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EDITORIAL |
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Addressing problems of dementia in India |
p. 147 |
Sanjeev V Thomas DOI:10.4103/0972-2327.85869 PMID:22028522 |
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REVIEWS |
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Nonpharmacological treatment of epilepsy  |
p. 148 |
VS Saxena, VV Nadkarni DOI:10.4103/0972-2327.85870 PMID:22028523Nonpharmacological treatment of epilepsy includes surgery, vagal nerve stimulation, ketogenic diet, and other alternative/complementary therapies, e.g., yoga, Ayurveda, electroencephalography (EEG) biofeedback technique, aerobic exercise, music therapy, transcranial magnetic stimulation, acupuncture, and herbal remedies (traditional Chinese medicine). Alternative therapies, despite the term, should not be considered as an alternative to antiepileptic medication; they complement accepted drug treatment. Alternative therapies like yoga, through techniques that relax the body and mind, reduce stress, improve seizure control, and also improve quality of life. Ketogenic diet is a safe and effective treatment for intractable epilepsies; it has been recommended since 1921. The diet induces ketosis, which may control seizures. The most successful treatment of epilepsy is with modern antiepileptic drugs, which can achieve control of seizures in 70-80% cases. Patients opt for alternative therapies because they may be dissatisfied with antiepileptic drugs due to their unpleasant side effects, the long duration of treatment, failure to achieve control of seizures, cultural beliefs and, in the case of women, because they wish to get pregnant Surgical treatment may lead to physical and psychological sequelae and is an option only for a minority of patients. This article presents supportive evidence from randomized controlled trials done to assess the benefit of non-pharmacological treatment. |
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N-methyl D-aspartate receptor encephalitis: A new addition to the spectrum of autoimmune encephalitis |
p. 153 |
Boby Varkey Maramattom, Anu Jacob DOI:10.4103/0972-2327.85872 PMID:22028524A large proportion of "encephalitis" is caused by unknown agents. Of late, a new category of disorders, "autoimmune encephalitis," has been described, which present with features similar to viral encephalitides. A well-delineated and common entity among this group is the recently described anti-NMDAR encephalitis (NMDARE). Although this entity was initially described in young women harboring ovarian teratomas, it is now characterised as well in children and men. Approximately 60% of the patients have an underlying tumor, usually an ovarian teratoma. In 40% of the patients, no cause can be found (idiopathic NMDARE). NMDARE typically presents with psychiatric features followed by altered level of consciousness, severe dysautonomia, hyperkinetic movement disorders, seizures and central hypoventilation. Orofacial dyskinesias resulting in lip and tongue mutilation are quite common. Seizures, are common and may be difficult to treat. The disease can be confirmed by serum and cerebrospinal fluid anti-NMDAR antibodies. Titers of these antibodies can also guide response to treatment. Tumor removal is necessary if identified, followed by immunological treatment. Intravenous methylprednisolone and immunoglobulins aim to suppress/modulate immune response while plasma exchange attempts to remove antibodies and other inflammatory cytokines. Rituximab and cyclophosphamide aim to suppress antibody production. Recovery is slow and often with neurological deficits if treatment is delayed. With many distinctive clinical features, a specific antibody that aids diagnosis, and early effective treatment with commonly available drugs leading to good outcomes, NMDARE is a diagnosis that should be considered early in any case of "unexplained encephalitis." |
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Confronting the stigma of epilepsy |
p. 158 |
