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Background: The study explored the differences in audiological and non-audiological characteristics between congenital, early-onset, and late-onset auditory neuropathy spectrum disorder (ANSD). Ninety-five individuals diagnosed with ANSD were included in the study. They were divided into three groups congenital ANSD—children (30 individuals, 60 ears), adults with early-onset ANSD (30 individuals, 56 ears), and adults with late-onset ANSD (35 individuals, 62 ears). The non-audiological characteristics (gender, laterality, and risk factors) and audiological characteristics (behavioral and electrophysiological measures) were compared between the three groups. Results: Discriminant analyses showed that the pure tone average, audiogram configuration, and speech thresholds were the best auditory predictors of onset-based group differences in ANSD (congenital and early-onset versus late-onset ANSD). While the congenital and early-onset group showed poorer pure-tone and speech thresholds, along with flat configuration, the late-onset group demonstrated relatively better thresholds and other configurations (rising, tent-shaped, cookie-bite). In addition, long latency responses were delayed or absent in children with congenital ANSD, indicative of onset coding deficits at the cortical level. Conclusions: The study highlights the audiological differences between congenital, early-, and late-onset ANSD groups. These differences could be because of variations in etiology, pathophysiology, site of lesion, or genetic variability between the groups, which needs to be explored further. [ABSTRACT FROM AUTHOR]

Purpose: Sensory gating is a phenomenon where the cortical response to the second stimulus in a pair of identical stimuli is inhibited. It is most often assessed in a conditioning--testing paradigm. Both active and passive neuronal mechanisms have been implicated in sensory gating. The present study aimed to assess if sensory gating is caused by an active neural mechanism associated with stimulus redundancy. Method: The study was carried out on 20 young neurotypical adults. We assessed the gating phenomenon using identical and nonidentical stimuli pairs presented in an electrophysiological conditioning--testing paradigm. We hypothesized that the novel stimulus in the nonidentical stimulus pair would not exhibit the sensory gating effects (reduction in the amplitude of cortical potentials to the second stimuli in the pair), owing to stimulus novelty. Results: Contrary to our expectations, the response analyses of the cortical auditory evoked potentials revealed that adults gated repetitive and novel stimuli similarly. Conclusions: The findings are discussed in relation to the significance of methodological factors in evaluating sensory gating. We believe that additional research using oddball presentation of novel stimuli along with appropriate analysis methods is necessary before drawing any conclusions on the mechanisms underlying sensory gating. [ABSTRACT FROM AUTHOR]

Background: Cochlear implant surgeries are performed by different surgical routes, Round window (RW), Extended round window (Ext RW) and Cochleostomy (C). Optimum intracochlear electrode placement is important to achieve a successful outcome. Intra-operative electrophysiological testing (Impedance and ECAP) is crucial to assess the device function and appropriate electrode placement. The variability of portal of electrode insertion might affect the neural response and its characteristics. Objective: To compare the intraoperative electrophysiological measurements (Impedance and ECAP) for possible differences on auditory nerve stimulation across the three electrode insertion routes. Materials and Methods: This is a retrospective data analysis of 47 cochlear implant recipients at a tertiary care centre in Central India over a period of 4 years. They were broadly divided into two groups depending on the electrode design as full banded and half banded. Intraoperative impedances were recorded for different modes of stimulation and ECAP measured at E5, E10, E15, E20 electrodes in both the groups across the three insertion routes. Results: In the half-banded group, the impedance values in different modes CG, MP1, MP2, MP1 + 2 did not differ significantly among the surgical routes at all four electrodes. While in the full banded group, for CG mode impedance values at E5 differ significantly. Rest for other modes in full banded group, there was no statistically significant difference across the three routes. Conclusion: The present study supports that there is no statistically significant difference in the intraoperative impedance and ECAP measurements in both, the full-banded and half- banded electrodes across the three surgical routes implying that, all the three surgical approaches provide equal stimulation of the auditory nerve. The CI surgeon can select the electrode insertion portal based on the surgical anatomy, the implant type and individual preferences. [ABSTRACT FROM AUTHOR]

Introduction: A lesser studied aspect of Parkinson's disease (PD) is its associated peripheral sensory motor neuropathy. Peripheral neuropathy is an intriguing aspect of PD, a problem not given sufficient attention and which if tackled properly could make a difference to the multifaceted sufferings of the PD patient. Studies regarding the prevalence of peripheral neuropathy and its risk factors in patients with PD are scarce from the Indian subcontinent. Methods: This prospective observational study was conducted in a tertiary care teaching hospital in South India. Patients diagnosed with idiopathic Parkinson's disease (IPD) were screened and enrolled. All the patients underwent detailed evaluation of symptoms, signs, and electrophysiology (Nerve conduction study, Sympathetic skin response), stimulated skin wrinkling with Eutectic Mixture of Local Anesthetics. Patients found to have large/small fiber neuropathy underwent additional tests to exclude other causes of neuropathy. Results: A total of 154 patients with IPD were enrolled in the study (mean age: 61.96 ± 9.15 years, mean duration of disease was 4.08 ± 3.16 years). The mean Hoehn and Yahr (H and Y) score was 2.3 ± 0.825 and the mean Unified Parkinsons Disease Rating Scale (UPDRS) 3 score in the ON state was 23.07 ± 11.14. The mean cumulative levodopa dose was 482.68 ± 651.76 (median: 292; range: 4728.57) grams. Peripheral neuropathy was found in 49 patients (31.8%), large fiber in 28 (18.2%) and small fiber in 47 (30.5%); an overlap of large and small fiber neuropathy was seen in 26 patients (16.9%). Around 34% of patients had serum homocysteine levels >20 mg/dl. In univariate analysis, duration of disease, levodopa cumulative dose, serum homocysteine level, H and Y score, UPDRS 3 ON score, Toronto Clinical Neuropathy Score (P < 0.001 for all), age at presentation, and rigidity predominant presentation (P = 0.02 for both) were associated with large fiber neuropathy. All of these variables were also associated with the presence of small fiber neuropathy (P = 0.004 for age at presentation and P < 0.001 for rest), except the type of PD presentation. However, in multivariate logistic regression analysis, only duration of disease, levodopa cumulative dose, and H and Y score were associated with the presence of large and small fiber neuropathy. Conclusions: In our cohort, majority of the patients were in early stage PD and around one fifth and one third of patients suffer from large and small fiber polyneuropathy, respectively. Large and small fiber neuropathy in PD is mainly associated with duration of disease, levodopa cumulative dose, and H and Y score. [ABSTRACT FROM AUTHOR]

