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EDITORIALS |
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Evolution of endovascular treatment of intracranial dural arteriovenous fistulas |
p. 1 |
Bulang Gao DOI:10.4103/0028-3886.60385 PMID:20228454 |
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Drug treatment of polymyositis and dermatomyositis |
p. 3 |
J.M.K Murthy DOI:10.4103/0028-3886.60386 PMID:20228455 |
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REVIEW ARTICLES |
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Congenital Chiari malformations  |
p. 6 |
Prasad Vannemreddy, Ali Nourbakhsh, Brian Willis, Bharat Guthikonda DOI:10.4103/0028-3886.60387 PMID:20228456Chiari malformation is the commonest anomaly of the craniovertebral junction involving both the skeletal as well as the neural structures. This entity has rapidly evolved over the past decade with newer visualization techniques, thus posing new challenges to diagnosis and management. This review includes the developmental theories, the latest nomenclature and existing treatment modalities of this interesting anomaly. Five theories tried to explain the malformation of the hindbrain and the neuraxis but no single theory completes the development of embryonic defects. Several atypical presentations have been reported with either incidental/asymptomatic features resulting in further classifications. The new magnetic resonance imaging flow techniques attempt to substantiate the clinical presentations and correlate with the abnormality which can be subtle in correlation. Surgical correction to improve the cranial volume, decrease the hydrocephalus and improve flow across the foramen magnum is the mainstay but needs to be tailored to a given type of malformation. Further clinical and imageological studies, especially longitudinal natural history, might improve our understanding of the atypical/asymptomatic presentations and the management that is currently available.
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Ophthalmoplegic migraine : Past, present and future  |
p. 15 |
Vivek Lal DOI:10.4103/0028-3886.60388 PMID:20228457Ophthalmoplegic migraine (OM) is a rare disorder characterized by childhood onset, ophthalmoplegia and migraine headaches. The 3rd cranial nerve is commonly involved in recurrent attacks. Involvement of the sixth and fourth nerves is uncommon. GdMRI discloses enhancement of the nerves. Adult cases are rare and confined to case reports. A viral pathogenesis is considered to be the cause of OM in view of nerve enhancement. We look at the various aspects of OM in children and adults. |
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ORIGINAL ARTICLES |
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Lycopene in treatment of high-grade gliomas: A pilot study |
p. 20 |
Tarun Puri, Shikha Goyal, Pramod K Julka, Omana Nair, Daya N Sharma, Goura K Rath DOI:10.4103/0028-3886.60389 PMID:20228458Background : The therapeutic benefit of lycopene is well established for carcinoma prostate in various clinical trials and has been proposed for other malignancies including high-grade gliomas. Setting and Design : Randomized placebo control study in the Department of Radiation Oncology of a teaching hospital. Patients and Methods : Fifty patients with high-grade gliomas were treated with surgery followed by adjuvant radiotherapy and concomitant paclitaxel. Patients were randomized to receive either oral lycopene (Group A) 8 mg daily with radiotherapy or placebo (Group B). Pre-and post-radiotherapy plasma lycopene levels were measured using high-precision liquid chromatography. McDonald's criteria were used for response assessment. Magnetic resonance imaging of brain and single photon emission computed tomograph were done three-monthly for two visits and six-monthly thereafter. Primary endpoint was response at six months post radiotherapy. Statistical Analysis Used : The data was analyzed using SPSS Software v10.0 (SPSS corporation Chicago IL) by applying Student's t-test, ANOVA F test, Chi-square test and Karl Pearson Correlation Coefficient. Results : Median age was 38 years. The commonest histology was glioblastoma multiforme (n = 32). Pre- and post-treatment plasma lycopene levels in the patients in Group A were 152 ng/ml and 316 ng/ml and in the patients in Group B were 93 ng/ml and 98 ng/ml (P = 0.009). There was non-significant differences in favor of lycopene between Group A and Group B with higher overall response at six months (P = 0.100), response at last follow-up (P = 0.171) and time to progression (40.83 vs. 26.74 weeks, P = 0.089)., The follow-up duration was significantly higher for Group A than Group B (66.29 vs. 38.71 weeks, P = 0.05). Conclusions : Addition of nutrition supplements such as lycopene may have potential therapeutic benefit in the adjuvant management of high-grade gliomas. |
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Anatomical variations of the sural nerve in the leg: A fetal study |
