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EDITORIAL |
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Welcome to the readers |
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Sanjeev V Thomas DOI:10.4103/0972-2327.78040 PMID:21633605 |
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REVIEW: MANAGEMENT UPDATES |
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Management of provoked seizure |
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Usha Kant Misra, Jayantee Kalita DOI:10.4103/0972-2327.78041 PMID:21633606A provoked seizure may be due to structural damage (resulting from traumatic brain injury, brain tumor, stroke, tuberculosis, or neurocysticercosis) or due to metabolic abnormalities (such as alcohol withdrawal and renal or hepatic failure). This article is a part of the Guidelines for Epilepsy in India. This article reviews the problem of provoked seizure and its management and also provides recommendations based on currently available information. Seizure provoked by metabolic disturbances requires correction of the triggering factors. Benzodiazepines are recommended for treatment of seizure due to alcohol withdrawal; gabapentin for seizure seen in porphyria; and antiepileptic drugs (AED), that are not inducer of hepatic enzymes, in the seizures seen in hepatic dysfunction. In severe traumatic brain injury, with or without seizure, phenytoin (PHT) may be given for 7 days. In ischemic or hemorrhagic stroke one may individualize the AED therapy. In cerebral venous sinus thrombosis (CVST), AED may be prescribed if there is seizure or computed tomographic (CT) abnormalities or focal weakness; the treatment, in these cases, has to be continued for 1 year. Prophylactic AED is not recommended in cases of brain tumor and neurosurgical procedures and if patient is on an AED it can be stopped after 1 week. |
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REVIEW: MANAGEMENT UPDATES (REVIEWS ON ADVANCES IN TREATMENT) |
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Treating children with attention-deficit/hyperactivity disorder and comorbid epilepsy |
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Shivanand Kattimani, S Mahadevan DOI:10.4103/0972-2327.78042 PMID:21633607Attention-deficit/hyperactivity disorder (ADHD) is one of common neurodevelopmental disorder often comorbid with epilepsy. There are no existing guidelines on how to manage these two conditions when they are comorbid. To identify relationship between epilepsy and ADHD and to know role of antiepileptics and safety of stimulant like methylphenidate in such conditions from existing literature, we searched articles published in clinical journals available online between 1990-2010, with these key words in medline:children, epilepsy, seizure, comorbid, ADHD, treatment. Relevant abstracts were further selected for their focus on current topic. Cross references were extracted. Finally relevant articles that included original research articles, reviews and abstracts of non-english literature were used. Children with epilepsy may manifest with symptoms of ADHD. Children with ADHD may develop epilepsy. Some antiepileptics like phenobarbitone, gabapentin, topiramate may not be helpful in controlling behavioral symptoms of ADHD. Stimulants are the main stay of pharmacotherapy for ADHD but there is risk of decreasing seizure threshold in children with comorbid epilepsy especially when their epilepsy is not well controlled. Existing evidence is not in favor of screening children with ADHD for EEG abnormality before starting stimulant therapy. |
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REVIEW: SYSTEMATIC (SYSTEMATIC REVIEW OF A SUBJECT) |
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Meniere's disease: Still a mystery disease with difficult differential diagnosis  |
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A Vassiliou, PV Vlastarakos, P Maragoudakis, D Candiloros, TP Nikolopoulos DOI:10.4103/0972-2327.78043 PMID:21633608One hundred and forty-six years after its first description, the differential diagnosis of Meniere's disease remains very challenging. The aim of the present study is to review the current knowledge on the advantages and disadvantages of the new diagnostic methods for Meniere's disease. The importance of accurate diagnosis for primary healthcare systems is also discussed. An extensive search of the literature was performed in Medline and other available database sources. Information from electronic links and related books were also included. Controlled clinical studies, prospective cohort studies, retrospective cohort studies, cross-sectional studies, case reports, written guidelines, systematic reviews, and books were selected. The typical clinical triad of symptoms from the vestibular and cochlear systems (recurrent vertigo, fluctuating sensorineural hearing loss and tinnitus) is usually the key for clinical diagnosis. Glycerol dehydration test and electrocochleography are the main diagnostic tests in current practice, while vestibular evoked myogenic potentials may be used in disease staging. Imagine techniques are not specific enough to set alone the diagnosis of Meniere's disease, although they may be necessary to exclude other pathologies. Recently developed 3D MRI protocols can delineate the perilymphatic/endolymphatic spaces of the inner ear and aid diagnosis. Meniere's disease is a continuous problem for the patients and affects their quality of life. Taking into account the frequent nature of the disease in certain countries, efforts for reliable diagnosis, prompt referral, and successful management are undoubtedly cost-effective for healthcare systems. |
