disease that affects both upper and lower motor neurons, causing a range of symptoms beyond the motor system. Recent research has shown that the autonomic nervous system can also be affected, with symptoms such as orthostatic hypotension, fluctuations in blood pressure, and dizziness being reported. Case presentation: A 58-year-old male presented with left lower limb limping, difficulty climbing stairs, and left foot weakness, followed by right upper limb weakness and was diagnosed with ALS and received edaravone and riluzole treatment. He presented again with right lower limb weakness, shortness of breath, and wide fluctuations in blood pressure, leading to ICU admission with new diagnosis of ALS with dysautonomia with respiratory failure and was managed with non-invasive ventilation, physiotherapy, and gait training exercises. Clinical discussion: ALS is a progressive neurodegenerative disease affecting motor neurons but non-motor symptoms can also occur, including dysautonomia, which can result in blood pressure fluctuations. Dysautonomia in ALS is caused by several mechanisms such as severe muscle atrophy, prolonged ventilatory support, and upper and lower motor neuron lesions. Management of ALS involves giving a definitive diagnosis, providing nutritional support, using disease-modifying drugs such as riluzole and non-invasive ventilation to improve survival and quality of life. Early diagnosis is essential for effective management of the disease. Conclusion: Early diagnosis, use of disease-modifying drugs, non-invasive ventilation, and maintaining the patient's nutritional status are crucial for managing ALS which can have non-motor symptoms as well.

Organizing pneumonia as an initial presentation in a male lupus: A rare case report.

Organizing pneumonia (OP) is one of the rare pulmonary manifestations of systemic lupus erythematosus (SLE) which is infrequently reported as a presenting manifestation. Early diagnosis of lupus-related OP with the help of imaging, can drive to prompt initiation of immunosuppressive therapy leading to a better prognosis. We present a case of a 34-year-old young male who presented with fever, myalgia, and a dry cough for 1 month and was later diagnosed as SLE-related organizing pneumonia.

Painful tonic spasms in a patient with neuromyelitis optica spectrum disorder: A case report.

Painful tonic spasms initially described in association with multiple sclerosis are actually more common in patients with neuromyelitis optica spectrum disorder. Characterized by fierce pain and tonic posture of limbs, painful tonic spasms are common in patients during the recovery phase after the first episode of myelitis. A 68-year-old man presented with painful tonic spasm after 2 months of diagnosis of neuromyelitis optica spectrum disorder. Eventual use of eslicarbazepine resulted in significant control of spasms. Early recognition of painful tonic spasms and appropriate therapeutic medications can significantly decrease the impact it can have on the quality of life among neuromyelitis optica spectrum disorder patients.

Severe hepatitis E infection in pregnancy: a case report.

Hepatitis E virus causes self limiting hepatitis most of the times but, during pregnancy it can lead to severe hepatitis along with various complications thereby increasing the mortality. Case presentation: A 27-year-old woman gravida two, para one at 38 weeks and 6 days of gestation presented with multiple episodes of nonbilious vomiting, severe dehydration, and later developed right upper quadrant abdominal pain. The patient had a positive serological test for the hepatitis E virus, and liver enzymes were severely elevated. Under supportive treatment she delivered a healthy baby, and her liver enzymes returned to normal levels after 2 weeks of delivery. Clinical discussion: Although the hepatitis E virus usually causes self-limiting hepatitis, it can quickly progress to severe hepatitis, liver failure, and even death during pregnancy. Immunological change with a Th2 biased response and increased hormonal levels during pregnancy could possibly facilitate the development of severe liver damage. No particular drug has been approved for the treatment of hepatitis E viral infection in pregnant women, and the commonly used drugs are contraindicated due to the risk of teratogenicity. Supportive therapy and intensive monitoring are the core management techniques for hepatitis E virus infection in pregnant women. Conclusion: Due to the high mortality risk, pregnant women should try to avoid possible exposure to the hepatitis E virus, but once infected, symptomatic therapy is the mainstay.

Guillain-Barré syndrome complicating early pregnancy: a case report from Nepal.