Sanjeev V Thomas, Aparna Nair DOI:10.4103/0972-2327.85873 PMID:22028525Stigma and resultant psychosocial issues are major hurdles that people with epilepsy confront in their daily life. People with epilepsy, particularly women, living in economically weak countries are often ill equipped to handle the stigma that they experience at multiple levels. This paper offers a systematic review of the research on stigma from sociology and social psychology and details how stigma linked to epilepsy or similar conditions can result in stereotyping, prejudice and discrimination. We also briefly discuss the strategies that are most commonly utilized to mitigate stigma. Neurologists and other health care providers, social workers, support groups and policy makers working with epilepsy need to have a deep understanding of the social and cultural perceptions of epilepsy and the related stigma. It is necessary that societies establish unique determinants of stigma and set up appropriate strategies to mitigate stigma and facilitate the complete inclusion of people with epilepsy as well as mitigating any existing discrimination. |
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ORIGINAL ARTICLES |
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An epidemiological study of dementia among the habitants of Eastern Uttar Pradesh, India |
p. 164 |
K Poddar, S Kant, A Singh, TB Singh DOI:10.4103/0972-2327.85874 PMID:22028526Dementia is one of the most serious health problems of the elderly and their caregivers. Objective: The objective of this study was to estimate the prevalence of dementia in the community, its sociodemographic and behavioral associates to determine the risk factors among resident of two districts of eastern Uttar Pradesh, India. Materials and Methods: This study was based on 2890 subjects aged 50 years and above, residing in rural areas of Mirzapur and urban areas of the Varanasi district of eastern Uttar Pradesh, India. Efforts were made to identify the subjects of age 50 years and above using cluster sampling. The Hindi Mental State Examination was used as the instrument tool to determine the score and a cut-off score of ≤23 was considered as the presence of dementia among the identified subjects. Results: The overall prevalence of dementia was found to be 5.1%. This percentage was increasing with age and decreasing with educational level. Among females, the prevalence of dementia was observed (7.2%) to be double than that in males (3.8%). Widows/widowers/unmarried had a double prevalence (9.3%) as compared with married (4.3%) people. The age, gender, marital status, education, occupation, number of family members in the household and liquor addiction were found to be significantly associated with dementia. The relative risk for the above-mentioned variables was found to be more than 1. |
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Knowledge and practice profile of obstetricians regarding epilepsy in women in Kerala state, India |
p. 169 |
Mamta Bhat, KN Ramesha, C Nirmala, PS Sarma, Sanjeev V Thomas DOI:10.4103/0972-2327.85877 PMID:22028527Purpose: To assess the knowledge of obstetricians about concerns of women with epilepsy. Materials and Methods: We surveyed 97 obstetricians (teaching hospitals-43, private hospitals-32, and community health centers-21) using knowledge of women's issues and epilepsy (KOWIE) questionnaire II with additional questions. Results: The mean duration of practice of the surveyed obstetricians was 12.4 ± 10.7 years and 94% were female doctors. Most of them were well informed about the teratogenic effects of AEDs (91%), need to continue antiepileptic drugs (AEDs) during pregnancy (95%), and the role of folic acid and vitamin K during pregnancy (95%). They agreed AEDs decrease the efficacy of oral contraception and it was safe for the woman to breast-feed the baby while on AEDs. Only 1/3 rd of them knew that steroid hormones could alter seizure threshold or that AEDs could predispose to osteomalacia. Fewer doctors knew that WWE could have increased sexual dysfunction (29.9%) or infertility (26.8%). The knowledge did not vary according to years of practice or practice settings. Conclusions: Obstetricians were well informed about the fetal complications of antenatal AED exposure, but were under informed of other complications such as osteomalacia, sexual dysfunction, and infertility. |
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Neurological complications in late-stage hospitalized patients with HIV disease |
p. 172 |
