Predictors of quality of life among adolescents with epilepsy in the state of Andhra Pradesh.

OBJECTIVES: Assessment of quality of life (QOL) reveals the impact of diseases and factors responsible for the impairment of quality of life. The purpose of this study was to evaluate the QOL among adolescents with epilepsy (AWE) in the state of Andhra Pradesh. MATERIALS AND METHODS: One hundred and fifty AWE aged 13-19 years were evaluated for QOL using the Telugu version of the Quality of Life in Epilepsy Inventory for Adolescents (QOLIE) AD-48 and the data were analyzed to predict the factors responsible for determining the QOL. RESULTS: The mean age of AWE was 15.86 ± 2.14 years. The age at onset of seizures among AWE was 9.28 ± 4.90 years. Generalized (45%) and partial seizures (34%) were the predominant types of seizures. The majority of AWE (77%) were taking anti epileptic medication for 1-8 years, were on monotherapy (55%), and were seizure free for the last 1 year (56%). The mean total QOL score in AWE was 72 ± 15. The high school-educated, seizure-free, and monotherapy-taking AWEs showed a significantly higher mean total QOL when compared to the primary school- educated, seizure-frequent, and polytherapy-taking AWEs (P < 0.01). Education (standardized beta [Sß] = 0.163 P < 0.05), seizure frequency (Sß-0.603; P < 0.01), and poly therapy (Sß-0.08; P < 0.01) were significant predictors of QOL in AWE. CONCLUSIONS: The results of the study suggest that in addition to seizure control, encouraging monotherapy and enhancing the education level may improve the QOL in AWE.

Evaluation of activity of hypothalamo-pituitary-gonadal axis in postmenopausal women suffering from severe acute illness.

BACKGROUND & OBJECTIVES: Postmenopausal women constitute an ideal model for studying the extent of hypothalamo-pituitary gonadal (HPG) axis suppression in critical illness as the gonadotropins are normally high and non-cyclical in them. The objective was to assess the impact of acute severe illness in postmenopausal women on the HPG axis and the activities of the hypothalamo-pituitary-adrenal (HPA), the hypothalamo- pituitary-thyroid (HPT) axes; and levels of serum prolactin, by comparison between critically ill postmenopausal women and otherwise healthy postmenopausal women. METHODS: Thirty five consecutive postmenopausal women older than 60 yr admitted to medical intensive care with a s0 implified a0 cute p0 hysiology s0 core II (SAPS II) more than 30 were included. On day five of their in-hospital stay, blood samples were collected for oestradiol, luteinizing hormone (LH), follicle stimulating hormone (FSH), cortisol, androstenedione, prolactin and thyroid profile. Thirty five apparently healthy postmenopausal women were selected as controls. RESULTS: Levels of LH, FSH, thyrotropin, free thyroxin (fT 4 ) and free tri-iodothyronine (fT 3 ) were lower while oestradiol, cortisol and dehydroepiandrosterone were higher among patients in comparison to healthy controls. Prolactin levels were similar in patients and controls. Among sick patients both FSH and fT 4 showed a negative correlation ( P<0.05) with the SAPS II score. INTERPRETATION & CONCLUSIONS: In critically ill postmenopausal women, paradoxically elevated oestrogen levels despite gonadotropin suppression suggests a non-ovarian origin. Prolactin remained unaltered in patients despite their illness, possibly reflecting atrophy of lactotrophs in menopause.

Consensus & Evidence-based INOSA Guidelines 2014 (First edition).

Obstructive sleep apnoea (OSA) and obstructive sleep apnoea syndrome (OSAS) are subsets of sleep-disordered breathing. Awareness about OSA and its consequences amongst the general public as well as the majority of primary care physcians across India is poor. This necessiated the development of the INdian initiative on Obstructive Sleep Apnoea (INOSA) guidelines under the auspices of Department of Health Research, Ministry of Health & Family Welfare, Government of India. OSA is the occurrence of an average five or more episodes of obstructive respiratory events per hour of sleep with either sleep related symptoms or comorbidities or ≥ 15 such episodes without any sleep related symptoms or comorbidities. OSAS is defined as OSA associated with daytime symptoms, most often excessive sleepiness. Patients undergoing routine health check-up with snoring, daytime sleepiness, obesity, hypertension, motor vehicular accidents and high risk cases should undergo a comprehensive sleep evaluation. Medical examiners evaluating drivers, air pilots, railway drivers and heavy machinery workers should be educated about OSA and should comprehensively evaluate applicants for OSA. Those suspected to have OSA on comprehensive sleep evaluation should be referred for a sleep study. Supervised overnight polysomnography (PSG) is the "gold standard" for evaluation of OSA. Positive airway pressure (PAP) therapy is the mainstay of treatment of OSA. Oral appliances are indicated for use in patients with mild to moderate OSA who prefer oral appliances to PAP, or who do not respond to PAP or who fail treatment attempts with PAP or behavioural measures. Surgical treatment is recommended in patients who have failed or are intolerant to PAP therapy.