Guillain-Barré syndrome (GBS) is an immunological demyelinating disorder characterized by progressive, ascending flaccid weakness, usually resulting after infection or some immune stimulation. Its occurrence during pregnancy is rare and due to attribution of its symptoms to pregnancy, diagnosis might be delayed. Case Presentation: A 39-year-old G4P3L2A0 woman at 13 weeks 6 days period of gestation presented with acute, symmetrical, ascending type of flaccid quadriparesis leading to slurring of speech, swallowing difficulty, and eventually respiratory failure. With the diagnosis of GBS, she was admitted to the intensive care unit, five sessions of plasma exchange were done along with physiotherapy and her symptoms started improving. After discharge she was on regular antenatal care visits and eventually, she delivered a healthy baby at term with an uneventful labor event and postpartum period. Clinical Discussion: There is a huge maternal-fetal risk of respiratory failure, aspiration pneumonia, preterm delivery, possible use of forceps or vacuum, and operative interference due to GBS in pregnancy, whose causative agents are similar to that of the general population. The swinging pattern of risk of GBS in pregnancy might be due to immunological changes with predominant Th2 response seen in pregnancy. Treatment measures are similar as in the general population with initial symptomatic care and administration of disease-specific therapy later which consists of intravenous immunoglobulin and/or plasmapheresis. Conclusion: A well-timed diagnosis of GBS in pregnancy might allow successful management with the help of intensive monitoring with or without immunotherapy.

Pregnancy in an isthmocele: A rare case from Nepal.

An infrequent form of ectopic pregnancy, pregnancy in an isthmocele can be hazardous due to hemorrhage or uterine rupture. With no clear guidelines for the management of this condition, surgery is the preferred option.

Porokeratosis of gluteal region: A case report.

Genitogluteal porokeratosis is a rare localized disorder of keratinization. Due to the rarity of the case and non-specific keratotic lesion, it is often misdiagnosed until a histological examination is performed. Treatment of this condition can be challenging, which comprises various topical and systemic drugs, lasers, cryotherapy, phototherapy, and also surgical intervention. Regular follow-up is necessary in the view of this disorder being a premalignant condition.

Cyst of rete ovarii: A case report.

Rete ovarii giving rise to cysts, and benign and malignant tumors is a rare entity. Radiological and histopathological examinations can be used to make the diagnosis of rete cyst and differentiate it from cystic tumor of ovarian origin, with surgery being the treatment of choice.

Successful management of postpartum hemorrhage and surgical site infection in a pregnant woman under warfarin for heart valve replacement: A case report.

INTRODUCTION AND IMPORTANCE: Pregnant women under warfarin for mechanical heart valves can pose a variety of challenges which requires fine tuning of various anticoagulants throughout the pregnancy and in the postpartum period as hemorrhage can lead to maternal and fetal morbidity and mortality. CASE PRESENTATION: A 36-year-old woman gravida two, para one at 35 weeks 5 days gestation, with hypothyroidism with mitral valve replacement and tricuspid valve repair due to rheumatic heart disease underwent emergency lower section cesarean section for fetal bradycardia. B-lynch suturing was eventually done to control atonic postpartum hemorrhage. During hospital stay she developed surgical site infection of abdominal skin incision site which was also subsequently managed. Postpartum anticoagulation was started late due to postpartum hemorrhage and finally the patient was discharged on warfarin. CLINICAL DISCUSSION: There is always a risk of both thromboembolic and hemorrhagic manifestations in a pregnant woman with a prosthetic heart valve which requires fine tuning of anticoagulants throughout the pregnancy and in the postpartum period. Hemorrhagic manifestation in the form of postpartum hemorrhage is common which can be difficult to manage and also poses a great dilemma in restarting the anticoagulation after delivery. Excessive blood loss can itself lead to mortality and morbidity, and also via increased risk of surgical site infection. CONCLUSION: Appropriate preconception counseling along with meticulous assessment, management and monitoring of pregnant women with prosthetic heart valves is necessary to decrease fetal and maternal morbidity and mortality.

Thoraco-omphalopagus conjoined twin: A rare case report.

INTRODUCTION AND IMPORTANCE: Conjoined twins represent a rare phenomenon and the etiology has not been clarified yet. There is a high rate of stillbirth and neonatal deaths resulting in very few cases surviving long enough for surgical separation. CASE PRESENTATION: A 33-year-old gravida 2 para 1 mother without any first and second trimester antenatal care visits was diagnosed to have conjoined twins in the third trimester. Mother and her family chose to terminate the pregnancy for which elective lower section cesarean section was done with the delivery of female conjoined twins, both of them subsequently declared dead within 4 h of birth. CLINICAL DISCUSSION: A conjoined twin gestation provides inimitable intricacy for obstetric management irrespective of the patient's areas of care. Early diagnosis through ultrasonography can be done and detailed evaluation is necessary along with fetal echocardiography regardless of site of fusion. Cesarean section is the recommended mode of delivery as this reduces various complications. CONCLUSION: The obstetricians' role in timely prenatal diagnosis, counseling, and organization of interdisciplinary medical care is indispensable in cases of conjoined twins.