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ORIGINAL ARTICLES |
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Long-term visual outcome in idiopathic intracranial hypertension |
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NN Baheti, M Nair, SV Thomas DOI:10.4103/0972-2327.78044 PMID:21633609Objective: To characterize the course, outcome, and risk of relapse or late worsening in a clearly defined cohort of idiopathic intracranial hypertension (IIH) over a long period of follow-up. Materials and Methods: Retrospective chart review of patients with definite IIH was evaluated at the Sree Chitra Tirunal Institute for Medical Sciences and Technology between 1998 and 2006. Patients' demographic data, clinical, neuro-ophthalmic examinations, and treatment details were abstracted. Patients were further categorized into three groups based on whether they improved, worsened, or relapsed on follow-up. Final visual outcome of each patient was defined according to grading of the worse eye at the last visit. Statistical analysis included t test to compare group means and chi-square test to compare proportions. Results: Of the 43 women included, visual impairment was observed in 80 eyes (93%) at presentation and it was moderate to severe in 14%. The mean CSF opening pressure at presentation did not differ significantly in those with visual impairment compared to those with normal vision. Those having early severe visual impairment had significantly higher (P = 0.015) likelihood of severe visual impairment on last follow-up. Of the total, 34 patients (79%) improved, 4 (9.3%) relapsed on follow-up after period of stability, and 5 (11.6%) worsened over 56 months follow-up (range, 26-132 months). The groups were comparable, except those who improved were younger (P<0.05). At last examination, 9% had significant vision loss. Conclusion: IIH patients can have delayed worsening or relapses and about tenth of patients can have permanent visual loss early or late in the course of the disease. All patients with IIH need to be kept under long-term follow-up, with regular monitoring of visual functions. |
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Intra-rater reliability of the modified Tardieu scale to quantify spasticity in elbow flexors and ankle plantar flexors in adult stroke subjects |
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Priyanka Singh, Abraham M Joshua, Sailakshmi Ganeshan, Sucharitha Suresh DOI:10.4103/0972-2327.78045 PMID:21633610Objectives: The purpose of this study was to investigate Iintra-rater reliability of the Modified Tardieu Scale (MTS) in elbow flexors and ankle plantar flexors in adult subjects with stroke. Materials and Methods: A total of 91 subjects with stroke participated in this test-retest study. Intra-rater reliability of the MTS was investigated by a qualified and trained physiotherapist for elbow flexors and ankle plantar flexors in two sessions. A rater was one who performed the procedure and an observer only records the angles so that the rater was blinded to findings. Outcome measures in this study were measurable components of MTS, which are angle of muscle reaction (R1), passive range of motion (R2), dynamic component (R2-R1), and quality of muscle reaction (grade 0 - 4) termed as MTS score. Results: Intra-rater reliability of MTS was very good for R1, R2, R2-R1, and MTS score (ICC > 0.85, P<0.0001) across two sessions in elbow flexors and ankle plantar flexors. Conclusion: MTS is a reliable clinical tool for measurement of spasticity in the elbow flexors and ankle plantar flexors in adult subjects with stroke. |
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Awareness of neurocysticercosis: A study from Northwest India |
p. 27 |
Mohit Girotra, Chanchal Gera, Rtika Ryfka Abraham, Rajat Gauba, Tino Bansal, Parmdeep Kaur, Yashpal Singh, Jeyaraj D Pandian DOI:10.4103/0972-2327.78046 PMID:21633611Background: Neurocysticercosis (NCC) is a common cause of epilepsy in developing countries. In order to plan and implement prevention programs, it is essential to study the awareness of NCC. Objective: To study the awareness of NCC among patients with NCC and compare with age- and gender-matched controls without NCC. Setting and Design: Hospital based case-control study. Materials and Methods: Two hundred and fourteen subjects were studied (109 NCC patients, and 105 age- and gender-matched controls without NCC). The participants were selected from neurology and medical wards of a tertiary referral hospital in northwest India. They were interviewed by trained medical interns using a questionnaire. Results: 64.2% of the NCC patients and 19% of control group had heard about NCC (P < 0.001). Knowledge regarding organ affected by NCC in the NCC group was 61.4% and in the control group was 80% (P = 0.09). Only 12.9% of the NCC group and none in the control group identified tape worm as a causative agent for NCC (P = 0.092). Negative effects of NCC on marriage and social life were more often cited by the NCC group but in the control group it was towards education (P = 0.004). Conclusions: The awareness of NCC was poor in both the groups. Educational programs are needed to improve the awareness about NCC among the patients and the public. |