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Nachiket Shankar, Robert Patrick Selvam, Nikhil Dhanpal, Ravikanth Reddy, Ashok Alapati DOI:10.4103/0028-3886.60390 PMID:20228459Background : Because of its clinical importance, numerous studies have been conducted worldwide documenting the anatomical variations of the sural nerve (SN). However, there is a paucity of literature from India concerning the anatomical variations of the SN in the leg. Aim : The aim of this study was to document the anatomical variations of the SN in the leg in Indian fetuses. Setting and Design : Anatomy department and an observational study. Materials and Methods : One hundred and two lower limbs from 51 fetuses of Indian origin (26 male and 25 female) with a crown-rump length ranging between 13 and 41 centimeters were dissected to expose the SN. The mode (Types A, B, C, D) and level of formation, bilateral symmetry, diameter of contributing nerves and presence of an intramuscular course were noted. Statistical Analysis : The relevant percentages were calculated and then tabulated. Results : When male and female fetuses were considered together, the SN was seen to be of Type A in 37.2%, Type B in 26.5%, Type C in 22.5% and Type D in 13.7% of the limbs. Bilateral symmetry of origin was noted in 60.8% of the fetuses. When the SN was formed by union of two or more nerves, the site of union was noted in the lower two-thirds of the leg in 95% of limbs. An intramuscular course of the SN or one of its contributors was seen in 16.7% of limbs. Conclusion : The present study showed differences in the anatomy of the SN compared to earlier studies, warranting further studies in the Indian population. |
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Conditional downregulation of brain- derived neurotrophic factor and tyrosine kinase receptor B blocks epileptogenesis in the human temporal lobe epilepsy hippocampus |
p. 29 |
Xiaohua Hou, Xiaoran Wang, Liming Zhang DOI:10.4103/0028-3886.60392 PMID:20228460Background : Brain-derived neurotrophic factor (BDNF) has been implicated as a potential therapeutic target in temporal lobe epilepsy (TLE). However, whether BDNF exerts an epileptogenic or antiepileptogenic function remains controversial. Materials and Methods : BDNF/tyrosine kinase receptor B (trkB) expression levels were comparatively assessed in the hippocampal tissue of TLE patients with (HS group) and without hippocampal sclerosis (non-HS group) as well as from non-epileptic controls. Results : Immunohistochemistry and immunoblot analysis revealed a marked increase in BDNF/trkB expression in the dentate gyrus and CA3 regions of HS and non-HS groups. The lack of any differences in expression levels was observed between HS and non-HS patients. Meanwhile, treatment with VPA (Valproic acid, anti-epileptic drug) resulted in a significant down-regulation of BDNF/trkB protein expression in sclerotic and non-sclerotic hippocampus (P < 0.001). In contrast, no marked change was noticed in VPA-untreated and OA-treated groups (sodium octanoate). Conclusion : These results suggest that the up-regulation of BDNF/trkB pathway might be at least in part responsible for the epileptogenesis. |
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Mapping of Indian neuroscience research: A scientometric analysis of research output during 1999-2008 |
p. 35 |
Adarsh Bala, BM Gupta DOI:10.4103/0028-3886.60393 PMID:20228461Objective : This study analyses the research output in India in neurosciences during the period 1999-2008 and the analyses included research growth, rank, global publications' share, citation impact, share of international collaborative papers and major collaborative partner countries and patterns of research communication in most productive journals. It also analyses the characteristics of most productive institutions, authors and high-cited papers. The publication output and impact of India is also compared with China, Brazil and South Korea. Materials and Method s: Scopus Citation database was used for retrieving the publications' output of India and other countries in neurosciences during 1999-2008. Results : India's global publications' share in neurosciences during the study period was 0.99% (with 4503 papers) and it ranked 21 st among the top 26 countries in neurosciences. The average annual publication growth rate was 11.37%, shared 17.34% of international collaborative papers and the average citation per paper was 4.21. India was far behind China, Brazil and South Korea in terms of publication output, citation quality and share of international collaborative papers in neurosciences. Conclusion : India is far behind in terms of publication output, citation quality and share of international collaborative papers in neurosciences when compared to other countries with an emerging economy. There is an urgent need to substantially increase the research activities in the field of neurosciences in India. |
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Quantitative videofluoroscopic analysis of penetration-aspiration in post-stroke patients |
p. 42 |
Li Bingjie, Zhang Tong, Sun Xinting, Xu Jianmin, Jiang Guijun DOI:10.4103/0028-3886.60395 PMID:20228462Background : Dysphagia is a common complication of stroke and is a potential cause for aspiration and malnutrition and is also associated with poor outcome. Videofluoroscopic Swallowing Study (VFSS) is the most objective method for evaluation of swallowing disorders. Aim : To investigate the incidence and characteristics of penetration-aspiration in post-stroke patients, and to study the relationship between penetration-aspiration and kinematic parameters of swallow. Patients and Methods : We prospectively studied swallowing function in 105 consecutive post-stroke patients and 100 normal adults by videofluoroscopic swallowing studies. The severity of airway invasion, penetration-aspiration, was studied quantitatively and kinematic parameters of swallow i.e. oral transit time, pharyngeal transit time (PTT), pharyngeal delay timem (PDT), maximal extent of vertical and anterior movement of larynx and hyoid bone for four kinds of boluses were also studied. Logistic regression was used to analyze the association between aspiration and kinematic parameters of swallow. Results : Stroke patients scored significantly higher scores on penetration-aspiration scale than the normal subjects (P < 0.001) during four bolus swallows. Logistic regression analysis showed that PTT, PDT, maximal extent of vertical laryngeal and hyoid movement were statistically associated with the prevalence of aspiration (P < 0.05). Conclusion : Penetration-aspiration is common in stroke patients. Several kinematic parameters of swallow are associated with the presence of aspiration on fluoroscopy. These data demonstrate that VFSS may be helpful for objective identification of dysphagia in stroke patients. |