Pure Sertoli cell tumor of the ovary: A case report.

Pure Sertoli cell tumors are an uncommon variant of rare ovarian Sertoli-Leydig cell tumors. Due to nonspecific clinical and imaging features, diagnosis is often made after histopathological examination. The prognosis is excellent as most are detected in the early stages and surgical resection is often curative in most cases.

Guillain-Barré syndrome associated with Coronavirus disease 2019: A case from Nepal.

Coronavirus disease 2019 (COVID-19) has now spread widely after the outbreak since December 31, 2019. Guillain-Barré syndrome is an immunological postinfectious neuropathy, which has been reported to be a rare but possible complication COVID-19. We report a case of Guillain-Barré syndrome associated with COVID-19 in Nepal.

Clinical spectrum and management of dystonia in patients with Japanese encephalitis: A systematic review.

BACKGROUND: Japanese encephalitis (JE) is a potentially fatal viral infection with a wide range of manifestations and can also present with a variety of movement disorders (MD) including dystonia. Dystonic features in JE are uncommon. Here, we have tried to summarize the clinical features and management of dystonia among JE patients with a comprehensive literature search. METHODS: Various databases, including PubMed, Embase, and Google Scholar, were searched against the predefined criteria using suitable keywords combination and boolean operations. Relevant information from observational and case studies was extracted according to the author, dystonic features, radiological changes in the brain scans, treatment options, and outcome wherever provided. RESULT: We identified 19 studies with a total of 1547 JE patients, the diagnosis of which was confirmed by IgM detection in serum and/or cerebrospinal fluid in the majority of the patients (88.62%). 234 (15.13%) of JE patients had dystonia with several types of focal dystonia being present in 131 (55.98%) either alone or in combination. Neuroimaging showed predominant involvement of thalami, basal ganglia, and brainstem. Oral medications including anticholinergics, GABA agonists, and benzodiazepines followed by botulinum toxin were the most common treatment modalities. CONCLUSION: Dystonia can be a disabling consequence of JE, and various available medical therapies can significantly improve the quality of life. Owing to insufficient studies on the assessment of dystonia associated with JE, longitudinal studies with a larger number of patients are warranted to further clarify the clinical course, treatment, and outcome of dystonia.

Prevalence of vancomycin-resistant enterococci in Asia-A systematic review and meta-analysis.

WHAT IS KNOWN AND OBJECTIVE: The rise of vancomycin-resistant enterococci (VRE) has been a major health problem in most countries of the world including Asia, since its discovery. There is a paucity of data on VRE in many countries of Asia as well as limited pooled estimates. Therefore, we performed a systematic review and meta-analysis to estimate a pooled prevalence of VRE in Asia. METHODS: A literature search in electronic databases like PubMed, Embase and Google Scholar and manual searching of references and grey literature, comprising the information on the prevalence of VRE with at least two species of enterococci, conducted in different countries of Asia from January 1, 2000, to September 20, 2020, was done. The random-effect model and 95% CIs was used to calculate the pooled prevalence. Subgroup, sensitivity and meta-regression analyses were performed to address heterogeneity while Egger's test for publication bias. RESULTS AND DISCUSSIONS: We identified 39 studies, comprising a total of 11,875 enterococcal isolates. The result of the analysis showed that the pooled prevalence of VRE in Asia was 8.10% (95% CI; 7-9; I2  = 93.79%; p < 0.001). Resistance to vancomycin was greater among strains of E. faecium compared to the strains of E. faecalis (22.40% vs. 3.70%). Amongst various regions of Asia, the highest prevalence of VRE was found in the Western Asian region and the lowest in the South-east Asian region. Moreover, the rate of VRE was higher than most European countries and lower than USA. WHAT IS NEW AND CONCLUSIONS: With an upsurge of VRE in Asia in recent years, efficient infection control programmes, robust surveillance systems and adherence to antibiotic stewardship are paramount to halt the further rise of VRE.