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The characteristic features of Madras motor neuron disease (MMND) are onset in the young in the first two decades, sporadic occurrence, facial and bulbar paralysis, sensorineural hearing impairment, asymmetrical weakness of limbs and pyramidal signs with a slow progression. The majority of the cases reported are from South India. MMND variant has the additional features of optic atrophy and cerebellar signs. We are reporting a 48 year old female of MMND who had persistent fasciculations of chin, with electromyographic features of fasciculations and fibrillations in mentalis muscle. Chin fasciculations, a rare clinical feature, is now described for the first time in Madras motor neuron disease adding a new feature to the clinical constellation of symptoms

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Aim: We aimed to study the cost of stroke, its predictors, and the impact on social determinants of the family. Settings and Design: This prospective study was done in the Stroke unit and Neurology clinic between April 2009 and October 2011. Materials and Methods: All first ever stroke patients during the study period were enrolled. Direct and indirect costs at admission, at 1 and 6 months follow-up were obtained. The follow-up included information about the patient's poststroke outcome using modified Rankin Scale (mRS), work status, modifications made at home, loan requirement, etc., Results: Two hundred patients were enrolled in this study and final analysis was performed on 189 patients. The mean age was 58 ± 13 years and 128 (67.7%) were men. Majority (54%) were living in a joint family. The mean overall cost of stroke per patient was rupees (INR) 80612 at 6 months. Higher income (P = 0.008), poor outcome (mRS >2) (P = 0.001), and length of hospital stay (P = 0.001) were the cost driving factors of total cost of stroke at 6 months. There was a decline in the requirement of help (P < 0.0001) and need for loan (P = 0.003) at 6 months follow-up. Conclusions: Direct medical cost or acute care of stroke accounted for a major component of cost of stroke. Poor outcome, length of hospital stay, and higher income were the cost driving factors. The socioeconomic impact on the family decreased at follow up probably due to joint family system.

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Idiopathic generalized epilepsy (IGE) is a common type of epilepsy. Strong support for a genetic role in IGE comes from twin and family studies. Several subtypes of IGE have been reported but families often have members affected with different subtypes. Major advances have been made in the understanding of genetic basis of monogenic inherited epilepsies. However, most IGEs are complex genetic diseases and some susceptible IGE genes are shared across subtypes that determine subtypes in specific combinations. The high throughput technologies like deoxyribonucleic acid microarrays and sequencing technologies have the potential to identify causative genes or loci in non-familial cases.

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Little was known about the human prefrontal cortex till recently. It was thus labeled as the "silent area," "uncommitted cortex." It not only constitutes the largest component of the human brain but is the latest evolutionary addition to the mammalian brain. It endows the human beings with qualities that differentiate humans from all other animals. During the last couple of decades the advent of modern electrophysiological and imaging (functional magnetic resonance imaging, proton emission tomography, SPECT) techniques have provided a wealth of insight into its role in memory, thought, emotions, moral judgment, social behavior, evaluating rewards, and assessing its fairness or otherwise and above all self-awareness. This brief review summarize the recent significant observations on its functions and connectivity which would interest the cognitive scientists and clinicians alike.

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Purpose: The aim of this study was to evaluate the feasibility of endovascular treatment (EVT) for unruptured small wide-necked ophthalmic segment aneurysms (OSAs) of the internal carotid artery (ICA). Materials and Methods: Between January 2010 and May 2013, 17 patients with 19 unruptured small wide-necked OSAs received EVT. Occlusion rates were classified as total/near-total (95-100%), subtotal (80-95%) and partial (<80%) occlusions. This was determined by immediate and follow-up angiography. Follow-up outcome was assessed by using modified ranking scale (mRS). Results: EVT was successfully performed in all the patients: Coiling only in 2 (10.5%) and stent-assisted coiling in 17 (89.5%) of the aneurysms. The immediate total/near-total occlusion was seen in 1 (5.3%), sub-total occlusion in 8 (42.1%) and partial occlusion in 10 (52.6%). Follow-up angiography performed at 9.4 (±4.7) months revealed total/near-total occlusion in 13 (68.4%), subtotal occlusion in 5 (26.3%) and partial occlusion in 1 (5.3%). At the end of the follow-up period of 17.4 (±6.9) months, no aneurysm rupture was found and 16 (94.1%) of the patients had mRS scores of: Grade 0 in (5.9%) an mRS 1 in the remaining. Conclusions: EVT may be feasible and effective treatment option for unruptured small wide-necked ophthalmic aneurysms of the ICA.