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Demographic and historical backgrounds of the elderly with nonepileptic seizures: A comparative study |
p. 48 |
Goksemin Acar, Martin C Salinsky DOI:10.4103/0028-3886.60396 PMID:20228463Background : Non-epileptic seizures (NES) are not infrequent in the elderly. However, the data on NES in the elderly is likited. Aim : To study the demographic and historical background of eldely patients with NES and compare the same with the data in the younger patients with NES. Patients and Methods : Patients with NES over 55 years of age and the next two consecutive patients with NES between ages 18 and 45 were compared in terms of demographic and historical features, psychiatric evaluation and MMPI testing. Results : Of all the 128 patients with NES, 13 (10.6%) were over 55 years of age. History of physical/sexual abuse was high in both the groups. The mean length of time for NES diagnosis was longer in the elderly (13.38 ± 15.33 vs. 6.15 ± 8.04 years; P < 0.05). Majority of the patients with NES were on AEDs without evidence of epilepsy and almost half in both the groups were using benzodiazepines. Conclusion : In demographic and historical aspects old and young patients do not display major differences; however, the diagnosis is significantly delayed in the elderly. Early diagnosis with video EEG is recommended to avoid potential long-term risks associated with inappropriate treatments. |
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Parkinson's disease: Functional changes in frontal and parietal cortex using 18 F- fluoro-deoxy glucose positron emission tomography/computed tomography |
p. 53 |
Zhongyu Hou, Shuhui Hong, Bo Sun, Xiangtao Lin, Qingwei Liu, Shuzhan Yao, Shuwei Liu DOI:10.4103/0028-3886.60397 PMID:20228464Background : In Parkinson's disease (PD) there is increasing evidence to suggest motor function changes of the cerebral cortex occur in addition to the pathological changes in the extrapyramidal system. Aims : To explore the functional changes in the frontal and parietal cortex in PD cat model. Settings and Design : Twenty-four healthy male cats were divided into four animal model groups with the injection of 1-methyl-4-phenyl-1, 2, 3, 6-tetrahydropyridine (MPTP), and one control group. Materials and Methods : Cats in both the animal model and control groups were observed for the behavioral changes. They were also examined by 18 F-fluoro-deoxy glucose positron emission tomography/computed tomography ( 18 F-FDG PET/CT). Region of Interest (ROI) was determined by 18 F-FDG intensity and semi-quantitative analysis was employed after detecting the standard absorption value (SUV). Statistical Analysis : Statistical significance was evaluated by ANOVA. Results : Compared to control group, the 18 F-FDG intensity and SUV were found normal on both the sides of frontal and parietal cortex in the PD models on the second day (P > 0.05), and on the fifth day, they were reduced significantly on both the sides of frontal cortex exclusively (P < 0.05). Moreover on the eighth day, the SUV of both frontal cortexes was reduced, while it was increased in both parietal cortex (P < 0.05). Finally on the eleventh day, the SUV remained stable in both the frontal cortex, and was back to normal level in both the parietal cortex. Conclusions : Functional disorders exist in the frontal cortex of PD animals and aggravate with time. Transient functional enhancement in the parietal cortex of PD cats might be a compensation for the dysfunction of frontal cortex. |
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Effect of early treatment in polymyositis and dermatomyositis |
p. 58 |
Peyman Naji, Farhad Shahram, Abdolhadi Nadji, Fereydoun Davatchi DOI:10.4103/0028-3886.60398 PMID:20228465Background : Idiopathic inflammatory myopathies, dermatomyositis (DM) and polymyositis (PM) are rare but are potentially treatable. Aim : To compare the effect of early and late treatment in patients with PM and DM. Patients and Methods : The study included all the adult patients with definite diagnosis of PM or DM treated for at least 12 months. The patients were divided into two groups: Early Group - treatment within three months and Late Group - treatment after three months. The number of patients with positive therapeutic response, remission in less than one year and the mean time elapsed for reaching the remission were assessed and compared between the two groups. Chi-square test, Fisher's exact test, t-test and Pearson correlation test were used for data analysis. Results : The analysis included 65 patients, 42 with DM and 23 with PM. Late Group included 24 patients (seven PM and 17 DM), while Early Group included 41 patients (16 PM and 25 DM). Positive therapeutic response, remission rate within one year was higher in Early Group (80% vs. 46%, P: 0.004). The mean time needed to achieve remission was much less with early treatment (5.5 vs. 11.9 months, P: 0.003). The relapse rate was also lower in Early Group (5% vs. 25%, P < 0.02). The comparison of treatment outcomes showed the same results in both PM and DM, but it was statistically significant in patients with DM. Conclusions : Early treatment in patients with PM and DM is associated with higher remission rates, shorter treatment period and low complication rates. |