Consensus & evidence-based INOSA Guidelines 2014 (first edition).

Obstructive sleep apnoea (OSA) and obstructive sleep apnoea syndrome (OSAS) are subsets of sleep-disordered breathing. Awareness about OSA and its consequences amongst the general public as well as the majority of primary care physcians across India is poor. This necessiated the development of the INdian initiative on Obstructive sleep apnoea (INOSA) guidelines under the auspices of Department of Health Research, Ministry of Health & Family Welfare, Government of India. OSA is the occurrence of an average five or more episodes of obstructive respiratory events per hour of sleep with either sleep related symptoms or co-morbidities or ≥ 15 such episodes without any sleep related symptoms or co-morbidities. OSAS is defined as OSA associated with daytime symptoms, most often excessive sleepiness. Patients undergoing routine health check-up with snoring, daytime sleepiness, obesity, hypertension, motor vehicular accidents and high risk cases should undergo a comprehensive sleep evaluation. Medical examiners evaluating drivers, air pilots, railway drivers and heavy machinery workers should be educated about OSA and should comprehensively evaluate applicants for OSA. Those suspected to have OSA on comprehensive sleep evaluation should be referred for a sleep study. Supervised overnight polysomnography (PSG) is the "gold standard" for evaluation of OSA. Positive airway pressure (PAP) therapy is the mainstay of treatment of OSA. Oral appliances are indicated for use in patients with mild to moderate OSA who prefer oral appliances to PAP, or who do not respond to PAP or who fail treatment attempts with PAP or behavioural measures. Surgical treatment is recommended in patients who have failed or are intolerant to PAP therapy.

Management of Guillain-Barré syndrome with plasmapheresis or immunoglobulin: our experience from a tertiary care institute in South India.

Guillain-Barré syndrome (GBS), an acute inflammatory demyelinating polyneuropathy is the most common generalized paralytic disorder. The objective was to study the outcome of disability grade in two groups of GBS treated with plasmapheresis alone and treated with IVIg alone. A retrospective analysis of all consecutive patients with GBS, admitted in our intensive care unit during the period of 3 years, 2009-2012 were included in the study. All patients of GBS who were to be treated with plasmapheresis or IVIg, the modality of management were always decided at their preference and consent after explaining the modalities to patient/family. The plasma exchange done was ∼200-250 mL of plasma per kilogram weight in five sessions (40-50 mL/kg per session) within 7-14 days. The replacement fluid contained 100 mL of 20% albumin diluted in 1000 mL of normal saline and 1000 mL of fresh frozen plasma. IVIg was administered as 0.4 g/kg body weight daily for 5 days. Our observations brought out the following, both the plasmapheresis and IVIg treatments were effective in reducing the disability grade amongst all time points, i.e., at presentation, immediate post-therapy and after 4 weeks. There was a marginal superiority in plasmapheresis over IVIg effect. However, whether the delay in presentation as noted in our study probably would have contributed to this effect was conjectural.

COVID-19 disease in peritoneal dialysis patients: a single centre experience from India.

BACKGROUND: This study presents our data on mortality in end stage renal disease (ESRD) patients on peritoneal dialysis (PD) who developed COVID-19. MATERIALS AND METHODS: Sri Padmavathi Medical College Hospital, Sri Venkateswara Institute of Medical Sciences University, was designated the State COVID Hospital in March 2020. In a retrospective observational study, we collected the data of ESRD patients on PD and identified the risk factors for mortality. RESULTS: Prior to the pandemic, 136 patients with ESRD were on peritoneal dialysis at our Institute. Among them, 27 (19.8%) eventually developed COVID-19, and 14 of them (51.8%) died. Serum albumin levels were lower and D-dimer levels were significantly higher in deceased patients than in survivors. DISCUSSION: The mortality rate in ESRD patients on PD with COVID-19 at our institution was higher than in other published studies.