Background : Transesophageal electrophysiological study (TEEPS) is a minimally invasive procedure that helps in evaluating the risk of recurrence of supraventricular tachycardia based on the inducibility of the tachycardia. The purpose of this study was to evaluate the diagnostic capability, negative predictive value of noninducibility of tachycardia and safety of TEEPS among the Indian pediatric population. Methods : It is a retrospective single-center observational study of all patients who underwent TEEPS from January 01, 2014, to December 31, 2020. Results : Twenty-two TEEPS procedures were done in eighteen children with three children undergoing more than one procedure. The median duration of follow-up was 42.97 months (8.52--82.12 months). Out of the 22 procedures, four were conducted for acute tachycardia termination, two for tachycardia suppression assessment, and sixteen to assess tachycardia inducibility off medications. Tachycardia was noninducible in nine children and inducible in six children. Two children had clinical recurrence of tachycardia on follow-up. Out of these two children, one had noninducibility of tachycardia on TEEPS. The negative predictive value of TEEPS was 88.89% in this study. None of the children had any postprocedure complication. Conclusions : TEEPS is a safe procedure. It is used to assess the efficacy of medication in the suppression of arrhythmia. The noninducibility of tachycardia has a high negative predictive value and is a good indicator of nonrecurrence of clinical tachycardia on midterm follow-up. [ABSTRACT FROM AUTHOR]

Background and Objectives: Charcot-Marie-Tooth (CMT) disease is the commonest inherited neuromuscular disorder and has heterogeneous manifestations. Data regarding genetic basis of CMT from India is limited. This study aims to report the variations by using high throughput sequencing in Indian CMT cohort. Methods: Fifty-five probands (M:F 29:26) with suspected inherited neuropathy underwent genetic testing (whole exome: 31, clinical exome: 17 and targeted panel: 7). Their clinical and genetic data were analysed. Results: Age at onset ranged from infancy to 54 years. Clinical features included early-onset neuropathy (n=23), skeletal deformities (n=45), impaired vision (n=8), impaired hearing (n=6), facial palsy (n=8), thickened nerves (n=4), impaired cognition (n=5), seizures (n=5), pyramidal signs (n=7), ataxia (n=8) and vocal cord palsy, slow tongue movements and psychosis in one patient each. Twenty-eight patients had demyelinating electrophysiology. Abnormal visual and auditory evoked potentials were noted in 60.60% and 37.5% respectively. Sixty two variants were identified in 37 genes including variants of uncertain significance (n=34) and novel variants (n=45). Eleven patients had additional variations in genes implicated in CMTs/ other neurological disorders. Ten patients did not have variations in neuropathy associated genes, but had variations in genes implicated in other neurological disorders. In seven patients, no variations were detected. Conclusion: In this single centre cohort study from India, genetic diagnosis could be established in 87% of patients with inherited neuropathy. The identified spectrum of genetic variations adds to the pool of existing data and provides a platform for validation studies in cell culture or animal model systems. [ABSTRACT FROM AUTHOR]

Background: Monomelic amyotrophy (MMA) is a benign, rare, sporadic disorder of adolescent and young adults with male predominance, where neurogenic amyotrophy is restricted to an upper or lower limb. It is a variant of lower motor neuron disorder with insidious onset and slow progression for 2-4 years. Paucity of cohort studies as well as relative unawareness among physicians in eastern India stimulated us to do this work. Material and Methods: Prospective observational study involving 140 cases of MMA from 2012 to 2016, conducted at S.C.B. Medical College, cuttack, Odisha to evaluate clinical profile, electrophysiology and radiological features. All the data were analysed & subjected to statistical analysis through SPSS software version 24. Results: Mean age at onset and presentation were 19.6yrs and 21.7yrs respectively and the average duration 3.2yrs. Upper limb involvement was more common (91.4%) with distal affection (83%) more than proximal (7%). Isolated Leg amyotrophy found in 12 cases (~9%) and 10 cases were having thigh weakness & atrophy. B/L Upper Limb distal involvement was present in 18.5% cases asymmetrically. Family history found in~2.8%. Autonomic symptoms were present in affected and bilateral homologous Limb in 21%, 5.8%(~6%) patients. Changes in electromyography (EMG) were present in affected limb (100%) and clinically unaffected limb (15%). Conclusion: MMA is a benign disease of young males with weakness and atrophy confined to unilateral limb or asymmetrical homologous limb and areflexia without sensory loss. It progresses variably for 2-3 years followed by stabilization without progression to Motor neuron disease. [ABSTRACT FROM AUTHOR]

Objective: To study impact of COVID-19 pandemic on frequency, clinical/electrophysiological profile and treatment outcomes in pediatric Guillain-Barré syndrome (GBS). Background: GBS is the most frequent cause of pediatric acute flaccid paralysis. The effect of the COVID-19 pandemic on pediatric GBS is unclear in the literature. Methods: We conducted an ambispective, multicentric, cohort study involving 12 of 27 centres in GBS Consortium, during two periods: pre-COVID-19 (March-August 2019) and during COVID-19 (March-August 2020). Children <12 years who satisfied National Institute of Neurological Diseases and Stroke criteria for GBS/variants were enrolled. Details pertaining to clinical/laboratory parameters, treatment and outcomes (modified Rankin Scale (mRS) at discharge, GBS Disability score at discharge and 3 months) were analysed. Results: We enrolled 33 children in 2019 and 10 in 2020. Children in 2020 were older (median 10.4 [interquartile range 6.75-11.25] years versus 5 (2.5-8.4) years; P = 0.022) and had more sensory symptoms (50% versus 18.2%; P = 0.043). The 2020 group had relatively favourable mRS at discharge (median 1 (1-3.5) versus 3 (2-4); P = 0.042) and GBS disability score at 3 months (median 0 (0-0.75) versus 2 (0-3); P = 0.009) compared to 2019. Multivariate analysis revealed bowel involvement (P = 0.000) and ventilatory support (P = 0.001) as independent predictors of disability. No child in 2020 had preceding/concurrent SARS-CoV2 infection. Conclusions: The COVID-19 pandemic led to a marked decline in pediatric GBS presenting to hospitals. Antecedent illnesses, clinical and electrophysiological profile of GBS remained largely unchanged from the pre-pandemic era. [ABSTRACT FROM AUTHOR]

Purpose: Auditory training is known to improve the speechperception- in-noise (SPIN) skills in children with auditory processing disorders (APDs); however, conventional non-- computer-based trainings are dreary and nonmotivating. This study intended to develop a computer-based noise desensitization training module in Indian English and test the efficacy of the same in children with APD having SPIN deficits. There are no such modules available at present to be used in Indian children. Method: The module incorporated words-in-noise training, with monosyllables and trisyllables as target words in the presence of speech-shaped noise as well as multitalker babble at different signal-to-noise ratios ranging from +20 to -4 dB SNR. The study included 20 children with SPIN deficits diagnosed with APD and who were recruited randomly to the experimental group who received the training (n = 10; Mage = 11.1 years) and to the control group who did not receive any training (n = 10; Mage = 11.4 years). The outcome measures included behavioral measures and electrophysiological measures (auditory long latency responses for speech in quiet and noise). Results: Compared to children in the control group, children in the experimental group exhibited improvements in the SPIN scores as well as temporal processing measures after training. Dichotic consonant--vowel tests and auditory memory and sequencing tests did not yield significant improvement post training. Also, auditory long latency responses in quiet and noise revealed significant reduction in the amplitudes after training. No significant difference was noted in the latencies of auditory long latency responses post training. Control group participants did not show any significant difference in any of the measures between the initial and follow-up evaluations. Conclusions: The developed training module was successful in fetching improvements in behavioral and electrophysiological measures. It would be a great addition to the evidence-based rehabilitation support inventory for children with SPIN deficits in India. [ABSTRACT FROM AUTHOR]

