Acute disseminated encephalomyelitis as the first presentation of CNS tuberculosis: report of a case with brief review

Acute disseminated encephalomyelitis (ADEM) also known as post infectious encephalomyelitis is a demyelinating disease of the central nervous system (CNS) that typically presents as a monophasic disorder associated with multifocal neurological symptoms and disability. It may follow vaccination in children or infection. Viral infection like measles, rubella, influenza, Epstein bar, HIV, herpes, cytomegalusvirus (CMV) and West Nile virus have been implicated in the causation. Among bacteria, group A hemolytic streptococcus, mycoplasma pneumonia, Chlamydia, Rickettesia and leptospira have been shown to cause ADEM. There are few reports of ADEM due to tuberculosis (TB). We describe acute disseminated encephalomyelitis due to tuberculosis in a 35 year old female who initially started with neuropsychiatric manifestations and later developed florid neurological deficit and classical magnetic resonance imaging (MRI) lesions suggestive of the disease. The patient recovered completely after antitubercular therapy and is following our clinic for the last 12 months now

Global, regional, and national burden of disorders affecting the nervous system, 1990–2021: a systematic analysis for the Global Burden of Disease Study 2021

BackgroundDisorders affecting the nervous system are diverse and include neurodevelopmental disorders, late-life neurodegeneration, and newly emergent conditions, such as cognitive impairment following COVID-19. Previous publications from the Global Burden of Disease, Injuries, and Risk Factor Study estimated the burden of 15 neurological conditions in 2015 and 2016, but these analyses did not include neurodevelopmental disorders, as defined by the International Classification of Diseases (ICD)-11, or a subset of cases of congenital, neonatal, and infectious conditions that cause neurological damage. Here, we estimate nervous system health loss caused by 37 unique conditions and their associated risk factors globally, regionally, and nationally from 1990 to 2021.MethodsWe estimated mortality, prevalence, years lived with disability (YLDs), years of life lost (YLLs), and disability-adjusted life-years (DALYs), with corresponding 95% uncertainty intervals (UIs), by age and sex in 204 countries and territories, from 1990 to 2021. We included morbidity and deaths due to neurological conditions, for which health loss is directly due to damage to the CNS or peripheral nervous system. We also isolated neurological health loss from conditions for which nervous system morbidity is a consequence, but not the primary feature, including a subset of congenital conditions (ie, chromosomal anomalies and congenital birth defects), neonatal conditions (ie, jaundice, preterm birth, and sepsis), infectious diseases (ie, COVID-19, cystic echinococcosis, malaria, syphilis, and Zika virus disease), and diabetic neuropathy. By conducting a sequela-level analysis of the health outcomes for these conditions, only cases where nervous system damage occurred were included, and YLDs were recalculated to isolate the non-fatal burden directly attributable to nervous system health loss. A comorbidity correction was used to calculate total prevalence of all conditions that affect the nervous system combined.FindingsGlobally, the 37 conditions affecting the nervous system were collectively ranked as the leading group cause of DALYs in 2021 (443 million, 95% UI 378–521), affecting 3·40 billion (3·20–3·62) individuals (43·1%, 40·5–45·9 of the global population); global DALY counts attributed to these conditions increased by 18·2% (8·7–26·7) between 1990 and 2021. Age-standardised rates of deaths per 100 000 people attributed to these conditions decreased from 1990 to 2021 by 33·6% (27·6–38·8), and age-standardised rates of DALYs attributed to these conditions decreased by 27·0% (21·5–32·4). Age-standardised prevalence was almost stable, with a change of 1·5% (0·7–2·4). The ten conditions with the highest age-standardised DALYs in 2021 were stroke, neonatal encephalopathy, migraine, Alzheimer's disease and other dementias, diabetic neuropathy, meningitis, epilepsy, neurological complications due to preterm birth, autism spectrum disorder, and nervous system cancer.InterpretationAs the leading cause of overall disease burden in the world, with increasing global DALY counts, effective prevention, treatment, and rehabilitation strategies for disorders affecting the nervous system are needed