Novel mutations in EPO-R and oxygen-dependent degradation (ODD) domain of EPAS1 genes-a causative reason for Congenital Erythrocytosis.

Congenital Erythrocytosis (CE) can be primary or secondary due to the mutations in genes involved in the erythropoietin receptor and oxygen sensing pathway. In this study, 42 patients with 38 unrelated patients and one family (4 patients) who were JAK-2 mutation (both exon 12 and exon 14) negative with high haematocrit values were investigated. The Endogenous Erythroid colony (EEC) assay was performed in all patients, interestingly EEC colonies were high in EPAS1 and EPOR mutated patients compared to non-mutated patients. The sequence analysis of EPAS1 (exon 12), EPO-R (exon-8), VHL (exon-3), and EGLN1 (exon-1) genes in all these patients showed 19% of patients (8/42) had mutations, in exon12 of EPAS1 and exon 8 of EPO-R genes. Two novel missense mutations MW_600850:c.1183G>C, MW_600851:c.1028A>C in EPO-R gene were observed in the study group. One new MW_600849:c.1969C>T nonsense mutation and five MW_619914:c.1715A>G, MW_619915:c.1694G>T, MW_619916:c.1634T>C, MW_600852:c.1771C>G, MW_600848:c.1859G>A novel missense mutations were observed in the EPAS1 gene. Among them, 4 mutations p. (Gln572Arg), p. (Ser565Ile), p. (Ile545Thr), p. (Gln591Glu) in the ODD (Oxygen-dependent degradation) domain of HIF2α, all these variations contributed to the formation of non-functional HIF2α. No mutations were observed in VHL and EGLN1 genes. Using in silico analysis we observed that these mutations contributed to major conformational changes in the HIF2α protein making it non-functional. The mutations in the EPAS1 gene were heterozygous and show autosomal dominant inheritance patterns and we observed in one family. These novel mutations in the EPAS1 (75% (6/8)) and 25% (2/8) EPO-R genes correlating with EEC positivity were observed for the first time in India in CE patients.

A randomised controlled trial of high-flow nasal oxygen versus non-rebreathing oxygen face mask therapy in acute hypoxaemic respiratory failure.

Background and Aims: A major limitation to providing oxygen therapy by high flow nasal oxygen (HFNO) delivery devices is its availability and therefore as an alternative many clinicians use a standard non rebreathing face mask (NRBM) in order to oxygenate their patients where low-flow nasal oxygen or simple facemask oxygen is not providing adequate respiratory support to achieve the target peripheral oxygen saturation (SpO2). We aimed to determine the clinical effectiveness of HFNO versus NRBM in terms of improving patient outcome among patients admitted to our intensive care unit (ICU) during coronavirus disease-2019 (COVID-19) outbreak. Methods: In this prospective open labelled study, 122 COVID-19 patients presenting with acute hypoxaemic respiratory failure (AHRF) were randomised to receive either HFNO or NRBM to achieve the target SpO2. The primary clinical outcome measured was device failure rate and secondary outcome was all-cause 28-day mortality rate. Results: The device failure rate was significantly higher in HFNO group (39% versus 21%, P = 0.030). Oxygen support with NRBM resulted in a reduced all mortality rate over HFNO (26.2% versus 45%) but the mortality rate after treatment failure in either group (HFNO or NRBM) remained high (91% versus 92%). Conclusion: Oxygen support with NRBM results in both reduced device failure rate and higher survival among patients of COVID-19 with AHRF.

Myasthenic crisis-like syndrome due to Cleistanthus collinus poisoning.

Poisoning with Cleistanthus collinus frequently causes cardiac manifestations such as rhythm disturbances and also results in other manifestations such as metabolic acidosis and hypokalemia. We present the case of a patient who presented with a rare myasthenic crisis-like syndrome requiring assisted ventilation due to Cleistanthus collinus poisoning, which responded to treatment with neostigmine.