Background: To study the incidence and time of onset of intensive care unit—acquired weakness in a prospective cohort of children (2–12 years) by serial simplified electrophysiological assessment (Pediatric Critical Illness Myopathy Polyneuropathy study, PEDCIMP). Methods: A single-center, prospective cohort study (Trial Registry Number: NCT02763709; PEDCIMP2016) was conducted at the pediatric intensive care unit of a tertiary care hospital in North India. A complete electrophysiological evaluation (4 motor nerves and 2 sensory nerves) was performed at baseline in children (2–12 years) admitted to the ICU with a pediatric risk of mortality (PRISM) of > 20 with more than 24-h stay. Following the entry evaluation, a minimal alternate day simplified electrophysiological testing of the unilateral common peroneal nerve and the sural nerve was assessed. A 25% reduction in compound muscle action potential (CMAP) and sensory nerve action potential from baseline was considered significant for ICUAW and was confirmed by complete electrophysiological re-evaluation. Results: Of the total 481 children assessed for eligibility, 97 were enrolled. The median age of the cohort was 7 years. Sepsis (81%); need for vasoactive support (43%); multiorgan dysfunction (26%) were the common reasons for admission. Of the 433 eligible patient ICU days, 380 electrophysiological observations were done. A significant decrease of > 25% in CMAP of common peroneal nerve was not detected in any of the 380 observations. However, two children unfit for inclusion were diagnosed with ICUAW during the study period. Conclusions: Children admitted with PRISM > 20 have a very low incidence of intensive care unit—acquired weakness by serial clinical and abbreviated electrophysiological evaluation. [ABSTRACT FROM AUTHOR]

Background: Guillian--Barre' Syndrome (GBS) has been shown to be associated with severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection. The aim of our study was to study the clinical profile and outcomes of GBS in COVID-19 from the Western region of India, the State of Maharashtra. Methods: This was a retrospective, multicenter observation study from different hospitals in Maharashtra beginning from March 2020 until November 2020. Results: We report 42 patients with COVID-19 GBS. Mean age was 59 years (range, 24--85 years). 31/42 (73.8%) were men. GBS was the presenting symptom in 14/42 (33%), while six of them remained asymptomatic for COVID-19 despite positive SARS-CoV-2 on nasopharyngeal swab reverse transcriptase polymerase chain reaction. The median interval between COVID-19 and GBS was 14 days (SD + 11), with minimum of 1 and maximum 40 days. Clinical presentation was like that of typical GBS. Electrophysiological studies showed a predominant demyelinating pattern in 25/42 (59.5%). Inflammatory markers were elevated in 35/42 (83.3%) and 38/42 (90.5%) had an Abnormal high-resolution CT (HRCT) chest. 14/42 (33.3%) patients required a ventilator, with nine deaths. Intravenous immunoglobulin was the mainstay of treatment for GBS. Majority had a good outcome and were walking independently or with minimal support at discharge. In subgroup analysis, the postinfectious group had a better outcome than the parainfectious group. Conclusion: GBS in COVID-19 occurs as both parainfectious and postinfectious GBS. Parainfectious GBS needs more rigorous monitoring and may benefit from COVID-19 specific treatment. Routine screening for SARS-CoV-2 should be implemented in patients with GBS in view of the ongoing pandemic. [ABSTRACT FROM AUTHOR]

Introduction: Rheumatoid Arthritis (RA) is a chronic multisystem immune mediated disease. Rheumatoid associated neuropathy causes significant disability and adds to the economic burden. Aim: To assess clinical determinants of peripheral neuropathy (diagnosed electrophysiologically using nerve conduction studies) among patients with RA. Additionally, it was also aimed to study the various patterns of peripheral neuropathy in patients with RA. Materials and Methods: A cross-sectional study was conducted on 100 consecutive adult patients with RA between 01st February, 2020 to 02nd January, 2021 at medicine and neurology departments of SMS Medical College and Hospital, Jaipur, Rajasthan, India. Inclusion and exclusion criteria were followed and eligible patients after appropriate laboratory evaluation underwent nerve conduction studies. Statistical analysis was performed using student's unpaired t-test and Chi-square test for continuous and categorical variables respectively. Results: Mean age of the study population was 42.4±14.2 years with 88 females and 12 males. Mean duration of RA was 7.0±7.4 years. Nerve conduction studies detected neuropathy in 18 patients, of these only four patients were symptomatic with tingling, pins and needles sensation and numbness. Fourteen patients had subclinical neuropathy. Patients with neuropathy had significantly longer disease duration (p=0.0001), were older (p=0.014) with more joint deformities (p=0.0008). Conclusion: Subclinical neuropathy is not infrequent in RA patients. Those with advanced age, longer disease duration, higher Erythrocyte Sedimentation Rate (ESR), erosions and deformities should be assessed electrophysiologically for neuropathy. [ABSTRACT FROM AUTHOR]

Introduction: Body postural changes are commonly used in the management of patients with acute cardiopulmonary dysfunction. Size, shape and position of heart are related to the body type, posture and respiration. Effect of change in body posture on electrical axis of heart has not been well documented. The present study was conducted to study the effect of posture on electrical axis of heart during different phases of breathing in normal subjects. Aim: To study the effect of posture on electrical axis of heart during different phases of breathing. Materials and Method: With the help of INCO RMS Vesta 101 electrocardiograph, ECG was recorded in the following normal breathing, deep inspiration and deep expiration in supine, standing and sitting posture. Results: The mean cardiac axis after deep expiration in supine posture was +47.29 degrees, in the sitting posture it was +32.67 degrees and in standing posture it was +30.69 degrees. The decrease in cardiac axis upon assuming sitting posture was significantly different from the value obtained during supine posture (P<0.05). The decrease in cardiac axis upon assuming standing posture was significantly different from the value obtained during supine posture (P<0.01). Conclusion: It was found inter-individual variability in electrical axis of heart which can be explained on the basis of orientation of heart in the chest. In the present study, the decrease in cardiac axis due to postural variation from supine to sitting and standing was more pronounced after deep expiration. There was decrease in electrical axis of heart (shift to left) when subject changed the posture from supine to sitting and standing. [ABSTRACT FROM AUTHOR]

Objective: to find out correlation between obesity and CTS and to check whether NCV can be used as a diagnostic tool in asymptomatic cases. Method: A 30 subjects out of 50 were undergone for nerve conduction studies and primary outcome measure is functional status scale symptoms severity scale. Result: results of this study failed to reveal a significant correlation between the BMI and CTS so null hypothesis is retained. results of NCV studies found no significant difference between symptomatic and asymptomatic group, so in that null hypothesis retained. [ABSTRACT FROM AUTHOR]