Rakendra Singh, Manjot Kaur, Deepak Arora DOI:10.4103/0972-2327.85878 PMID:22028528Background and Objective: The nervous system is the most frequent and serious targets of human immunodeficiency virus (HIV) infection. In spite of a wide prevalence of neurological manifestations in HIV there are not many studies to look into it, especially from this part of the world. We investigated various neurological manifestations of HIV and their association with CD4 and CD8 counts at the time of presentation. Materials and Methods: All HIV-infected patients who presented to 750 bedded teaching hospital in North India were subjected to thorough neurological and neuropsychological evaluation. Wherever indicated, neuroimaging, cerebrospinal fluid study, electromyography, and nerve-conduction studies were performed to confirm the diagnosis. CD4 and CD8 counts were calculated. Results: A total of 416 HIV-positive patients were seen. Of them 269 were males. A total of 312 neurological events were identified in 268 patients having evidence of neurological involvement. HIV-associated dementia (HAD) was the most common cause of morbidity (33.65%), followed by CNS infections (21.63%). Most common CNS infection was tuberculosis (65.56%). CD4 counts in CNS infections and HAD were 64.8/ml and 83.52/ml, respectively. Most of the patients in our study had low scores on MMSE (22.32). Conclusions: Even in the absence of overt neurological disease, subclinical involvement in the form of subtle cognitive and motor decline is found to occur with greater frequency. Most of these patients have lower CD4 and CD8 counts, thus substantiating the proposition that neuroAIDS is a late manifestation. Significant correlation exists between CD4 counts and type of neurological manifestation. We concluded that neuropsychological assessment should be mandatory for all HIV-positive patients. |
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Severe myoclonic epilepsy of infancy (Dravet syndrome): Clinical and genetic features of nine Turkish patients |
p. 178 |
Meral Özmen, Cengiz Dilber, Burak Tatli, Nur Aydinli, Mine Çaliskan, Baris Ekici DOI:10.4103/0972-2327.85879 PMID:22028529Purpose: Mutations of the a-1 subunit sodium channel gene (SCN1A) cause severe myoclonic epilepsy of infancy (SMEI). To date, over 300 mutations related to SMEI have been described. In the present study, we report new SCN1A mutations and the clinical features of SMEI cases. Materials and Methods: We studied the clinical and genetic features of nine patients diagnosed with SMEI at the Pediatric Neurology Department of Istanbul Medical Faculty. Results: Five patients had nonsense mutations, two had missense mutations, one had a splice site mutation and one had a deletion mutation of the SCN1A gene. Mutations at c.3705+5G splice site, p.trip153X nonsense mutation and deletion at c.2416_2946 have not been previously described. The seizures started following whole cell pertussis vaccination in all patients. The seizures ceased in one patient and continued in the other eight patients. Developmental regression was severe in three patients, with frequent status epilepticus. The type of mutation was not predictive for the severity of the disease. Two of the three patients with severe regression had nonsense and missense mutations. Conclusions : Dravet syndrome can be result of several different types of mutation in SCN1A gene. Onset of the seizures after pertussis vaccination is an important clue for the diagnosis and neuro- developmental delay should be expected in all patients. |
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Being ambulatory does not secure respiratory functions of Duchenne patients |
p. 182 |
Baris Ekici, Yakup Ergül, Burak Tatli, Feride Bilir, Fatih Binboga, Ayse Süleyman, Zeynep Tamay, Mine Çaliskan, Nermin Güler DOI:10.4103/0972-2327.85889 PMID:22028530Aim: The aim of this work was to assess the respiratory functions of ambulatory Duchenne patients and to propose an earlier time period for intervention. Materials and Methods: Lung functions and North Star Ambulatory Assessment (NSAA) scores of Duchenne patients were evaluated simultaneously. Results: Thirty ambulatory Duchenne patients were included in this study. NSAA scores of the patients were directly correlated with arm abduction, arm adduction, and shoulder flexion strengths. Forced expiratory volume in 1 second percent predicted and forced vital capacity (FVC) percent predicted correlated inversely to age and to the NSAA score. Twelve of 13 patients with FVC values lower than 80% of predicted had NSAA scores below 24 points. None of the patients who were younger than 7 years had FVC values lower than 80% of predicted. Conclusion: Annual spirometry is necessary for Duchenne patients older than 6 years regardless of the ambulatory status. |
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SHORT COMMUNICATIONS |
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Global aphasia without hemiparesis: A case series |
p. 185 |