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Introduction: Suprasellar lesions present a surgical challenge due to their complex relationship with surrounding neurovascular structures. Of the approaches for these lesions, bifrontal basal interhemispheric approach (BBIA) gives a midline perspective of suprasellar anatomy and has certain advantages over lateral approaches. Materials and Methods: We retrospectively reviewed 48 patients with suprasellar lesions operated over 7 years via BBIA. Patient records, operation notes, radiology, and outpatient files were scrutinized to collect data. Results: During the study period 48 patients (mean age 33 years, M:F 1.5:1) were operated by this approach. The clinical features included: Visual field deficits in 33 (69%) patients, with 6 of them being blind, diabetes insipidus in 7, growth retardation in 5, and subarachnoid hemorrhage in three patients. Cranopharyngiomas (52%) and meningiomas (16.7%) were the most common pathologies. Dyselectrolytemia (18, 40%) and diabetes insipidus (15, 33%) were the most common complications. Postoperative seizures, meningitis, subdural effusion, and retraction site contusion were seen in 12 (27%), 5 (11%), 4 (9%), and 1 (2.2%) patient, respectively. Three patients died postoperatively and 19 (40%) patients required hormone replacement therapy. Amongst the patients with preoperative visual deficits, 23 (70%) had improvement in visual functions, in six (20%), there was no change and four (8.3%) patients had visual deterioration. Conclusion: BBIA provides a true midline perspective and orientation, and permits complete and safe removal of midline suprasellar lesions in majority of cases. This approach is especially useful in retrochiasmatic tumors and in residual/recurrent tumors providing virgin plane of dissection.

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Objective: Neurosurgeons often have to rely on judgments of junior staffs to decide on patients whom they cannot attend immediately. Viber is a free to use application for image transfer on Internet. We evaluated the use of viber in neurosurgical scenario, to show it is cheap, fast, accessible, reliable and feasible. Materials and Methods: We conducted a prospective study from March 2013 to July 2013. Residents were taught to take sharp pictures and upload them immediately using viber on Internet. Primary endpoints were discordance between opinion of residents and consultants on viber images and subsequent actual image evaluation and time delay in decision-making. Discordance was considered significant if it changed management decision. Results: During the study period, 120 (mean age: 42 years, 58% males) patients were enrolled. Wi-Fi is freely available in the institute and thus no costs were involved. Decision could be made on images received on viber at an average of 20 min. There was discordance in 56.7% cases between residents' reports and images on viber, which was significant in 88.2% cases. However in 5% cases decision changed after actual images were reviewed. Of all imaging modalities, computed tomography angiographic images were associated with statistically significant discordance (P <0.05). Conclusion: This study suggests that the use of viber app in neurosurgery can be an easy fast reliable and almost free mode of communicating images enabling a quick decision. However this cost-effective method should be used with caution particularly with imaging modalities, which require processing and review on console.

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Background: The transpedicular route in percutaneous vertebroplasty (PVP) is a well-established approach for the treatment of vertebral compression fractures (VCFs). However, the value of simple transpedicular biopsy in VCFs is less addressed. The purpose of this study is to evaluate the value of transpedicular biopsy during PVP for uncovering the malignancy in VCFs in a 10-year retrospective study. Materials and Methods: During the study period of the 1019 patients who underwent PVP for VCFs, 450 patients comprising of 127 male and 323 female underwent transpedicular biopsy during PVP for 705 fractured vertebras. The medical records were analyzed for age, gender, imaging studies, operation notes, pre-operative and post-operative diagnoses, date of vertebroplasty and biopsy, vertebral level and pathological reports. Results: Pathology of the specimens of the 450 patients confirmed non-malignant VCFs in 389 (86.44%) and malignancy in 61 (13.56%). The malignant pathology included: 52 (11.56%) distant metastases to vertebra, in 3 (0.67%) of the spinal metastases was unsuspected and in 49 (10.89%) of them the malignancy was suspected pre-operatively. There were 9 (2%) primary spinal malignancies, 2 (0.44%) unsuspected multiple myeloma and 7 (1.56%) pre-operatively suspected primary malignancies. The frequency of unsuspected malignancy was 1.11% (5/450) in this study. There was no complication associated with transpedicular biopsy during PVP. Conclusions: VCFs harbored 1.11% of unexpected malignancy. During the vertebroplasty, concomitant transpedicular vertebral biopsy is a safe and useful procedure for distinguishing non-malignant from malignant compression fractures, especially in diagnosing unsuspected malignancy.

