Annals of Indian Academy of Neurology
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   Table of Contents - Current issue
January-March 2017
Volume 20 | Issue 1
Page Nos. 1-85

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The stretch reflex and the contributions of C David Marsden p. 1
Kalyan B Bhattacharyya
The stretch reflex or myotatic reflex refers to the contraction of a muscle in response to its passive stretching by increasing its contractility as long as the stretch is within physiological limits. For ages, it was thought that the stretch reflex was of short latency and it was synonymous with the tendon reflex, subserving the same spinal reflex arc. However, disparities in the status of the two reflexes in certain clinical situations led Marsden and his collaborators to carry out a series of experiments that helped to establish that the two reflexes had different pathways. That the two reflexes are dissociated has been proved by the fact that the stretch reflex and the tendon reflex, elicited by stimulation of the same muscle, have different latencies, that of the stretch reflex being considerably longer. They hypothesized that the stretch reflex had a transcortical course before it reached the spinal motor neurons for final firing. Additionally, the phenomenon of stimulus-sensitive cortical myoclonus lent further evidence to the presence of the transcortical loop where the EEG correlate preceded the EMG discharge. This concept has been worked out by later neurologists in great detail , and the general consensus is that indeed, the stretch reflex is endowed with a conspicuous transcortical component.
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Transforming growth factor-β1 (C509T, G800A, and T869C) gene polymorphisms and risk of ischemic stroke in North Indian population: A hospital-based case-control study p. 5
Pradeep Kumar, Shubham Misra, Amit Kumar, Mohammad Faruq, Sunil Shakya, Gyan Vardhan, Subiah Vivekanandhan, Achal Kumar Srivastava, Kameshwar Prasad
Background: Transforming growth factor-beta 1 (TGF-β1) is a multifunctional pleiotropic cytokine involved in inflammation and pathogenesis of cerebrovascular diseases. There is limited information on the association between variations within the TGF-β1 gene polymorphisms and risk of ischemic stroke (IS). The aim of this study was to investigate the association of the TGF-β1 gene (C509T, G800A, and T869C) polymorphisms, and their haplotypes with the risk of IS in North Indian population. Methods: A total of 250 IS patients and 250 age- and sex-matched controls were studied. IS was classified using the Trial of Org 10172 in Acute Stroke Treatment classification. Conditional logistic regression analysis was used to calculate the strength of association between TGF-β1 gene polymorphisms and risk of IS. Genotyping was performed using SNaPshot method. Results: Hypertension, diabetes, dyslipidemia, alcohol, smoking, family history of stroke, sedentary lifestyle, and low socioeconomic status were found to be associated with the risk of IS. The distribution of C509T, G800A and T869C genotypes was consistent with Hardy-Weinberg Equilibrium in the IS and control groups. Adjusted conditional logistic regression analysis showed a significant association of TGF-β1 C509T (odds ratio [OR], 2.1; 95% CI; 1.2–3.8;P= 0.006), G800A (OR, 4.4; 95% CI; 2.1–9.3;P< 0.001) and T869C (OR, 2.6; 95% CI; 1.5–4.5;P= 0.001) with the risk of IS under dominant model. Haplotype analysis showed that C509-A800-T869 and T509-G800-C869 haplotypes were significantly associated with the increased risk of IS. C509T and T869C were in strong linkage disequilibrium (D' =0.51, r2 = 0.23). Conclusion: Our results suggest that TGF-β1 polymorphisms and their haplotypes are significantly associated with the risk of IS in North Indian population.