Aparna R Pai, Gopee Krishnan, S Prashanth, Suryanarayana Rao DOI:10.4103/0972-2327.85890 PMID:22028531Background: Global aphasia without hemiparesis (GAWH) is a rare stroke syndrome characterized by the unusual dissociation of motor and language functions. Issues regarding its etio-pathogenesis, lesion sites, and recovery patterns are extensively being debated in contemporary neuroscience literature. Materials and Methods: Four patients admitted in our hospital between 2005 and 2009 with GAWH caused by ischemic stroke were studied retrospectively with emphasis on number and site of lesions, etiology, and recovery patterns. Results: The clinical findings from our subjects showed that GAWH could result from either single/multiple lesions including subcortical lesions. The recovery was rapid, although not complete. One case evolved into Wernicke's aphasia as seen in earlier studies. Two subjects revealed evolution to transcortical sensory aphasia and one to Broca's aphasia which is distinct from previous proposals. Two cases showed lack of clinico-anatomic correlation during recovery. Conclusions: GAWH could result from both embolic and large vessel strokes and single or multiple lesions. The recovery pattern may be variable and may show lack of clinico-anatomical correlation indicating anomalous cerebral functional reorganization, questioning the conventional teaching of language representation in the brain. |
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Idiopathic hypertrophic cranial pachymeningitis: Three biopsy-proven cases including one case with abdominal pseudotumor and review of the literature |
p. 189 |
KM Hassan, Prabal Deb, HS Bhatoe DOI:10.4103/0972-2327.85891 PMID:22028532Hypertrophic pachymeningitis (HP) is a rare disorder of diverse etiology. It presents with headaches, cranial neuropathies and ataxia occurring alone or in combination. Dural biopsy is essential to exclude secondary causes of pachymeningitis. There is paucity of data on biopsied cases of HP. We report three biopsy-proven cases of idiopathic hypertrophic cranial pachymeningitis. All our patients had headaches and multiple cranial neuropathies; ataxia was seen in one patient. One patient had recurrent anterior and posterior cranial neuropathies, while one each had recurrent anterior and posterior cranial neuropathies. Two patients had profound irreversible mono-ocular visual loss. All of them showed prominent pachymeningeal thickening on imaging. Infarcts were seen in one patient, which have rarely been documented. All patients showed biopsy evidence of meningeal thickening and nonspecific chronic inflammation of the dura. The disease may have a remitting and relapsing course, and usually responds to steroids. Clinical improvement was excellent in two patients and modest in one on steroid therapy. All our patients required azathioprine during the course of therapy. Early institution and long-term maintenance of steroid therapy prevents neurologic sequelae. Occurrence of abdominal inflammatory pseudotumor in a patient of HP possibly as part of multifocal fibrosclerosis has not been described earlier. |
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Prevalence, types, clinical associations, and determinants of peripheral neuropathy in rheumatoid patients |
p. 194 |
Monodeep Biswas, Arghya Chatterjee, Sudip Kumar Ghosh, S Dasgupta, Kartik Ghosh, PK Ganguly DOI:10.4103/0972-2327.85893 PMID:22028533Background: 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. |
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TECHNICAL NOTE |
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Kinaesthetic ipsilateral and crossed extensor plantar response: A new way to elicit upgoing toe sign (Babinski response)? |
p. 198 |
Abraham Kuruvilla, Pandurang R Wattamwar DOI:10.4103/0972-2327.85894 PMID:22028534We describe a phenomenon of "kinaesthetic extensor plantar response" in advanced pyramidal dysfunction, an interesting observation noted in a patient with dorsal myelopathy. A 44-year-old woman presented with one-year history of gradually progressive weakness and stiffness of both lower limbs along with urge incontinence of urine. Examination showed spontaneous elicitation of extensor plantar response while assessing the tone by rolling method as well as on noxious stimulation of the thigh. Magnetic resonance imaging (MRI) of the dorsal spine and digital subtraction angiography showed the presence of spinal dural arteriovenous fistula causing myelopathy. This case exemplifies the fact that in advanced pyramidal dysfunction, not only the receptive field of Babinski reflex may extend to the leg or thigh, but may also integrate with other modalities of stimulation, such as the rolling movement. The possible underlying pathophysiology of such a phenomenon is discussed. |
