Clinical profile and dynamic magnetic resonance imaging in Hirayama disease: a single-centered cross-sectional study in Nepal.

Hirayama disease (HD) is juvenile monomelic amyotrophy of the distal upper limb first described by Hirayama in 1959 AD. HD is a benign condition with chronic microcirculatory changes. The hallmark of HD is necrosis of the anterior horns of the distal cervical spine. Materials and Methods: Eighteen patients were assessed for clinical and radiological Hirayama disease. Clinical criteria included insidious onset nonprogressive chronic upper limb weakness and atrophy in teens or early twenties without sensory deficits and coarse tremors. MRI was done in a neutral position followed by neck flexion to evaluate cord atrophy and flattening, abnormal cervical curvature, loss of attachment between the posterior dural sac and subjacent lamina, anterior shifting of the posterior wall of the cervical dural canal, posterior epidural flow voids, and an enhancing epidural component with its dorsal extension. Results: The mean age was 20.33 years, and the majority, 17 (94.4%), were male. Neutral-position MRI revealed loss of cervical lordosis in 5 (27.8%) patients, cord flattening in all patients with asymmetry in 10 (55.5%), and cord atrophy was observed in 13 (72.2%) patients with localized cervical cord atrophy in only 2 (11.1%) and extension of atrophy to dorsal cord in 11 (61.1%) patients. Intramedullary cord signal change was seen in 7 (38.9%) patients. Loss of attachment of posterior dura and subjacent lamina and anterior displacement of dorsal dura was seen in all patients. A crescent-shaped epidural intense enhancement was noted along the posterior aspect of the distal cervical canal in all patients, with dorsal level extension in 16 (88.89%) patients. The mean thickness of this epidural space was 4.38±2.26 (mean±2SD), and the mean extension was 5.5±4.6 vertebral levels (mean±2SD). Conclusion: The high degree of clinical suspicion can guide additional contrast studies in flexion as a set MRI protocol for early detection and avoiding false negative diagnoses of HD.

Japanese encephalitis among patients with acute encephalitic syndrome admitted to a tertiary hospital in Chitwan, Nepal--a prospective observational study.

INTRODUCTION: The reported incidence of JE among patients with acute encephalitic syndrome (AES) in Nepal ranges between 20% to 62%. In light of the lack of up-to-date data, we sought to describe the epidemiology of JE in Chitwan, Nepal. METHODS: A prospective observational study was conducted during 2010-2012 in the College of Medical Science in the Chitwan District. Patients with suspected JE were tested for anti-JE IgM in serum and cerebrospinal fluid (CSF). RESULTS: Of 227 all patients tested, 18 (7.9%) were found positive for JE. 17/202 (8.4%) patients with AES had JE. All, with the exception of two patients, were diagnosed on the basis of positive a serologic test, both in serum and CSF samples. Patients with JE were significantly older (42.1±27.6 years) than patients without JE (25.6±25.2 years, p = 0.02). Half of JE cases occurred in adults older than 50. More of the JE cases (11/18, 61.1%) occurred during the rainy season when compared to the JE negative patients [71/209, (34%), p = 0.01]. None of the JE patients had a relevant travel history, and one recalled having been immunized against JE. There was a variation in the geographic distribution of cases across the districts of the central Terai. CONCLUSIONS: In this cohort, the proportion of patients with AES who had JE was lower than in previous studies. In addition, most patients were adults, and cases were not distributed uniformly across the central Terai region. The risk of acquiring JE by short-term travelers in the area is likely to be low. Vector-control programs and the promotion of mosquito avoidance behavior in the Terai region should continue. The high proportions of adults among patients with JE may suggest recent changes in the epidemiology of JE in the central Terai region, and routine immunization of all adults should be considered.

Intravenous Thrombolysis and Risk Factors for Ischemic Stroke.

Thrombolysis is one of the proven potential treatments for the management of acute ischemic stroke. Intravenous recombinant tissue-plasminogen activator (rt-PA) is the only medically approved biological thrombolysing agent for the treatment of acute ischemic stroke within 4.5h of stroke (2.2% symptomatic intracerebral haemorrhage, 12.7% mortality and 58.0% functional independence), but following the guideline and criteria provided by National Institute of Neurological Disorder and Stroke (NINDS) and SITS (Safe Implementation of Thrombolysis in Stroke) studies. Nepal needs to evidently introduce intravenous rt-PA in its clinical setting for treatment of acute ischemic stroke, which has been approved for more than a decade ago in developed countries. Several modifiable and non-modifiable risk factors can affect the outcomes of the treatment with intravenous rt-PA. Early modification of factors predicting the risk outcomes can be a beneficial tool to justify the thrombolytic treatment. This review aims to discuss the major studies on thrombolysis using rt-PA and main factors that can affect the outcomes of treatment in ischemic stroke.

Acute coronary syndrome in an intensive care unit of a tertiary care centre: the spectrum and coronary risk factors.

INTRODUCTION: Acute coronary syndrome is the major leading cause for coronary care unit admission. Its spectrum comprises a variety of disorders including unstable angina, non ST elevation and ST elevation myocardial infarction. Its spectrum and incidence is crucial as a part of need assessment of cardiac catheterization laboratories. METHODS: An observational study was designed to study the spectrum of acute coronary syndrome and associated coronary heart disease risk factors in subjects admitted in intensive care unit of College of Medical Sciences Teaching Hospital, Bharatpur, Nepal from August 2009 to September 2010. Details including coronary risk factors and the categories and outcomes of acute coronary syndrome were analyzed. RESULTS: A total of 57 subjects were included in the study. The majority 36 (63.16%) were males. The mean age was 64.54±13.8 years. Five (8.8%) patients were ≤45 years and 29 (50.88%) patients were ≥65 years. The major coronary heart disease risk factors were smoking 29 (50.88%), diabetes 25 (43.85%), hypertension 21 (36.87%), and previous history of coronary heart disease 18 (31.58%). ST elevation myocardial infarction was the major category 24 (42.11%) followed by non-ST elevation myocardial infarction and unstable angina 18 (31.58%) and 15 (26.32%), respectively. Myocardial infarction complicated with cardiogenic shock had very high mortality (83.33%). CONCLUSIONS: The ST elevation myocardial infarction was the major clinical form of acute coronary syndrome admitted in intensive care unit. Prevention should be targeted on modifi able risk factors such as the management of hypertension, dyslipidemia, diabetes and obesity. In addition, the improvement in cardiology service with the establishment of CCU and cathlab might improve the mortality and morbidity in such cases.