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Evolution of endovascular management of intracranial dural arteriovenous fistulas: Single center experience |
p. 62 |
Rashmi Saraf, Manish Shrivastava, W Siddhartha, Uday Limaye DOI:10.4103/0028-3886.60400 PMID:20228466Aim : We aim to focus on the treatment of intracranial dural arteriovenous fistulas (DAVF) with emphasis on the evolution of endovascular management at our center over the last 13 years. We also aim to highlight the present treatment strategy, considering all the embolic agents available with us. Setting and Design : This is a retrospective study of 99 patients of DAVFs treated from December 1995 to March 2009. Patients and Methods : Seven patients were found to have spontaneous thrombosis when taken up for treatment. The other 92 patients underwent endovascular treatment through transarterial or transvenous routes using polyvinyl alcohol particles, glue, detachable platinum coils or injection onyx as embolic agents. The treatment strategies have evolved over a period of time with changing philosophies and availability of different embolic agents. Results : Transverse- sigmoid and cavernous sinuses were the commonest sites of DAVFs. Intracranial hemorrhage was common presentation. Transarterial PVA embolization was performed in four patients, transarterial glue in 15, transvenous embolization in 33 and transarterial Onyx in 36. Direct puncture and packing of the sinuses was done in four patients. Cure was achieved in 80 out of 92 patients (cure rate of 87%). Patients who had Onyx embolization had cure rate of 92% (33 out of 36 patients). 14 complications were seen of which two were in the Onyx group. Conclusion : Embolization of DAVFs has evolved over the last decade and has become the treatment of choice with high cure rates and improved safety. We propose the use of Onyx as the embolic agent of choice in the treatment of DAVFs. |
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Is there a need to diagnose Rathke's cleft cyst pre operatively? |
p. 69 |
A Raghunath, S Sampath, B Indira Devi, BA Chandramouli, G Jagath Lal, Yasha T Chickabasaviah, Rose Dawn Bharath DOI:10.4103/0028-3886.60402 PMID:20228467Background: Rathke's cleft cyst is a rare benign sellar lesion. The exact preoperative diagnosis of this lesion by clinical and radiological features is difficult. Hence it is often misdiagnosed as craniopharyngioma. Aim: To identify the radiological pointers for pre operative diagnosis of Rathke's cleft cyst. Materials and Methods: This study presents the details of nine patients who were operated in our institution between 1998 and 2008. Radiological and histopathological variations were studied. Results: The possibility of Rathke's cleft cyst was considered pre operatively in one patient only. On reviewing the images, characteristic imaging findings were observed in a few cases. Conclusion: As minimally invasive trans-sphenoidal approach is sufficient for treating these lesions, pre operative diagnosis is important. |
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Quality of life with special respect to depression after surgical treatment of hypertensive basal ganglia hemorrhage |
p. 74 |
Jian Hai, Lin Zhang, Fei Wang, Jue-Feng Wan, Qing-Gang Pan DOI:10.4103/0028-3886.60403 PMID:20228468Background : Hypertensive intracerebral hemorrhage is associated with high mortality and morbidity. Place of surgery in the primary supratentorial intracerebral hemorrhage is uncertain and the data on the long-term functional outcome of surgery in these patients is limited. Aim : The aim of the study was to determine long-term functional outcome of patients undergoing surgical treatment for hypertensive basal ganglia hemorrhage, especially in respect to depression. Study Design and Settings : Retrospective analysis of database of 44 patients undergoing craniotomy for hypertensive basal ganglia hemorrhage between December 2002 and May 2007. Materials and Methods : Long-term was defined as at least 18 months after craniotomy. Neurological status of the patients at admission was assessed by National Institute of Health Stroke Scale (NIHSS) and Glasgow Coma Scale (GCS). Outcome data consisted of the items including functionality, depression and quality of life. Tests applied included Barthel Index (BI), modified Rankin Scale (mRS), Beck Depression Inventory (BDI) and stroke-specific quality of life (SSQOL) scale. Results : The long-term mortality rate was 29.5% (13/44). Of the 31 survivors, 21 (67.7%) patients had a BI $ 60, 23 (74.2%) patients had a mRS <4 and 21 (67.7%) patients had a SSQOL $ 60%, each representing a favorable outcome. In retrospect, 19 (61.3%) patients approved the surgery. Eighteen (58.1%) patients developed depression (BDI > 9), which was related to high NIHSS and low GCS score preoperatively, low BI, high mRS and low SSQOL postoperatively. Conclusions : The study reveals that depression is a common long-term complication after surgical treatment of hypertensive basal ganglion hemorrhage. Both the NIHSS and GCS scores before operation have critical roles in patient's quality of life associated with depression. |