Context: The amplitude ratio of sural radial sensory nerve action potential is used as a sensitive measure for the diagnosis of an early distal axonal peripheral neuropathy. There is no age-stratified reference data available. Aim: To establish age-stratified sural radial amplitude ratio (SRAR) reference data in healthy Indian subjects. Study Setting and Design: The study was conducted in the electrodiagnostic laboratory of a tertiary city hospital and is an analytical, prospective, and field trial study. Materials and Methods: A prospective study was conducted on 146 healthy volunteers between 18 and 86 years, stratified into 6 groups, a = 18–30 years, b = 31–40 years, c = 41–50 years, d = 51–60 years, e = 61–70 years, and f = >70 years. Sural: Radial amplitude ratio was calculated. Statistical Methods: Stata 12.1 statistical program was used. Lower limit of SRAR was obtained (mean-2SD of transformed data). ANOVA defined the intergroup variability, and linear regression and Pearson's correlation assessed the statistical significance. Results: The lower limit of normal SRAR, for each age group is as follows: a: 0.30, b: 0.23, c: 0.20, d: 0.17, e: 0.17, and f: 0.08. SRAR of groups a, b, c was significantly different from groups e and f. Similarly, SRAR was significantly different between groups d and f but not between groups d and e or a, b, c, d. Conclusion: This study provides age-stratified reference data for SRAR. There is evidence to suggest that SRAR varies with age; hence, a single value of SRAR should not be used when diagnosing a peripheral neuropathy based on this criterion. [ABSTRACT FROM AUTHOR]

Background: Multiple mutations in the voltage-gated sodium channel have been associated with knockdown resistance (kdr) to DDT and pyrethroid insecticides in a major human disease vector Aedes aegypti. One mutation, V1016G, confers sodium channel resistance to pyrethroids, but a different substitution in the same position V1016I alone had no effect. In pyrethroid-resistant Ae. aegypti populations, V1016I is often linked to another mutation, F1534C, which confers sodium channel resistance only to Type I pyrethroids including permethrin (PMT), but not to Type II pyrethroids including deltamethrin (DMT). Mosquitoes carrying both V1016G and F1534C exhibited a greater level of pyrethroid resistance than those carrying F1534C alone. More recently, a new mutation T1520I co-existing with F1534C was detected in India. However, whether V1016I or T1520I enhances pyrethroid resistance of sodium channels carrying F1534C remains unknown. Methodology/Principal findings: V1016I, V1016G, T1520I and F1534C substitutions were introduced alone and in various combinations into AaNav1-1, a sodium channel from Aedes aegypti. The mutant channels were then expressed in Xenopus oocytes and examined for channel properties and sensitivity to pyrethroids using the two-electrode voltage clamping technique. The results showed that V1016I or T1520I alone did not alter the AaNav1-1 sensitivity to PMT or DMT. However, the double mutant T1520I+F1534C was more resistant to PMT than F1534C, but remained sensitive to DMT. In contrast, the double mutant V1016I+F1534C was resistant to DMT and more resistant to PMT than F1534C. Furthermore, V1016I/G and F1534C channels, but not T1520I, were resistant to dichlorodiphenyltrichloroethane (DDT). Cryo-EM structures of sodium channels suggest that T1520I allosterically deforms geometry of the pyrethroid receptor site PyR1 in AaNav1-1. The small deformation does not affect binding of DDT, PMT or DMT, but in combination with F1534C it increases the channel resistance to PMT and DDT. Conclusions/Significance: Our data corroborated the previously proposed sequential selection of kdr mutations in Ae. aegypti. We proposed that mutation F1534C first emerged in response to DDT/pyrethroids providing a platform for subsequent selection of mutations V1016I and T1520I that confer greater and broader spectrum of pyrethroid resistance. [ABSTRACT FROM AUTHOR]

Introduction Dynamic cervical cord compression as the pathogenetic mechanism of Hirayama disease has been recently evaluated on dynamic magnetic resonance imaging of cervical spine. Objectives To study clinical, radiological and electrophysiological profile of patients with Hirayama disease. Materials and Methods This was a prospective study of 17 cases of Hirayama disease carried out at SMS hospital, Jaipur, from June 2016 to October 2017. Results In our study, predominantly male (94.1%) patients were affected and average age of onset was 23.1 years. Unilateral involvement was seen in 71% cases (n = 12). Brisk deep tendon reflexes and dysesthesia, not characterstics of Hirayama disease, were found in 3 patients (18%) and 2 patients (12%) respectively. Long neck was found in our study in 7 patients (41%). On dynamic cervical spine MRI, anterior shifting of posterior cervical dura on flexion, asymmetric cord flattening and loss of cervical lordosis were found in 82% patients. Nerve conduction study was abnormal in 14 patients (82%) and electromyographic evidence of neurogenic affliction was present in all patients in involved segments (C5-T1). Conclusion Hirayama disease, a benign variant of motor neuron disorder can be diagnosed with increasing sensitivity with use of dynamic cervical MRI spine study. [ABSTRACT FROM AUTHOR]

Background: The factors associated with the spontaneous remission (SR) of symptoms in carpal tunnel syndrome (CTS) are not well known. Objectives: To look for determinants of SR in medically treated, electrophysiologically proven patients of CTS. Methods: We revisited the medical records and nerve conduction study data of 130 hands with CTS and divided them into two groups as per the absence or persistence of the symptoms when contacted after a median time lapse of 3 years following the diagnosis. Results: SR occurred in 46.1% of the hands. Higher odds of SR were linked with female gender, symptoms restriction to lateral fingers, symptom duration <10 months, mildly delayed median motor and sensory distal latencies, and median sensory amplitude >20 µV. We developed a seven-point scale, on which a score of ≥4 had a strong association (odds ratio 4.31) with SR. Discussion and Conclusion: No single risk factor, standalone, can predict SR in patients with CTS, which could lead to an invasive treatment (Surgery or local injection) to them. We propose that patients scoring ≥4 on our 7 point scale should be treated medically for the initial 10 months after the symptom onset. [ABSTRACT FROM AUTHOR]

Background: Nerve conduction studies are considered to be the gold standard for diagnosing secondary tarsal tunnel syndrome (sTTS), but their utility in the diagnosis of idiopathic tarsal tunnel syndrome (iTTS) is largely unknown. Objective: We sought to investigate the value of motor nerve conductions studies (MNCS) in the diagnosis of clinically suspected iTTS. Materials and Methods: Twenty-six (52 limbs) adult patients of clinically suspected iTTS were subjected to motor nerve conductions of posterior tibial nerve, and its branches and motor conduction parameters were compared with those of 45 healthy controls. Results: Symptoms were bilateral in 70% (P = 0.02), with heel pain in 95% of symptomatic limbs. MNCS was abnormal in 32 (80%) of symptomatic limbs and 8 (66.6%) of asymptomatic limbs (P = 0.004). Out of electrophysiologically abnormal nerves (n = 67), the pathological process could be identified in all the nerves with abnormal MNCS (P = 0.02). Probable demyelination was seen in 58.2% of the electrophysiologically abnormal nerves. Discussion: The present study shows that iTTS are gender and Body Mass Index neutral with bilateral symptoms being common. Tinel's sign was inconsistent. Heel pain did not correlate with abnormal inferior calcaneal nerve conductions. Motor nerve conduction study was abnormal in a significant number of symptomatic limbs. "Probable demyelination" was more frequent in symptomatic limbs. Conclusion: MNCS is significantly abnormal in symptomatic limbs of subjects with iTTS. Demyelination is slightly more common than axonopathy in iTTS. With a sensitivity of 80% and specificity of 33.3%, MNCS seems to be useful as a screening tool in clinically suspected iTTS. This study is Level II: Lesser quality randomized controlled trial or prospective comparative study. [ABSTRACT FROM AUTHOR]