A clinical and electrophysiological study of peripheral neuropathies in peritoneal dialysis patients: Our experience from rural South India.

The objective was to study the prevalence, clinical features, electrophysiological features, and severity of peripheral neuropathy in chronic kidney disease (CKD) patients on peritoneal dialysis (PD) and effect of the presence of diabetes mellitus (DM). Between May 2015 and December 2016, 100 CKD patients on PD were assessed. The prevalence of peripheral neuropathy was 65% based on clinical symptoms and 92% based on electrophysiological parameters. The mean age was 55.7 ± 10.9 years. About 64% were male. Twelve patients (12%) had motor weakness, 64 patients (64%) had positive symptoms and 60 patients (60%) had negative symptoms. Autonomic symptoms were seen in 14 patients (14%). Definite damage was seen in 68 patients (68%), early damage was seen in 16 patients (16%). In PD patients with DM (n = 50), 50 patients (100%) had definite damage. In PD patients without DM (n = 50), 18 patients (36%) had definite damage, 16 patients (32%) had early damage. In CKD patients on PD, patients aged >50 years (definite damage in 75.7%) showed more severe peripheral neuropathy when compared to patients aged ≤50 years (definite damage in 53%). Most common nerves involved in the present study were median motor nerve, sural nerve, ulnar sensory nerve, common peroneal nerve, posterior tibial nerve followed by the median sensory nerve. Peripheral neuropathy is common in CKD patients on PD, with higher prevalence and severity in elderly females and diabetics. Rationale management of diabetes in CKD patients on PD probably lowers the prevalence and severity of peripheral neuropathy.

Nerve and vein preserving neck dissections for oral cancers: a prospective evaluation of spinal accessory nerve function and internal jugular vein patency following treatment.

Nerve and vein preserving modification of the radical neck dissection is commonly used in the management of oral squamous cell cancers. There is limited literature addressing nerve function and vein patency following treatment. We prospectively analysed 65 patients with nerve conduction study using surface electromyography at baseline, 1 month and 6 months post-surgery and colour Doppler of the internal jugular vein at baseline and 1 month post-surgery. We also studied functional outcomes of nerve sparing with arm abduction test and Neck Dissection Quality of Life questionnaire. There was a statistically significant increase in mean latency of motor action potential and decrease in the mean amplitude of the motor action potential following surgery, suggesting nerve dysfunction. Following surgery, there was a significant decrease in the diameter of the vein as well as an increase in the velocity of blood flow; there was partial thrombus in 5% of individuals. In conclusion, even though nerve dysfunction compromised shoulder abduction, vein dysfunction rarely resulted in any significant clinical impact.

Quality of Sleep and Sleep Disorders in Patients with Parkinsonism: A Polysomnography Based Study from Rural South India.

OBJECTIVE: The objective of this study is to study the quality of sleep, sleep disorders, and polysomnographic profile in Parkinsonism patients from rural areas and to correlate polysomnographic profile with the staging of disease and with sleep questionnaire. MATERIALS AND METHODS: Between May 2014 and December 2015, 168 Parkinsonism patients were prospectively screened using sleep questionnaire; Epworth Sleepiness Scale (ESS), Pittsburgh Sleep Quality Index (PSQI), Parkinson Disease Sleep Score-2 (PDSS-2). Sixty patients underwent overnight polysomnography subsequently. RESULTS: The mean age of 168 patients in the study was 65.3 ± 12.8 years. The mean duration of Parkinsonism was 4.6 ± 3.1 years. The mean ESS, PSQI and PDSS-2 were 12.4 ± 3.2, 7.9 ± 2.1 and 44.7 ± 5.8, respectively. A total of 148 patients (88.1%) had poor quality sleep, which was reported only in 37 patients (22%). Excessive daytime sleepiness (80%) and insomnia (76.7%) were most common symptoms. Polysomnographic profile showed poor sleep efficiency (median interquartile range [IQR] 74.8% [17.8%-99.5%]), reduced slow wave sleep (median [IQR] 0% [0%-9.5%]), and reduced rapid eye movement [REM] sleep (median (IQR) 4.9% [0.1%-24.2%]). Sleep disorders in the study were sleep fragmentation (n = 60, 100%), obstructive sleep apnea syndrome (n = 40, 66.7%), central sleep apnea syndrome (n = 6, 10%), and periodic limb movement disorder (n = 52, 86.7%). Two patients had REM sleep behavioral disorder clinically. There was statistically significant positive correlation between staging of disease, sleep latencies, and sleep questionnaire. CONCLUSION: Sleep is impaired in majority of Parkinsonism patients which needs to be diagnosed early and managed effectively. Patient education and awareness programs in rural areas regarding sleep disorders in Parkinsonism are required for early diagnosis.