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Can infusion tests be recommended for patients with giant hydrocephalus? |
p. 78 |
Krzysztof Cieslicki, Ryszard Czepko DOI:10.4103/0028-3886.60405 PMID:20228469Background : The problem of adequate diagnosis of hydrocephalus followed by administration of an effective treatment has not yet been properly solved. Specifically, this pertains to the decision about the surgical insertion of a flow diverting device. Aims : A lumbar infusion test was used to examine the compensatory parameters of intracranial space in giant hydrocephalus. The early and late results of shunt implantation were analyzed together with complications after surgery. Settings and Design : In-house software was used offline to adjust the dynamic intracranial pressure (ICP) response to infusion. Patients and Methods : Nine patients with giant hydrocephalus were the subjects for the study. We analyzed recordings of the response in ICP to the 2 ml/min infusion of saline. We performed computerized identification of outflow resistance and intracranial compliance based on the truncated (30- 100%) ICP response and assessed the stability of estimates over time. Eight out of nine patients were shunted. Monitoring of patients was followed for a period of up to 9 months. Results : Five out of eight shunted patients improved within a few days of surgery. During follow-up five patients developed various complications. A definite improvement was noted in four patients. The improvement rate did not correlate with any of the compensatory parameters. Most of the patients studied exhibited a lack of intracranial space reserve, a significantly reduced rate of CSF secretion, and a slightly elevated value of outflow resistance. Conclusions : The infusion test showed itself to be more useful as a way of revealing the compensatory parameters of the intracranial space than as a prognostic tool. The outcome of shunted patients with giant hydrocephalus was uncertain, owing to the relatively high rate of complications. We may therefore suggest that the diagnosis of giant hydrocephalus is a relative contraindication to implantation, as well as to the performance of an infusion test. |
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BRIEF REPORTS |
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The epidemiology and clinical management of craniocerebral injury caused by the Sichuan earthquake |
p. 85 |
Lu Jia, Guo-ping Li, Chao You, Hao Li, Si-qing Huang, Chao-hua Yang, Hai Xiong, Yi-jun Zeng DOI:10.4103/0028-3886.60406 PMID:20228470Earthquake is one of the most devastating natural disasters that threaten human lives. Worldwide more than 3 million deaths have been caused by earthquakes in recent 20 years. To analyze clinical features of head injuries after Sichuan earthquake. From May 12 to June 12, 2008, Departments of Neurosurgery in major Hospitals in Sichuan Province admitted 1368 patients with head injuries caused by the Sichuan earthquake; the epidemiology, mechanism, severity, complications, treatments and outcome of head injury were retrospectively analyzed. Of the 1368 patients, 755 were men and 613 women. Collapsing building was the most important cause of head injury. Most of the patients, 85% had mild to moderate head injury. The type of injury was open scalp injury in 65% of patients. About 47% of the head-injured patients were admitted within 72 h after earthquake. Skeletal bone fracture was the most common associated injury (9%). Only 98 patients received surgery. Glasgow Outcome Scale on discharge or transfer was: 5 in 1121 (82%) patients, 4 in 173 (13%) patients, and 3 or less in 74 (5%) patients. Overall 33 (2%) patients died. The characteristics of Sichuan earthquake-related head injury are quite distinct. Early standardized treatment is important to have better outcomes. |
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Ischemic hyperintensities on T1-weighted magnetic resonance imaging of patients with stroke: New insights from susceptibility weighted imaging  |
p. 90 |
Neeraj N Baheti, Ajith Cherian, PR Wattamwar, Chandrasekharan Kesavadas, Bejoy Thomas DOI:10.4103/0028-3886.60408 PMID:20228471Hyperintensities on T1-weighted magnetic resonance imaging (MRI) in the setting of brain ischemia are usually considered hemorrhagic transformations. Such changes can also be seen due to "incomplete infarction" with selective neuronal loss. Arguments regarding the cause of these T1 hyperintensities have shuttled between gemistocytic astrocyte accumulation, tissue calcification and paramagnetic substance deposition. Susceptibility weighted imaging (SWI), a sensitive modality for detecting paramagnetic agents and blood products, has never been used to resolve this issue. The study was aimed to evaluate the SWI signal changes of T1 hyperintense lesion in stroke patients and understand its usefulness in differentiating a hemorrhagic infarct and an incomplete infarct. All the seven patients with infarct, having hyperintensities on T1 weighted MRI seen over the last one year were subjected to SWI. In none of the patients SWI failed to show any blooming. By doing SWI for T1-weighted hyperintensities, we can differentiate hemorrhagic infarct and a non-hemorrhagic "incomplete infarct". This differentiation will immensely help in planning management strategy and prognostication. |
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Spectrum of emergency department presentation in patients of acute intermittent porphyria: Experience from a North Indian tertiary care center |
p. 95 |