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CASE REPORTS |
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Magnetic resonance spectroscopy findings in non-enhancing desmoplastic medulloblastoma |
p. 200 |
Puneet Mittal DOI:10.4103/0972-2327.85895 PMID:22028535Medulloblasoma is a common posterior fossa tumor seen in children and presents with some typical features like midline vermian location and fairly homogeneous enhancment. Desmoplastic variety of medulloblastoma is usually seen in the adults and is known to show some atypical features like lateral cerebellar location, variable enhancement, and early meningeal infilteration. Therefore medulloblastoma should always be considered in differential diagnosis of posterior fossa mass in adults even when typical imaging findings are not that of medulloblastoma. Enhancement pattern can be variable in these tumors varying from mild to striking. Occasionally, totally non-enhancing tumors are encountered, which can cause further diagnostic confusion. We describe the magnetic resonance (MR) and MR spectroscopy findings in a case of midline vermian mass, which did not show any enhancement on post-contrast images, and was subsequently proven to be desmoplastic medulloblastoma. On MR spectroscopy, the mass showed elevated choline peak consistent with mitotic lesion. No significant lipid lactate leak was seen, which is also consistent with the ususally homogeneous nature of these tumors. Moreover, it displayed taurine peak at 3.4 ppm which is considered fairly specific for medulloblastoma. Therefore, MR spectroscopy findings can be helpful in the diagnosis of medulloblastoma in adults when MR imaging findings can be nonspecific. |
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Hypertrophic pachymeningitis |
p. 203 |
SN Karthik, K Bhanu, Sakthi Velayutham, M Jawahar DOI:10.4103/0972-2327.85896 PMID:22028536 |
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Steroid-responsive encephalopathy in autoimmune thyroiditis: Clinical spectrum and MRI observations in three cases |
p. 205 |
K Dinkar, Sanjib Sinha, AB Taly, PS Bindu, RD Bharath DOI:10.4103/0972-2327.85897 PMID:22028537Hashimoto's encephalopathy (H.E.) is probably of autoimmune etiology, and manifests with seizures, stroke-like episodes, cognitive decline, neuropsychiatric symptoms, myoclonus. It is presumed to be autoimmune in origin with high serum titers of antithyroid peroxidase antibodies (anti-TPA). Thyroid function might often be normal. The diagnosis is arrived at by excluding other toxic, metabolic and infectious causes of encephalopathies, supportive clinical profile, elevated thyroid antibodies and optimum steroid response. We present the characteristic phenotypic manifestations, magnetic resonance imaging and electroechography observations and response to immunomodulation with follow-up in three cases of H.E. All the three cases manifested with subacute to chronic progressive encephalopathy, cerebellar dysfunction, seizures, behavioral abnormalities and oculomotor disturbances and had evidence of hypothyroidism, elevated titers of anti-TPA and positive thyroid anti-microsomal antibodies. Atypical and uncommon presentations are known. This report emphasizes that a high index of suspicion is often required in cases with "investigation negative encephalopathy" for early diagnosis of H.E. |
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Cysticercosis of midbrain presenting with fluctuating ptosis |
p. 208 |
M Netravathi, AS Banuprakash, Renu Khamesra, N Hemantakumar Singh DOI:10.4103/0972-2327.85898 PMID:22028538Fluctuating ptosis is usually caused by Myasthenia gravis. There are a few case reports of central causes of fluctuating ptosis. A 58-year-old man presented with fluctuating ptosis of one year duration. He was diagnosed as having ocular myasthenia and investigated. On evaluation, his electrophysiological tests revealed negative decremental response and results of neostigmine test was negative. During follow-up, patient developed headache. Imaging of the brain revealed midbrain cysticercosis granuloma. The focal encephalitis and edema was responsible for fluctuating ptosis. It is therefore essential to be aware of conditions that cause such pseudomyasthenic features. Patients with ptosis need to be evaluated for other rare central cause especially when neostigmine test is negative. |
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Acute movement disorder with bilateral basal ganglia lesions in diabetic uremia |
p. 211 |