Stigma and Polytherapy: Predictors of Quality of Life in Patients with Epilepsy from South India.

BACKGROUND: Apart from unpredictable seizures and consequent injuries, people with epilepsy (PWE) confront with psychosocial adjustments. Quality of life (QOL) varies with culture and socioeconomic milieu. Identification of predictors for QOL enables comprehensive and effective care. OBJECTIVE: The objective of this study was to investigate the role of stigma, demographic, socioeconomic, and clinical factors in QOL among PWE. SUBJECTS AND METHODS: In this prospective observational study, 170 PWE answered QOL in epilepsy-31 (QOLIE-31) and stigma questionnaire. Internal consistency of instruments was evaluated by Cronbach's alpha and reproducibility by intracorrelation coefficient (ICC). Descriptive statistics were calculated, and predictors were identified by regression analysis. RESULTS: Mean age of the PWE was 34.39 ± 11.49. Cronbach's alpha and ICC of the QOLIE-31 were 0.946 and 0.974 and stigma scale was 0.903 and 0.954, respectively. Mean total QOL was 60.29 ± 14.12. Highest and lowest scores of subscales of QOL were observed in medication effects and social functioning. Mean stigma score of PWE was 22.21 ± 14.64. Majority of PWE had mild stigma (75%) followed by moderate (22%) and high stigma (1%). Stigma score correlated with total and subscales of QOL (P < 0.01). Statistically significant decrease in scores of total and subscales of QOL was observed in high and moderate stigma when compared to mild stigma (P < 0.01). Stigma (standardized beta coefficient = -0.652, P < 0.00) and polytherapy (standardized beta coefficient = -0.180, P < 0.02) were found to be the significant predictors of QOL. Significant decrease in total and subscale scores of QOL was observed in PWE under polytherapy when compared to monotherapy and also in seizure frequent against seizure-free PWE (P < 0.01). CONCLUSIONS: Besides control of seizures, encouragement of monotherapy and destigmatization campaigns may improve the QOL of PWE.

A Clinical and Electrophysiological Study of Peripheral Neuropathies in Predialysis Chronic Kidney Disease Patients and Relation of Severity of Peripheral Neuropathy with Degree of Renal Failure.

OBJECTIVE: To study the prevalence, clinical features, electrophysiological features, and severity of peripheral neuropathy in predialysis chronic kidney disease (CKD) patients with respect to severity of renal failure and presence of diabetes mellitus. MATERIALS AND METHODS: Between May 2015 and December 2016, 200 predialysis CKD patients were assessed prospectively. RESULTS: The prevalence of peripheral neuropathy in predialysis CKD patients in the present study was 45% based on clinical symptoms and 90% electrophysiologically. Mean age of 200 predialysis CKD patients who participated in the study was 53.2 ± 13.2 years. One hundred and thirty-six (68%) patients were male and 64 (32%) patients were female. Mean duration of disease was 2.2 ± 1.6 years. Nearly 45% patients of patients had asymptomatic peripheral neuropathy in the present study, which was more common in mild-to-moderate renal failure group. One hundred twenty-six patients (63%) had definite damage and 54 patients (27%) had early damage. In mild-to-moderate renal failure (n = 100) and severe renal failure patients (n = 100), 88% and 92% had significant peripheral neuropathy, respectively. Most common nerves involved were sural nerve, median sensory nerve, and ulnar sensory nerve. Diabetic patients (97%) showed more severe and high prevalence of peripheral neuropathy when compared to nondiabetic patients (83%). Most common patterns were pure axonal sensorimotor neuropathy and mixed sensorimotor neuropathy. CONCLUSION: Peripheral neuropathy is common in predialysis patients, prevalence and severity of which increases as renal failure worsens. Predialysis patients with diabetes show higher prevalence and severity of peripheral neuropathy when compared with nondiabetics.