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Sleep in Wilson's disease: Questionnaire based study |
p. 31 |
Archana B Netto, Sanjib Sinha, Arun B Taly, Samhita Panda, Shivaji Rao DOI:10.4103/0972-2327.78047 PMID:21633612Objective: We proposed to detect sleep abnormalities in Wilson's disease, (WD) using sleep questionnaires. Materials and Methods: Twenty-five patients (M:F = 18:7; age: 24.4 ± 9.2 years) with WD and 24 controls (all males; age: 33.1 ± 9.7 years) were recruited. They underwent phenotypic/magnetic resonance imaging (MRI) evaluation followed by administration of Pittsburg Sleep Quality Index (PSQI) and Epworth Sleepiness Scale (ESS) questionnaires. Results: The mean age at presentation and diagnosis was 24.4 ± 9.2 and 17.6 ± 7.5 years, respectively. The duration of illness at diagnosis was 14 ± 21.9 months. On PSQI, 15 patients with WD had abnormal PSQI scores of >5 as compared to 6 patients among the controls. The mean PSQI score was significantly more (P = 0.03) in patients compared to the controls. The PSQI worst scores were noted only in WD. Evaluation with ESS showed that three patients with WD scored >10/24, while two among the controls qualified for excessive daytime sleepiness. Overall, assessment by sleep questionnaires detected abnormality in 16 patients with WD as compared to 8 controls (P = 0.004). Subgroup analysis revealed that patients whose duration of illness was >8 years and who were on decoppering treatment had significantly lesser excessive daytime somnolence. Conclusions: Sleep disturbances were observed more often in WD than in controls. Better designed studies will provide a better understanding. |
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Multidetector computed tomography angiography: Application in vertebral artery dissection |
p. 35 |
Evelyn Teasdale, Peter Zampakis, Celestine Santosh, Saif Razvi DOI:10.4103/0972-2327.78048 PMID:21633613Background and Purpose: Multidetector computed tomography angiography (MDCTA) is a minimally invasive radiological technique providing high-resolution images of the arterial wall and angiographic images of the lumen. We studied the radiological features of vertebral artery dissection (VAD) in a consecutive series of patients investigated for acute stroke and subarachnoid hemorrhage (SAH) in order to confirm and define the diagnostic features of VAD on MDCTA. Patients and Methods: Review of patients identified prospectively over a 4-year period with VAD assessed by MDCTA was conducted. Radiological features of VAD on MDCTA were reanalyzed utilising previously reported criteria for VAD. Results: Thirty-five patients (25 males, mean age 49.6 years) with a total of 45 dissected vertebral arteries were reviewed. MDCTA features of VAD included increased wall thickness in 44/45 (97.7%) arteries and increased total vessel diameter in 42/45 arteries (93.3%). All dissected arteries had either lumen stenosis (21/45) or associated segmental occlusion (24/45). An intimal flap was detected in 6/45 (13.3 %) vessels. Twenty-five patients had follow-up imaging, 14/32 vessels returned to normal, 4 showed improvement in stenosis but did not return to normal and 14 demonstrated no change. The majority of non-occluded vessels became normal or displayed improved patency. Only 4/17 occluded arteries demonstrated re-establishment of flow. No adverse effects were recorded. Conclusions: MDCTA is a safe and reliable technique for the diagnosis of VAD. Increased wall thickness (97.7%) and increased vessel wall diameter (93.3%) were the most frequently observed features. |
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CASE REPORTS |
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Vocal cord palsy: An uncommon presenting feature of myasthenia gravis |
p. 42 |
Prahlad K Sethi, Anuradha Batra, Nitin K Sethi, Josh Torgovnick, Edward Arsura DOI:10.4103/0972-2327.78049 PMID:21655204Vocal cord palsy can have myriad causes. Unilateral vocal cord palsy is common and frequently asymptomatic. Trauma, head, neck and mediastinal tumors as well as cerebrovascular accidents have been implicated in causing unilateral vocal cord palsy. Viral neuronitis accounts for most idiopathic cases. Bilateral vocal cord palsy, on the other hand, is much less common and is a potentially life-threatening condition. Myasthenia gravis, an autoimmune disorder caused by antibodies targeting the post-synaptic acetylcholine receptor, has been infrequently implicated in its causation. We report here a case of bilateral vocal cord palsy developing in a 68-year-old man with no prior history of myasthenia gravis 2 months after he was operated on for diverticulitis of the large intestine. Delay in considering the diagnosis led to endotracheal intubation and prolonged mechanical ventilation with attendant complications. Our case adds to the existing literature implicating myasthenia gravis as an infrequent cause of bilateral vocal cord palsy. Our case is unusual as, in our patient, acute-onset respiratory distress and stridor due to bilateral vocal cord palsy was the first manifestation of a myasthenic syndrome. |