Susheel Kumar, Navneet Sharma, Manish Modi, Aman Sharma, Sushil Mahi, Subhash Varma DOI:10.4103/0028-3886.60410 PMID:20228472Of the porphyrias, acute intermittent porphyria (AIP) is the most frequently encountered porphyria. The clinical characterestics of thirteen patients of AIP who presented to the emergency department were analyzed. The most common precipitating factor was drugs. Eleven patients presented with pain abdomen. Neurological manifestations included: Seizures in six and motor weakness in six. Of the four patients with hyponatremia, three had associated neuropathy and the fourth patient demonstrated a severe course marked by pontine-extrapontine myelinolysis and profound adrenergic activity. In conclusion, even though AIP is less frequently reported from India the emergency physicians should be vigilant to exclude the diagnostic possibility of AIP in a patient with an appropriate clinical setting. |
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A rapid polymerase chain reaction-based test for screening Steinert's disease (DM1) |
p. 99 |
Khalil Hamzi, Hanane Bellayou, Ilham Slassi, Sellama Nadifi DOI:10.4103/0028-3886.60411 PMID:20228473Myotonic dystrophy (DM) is a multisystemic neuromuscular disorder caused by a dynamic mutation of (CTG) trinucleotide repeats in the 3' untranslated region of the myotonic dystrophy protein kinase gene (DMPK). The aim of the present study was to establish the use of polymerase chain reaction (PCR)-based simple and rapid method for initial sample screening. Only a minority of samples were tested positive with the above method and need to be detected by tri primer (TP)-PCR and southern blotting which is more time consuming and involves use of radioactive material. This study concerned 24 patients from nine families with a clinical diagnosis of the DM1. DNA extracted from the blood was used for amplification of the triplet repeat sequences at the DMPK loci. We obtained two bands for the normal subjects and one band for patients corresponding to normal DMPK allele, confirmed by the TP-PCR and the southern blot. This rapid test for initial screening of samples for the presence of DMPK mutations is economical and reliable method. This method reduces the number of samples needing TP-PCR and southern blotting. |
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Recovery of oculomotor nerve palsy following surgical clipping of posterior communicating artery aneurysms |
p. 103 |
Vamsi K Yerramneni, P Sarat Chandra, Manish K Kasliwal, Sumit Sinha, Ashish Suri, Aditya Gupta, Bhawani S Sharma DOI:10.4103/0028-3886.60413 PMID:20228474Oculomotor nerve palsy (ONP) can be the presenting feature of ruptured or an unruptured posterior communicating artery (PComA) aneurysm. Etiopathogenesis and recovery of ONP following treatment of PComA aneurysm has been a subject of controversy. Case records of thirteen patients (mean age 42 years (range 19-65 years), M:F: 3:10) with PComA aneurysm and ONP who underwent surgery over a period of eight years were analyzed. Twelve patients presented with subarchnoid hemorrhage and one had unruptured aneurysm. The interval between the onset of symptoms and surgery ranged between 4 and 70 days (mean 16.7 days). All the four patients with partial ONP had complete recovery and of the nine patients with complete ONP, six had complete recovery and three had partial recovery. The recovery of the ONP is influenced by the degree of preoperative deficit. Although clipping of the aneurysm probably expedites the recovery of the third nerve palsy, age, sex and timing of surgery may not have any influence on the recovery patterns. |
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CASE REPORTS |
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Renal tubular acidosis presenting as respiratory paralysis: Report of a case and review of literature |
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J Kalita, PP Nair, G Kumar, UK Misra DOI:10.4103/0028-3886.60415 PMID:20228475Respiratory paralysis due to renal tubular acidosis (RTA) is rare. We report a 22-year-old lady who developed severe bulbar, respiratory and limb paralysis following respiratory infection. She had hypokalemia (1.6 meq/L) and hyperchloremic (110 meq/l) acidosis (pH 7.1). She was diagnosed as distal RTA by ammonium chloride test. She improved following sodium bicarbonate and potassium supplementation. RTA should be differentiated from familial periodic paralysis (FPP) because acetazolamide used in FPP aggravates RTA and sodium bicarbonate used in RTA aggravates hypokalemic periodic paralysis. |
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Intra familial phenotypical variations in adrenoleukodystrophy |
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Jayaprakash Gosalakkal, Anand Prasad Balky DOI:10.4103/0028-3886.60418 PMID:20228476Adrenoleukodystrophy (ALD) is an X-linked recessively inherited peroxisomal disorder, characterized by progressive white-matter demyelination of the central nervous system and adrenocortical insufficiency. It has a wide phenotypical variability ranging from symptomatic childhood cerebral form to the asymptomatic with biochemical defects only; sometimes within the same family. We report a family of three siblings diagnosed with ALD confirmed with the mutations in ABCD1 gene having phenotypical variability ranging from pure adrenal insufficiency to progressive neurodegeneration in the same family. The mother was identified as the carrier and maternal uncle was diagnosed with Adrenomyeloneuropathy. We discuss the variable presentation in our family and the possible causes of phenotypical variability. |