History helps us to become better students, judge wisely, understand change, and most importantly, it tells us who we are. It helps us to understand what happened, why it happened and what its ramifications are. Winston Churchill once said: "Study history, study history. In history lie all the secrets of statecraft." Here, we take this opportunity to pay our gratitude to our esteemed teachers who worked relentlessly for uplifting of the department of Neurology, PGIMER, Chandigarh; and, narrate chronicles of all those people who made this department reach the heights where it stands today. [ABSTRACT FROM AUTHOR]

Background: Charcot–Marie–Tooth (CMT) disease is clinically and genetically heterogeneous. There are no published series describing clinical, electrophysiological, and genetic information on CMT from the Indian subcontinent. Magnetic resonance imaging (MRI) neurography technique provides useful information about the plexus and roots and can be employed in patients with CMT. Settings and Design: A prospective, observational study carried out at a tertiary care hospital in Western India. Subjects and Methods: CMT patients fulfilling the UK Genetic Testing Network criteria were included. They underwent clinical, electrophysiological, radiological, and multigene panel testing. Results: Totally 22 patients (19 males, 3 females; 18 sporadic and 4 familial cases) were studied. Pes cavus (19), hammer toes (16), and scoliosis was seen in 1 patient. Electrophysiology revealed motor predominant neuropathy with 15 demyelinating (10 uniform and 5 multifocal) and 7 axonal patterns. Thickened lumbosacral plexuses on MRI neurography were evident in 6/10 studied patients, all 6 having demyelinating neuropathy. Genetic analysis identified PMP22, GJB1, SH3TC2, HSPB1, SPTLC2, MPZ, AARS, and NEFH gene mutations. Conclusions: This small series documents the pattern of CMT neuropathies as seen in Western India. Clinico‑electrophysiological and genetic diagnosis showed general concordance some overlaps and reiterated advantages of gene panel testing in this heterogeneous group of neuropathies. MRI neurography was useful as an additional investigation to detect nerve enlargement in patients with demyelinating neuropathies. [ABSTRACT FROM AUTHOR]

Objectives To study demographic, clinical, electrophysiological characteristics, prevalent subtypes and factors associated with outcome of Guillain–Barré syndrome in north-western region of India. Material and methods It was a hospital-based prospective observational study. 60 patients with Guillain–Barré syndrome diagnosed as per Asbury and Cornblath criteria were enrolled and followed up for 3 months. Various epidemiological, clinical and electrophysiological parameters were evaluated. Result Most common variant in our study was acute motor axonal neuropathy (41.6%) while the most common age group was 15–29 years. There was history of antecedent event in 22 patients (37%) and sensory deficit in 31.7% (proprioceptive > exteroceptive). Cranial nerve involvement was seen in 40% (facial > bulbar > ophthalmoplegia) and autonomic dysfunction in 11.7%. Respiratory involvement was seen in 22 patients (36.7%). Nerve conduction abnormalities were observed in 96% of the cases. Sural sparing was found in 15% of cases at admission predominantly in acute inflammatory demyelinating polyneuropathy and F-wave abnormality (prolonged/delayed) in 72% of patients at admission which was most frequently seen in peroneal nerve. Severity at admission (high GBS disability score), need for ventilation, high modified Erasmus GBS outcome score were associated significantly ( p -value <0.05) while age (>55 years) and axonal lesion were non-significantly ( p -value >0.05) with poor outcome. [ABSTRACT FROM AUTHOR]

Carpal tunnel syndrome (CTS) is the most common entrapment neuropathy seen in uremic patients. The study was undertaken to estimate the frequency of CTS in uremic patients and to identify the most sensitive electrodiagnostic test. Study was conducted on 80 subjects of age 30-60 years. End‑stage kidney disease patients were recruited for the clinical evaluation, motor nerve conduction studies (NCS), sensory NCS, F wave study and median‑versus‑ulnar comparison studies (palm‑to‑wrist mixed comparison study, digit 4 sensory latencies study and lumbrical‑interossei comparison study). Among three different diagnostic modalities, frequency of CTS was found to be 17.5% with clinical evaluation, 15% with routine NCS studies and 25% with median‑versus‑ulnar comparison studies. Among the median‑versus‑ulnar comparison studies, lumbrical‑interossei comparison study was found to be most sensitive (90%). The comparative tests for CTS are more sensitive compared to routine NCS and clinical examination. Among the comparative tests, lumbrical‑interossei comparison study is the most sensitive. Early diagnosis of CTS may help patients of uremia to seek proper treatment at an appropriate time. [ABSTRACT FROM AUTHOR]

Context: Cirrhosis of liver is an important cause of morbidity and mortality and if associated with peripheral neuropathy (PN) it also poses a huge financial, psychological burden for the patients and their families. Aim: The aim of the present study was to study the magnitude of PN among subjects with cirrhosis of liver presenting to tertiary care teaching hospital in central rural India. Settings and Design: A cross-sectional study was performed in a tertiary care teaching hospital. Materials and Methods: In all patients of cirrhosis of liver irrespective of etiology, aged 15 and above, undergone clinical assessment for peripheral nervous systems damage and confirmed by nerve conduction studies. Statistical Analysis Used: We used chi square test to study associations. P value ≤0.05 was considered as significant. Crude odds ratios were computed to assess the strength of association between independent variables and dependent variables along with their 95% confidence intervals. Results: We included 207 of cirrhosis of liver patients admitted in medicine department from November 2010 through November 2013. Nearly 83% patients were male and 63.2% patients were under the age of 45 years. Common features in these patients were ascites (71%) splenomegaly (63.3%) pedal edema (61.4%) icterus (46.4%) tingling (44.9%) gastrointestinal bleeding(39.1%), ataxia (26.6%),numbness(26.6%),distal motor weakness (21.7%) and paresthesia(20.8%). Among the manifestation of peripheral nerve involvement, loss of ankle reflex was the most common feature in 51.7%, followed by loss of temperature sense 29.5%, loss of vibration sense 20.8%, loss of touch 16.4%, loss of position sense 14.5% and loss of pain in 6.3% of the patients. Peripheral neuropathy was found in 53.6% [95% CI: 46.58- 60.56] study subjects on electrophysiological study. Conclusions: Analysis of electrophysiological study shows that the PN is very common in study subjects with cirrhosis of liver, especially in male subjects, during the middle age group. [ABSTRACT FROM AUTHOR]