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A study of acute muscle dysfunction with particular reference to dengue myopathy p. 13
Rajesh Verma, Vikram V Holla, Vijay Kumar, Amita Jain, Nuzhat Husain, Kiran Preet Malhotra, Ravindra Kumar Garg, Hardeep Singh Malhotra, Praveen Kumar Sharma, Neeraj Kumar
Background: Acute myopathy is a common cause of acute motor quadriparesis which has various etiologies with different courses of illness and prognosis depending on the cause. Understanding this diversity helps us in proper approach toward diagnosis, predicting the prognosis, and possible complications and in improving the treatments that are being provided. This study was planned to study the clinical, electrophysiological, and etiological profile of patients presenting with acute myopathy. We also studied how dengue-related acute myopathy differs from other causes and also difference between myopathy due to myositis and hypokalemia in cases of dengue. Materials and Methods: This was a prospective, observational study involving all clinically suspected cases of acute myopathy of not more than 4 weeks duration with raised serum creatine kinase (CK) level. They were subjected to detailed clinical evaluation along with hematological, biochemical, microbiological, and electrophysiological studies and followed-up for outcome at 1 and 3 months. Muscle biopsy and histopathological examination were done in selected patients after taking informed consent. Statistical analysis was performed by appropriate methods using SPSS version 16.0 (Chicago, IL, USA). Results: We evaluated thirty patients of acute myopathy with raised CK level. Seventeen patients had fever, 11 had myalgia, and 5 had skin lesions. All presented with symmetric weakness, 17 (56.7%) patients having predominantly proximal weakness, neck or truncal weakness in 6 (20%), hyporeflexia in 12 (40%), with mean Medical Research Council (MRC) sum score of 46.67 ± 6.0. Eight (mean modified Barthel index [MBI] at presentation - 15 ± 3.7) patients had poor functional status according to MBI and 15 according to modified Rankin scale (MRS) (mean MRS score - 2.5 ± 1.2). Etiology was dengue viral infection in 14 patients; hypokalemia due to various causes other than dengue in 8; pyomyositis in 3; dermatomyositis, polymyositis, thyrotoxicosis, systemic lupus erythematosus, and unknown etiology in one each. Only eight patients had abnormal electrophysiology and seven among nine biopsies done were abnormal. At 1 month, 24 (80.0%) and 23 (76.7%) patients had achieved normal MBI and MRS scores with 28 (93.3) and 27 (90%) patients, respectively, at 3 months. Dengue with hypokalemia had less myalgia, more of hyporeflexia, and lower serum CK compared to those without hypokalemia. Conclusion: Dengue infection and hypokalemia due to various causes are the most common causes of acute myopathy and are associated with rapid and complete recovery within 1 month. Shorter duration of illness, higher MRC sum score, better disability status at presentation, lower serum CK correlate with better outcome. Biopsy was decisive in <20% cases; hence, it is not primary investigation in acute myopathy.
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Median and common peroneal neuropathy in coir workers of Alappuzha district, Kerala p. 23
Sadanandavalli Retnaswami Chandra, Biji Anand, Thomas Gregor Issac
Introduction: Coir work, in a large number of people involves mechanically rolling the coconut fibers into coir which is later subjected to various processes. The primary work is done as a cottage industry specially by women in the sitting posture for several hours. This study reports evidence of median and common peroneal neuropathy electrophysiologically in people who had been engaged in this job for several years. This study was initiated to establish the possible relationship between coir work and symptomatic neuropathies which was seen in that region with all investigations “ for other causes not” contributing to the etiological diagnosis. Subjects and Methods: One hundred and forty-two upper limbs and 142 lower limbs in patients engaged in long years of coir work but having no symptoms were evaluated electrophysiologically with informed consent and financial compensation, appropriate inclusion and exclusion criteria were followed as described in the text. Results: There is electrophysiological evidence for median and common peroneal neuropathy in persons engaged in long years of coir work. Conclusions: Coir workers neuropathy appears to be a new occupational neuropathy which can be prevented by following simple preventive measures.
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Correlation between hippocampal volumes and medial temporal lobe atrophy in patients with Alzheimer's disease p. 29
Vikas Dhikav, Sharmila Duraiswamy, Kuljeet Singh Anand
Introduction: Hippocampus undergoes atrophy in patients with Alzheimer's disease (AD). Calculation of hippocampal volumes can be done by a variety of methods using T1-weighted images of magnetic resonance imaging (MRI) of the brain. Medial temporal lobes atrophy (MTL) can be rated visually using T1-weighted MRI brain images. The present study was done to see if any correlation existed between hippocampal volumes and visual rating scores of the MTL using Scheltens Visual Rating Method. Materials and Methods: We screened 84 subjects presented to the Department of Neurology of a Tertiary Care Hospital and enrolled forty subjects meeting the National Institute of Neurological and Communicative Disorders and Stroke, AD related Disease Association criteria. Selected patients underwent MRI brain and T1-weighted images in a plane perpendicular to long axis of hippocampus were obtained. Hippocampal volumes were calculated manually using a standard protocol. The calculated hippocampal volumes were correlated with Scheltens Visual Rating Method for Rating MTL. A total of 32 cognitively normal age-matched subjects were selected to see the same correlation in the healthy subjects as well. Sensitivity and specificity of both methods was calculated and compared. Results: There was an insignificant correlation between the hippocampal volumes and MTL rating scores in cognitively normal elderly (n = 32; Pearson Correlation coefficient = 0.16, P> 0.05). In the AD Group, there was a moderately strong correlation between measured hippocampal volumes and MTL Rating (Pearson's correlation coefficient = −0.54;P< 0.05. There was a moderately strong correlation between hippocampal volume and Mini-Mental Status Examination in the AD group. Manual delineation was superior compared to the visual method (P < 0.05). Conclusions: Good correlation was present between manual hippocampal volume measurements and MTL scores. Sensitivity and specificity of manual measurement of hippocampus was higher compared to visual rating scores for MTL in patients with AD.