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Cardioembolic stroke on unaffected side during thrombolysis for acute ischemic stroke |
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Arun Garg, Amitabh Yaduvanshi, Kapil Dev Mohindra DOI:10.4103/0028-3886.60419 PMID:20228477Cardiac thrombus is not an established contraindication to thrombolysis with intravenous tissue plasminogen activator in acute ischemic stroke. Recurrent ischemic stroke involving an initially unaffected arterial territory during the course of thrombolysis in stroke has been reported, but remains exceptionally rare. We present a case that developed cardioembolic stroke on the previously unaffected side during thrombolysis for acute ischemic stroke. |
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Intrathecal baclofen pump implantation in a case of stiff person syndrome |
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Bobby Varkey Maramattom DOI:10.4103/0028-3886.60420 PMID:20228478Stiff person syndrome (SPS) is a rare disorder characterized by severe axial and/or limb rigidity, spasms and continuous motor unit activity on EMG. Symptomatically these patients are usually treated with GABAergic medications. We present a 45-year-old woman with SPS, who failed high dose of GABAergic medication. With intrathecal baclofen by intrathecal baclofen pump she had a significant improvement in her symptoms and quality of life. |
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Progressive multifocal leukoencephalopathy in a patient with idiopathic CD4+T lymphocytopenia |
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Vinod Puri, Neera Chaudhry, Parveen Gulati, Nisheeth Patel, Medha Tatke, Sanjiv Sinha DOI:10.4103/0028-3886.60423 PMID:20228479Progressive multifocal leukoencephalopathy (PML) is demyelinating of central nervous system caused by JC virus infection and often occurs in immunodeficient individuals. We report progressive PML in a 30-year-old male with idiopathic severely depressed CD4+T lymphocyte count. He was sero-negative for human immunodeficiency virus (HIV) infection. |
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Non-traumatic intraosseous cerebrospinal fluid cyst with associated craniovertebral anomalies: A case report and suggested mechanism of formation |
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Tony Abraham Thomas, Damodar Rout DOI:10.4103/0028-3886.60424 PMID:20228480Intraosseous cerebrospinal fluid (CSF) cysts, also known as intra-diploic arachnoid cysts, are usually of post-traumatic origin. Lesions presenting without a history of trauma are rare and are presumed to be of congenital origin. We report a young man with an intraosseous cystic lesion containing CSF and communicating with the intracranial subarachnoid space through a dural opening. The presence of other congenital defects of the skull and spine could be a pointer in this direction. Surgical management involves craniectomy and repair of the dural defect followed by cranioplasty for the bony defect. |
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Vertebral body hyperostosis as a presentation of Pott's disease: A report of two cases and literature review |
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Alok Umredkar, Sandeep Mohindra, Rajesh Chhabra, Rahul Gupta DOI:10.4103/0028-3886.60426 PMID:20228481Vertebral body tuberculosis has numerous forms of presentations. We present two patients of Pott's disease, in whom vertebral body hyperostosis was the radiological presentation. Both the patients had massive hyperostosis of vertebral bodies leading to the obliteration of the spinal canal and neurological deficits. The first case had associated lupus vulgaris, while the second patient had milliary mottling of lungs and calcified bilateral psoas muscles. Surgical decompression, followed by full-dose chemotherapy, remains the hallmark of management of this disease. |
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Dural based primary osteosarcoma in right fronto-temporal region:Case report with review of literature |
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Nandita Ghosal, Ravi Dadlani, Sunil V Furtado, Naman Bagdi, AS Hegde DOI:10.4103/0028-3886.60428 PMID:20228482We report a case of primary dural based osteosarcoma in the right fronto-temporal convexity in a 43-year-old female who presented with a short history of seizure and headache. Radiologic evaluation revealed a well defined brightly enhancing extra-axial lesion in the right fronto-temporal region with a dural tail around the sylvian fissure. The overlying bone was uninvolved. Paraffin section of the tumor showed plump cells with moderate nuclear and cellular pleomorphism with eosinophilic extracellular material (osteiod) between the cells. At a few places, lace like osteiod was seen encasing individual cells signifying osteiod being formed by tumor cells. Immunohistochemistry for epithelial membrane antigen was focally positive and negative for S-100 protein and glial fibrillary acidic protein. A final histopathological diagnosis of dural based primary osteosarcoma of the right fronto-temporal region was rendered. To the best of our knowledge this will be the eighth such case in literature. |
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Infectious intracranial aneurysm: Endovascular treatment with Onyx case report and review of the literature |