Objective: The objective of the study is to develop and validate a predictor score for assessing the requirement of mechanical ventilation (MV) in patients with Guillain-Barre syndrome (GBS). Study design: The study was conducted in patients admitted with GBS in neurointensive care unit in a tertiary care hospital. The demographic, clinical factors, electrophysiological, and spirometric data of all consecutive patients were prospectively collected. The study was undertaken in 2 stages. In the first stage, data were collected for development of a predictor score. In the second stage, the score developed was validated on a separate set of patient data. Results: The data collected were compared between the 2 groups (ventilated vs nonventilated). On univariate analysis, time taken to reach maximum deficit, neck weakness, bulbar weakness, facial weakness, single breath count (SBC), forced vital capacity, and phrenic nerve latency predicted the need for MV. On multivariate analysis, only neck weakness, bulbar weakness, SBC, and forced vital capacity were independent predictors of MV. There was a good correlation between SBC and the spirometric tests and phrenic nerve distal motor latency, as reflected in receiver operating characteristics curve. The predictor score developed using the regression coefficient of independent predictors showed that the best cutoff score for prediction of ventilation was 60 (sensitivity, 0.95; 1 - specificity, 0.065). Internal cross validation of the neck weakness, SBC, and bulbar palsy (NSB) score showed good correlation (Pearson R = 0.76; P = .00). There was no statistically significant difference between predicted and observed outcomes (sensitivity, 95%; specificity, 93%). Conclusion: Several independent risk factors were found to predict the requirement for MV in patients with GBS at admission. However, after scoring and analyzing them, it was found that combining a few of them was more useful to predict the need for MV. A model using NSB score, developed using clinical variables, accurately predicted the requirement of MV. In addition, among the NSB score parameters, simple bedside SBC could adequately assess the adequacy of vital capacity. [ABSTRACT FROM AUTHOR]

Concreteness is a phenomenon which refers to the linguistic stimuli to indicate concrete concepts generally show an advantage over abstract concepts. Dual-coding theory is a classical explanation for concreteness effect (Paivio, 1971, 1986). The aim of the study was to measure the reaction time (Judgment task) in processing abstract and concrete words and to find out the gender differences if any. Twenty young adults within the age range of 18-25years (10 males and 10 females) were considered in this study. Stimuli used were abstract and concrete words and total of 100 words were taken from various resources and was given for rating to twenty speech language pathologist and asked them to rate on 3 point rating scale. Out of which 50 words in each category were considered for experiment. Stimuli were audio recorded by a native Kannada adult female speaker and the recorded stimuli were normalized. Separate program was done loaded in DMDX and the stimulus was presented through Headphones and all the participants were asked to judge the type of word. Mixed analysis of variance repeated measures analysis of variance was done for comparison between abstract and concrete words and gender differences. The results of the present study revealed that there were faster reaction times for concrete words compared to abstract words and no gender differences were observed. Conclusion of the study explains that concreteness is an important variable in differentiating the processing abstract and concrete words. [ABSTRACT FROM AUTHOR]

Background: Rheumatoid arthritis is a multi-system autoimmune disorder predominantly involving multiple small and large joints along with certain extra-articular manifestations. The presence of peripheral neuropathy in patients with rheumatoid arthritis contributes significantly to the functional limitation in patients with rheumatoid arthritis. Objectives: To study the prevalence, types, and determinants of peripheral neuropathy in patients with rheumatoid arthritis. Materials and Methods: We studied 74 patients with rheumatoid arthritis of at least 2 year duration for the presence of peripheral neuropathy both clinically and electrophysiologically. The data obtained were entered into a database and continuous variables were analyzed using the Student t test and categorical variables were analyzed using the chi-square test. Results: Peripheral neuropathy was detected in 39.19% (29 out of 74 patients) patients on electrophysiologic testing and 82.76% (24 out of 29 patients) of the patients were asymptomatic. There was significant association between the presence of peripheral neuropathy and disease duration and rheumatoid factor positivity by the latex agglutination method. Sensory neuropathy was the most common form detected. Conclusions: Our study shows that subclinical peripheral neuropathy particularly sensory neuropathy which is not related to disease severity is very common in patients with prolonged disease duration. [ABSTRACT FROM AUTHOR]

Background: The Bioelectrical Impedance analysis is an easy, applicable method for assessing Total Body water and Fat Free Mass of various groups of people. It has many advantages over other methods and is safe, rapid, portable, easy to perform and require minimum operator training. It has been used e×tensively for developing specific prediction equation for different ethnicity, age, gender, level of body fatness and physical activity. Regression equations play great role to estimate the body density and fatness specific to the owing to methodological and biological factors. The purpose of the study was to investigate the utility of multifrequency BIA for the estimation of TBW and FFM of Indian females at different frequencies. Earlier scientists have measured various parameters of body composition of Indian population using MALTRON-II. However, literature shows that prediction equations have not been developed for Indian females. In the present research paper, an attempt has been made to develop BIA equations using data taken from PhD thesis (1). Data of reference (1) included vital information about body composition of 60 Indian females such as age, TBW, FFM, height, weight, impedance and phase at 5KHz., 50KHz., 100KHz. and 200 KHz.. This data was then used to calculate the impedance inde× i.e. ( height2/impedance) at the respective frequencies. This method uses multiple regression analysis to drive prediction equation of Indian females at frequencies of 5KHz.,50KHz.,100KHz.,200KHz., wherein the main aim is to predict one variable which is called dependent variable from 1 or more independent variable also called as predictor variable. Here in this study we used TBW and FFM as dependent variable and Weight of females and Impedance inde× i.e.(height2/impedance) as independent variable at different frequencies of 5 KHz.,50KHz.,100KHz.,200KHz respectively. Data was fed then into R software, an integrated suite of software facilities used for data manipulation, calculation and graphical display. It is used for effective data handling and storage facility. The main use of R software is for regression analysis, which was then used to generate the prediction equation. The prediction developed here is through multiple regression analysis. In multiple regression analysis the equation is of the form given below; Y=m1×1+ m2×2+ m3×3+...........+mn×n+ c; where c is intercept and m1,m2,m3…mn are the weight's assigned to each of the predictor variables by the regression solution. Data used in commercial software provided 8 BIA equations;4 for TBW and 4 for FFM at frequencies of 5 KHz,50KHz,100 KHZ and 200 KHz. This data included other statistical data such as mean, standard deviation and correlation of Total Body Water (TBW) and Fat Free Mass(FFM) with Impedance inde× and Weight. Besides this scatter matri× plot for Total Body Water (TBW) and Fat free Mass (FFM) ,Normal distribution of standardised residuals showing the relationship between TBW and FFM ,Scale location plot, Residual verses leverage plot, standardised residual verses cook's distance plot at frequencies of 5 KHz,50KHz,100 KHZ and 200 KHz are plotted. These plots for linear model objects give the diagnostic information about the linear model. The final race-combined TBW prediction equations included stature2/resistance and body weight. Multiple regression analysis was carried out on clinical data through R 2.9 software. The BIA prediction equation for Total Body water and Fat Free mass was developed at different frequencies of 5 KHz. and 50 KHz. respectively. The data was taken for Indian females lying in a limited age span of 17-22 years. [ABSTRACT FROM AUTHOR]

The article presents abstracts of research papers related to cardiac pacing that will be presented at the Asian-Pacific Symposium on Cardiac Pacing and Electrophysiology. The symposium will be held in New Delhi, India from October 25, 1997 to October 28, 1997. One of the research papers is related to ventricular tachycardia. Four of the 53 patients with idiopathic left ventricular tachycardia manifested characteristics identical to those of adenosine-sensitive ventricular tachycardia originating from the right ventricular outflow tract. In all 4 patients, spontaneous ventricular tachycardia was related to exercise or emotional stress.