Experience with oral mexiletine in primary erythromelalgia in children

Primary erythromelalgia is characterized by burning pain, redness, and warmth in the extremities. We present two cases of primary erythromelalgia both of whom presented with a history of several months of severe burning pain in both hands and feet. Both patients had received multiple pain medications with no improvement in symptoms. Pain was relieved by putting affected parts in ice cold water, which resulted in immersion injury of the affected parts. Both patients stopped taking part in school and social activities. We tried oral mexiletine, a class Ib antiarrythmic agent, in view of its reported role in various chronic painful conditions. Dramatic improvement was observed with its use. Both patients improved after several weeks of use, and there were fewer soaking episodes. We observed no adverse effects with mexilitine therapy

Nosocomial sepsis-induced late onset thrombocytopenia in a neonatal tertiary care unit

BACKGROUND AND OBJECTIVES: Late onset sepsis (LOS)(onset of sepsis >72 hours of age or nosocomial sepsis) is an important cause of morbidity and mortality in the neonatal intensive care unit (NICU). Thrombocytopenia is an important complication of sepsis. We investigated the incidence of thrombocytopenia in LOS patients and studied the influence of various parameters on platelet response. PATIENTS AND METHODS: Infants born in the level 3 neonatal intensive care unit between January 2002 and December 2006 with documented LOS were included in this prospective study. Multiple hemograms with platelet counts, bacterial blood culture and fungal blood culture were obtained in all patients. Demographic and clinical data were compared between patients without thrombocytopenia and with mild, moderate and severe thrombocytopenia. Duration of thrombocytopenia in relation to type of organism and mortality with respect to degree of thrombocytopenia were also studied. RESULTS: Of 200 patients with culture-proven nosocomial sepsis, 119 (59.5%) patients developed thrombocytopenia (platelet count <150*109/L). In our series Klebsiella pneumoniae was the most frequently isolated organism (125/200, 62.5%) and the incidence of thrombocytopenia was 60.0% (75/125). However, the incidence of thrombocytopenia was highest among patients who had concurrent bacterial and fungal sepsis (28/31, 90.3%). Coagulase-negative staphylococcal (CoNS) sepsis was present in 21 (10.5%) patients and the incidence of thrombocytopenia was 33.3%. Isolated fungal sepsis was present only in 6 (3%) patients and the incidence of thrombocytopenia was 66.0%. The incidence of thrombocytopenia was highest among preterm babies and low-birth weight (LBW) babies. Twenty-seven percent (54/200) of babies presented with mild thrombocytopenia, 20% (40/200) presented with moderate thrombocytopenia, and 12.5%(25/200) developed severe thrombocytopenia. Severity of thrombocytopenia was also directly related to the presence of necrotizing enterocolitis (NEC) and disseminated intravascular coagulation (DIC). The mortality rate was significantly associated with the degree of thrombocytopenia. CONCLUSION: LOS sepsis is an important risk factor for thrombocytopenia in the NICU. Fungal and gram-negative sepsis are frequently associated with a decreased platelet count. Sepsis-induced thrombocytopenia is more common among LBW babies and preterm babies. The mortality rate is significantly related to degree of thrombocytopenia

Dapsone as a cheap and safe second-line drug for chronic immune thrombocytopenia in developing countries: A prospective cohort study

Objective: The aim was to evaluate the efficacy and safety profile of dapsone as a cheap second-line treatment for chronic immune thrombocytopenia (ITP) in developing countries. Materials and Methods: A prospective study on 100 chronic ITP patients. These patients were put on dapsone after ruling out glucose 6 phosphate dehydrogenase deficiency and secondary causes of ITP. Results: The basic work up for secondary causes of ITP was negative. All these patients had been treated with steroids in the past. Anti-D had been given acutely in 20 patients, and intravenous immunoglobulin G had been given in 10 patients. Vincristine had been given to 20 of these patients. Dapsone was started in these patients, and 44% patients showed a response to treatment. The mean time to onset of response was 21 days. Out of these 44 patients, 21 (47.7%) went into remission and had platelet count >100,000/μl at 2 years post tapering of the treatment. Remaining 23 patients were kept on low dose dapsone and maintained their platelet counts. Adverse drug reactions included mild skin eruptions in 5% of patients, pruritus in 10% of patients, dose-related hemolysis in 1% of patients, methemoglobinemia in 1% of patients and Stevens Johnson syndrome in 1% of patients. 56 patients were nonresponders to the available, affordable conventional medical treatment and were referred to the surgical department for splenectomy, with a cure rate of 86% postsurgery. Conclusions: Dapsone is a safe, cheap and effective treatment option for patients with chronic ITP, who cannot afford the usual costlier second-line drugs