Consensus and evidence-based Indian initiative on obstructive sleep apnea guidelines 2014 (first edition).

Obstructive sleep apnea (OSA) and obstructive sleep apnea syndrome (OSAS) are subsets of sleep-disordered breathing. Awareness about OSA and its consequences among the general public as well as the majority of primary care physicians across India is poor. This necessitated the development of the Indian initiative on obstructive sleep apnea (INOSA) guidelines under the auspices of Department of Health Research, Ministry of Health and Family Welfare, Government of India. OSA is the occurrence of an average five or more episodes of obstructive respiratory events per hour of sleep with either sleep-related symptoms or co-morbidities or ≥15 such episodes without any sleep-related symptoms or co-morbidities. OSAS is defined as OSA associated with daytime symptoms, most often excessive sleepiness. Patients undergoing routine health check-up with snoring, daytime sleepiness, obesity, hypertension, motor vehicular accidents, and high-risk cases should undergo a comprehensive sleep evaluation. Medical examiners evaluating drivers, air pilots, railway drivers, and heavy machinery workers should be educated about OSA and should comprehensively evaluate applicants for OSA. Those suspected to have OSA on comprehensive sleep evaluation should be referred for a sleep study. Supervised overnight polysomnography is the "gold standard" for evaluation of OSA. Positive airway pressure (PAP) therapy is the mainstay of treatment of OSA. Oral appliances (OA) are indicated for use in patients with mild to moderate OSA who prefer OA to PAP, or who do not respond to PAP or who fail treatment attempts with PAP or behavioral measures. Surgical treatment is recommended in patients who have failed or are intolerant to PAP therapy.

Epilepsy care in six Indian cities: a multicenter study on management and service.

BACKGROUND: Epilepsy care in developing countries is lagging behind than in the developed countries. Precise data on delivery of neurological services for epilepsy is essential to optimize the medical services for epilepsy care with limited resources. OBJECTIVE: This study was carried out in order to examine the management practices and utilization of various medical services for epilepsy in different parts of India. METHODOLOGY: University centers with epilepsy clinics, one each from six states of India, had participated in this study. Demographic data, clinical details, and data on epilepsy care were collected simultaneously on standard proforma. RESULTS: Data on 285 patients with epilepsy (generalized epilepsy: 49.1%, localization-related epilepsy: 49.9%, others: 1%) were included. Mean age of onset of epilepsy was 14.8+11.1 years. Mean delay in diagnosis was 1.5+/-4 years. Mean distance from place of residence to the consulting neurologist was 70+/-82 km. Medical consultations before referral to epilepsy center included general practitioners (54.1%) and specialists (43.3%). Very few patients received services from clinical psychologist or social worker. Investigations included, EEG (63.2%), CT Scan (36.2%). MRI brain (8.5%) and video EEG (2.1%) were limited to a few. Nearly 75.5% were on monotherapy. Newer Anti-Epileptic Drugs (AEDs) were used only in less than 5% patients. CONCLUSION: The services for epilepsy are urban-based and there is underutilization of services, general practitioners and specialists. Newer AEDs (although expensive) are gradually emerging in Indian market. Facilities for epilepsy surgery, therapeutic drug monitoring and services of clinical psychologist or medical social workers are limited.

Clinical and electrophysiological study of intermediate syndrome in patients with organophosphorous poisoning.

Intermediate syndrome (IS) developed in 38 of 214 cases with organophosphorous compound poisoning (OPCP). Neck muscle weakness, motor cranial nerve palsy, respiratory muscle paralysis, proximal limb weakness were the chief neurological signs developed 16-120 hours after consumption of the insecticide. Two patients had pyramidal tract signs. Mean duration of IS was 9.26 (+/- 4.84) days. Electrophysiological study (EPS) was done in 21 patients. 18 patients showed decremental response to repetitive stimulation at 3Hz 5 pulses and absence of post tetanic facilitation. Motor conduction studies were abnormal in on (prolonged distal latency and reduced conduction velocity), 'F' responses were abnormal in, sensory nerve conduction was abnormal in two, and simple repetitive response were observed in 11 patients. 4 patients died. In IS neuromuscular junctional dysfunction is the predominant factor.