The article presents information on the Asian-Pacific Symposium on Cardiac Pacing and Electrophysiology that will be held in New Delhi, India from October 25, 1997 to October 28, 1997. Various topics that will be discussed at the symposium are: ventricular tachycardia; ventricular arrhythmias; selection of optimal pacemaker and pacing mode; indications for pacemaker implantation; organizing a pacemaker clinic and follow up program; atrial flutter and fibrillation; catheter ablation of atrial flutter and fibrillation; etc.

The present report deals with the clinical phenomenology of three members (brothers) of one family with McLeod syndrome (MLS). In two, the clinical pictures were of choreiform disorders with amyotrophy, which were found to be neurogenic in origin by detailed electrophysiological study. The index case had peripheral acanthocytosis; immunohematological and molecular genetic studies confirmed diagnosis of MLS. However, one brother only had a slowly progressive motor neuron disease like picture but no abnormal movement disorder. He had peripheral acanthocytes as well. The inheritance seems to be X-linked recessive in nature. The affected family members exhibited much phenotypic heterogeneity. This appears to be the first report of MLS from India. [ABSTRACT FROM AUTHOR]

Two siblings who were born out of consanguineous marriage in an Indian family and had typical clinical manifestations of Sjögren-Larsson syndrome were found to have normal electroretinogram and abnormal visual evoked potential results. Abnormal visual evoked potential results along with the presence of distinctive foveal and parafoveal deposits, which could have a prognostic bearing on the visual acuity of the patients, emphasize the importance of a complete ophthalmological and electrophysiological evaluation in all patients with Sjögren-Larsson syndrome. [ABSTRACT FROM AUTHOR]

Objective: To describe the clinical-laboratory profile of pediatric Guillain-Barre syndrome and delineate features associated with need of mechanical ventilation., Methods: In a prospective observational study at tertiary care hospital, clinical-laboratory assessment and nerve conduction studies were documented in consecutive children hospitalized with Guillain-Barre syndrome according to Brighton criteria. Clinical-laboratory features were compared between ventilated and nonventilated patients using univariate and multivariate analysis., Results: Forty-six children (27 boys) with a mean age of 69.1±35.2 months were enrolled. History of preceding infection was present in 47.8%, bulbar palsy in 43.5%, feeble voice in 41.3%, sensory involvement in 13%, and autonomic involvement in 39.5%. Tetraparesis was noted in 87% of cases. Hughes disability scale >3 was noted in 44 children at admission and 39 (84.7%) at discharge. The most common electrophysiological type was acute motor axonal neuropathy (46.5%) followed by acute motor sensory axonal neuropathy (39.5%), acute inflammatory demyelinating polyneuropathy (7%), and inexcitable nerves (7%). Nine (19.7%) children were ventilated, 3 (6.5%) died or were lost, and 43 were discharged. Factors associated with need of mechanical ventilation on univariate analysis were older age, hypertension, bulbar palsy, feeble voice, lower Medical Research Council (MRC) sum, raised total leucocyte count, and history of preceding infection. Logistic regression revealed older age, history of predisposing illness, lower MRC sum at presentation, and bulbar palsy as independent predictors of mechanical ventilation., Conclusions: The most common electrophysiological subtype in northern Indian children is acute motor axonal neuropathy. Older age, preceding infection, low MRC sum, and bulbar palsy are predictors of mechanical ventilation in pediatric Guillain-Barre syndrome.

Objective: To investigate the electro-clinical, imaging, pathologic characteristics and post- operative outcome of patients with focal cortical dysplasia (FCD) associated medically refractory epilepsy. Methods: Study cohort comprised all patients with pathologically proven FCD who underwent surgery for medically refractory epilepsy and had >1 year postoperative follow-up. All underwent standard pre-surgical evaluation including long-term video-EEG and high resolution MRI, and evaluation with SPECT, PET, f-MRI, cortical stimulation-mapping and invasive monitoring when indicated. We assessed seizure outcome by Engel Scoring System. Results: For the 33 patients (22 males, 11 females), mean age at surgery was 18.2 years and mean duration of epilepsy 12.7 years. Six patients had temporal lobe, 13 had frontal lobe each and 14 had multilobar involvement. Twenty-three underwent lesionectomy, 6 anterior temporal lobectomy, 2 hemispherotomy and 2 multilobar resection. Pathologically, 12 patients had FCD type IIA, 11 had IIB, 2 had IA and IB each and 6 patients had dual pathology in the form of FCD with hippocampal sclerosis. Twentythree patients became seizure-free at 1year follow-up. Linear regression analysis showed a positive correlation between duration of epilepsy and post-operative seizure score (R= 0.32, P= 0.05) and negative correlation between age of onset and severity of epilepsy (R= -0.42, P= 0.01). Pathological subtypes and electrophysiological variables did not predict postoperative outcome. Conclusions: This study is the first from India with a sizeable number of patients with FCD. An earlier onset and longer duration of epilepsy are associated with more refractory seizures, thereby stressing on early identification and a timely surgical intervention. [ABSTRACT FROM AUTHOR]

Myasthenia gravis is recognized the world over. There are several studies from India dealing with various aspects such as electrophysiology, response to treatment and comparison of juvenile with adult myasthenia. However, there is no epidemiological data on myasthenia from India. We present the findings from the largest hospital based studies on myasthenia gravis from India, using data collected retrospectively over a period of 43 years (1965 to 2008) from the Neurology Department in a tertiary referral centre. The objective was to study the age at onset, gender distribution, serological status and thymic pathology in patients with myasthenia gravis. Of the 841 patients, 614 were males and 227 were females. The M:F ratio was 2.70:1. The median age at onset in the total group was 48 years (males: 52 years and females: 33 years). The mean age at onset was 45.89 years, (males 49.88 years and females 35.15years). The peak age at onset for males was in the sixth and seventh decade. In females, it occurred earlier in the third decade. Serological studies were done in 282 patients for Acetylcholine receptor (AchR) antibodies. Seropositivity was seen in 84.39% patients. Electrophysiological studies using Repetitive Nerve Stimulation (RNS) were documented for 513 patients of whom 393 (76.60%) patients showed a positive decremental response. Thymoma was more common in males while thymic hyperplasia was more common in females. Although in several reported series, Myasthenia gravis is reported to be more prevalent in females, in our study myasthenia was more common in male population. [ABSTRACT FROM AUTHOR]

The article presents information on the Asian-Pacific Symposium on Cardiac Pacing and Electrophysiology that will be held in New Delhi, India from October 25, 1997 to October 28, 1997. Highlights of the symposium are: special clinical tutorials in small groups to facilitate close interaction with experts; controversies on topics particularly relevant to the Asia-Pacific Region; scientific exposition featuring technologies for the next millennium; and a once-in-a-lifetime opportunity for Associate Professionals from South East Asia to participate in an international convention on the subject.