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The need for a population-based, dose optimization study for recombinant tissue plasminogen activator in acute ischemic stroke: A study from a tertiary care teaching hospital from South India p. 36
Siju V Abraham, Fazil Thaha, S Vimal Krishnan, Athulya Shajan, Jayaraj Mymbilly Balakrishnan, Babu Urumese Palatty
Context: The guideline recommended dose of intravenous (i.v) recombinant tissue-type plasminogen activator (rt-PA) for acute ischemic stroke is 0.9 mg/kg in the European and American populations. In Asiatic population, some studies have shown that a lower dose of i.v rt-PA is equally efficacious. Aims: To assess if there is a need for a dose optimization for i.v rt-PA study among Indians. Setting and Design: A prospective, observational database of acute stroke cases that presented to a tertiary care institute over a period of 1 year was made. Methods: The data procured using a prestructured elaborate pro forma. Based on the dose of rt-PA received, the individuals were divided into three groups; Group 1 (0.6–0.7 mg/kg), Group 2 (0.7–0.8 mg/kg), and Group 3 (0.8–0.9 mg/kg). Improvement was assessed in each group and between the thrombolysed and nonthrombolysed individuals. Statistical Analysis Used: The nonparametric Mann–Whitney U-test (Wilcoxon rank-sum test) was applied for assessing improvement of National Institutes of Health Stroke Scale score with significance level of α < 0.05 (P < 0.012) and compliance level at 95%. Results: Between the thrombolysed (n = 46) and nonthrombolysed (n = 113) group, there was a statistically significant neurological improvement in the thrombolysed group. Clinical improvement was noted in 75%, 85.7%, and 66.7% of individuals receiving rt-PA in Groups 1, 2, and 3, respectively. Four out of the five who developed a clinically significant intracranial hemorrhage were thrombolysed at a dose of 0.8–0.9 mg/kg rt-PA (Group 3). Conclusion: There is a need for a properly randomized, dose optimization study of i.v rt-PA in the Indian subcontinent.
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Etiological profile of noncompressive myelopathies in a tertiary care hospital of Northeast India p. 41
Ashok Kumar Kayal, Munindra Goswami, Marami Das, Lakhshya Jyoti Basumatary, Suvorit Subhas Bhowmick, Baiakmenlang Synmon
Background: The discovery of antibodies against aquaporin-4 and evolving concepts of noncompressive myelopathies in the 21st century have made a major impact on the etiological profile of these diseases, with few cases turning out to be idiopathic. Objective: To find causes of noncompressive myelopathy in a tertiary care hospital of Northeast India. Materials and Methods: An observational study was carried out in the Neurology Department of Gauhati Medical College, Guwahati, from September 2013 to February 2016. Patients of noncompressive myelopathies who underwent magnetic resonance imaging (MRI) of the spine were segregated into two categories: acute-to-subacute myelopathy (ASM) and chronic myelopathy (CM). In addition to routine blood tests, chest X-ray, urinalysis, and visual evoked potentials, investigations included MRI of the brain, cerebrospinal fluid analysis, and immunological, infectious, and metabolic profile based on the pattern of involvement. Results: The study had 151 patients (96 ASM and 55 CM) with a median age of 35 years and male: female ratio 1.4:1. The causes of ASM were neuromyelitis optica spectrum disorder (23), multiple sclerosis (MS) (8), systemic lupus erythematosus (1), Hashimoto's disease (1), postinfectious acute disseminated encephalomyelitis (6), postinfectious myelitis (8), infections (9), spinal cord infarct (5), and electrocution (1). The causes of CM were MS (1), probable or possible sarcoidosis (7), mixed connective tissue disease (1), Hashimoto's disease (2), infections (9), Vitamin B12deficiency (4), folate deficiency (2), hepatic myelopathy (2), radiation (11), and paraneoplastic (1). No etiology could be found in 48 (31.8% ) patients (34 ASM and 14 CM). In 21/96 (21.9% ) patients of ASM, acute transverse myelitis was idiopathic based on current diagnostic criteria. Conclusion: Underlying etiology (demyelinating, autoimmune, infectious, vascular, metabolic disorder, or physical agent) was found in 68% patients of noncompressive myelopathy.