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Carcinoma of gall bladder presenting as dermatomyositis |
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Deepti Akkihebbal Narasimhaiah, Jennifer Anne Premkumar, Viju Moses, Geeta Chacko DOI:10.4103/0972-2327.78050 PMID:21655205Cancer-related muscle diseases are usually paraneoplastic disorders. Dermatomyositis (DM) is a type of inflammatory myopathy that is strongly associated with a broad range of malignant disorders. The malignancy can occur before, concomitantly or after the onset of myositis. The malignancies most commonly associated with DM are carcinomas of ovary, lung, stomach, colorectal and pancreas, as well as non-Hodgkin's lymphoma. An association of DM with carcinoma of the gall bladder (GB) is extremely rare with only two previously reported cases in the literature. We report a case of carcinoma of GB with DM as the paraneoplastic manifestation. |
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Macroglossia associated with 271 bp deletion in exon 50 of dystrophin gene |
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Hardeep Singh Malhotra, Ratish Juyal, Kiran Preet Malhotra, Rakesh Shukla DOI:10.4103/0972-2327.78051 PMID:21655206Macroglossia is rare in patients of Duchenne muscular dystrophy (DMD), and its occurrence without any endocrinologic abnormality, seizures or an abnormal karyotype is even rarer. We describe a patient of DMD with isolated macroglossia with 271 bp deletion in exon 50 of the dystrophin gene and speculate a relationship in this regard. |
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Kleine-Levine syndrome in an adolescent female and response to modafinil |
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Ashish Aggarwal, Amit Garg, RC Jiloha DOI:10.4103/0972-2327.78052 PMID:21655207Kleine-Levine Syndrome (KLS) is a disorder characterized by a triad of periodic hypersomnia, hyperphagia, and hypersexuality. KLS, although more common in young males, it has also been seen in females. Treatment options available for its management include mood stabilisers like lithium, stimulants like amphetamines, antidepressants and other options including electroconvulsive therapy. Modafinil is one of the new stimulant medications approved for narcolepsy. Herein, we report a young female with KLS and showing favorable response to modafinil. More data is required to establish the effectiveness of modafinil in this syndrome. |
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Mcleod syndrome: Report of an Indian family with phenotypic heterogeneity |
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Ambar Chakravarty, P Bhattacharya, D Banerjee, S Mukherjee DOI:10.4103/0972-2327.78053 PMID:21655208The 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. |
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IMAGES IN NEUROLOGY |
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A case of disseminated neurocysticercosis |
p. 56 |
D Saranya, M Jawahar, K Bhanu DOI:10.4103/0972-2327.78054 PMID:21655209 |
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Superficial siderosis |
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Sameer Vyas, Suresh Giragani, Paramjeet Singh, Anil Bansali, Niranjan Khandelwal DOI:10.4103/0972-2327.78055 PMID:21655210 |
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Superficial siderosis in cerebral amyloid angiopathy |
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Nisar Ahmad Wani, Tasleem L Kosar, Aijaz A Rawa, Abdul K Qayum DOI:10.4103/0972-2327.78056 PMID:21654928 |
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Molar tooth sign with ataxia and see-saw nystagmus (Joubert syndrome) |
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N Byju, James Jose, K Saifudheen, Mohammed Musthafa DOI:10.4103/0972-2327.78057 PMID:21654929 |
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TECHNICAL NOTE |
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Single-fiber EMG: A review |
p. 64 |
V Arul Selvan DOI:10.4103/0972-2327.78058 PMID:21654930 |
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LIGHTER MOMENTS |
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Ode to Epileptologists! |
p. 68 |
Ajith Cherian PMID:21654931 |
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OBITUARY |
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Dr. H. S. Swamy |
p. 69 |
P Satishchandra, AB Taly |
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Dr. Ravi Nehru: The life, the man, the loss |
p. 70 |
Amit Batla, Farah Khanam |
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