The article presents information on the VIth Asian-Pacific Symposium on Cardiac Pacing and Electrophysiology that will be held in New Delhi, India from October 25, 1997 to October 28, 1997. The symposium offers 4 days of comprehensive, meticulously chalked out educational programs, designed in concordance with the growing demands of the discipline. Besides the regular plenary sessions and presentations, the meeting will also afford an excellent opportunity to renew old friendships, foster new ones, and to interact with people who have been forced by distance to evolve a different variety of practice.

Objective: The study aims to characterise the electrodiagnostic findings of patients with traumatic brachial plexus injuries (BPIs) in India and to analyse the association between aetiologies and levels of injuries., Methods: A total of 111 consecutive electrodiagnostic studies done between January 2009 and June 2011 on persons with traumatic BPI were retrospectively analysed., Setting: Electrodiagnostic Laboratory, Department of Physical Medicine and Rehabilitation in a tertiary care university teaching hospital in South India., Main Outcome Measures: Nerve conduction velocities and electromyography (EMG) to locate the level of BPI, Dumitru and Wilbourne scale to assess the severity of BPI., Results: We studied 106 males and five females, ranging from 11 to 59 years of age. All but one had unilateral BPI. Motorcycle crashes were the most frequent cause (n=64, 58%). Isolated supraclavicular injury was found in 98 arms (88%) and infraclavicular injury in seven arms (6%). Root-level injuries were more common in motorcycle crashes and occupation-related trauma, while trunk-level injuries were more often found in automobile crashes, falls, bicycle-related trauma and penetrating wounds. Pan root (C5-T1) involvement was more common in the motorcycle trauma group (74%). There was no significant association between aetiologies and levels of BPIs. A total of 73 (65%) plexus injuries were of 'severe' category as per Dumitru and Wilbourn scale., Conclusions: Motorcycle crash is the most common cause of traumatic BPIs. Supraclavicular injury is the rule in most cases. Proper attention needs to be given to differentiate the mild to moderate injuries from the severe injuries with EMG techniques since most of the cases are severe. There was no significant association found between aetiologies and levels of injury., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

To evaluate the presence of peripheral neuropathy (PN) in newly diagnosed treatment-naive patients with multiple myeloma (MM), 29 patients and 25 age and sex matched controls underwent electrophysiological studies. Patients with associated diabetes, alcoholism, peripheral vascular disease and POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M component and skin changes) were excluded from the study. The median age of patients was 56 years (range 28-82) and the sex ratio was 2.6:1. Eighteen (62.1%) patients were found to have evidence of PN by history and clinical examination alone (two patients), both clinical and electrophysiological evidence (five patients) and only electrophysiological evidence (11 patients). Out of 25 healthy controls, only two patients had evidence of PN by electrophysiological studies (cases vs. controls, p < 0.002). The commonest nerve involvement was in the form of sensory-motor axonal neuropathy followed by sensory-motor demyelinating neuropathy. This information may impact therapeutic and prognostic decision-making in newly diagnosed patients with MM.

Electroantennograms (EAGs)were recorded from males and females of the potato tuber moth,Phthorimaea operculella in response to a broad range of plant volatile compounds belonging to diverse chemical classes.The responses to 27 compounds were evaluated,which indicated significant differences in EAGs between chemicals as well as between sexes.The fatty acid derivatives comprising essentially green leaf volatile components elicited significantly greater responses in females.The response profile of males was,in general,lower than that of females.EAG responses to the oxygenated and hydrocarbon monoterpenes were lower in both males and females.Dose -response studies indicate differences in response between the sexes and concentrations,suggesting the existence of sexual dimorphism. Compounds belonging to the fatty acid derivatives class appear to be important for an oligophagous pest such as the potato tuber moth and the findings are discussed in relation to host plant selection in this species.

Progressive myoclonic epilepsy (PME) is a syndrome complex encompassing different diagnostic entities and often cause problems in diagnosis. We describe the clinical, electrophysiological and pathological features of 97 patients with the diagnosis of PME evaluated over 25 years. Case records of confirmed patients of Neuronal ceroid lipofuscinosis (NCL = 40), Lafora body disease (LBD = 38), Myoclonic epilepsy with ragged red fibers (MERRF = 10), and probable Unverricht-Lundberg disease (ULD = 9) were reviewed. The mean age at onset in patients with NCL (n = 40) was 5.9+/-9.1 years (M:F:: 28:12). Subtypes of NCL were: late infantile (n = 19), infantile (n = 8), juvenile (n = 11) and adult (n = 2) NCL. EEG (n = 37) showed varying degree of diffuse slowing of background activity in 94.6% and epileptiform discharges in 81.1% of patients. Slow frequency photic stimulation evoked photo-convulsive response in 5 patients only. Giant SSEP was demonstrated in 7 and VEP study revealed a prolonged P100 (2) and absent waveform (7). Electrophysiological features of neuropathy were present in 3 patients. Presence of PAS and Luxol Fast Blue (LFB) positive, auto fluorescent (AF) ceroid material in brain tissue (n = 12) and electron microscopy of brain (n = 5), skin (n = 28) and muscle (n = 1) samples showing curvilinear and lamellar bodies established the diagnosis. Patients of LBD (mean age of onset at 14.4+/-3.9 years, M:F:: 24:14) with triad of PME symptoms were evaluated. EEG (n = 37) showed variable slowing of background activity in 94.6% and epileptiform discharges in 97.4%. Photosensitivity with fast frequency was observed only in 5 patients. CT (n = 32) and MRI (n = 4) revealed diffuse cortical atrophy. Giant SSEP was demonstrated in 24 patients of LBD while VEP study revealed a prolonged P100 (4) and absent waveform (8). Electrophysiological features of neuropathy were present in one patient. Diagnosis was established by the presence of PAS positive diastase resistant, Lugol's Iodine labeled inclusions in sweat glands of axillary skin (n = 35), brain (n = 2) and liver (n = 1). Ten patients with MERRF (mean age at onset: 14.6+/-5.8 years; M: F:: 3:2) had triad of PME symptoms. Muscle biopsy revealed oxidative reaction product and classical ragged red fibers. In nine patients of PME without cognitive decline, probable diagnosis of ULD (mean age at onset: 13.8+/-9.5 years) was considered after biopsy of skin and/or muscle excluded other forms of PMEs. Neuronal ceroid lipofuscinosis and Lafora body diseases were the common causes of PME in the series from south India. This is one of the largest series from the Indian subcontinent to the best of our knowledge. Photosensitivity is notably less common in LBD/NCL in this series distinctly different from those reported in the literature. Further exploration is required to determine whether different genotype is responsible. Morphological changes were helpful in diagnosis and could be confirmed by biopsy of peripheral tissues like skin and muscle in majority (60%). Electron microscopy was helpful in the diagnosis NCL and MERRF.