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Preoperative parameters and their prognostic value in amyotrophic lateral sclerosis patients undergoing implantation of a diaphragm pacing stimulation system p. 51
Aydin Sanli, Ihsan Şükrü Şengün, Volkan Karaçam, Aylin Özgen Alpaydın, Kemal Can Tertemiz, Sevgi Özalevli, Bahar Ağaoğlu Şanlı, Alper Kaya, Nezih Özdemir
Introduction: Amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disease with devastating and fatal respiratory complications. Diaphragm pacing stimulation (DPS) is a treatment option in diaphragm insufficient ALS patients. Ventilatory insufficiency depending on diaphragmatic failure is treated by the present study aimed to investigate prognostic value of preoperative clinical and functional characteristics of ALS patients undergoing implantation of a DPS system and to determine appropriate indications for the DPS system. Methods: The study included 34 ALS patients implanted with DPS system. All patients underwent multidisciplinary and laboratory evaluations before the surgery. The laboratory examinations included pulmonary function tests and arterial blood gas analysis. Survival rates were recorded in a 2-year follow-up after the surgery. Results: Twenty-eight of 34 patients with ALS survived after a 2-year follow-up. These patients were younger than those who died and had the disease for a longer time; however, the differences were not significant. Both right and left hemidiaghragms were thicker in the survived patients (P < 0.0001 for each). Pulmonary function tests revealed no significant differences between the patients who survived. Arterial blood gas analysis demonstrated lower partial pressure of carbon dioxide in the survived patients (P = 0.025). Conclusions: DPS implantation was more efficacious in ALS patients with mild respiratory failure and thicker diaphragm. Predictors of long-term effectiveness of DPS system are needed to be addressed by large-scale studies.
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Ictal asystole: A rare cardiac manifestation of temporal lobe epilepsy, treated with epilepsy surgery p. 55
Shreyas Hasmukh Ravat, Amit Ashok Bhatti, Mansi Viraj Shah, Dattatraya P Muzumdar, Sangeeta Hasmukh Ravat
Seizures are associated with fascinatingly varied cardiac and autonomic manifestations, of which ictal tachycardia is common, and asystole and bradycardia are rare. Ictal asystole (IA), an often unsought autonomic phenomenon, occurs most commonly with temporal followed by frontal lobe seizures. Prolonged IA may lead to cerebral anoxic ischemia. As the mysteries of sudden unexplained death in epilepsy are unraveled, it is quite possible that the key to it lays within these seizure-induced cardiac rhythm abnormalities. We present a case of a young male with temporal lobe epilepsy due to left mesial temporal sclerosis with prolonged IA, which was successfully managed with epilepsy surgery.
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Nocardial pyomyositis p. 58
Boby Varkey Maramattom, Rony K Varghese, Anila Sudhakaran, Kurian Ninan
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Labrune syndrome: A unique leukoencephalopathy p. 59
Leena Pahuja, Elisheba Patras, Sachin Sureshbabu, Nittin Parkhe, Laxmi Khanna
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Menkes disease and response to copper histidine: An Indian case series p. 62
Sangeetha Yoganathan, Sniya Valsa Sudhakar, Gautham Arunachal, Maya Thomas, Annadurai Subramanian, Renu George, Sumita Danda
Background: Menkes disease (MD) is an X-linked recessive neurodegenerative disorder caused by mutations in ATP7A gene. Depending on the residual ATP7A activity, manifestation may be classical MD, occipital horn syndrome, or distal motor neuropathy. Neurological sparing is expected in female carriers. However, on rare occasions, females may manifest with classical clinical phenotype due to skewed X-chromosome inactivation, X-autosome translocation, and XO genotype. Here, we describe a small series of probands with MD and their response to copper histidine therapy. This series also includes a female with X-13 translocation manifesting neurological symptoms. Methods: The clinical profile, laboratory and radiological data, and follow-up of four children with MD were collected from the hospital database and are being presented. Results: All the four children in our series had developmental delay, recurrent respiratory tract infections, hair and skeletal changes, axial hypotonia, tortuous vessels on imaging, low serum copper, ceruloplasmin, and elevated lactate. Fetal hypokinesia and fetal growth retardation were present in two cases. Failure to thrive was present in three children and only one child had epilepsy. Subcutaneous copper histidine was administered to all children. The average time lapse in the initiation of treatment was 20.3 months, and average duration of follow-up was 14.3 months. Conclusion: We conclude that copper histidine therapy is beneficial in reversing the skin and hair changes, improving appendicular tone, socio-cognitive milestones, and improving weight gain, and immunity. Early diagnosis and management of MD are essential to have a better clinical outcome. More research is needed to explore and devise new strategies in the management of patients with MD.