Gurusidheshwar M Wali, Mallikarjun S Khanpet, Rajendra V Mali DOI:10.4103/0972-2327.85899 PMID:22028539Acute movement disorder associated with symmetrical basal ganglia lesions occurring in the background of diabetic end stage renal disease is a recently described condition. It has distinct clinico-radiological features and is commonly described in Asian patients. We report the first Indian case report of this potentially reversible condition and discuss its various clinico-radiological aspects. |
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Unusual presentation of Takayasu's arteritis as posterior reversible encephalopathy syndrome |
p. 214 |
Syed Ahmed Zaki, Vishal Chavan, Preeti Shanbag DOI:10.4103/0972-2327.85900 PMID:22028540Takayasu's arteritis is a chronic, idiopathic, inflammatory disease primarily affecting aorta and its branches. It mainly affects young females in the age group of 10-30 years. Various atypical presentations of Takayasu's arteritis have been described in children. These atypical presentations can cause delayed diagnosis resulting in increased morbidity and mortality. Posterior reversible encephalopathy syndrome (PRES) is a neuroradiologic condition associated with headache, seizures, altered sensorium, visual disturbances, and characteristic lesions on neuroimaging. We report a child with Takayasu's arteritis who presented a posterior reversible encephalopathy syndrome. He also had associated abdominal tuberculosis for which anti-tuberculous treatment was started. PRES was diagnosed by magnetic resonance imaging with fluid-attenuated inversion recovery sequences. The child was started on nifedipine and propranolol. The child regained his consciousness within 48 h of admission. Prompt treatment of hypertension led to rapid reversal of neurological symptoms. In view of hypertension a computed tomography aortogram was done, which showed features suggestive of high grade (>75%) focal proximal left renal artery stenosis. EULAR (European League Against Rheumatism)/PReS (Paediatric Rheumatology European Society) consensus criteria was used for the diagnosis of Takayasu's arteritis in our patient. Percutaneous transluminal balloon angioplasty of the stenotic left renal artery was performed. Post-angioplasty, nifedipine was gradually omitted and oral propranolol was continued. |
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IMAGES IN NEUROLOGY |
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Cerebral hydatid cyst showing pathognomonic daughter cysts |
p. 217 |
Mukund R Vidhate, Dilip Singh, Pawan Sharma, Maneesh K Singh DOI:10.4103/0972-2327.85903 PMID:22028541 |
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Unilateral symptomatic palatal tremor due to pontocerebellar infarction |
p. 219 |
Karkal Ravishankar Naik, Aralikatte Onkarappa Saroja DOI:10.4103/0972-2327.85905 PMID:22028542 |
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LIGHTER MOMENTS |
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Choice is simply yours-Physician's perception |
p. 221 |
Shridhar Dwivedi PMID:22028543 |
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LETTERS TO THE EDITOR |
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Comment on: Central nervous system inflammatory demyelinating disorders of childhood |
p. 222 |
H Radhakrishna DOI:10.4103/0972-2327.85909 PMID:22028544 |
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Authors' reply |
p. 222 |
Mahesh Kamate, Vivek Chetal, Venkatesh Tonape, Niranjana Mahantshetti, Virupaxi Hattiholi PMID:22028545 |
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Intravenous tissue plasminogen activator, ischemic stroke, and the risk of Kounis Syndrome |
p. 223 |
Nicholas G Kounis, Sophia N Kouni, George N Kounis, Sophia A Kounis DOI:10.4103/0972-2327.85913 PMID:22028546 |
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Dengue and rhabdomyolysis |
p. 224 |
S Vivekanandan DOI:10.4103/0972-2327.85914 PMID:22028547 |
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Noninvasive investigations are essential tools for epilepsy surgery in developing countries: Extrapolating results from Chinese studies |
p. 225 |
Feng Chang, Qin Huang DOI:10.4103/0972-2327.85918 PMID:22028548 |
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Decompression syndrome, ear-plug and barotrauma |
p. 226 |
Gunalp Uzun, Mesut Mutluoglu, Mehmet G Senol DOI:10.4103/0972-2327.85921 PMID:22028549 |
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OBITUARY |
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Dr. Shyamal Kumar Sen |
p. 228 |
Ambar Chakravarty |
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