Vitamin D status correlates with the markers of cystic fibrosis-related pulmonary disease

Background: The prevalence of Vitamin D deficiency remains high in cystic fibrosis despite daily supplementation. Vitamin D as an immunomodulator has been related to lower respiratory tract infections in children. The present study was undertaken to examine the association between vitamin D status and markers of cystic fibrosis-related pulmonary disease including exacerbations, bacterial colonization and pulmonary function. Methods: The study includes review of records of 51 cystic fibrosis patients. Baseline patient variables and serum vitamin D levels were recorded. Based on vitamin D levels study patients were divided into three groups: vitamin-D sufficient (≥20 ng/mL), vitamin-D insufficient (12 to 20 ng/mL), and vitamin D-deficient (≤12 ng/ml). Results: The proportion of children with deficient, insufficient and sufficient vitamin D levels were 47.1%, 15.7%, and 37.2%, respectively. Female sex, bacterial colonization and a greater number of exacerbations were associated with highest odds of developing vitamin D deficiency in patients with CF with 1.77 (0.22–4.61) (p = 0.002), 2.9(0.57−14.82) (p = 0.011), and 5.12 (1.28−20.50) (p = 0.021) respectively. The comparison of vitamin-D levels taken during exacerbations, colonization and during routine follow-up were significant [16.04 (7.42−27.91), 24.3 (15.5−32.4) and 48.54 (18.37−78.7) ng/ml, p < 0.001]. The FEV1 was determined in 24 patients; the comparison was significant between vitamin D-deficient and -sufficient groups [0.75 (0.717−0.777) vs. 0.82 (0.74−0.92) p < 0.05]. Conclusion: We concluded that vitamin D deficiency was highly prevalent in children with CF, despite daily supplementation of the vitamin in diet. Further, vitamin D deficiency was associated with a higher rate of pulmonary exacerbations and higher incidence of pulmonary bacterial colonization. In addition, in younger patients, low vitamin D levels were associated with reduced pulmonary function. Key Words: bacterial colonization, cystic fibrosis, lung function, pulmonary exacerbation, vitamin D deficienc

Shoshin beriberi-thiamine responsive pulmonary hypertension in exclusively breastfed infants: A study from northern India

AbstractObjectiveTo study the effect of thiamine administration on the resolution of pulmonary hypertension in exclusively breastfed infants.DesignProspective cohort study.SettingHospital based study of a tertiary care hospital.PatientsA total of 29 infants with 17 males (58.6%) and 12 females (41.4%) were included in the study.InterventionIn addition to the management of shock, right heart failure and renal failure, patients received intravenous thiamine 100mg/kg IV followed by 10mg/day till introduction of supplementary feeds.Main outcomes measuresResolution of shock, metabolic complications and pulmonary hypertension.ResultsMean age at presentation was 78.45±30.7 days. All infants were exclusively breastfed. 86.2% of mothers were on customary dietary restrictions. Biventricular failure and tachycardia was commonly present. There were four deaths in our series. Acute metabolic acidosis was a universal feature with a mean pH of 7.21±0.15. Pulmonary hypertension was present in all patients on admission. Intravenous thiamine 100mg/kg IV stat was given immediately after documenting pulmonary hypertension. Repeat echocardiography showed complete resolution of pulmonary hypertension.ConclusionMany infants present to us with Shoshin beriberi with unusually high pulmonary pressures. These patients respond to thiamine challenge with prompt resolution of metabolic complications and reversal of pulmonary hypertension. We believe this is first of its kind from the region, which is reported