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Subjective symptoms of carpal tunnel syndrome correlate more with psychological factors than electrophysiological severity p. 69
Firosh Khan, Abdulkhader Shehna, Sivaramakrishnan Ramesh, Kakkassery Sankaran Sandhya, Reji Paul
Aim: Carpal tunnel syndrome (CTS) is the most common entrapment neuropathy and is one of the most common requests for electrodiagnosis. We aimed to note the relationship of subjective symptom severity of CTS, with objective electrophysiological severity and psychological status of patients. Patients and Methods: One hundred and forty-four consecutive patients of CTS referred to neurophysiology laboratory of a tertiary care hospital over 1 year were prospectively studied. Boston CTS Assessment Questionnaire (BCTSAQ) and visual analog scale (VAS) were used to assess subjective symptom severity. Psychological status was assessed by Hospital Anxiety and Depression Scale (HADS). Electrophysiological severity of CTS was estimated by median motor distal latency and median to ulnar peak sensory latency difference across the wrist. Each parameter in both hands was scored from 0 to 3 depending on the severity grade, and a composite electrophysiological severity score (CEPSS) was calculated for each patient by summing up the scores in both hands. Statistical analysis was done by Spearman's rank correlation test. Results: There was significant correlation of BCTSAQ with VAS (P = 0.001), HADS anxiety score (P < 0.001), and HADS depression score (P = 0.01). CEPSS had no significant correlation with VAS (P = 0.103), HADS anxiety score (P = 0.211), or HADS depression score (P = 0.55). CEPSS had a borderline correlation with BCTSAQ (P = 0.048). Conclusions: While the subjective symptoms of CTS are well correlated with psychological factors, their correlation with objective electrophysiological severity is weak. Hence, prompt treatment of psychological comorbidity is important in symptomatic management of CTS; decision about surgical intervention should be based on electrophysiological severity rather than symptom severity.
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Superficial siderosis presenting as chronic migraine: Rare presentation of a rare disease p. 73
Debashish Chowdhury, Amit Shankar Singh, Geeta Anjum Khwaja
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Multidrug-resistant Acinetobacter meningitis treated by intrathecal colistin p. 74
Rajesh K Singh, Sanjeev K Bhoi, Jayantee Kalita, Usha K Misra
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Pregnancy reduces severity and frequency of attacks in hyperkalemic periodic paralysis due to the mutation c.2111C>T in the SCN4A gene p. 75
Josef Finsterer, Salma Majid Wakil, Franco Laccone
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Amyloid myopathy as the presenting feature of lymphoplasmacytic lymphoma p. 77
Zain Guduru, Abhishek Purohit, Cunfeng Pu, Sandeep Rana
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Leber's hereditary optic neuropathy: Report of a simple case associated with a rare variant mutation p. 78
Ravinder Mohan Malhotra, Mousa Ali Al Mejally, Hanadi Mahmoud Abualela, Marwa Ahmed Eltemamy
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A new algorithm of suspected stroke patient management with brain natriuretic peptide/N-terminal pro-brain natriuretic peptide point of care testing platform in the emergency department p. 81
Mingfeng He, Zhixin Wu, Yingying Li, Junna Lei
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An unusual cause of reversible parkinsonism p. 82
Sahil Mehta, Amith Kumar, Aastha Takkar, Manoj Kumar Goyal, Vivek Lal
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Brain and neurological disorders: A simplified health education guide p. 84
Rahul T Chakor
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The sleep solution: Secrets for a good night's sleep p. 85
Vibhor Pardasani
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