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Pei Chao Zhao, Jin Li, Min He, Chao You DOI:10.4103/0028-3886.60429 PMID:20228483Intracranial infectious (mycotic) aneurysms are very rare, but continue to be challenging and technically demanding, which need careful diagnosis and therapy. We present an 18-year-old man with an intracranial infectious aneurysm located on the left posterior cerebral artery who was successfully treated with endovascular embolization by a liquid embolic agent (Onyx) and who recovered well. |
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LETTERS TO EDITOR |
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Paroxysmal exercise-induced dystonia with optic atrophy: A-30-year follow-up |
p. 135 |
AJ Larner, A Jacob DOI:10.4103/0028-3886.60399 PMID:20228484 |
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Bilateral carpal tunnel syndrome in a child with type 1 diabetes mellitus |
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Nilgun Selcuk Duru, Hurtan Acar, Semih Ayta, Murat Elevli DOI:10.4103/0028-3886.60401 PMID:20228485 |
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Coil embolization of a ruptured anterior communicating aneurysm in Takayasu's arteritis |
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Boby Varkey Maramattom DOI:10.4103/0028-3886.60404 PMID:20228486 |
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Fatal course of cerebral vasculitis induced by neuroborreliosis |
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Fredrik Buchwald, Kasim Abul-Kasim, Johan Tham, Bjarne U Hansen DOI:10.4103/0028-3886.60407 PMID:20228487 |
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Facio-auriculo-vertebro-cephalic spectrum of Goldenhar syndrome |
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Amit Mahore, Nitin Dange, Santhosh Nama, Atul Goel DOI:10.4103/0028-3886.60409 PMID:20228488 |
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Gorham's disease of the calvarium |
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Ji Zhang, Jin Li, Liu Ling, Yue Kang Zhang DOI:10.4103/0028-3886.60412 PMID:20228489 |
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Complete heart block complicating intracranial aneurysm surgery in a pregnant patient |
p. 146 |
Pragati Ganjoo, Deepa V Navkar, Monica S Tandon DOI:10.4103/0028-3886.60414 PMID:20228491 |
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Lumbosacral epidural lipomatosis due to prolonged steroid intake causing cauda equina syndrome |
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Nabil S Mahmood, Shiran Shetty, Sajan Andrews DOI:10.4103/0028-3886.60416 PMID:20228490 |
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Primary angiitis of central nervous system: Tumor-like lesion |
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R Shiva Kumar, Atampreet Singh, Chaturbhuj Rathore, Chandrasekharan Kesavadas DOI:10.4103/0028-3886.60417 PMID:20228492 |
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Compound "elevated" fracture of the cranium |
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S Bhaskar DOI:10.4103/0028-3886.60421 PMID:20228493 |
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Intracranial moving bullet syndrome |
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Alok A Umredkar, Sandeep Mohindra DOI:10.4103/0028-3886.60422 PMID:20228494 |
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Contrecoup epidural hematoma |
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Satoru Takeuchi, Yoshio Takasato, Hiroyuki Masaoka, Naoki Otani DOI:10.4103/0028-3886.60425 PMID:20228495 |
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A rare case of metachronous double spinal dural arteriovenous fistula |
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Amit Dagar, Manish K Kasliwal, Ashish Suri, Subhash Kumar, Ajay Garg, Bhawani S Sharma DOI:10.4103/0028-3886.60427 PMID:20228496 |
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Spinal schwannoma: An unusual cause of acute subarachnoid hemorrhage |
p. 155 |
Liyong Sun, Zan Chen, Fengzen Jian, Feng Ling DOI:10.4103/0028-3886.60430 PMID:20228497 |
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Cerebrospinal fluid rhinorrhea and acquired anterior basal encephalocoele in a patient with colloid cyst of the third ventricle |
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Kythasandra Shivakumar Deepak, Chandrasekharan Kesavadas, Tirur Raman Kapilamoorthy, Gireesh Menon DOI:10.4103/0028-3886.60431 PMID:20228498 |
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Post gamma knife subarachnoid hemorrhage and hydrocephalus |
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Chandrasekharan Kesavadas, Biji Bahuleyan, Somenath Chatterjee, Tirur Raman Kapilamoorthy DOI:10.4103/0028-3886.60432 PMID:20228499 |
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Melanotic craniopharyngioma, an unusual variant in a young female |
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Nandita Ghosal, Ravi Dadlani, Sai Kiran, AS Hegde DOI:10.4103/0028-3886.60433 PMID:20228500 |
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NEUROIMAGES |
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Simultaneous involvement of fifth and seventh nerves by vertebrobasilar dolichoectasia presenting as painful tic convulsif - Demonstrated by MR imaging |
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Pallavi Aga, Umesh Parashari, Anit Parihar, Rajesh Yadav, Ragini Singh, Neera Kohli DOI:10.4103/0028-3886.60394 PMID:20228501 |
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Spontaneous rupture of an intracranial teratoma |
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Chen Xuzhu, Jiang Tao, Wang Junmei, Dai Jianping DOI:10.4103/0028-3886.60391 PMID:20228502 |
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