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In this report, we describe a new mutation located in the coiled 1B domain of desmin and associated with a predominant cardiac involvement and a high degree of cardiac sudden death in a large Indian pedigree with 12 affected members. The index cases was 38-year-old man who presented with progressive difficulty in gripping footwear of 5 years duration with the onset in the left lower limb followed by right lower limb in 6 months. 3 years from onset, he developed lower limb proximal and truncal muscle weakness. There was mild atrophy of the shoulder girdle muscles with grade 3 weakness, moderate wasting of thigh and anterior leg muscles with proximal muscle weakness and foot drop. At 40 years, he had a pacemaker implanted. The 9 exons and intronic boundaries of the desmin gene were sequenced and a heterozygous nucleotide change c. 734A > G in exon 3 was identified.

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Background: Severe traumatic brain injury (TBI) is a major cause of morbidity and mortality. Reduction of thalamic volumes were seen in upto 80% of patients who survived for more than 3 months after TBI. However, the same may not be true in patients who died earlier following TBI. Aims: To to study the thalamus for evidence of any injury in short term survivors of TBI (<5 days) using immunohistochemistry to look for evidence of acute thalamic injury. Materials and Methods: A cross sectional prospective study was done in which autopsy specimens of short term survivors of TBI (<5 days) were studied for histopathological changes. Results: A total of 16 patients with a mean age of 37.8 years were included in the study. CT scan revealed acute subdural haematoma in 10, contusions in 4 patients, extradural haematoma and depressed fracture in 1 each, and diffuse axonal injury in 1 patient. Seven patients required surgery in the form of a decompressive hemicraniectomy. The histopathological analysis of the bilateral thalami showed evidence of congestion of the cerebral capillaries in 8 patients. Axonal retraction balls were seen in 8 patients, myelin breakdown products were seen in 14 patients and axonal swelling was seen in 14 patients. Conclusions: Thalamic injury is universal in the setting of severe TBI in patients who have decreased survival and may be a significant factor for the poor outcome in these patients.

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An inflammatory myofibroblastic tumor (IMT) is a rare lesion with an uncertain prognosis and a disorder difficult to classify. IMTs are a heterogeneous group of lesions, sometimes indistinguishable from meningiomas and other expanding or inflammatory lesions of the central nervous system. This report presents a patient with IMT, who presented with recurrent retroocular pain radiating to the occipital region and no neurologic deficits. He had early recurrence in spite of total resection of the lesion. The clinical profile of 18 patients with either progression or recurrence has been reviewed

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Aim: To present a prospective series of 86 patients with solitary cerebral cysticerci who underwent both contrast computed tomography (CT) and contrast magnetic resonance imaging (MRI), and to correlate and compare the imaging findings using these two modalities. Materials and Methods: Lesion characteristics, staging of the cyst and perilesional hypodensity on CT and hyperintensity on MRI were analyzed, and the lesion was described with regard to the appearance of the scolex, cyst fluid, cyst wall and the perilesional area. Results: Patients were largely children and adolescents (mean age 17.4 years, range 6-52) with 52.3% males. MRI was performed, on average, 10.7 days after CT. MRI was more sensitive than plain CT scan in detecting cysticercal lesions (P = 0.003), but there was no statistically significant difference between contrast CT and MRI. None of the patients were detected with an alternative disease on serial MRI. No cyst showed significant mass effect. Non-contrast CT was less sensitive than either contrast CT or MRI in detecting the scolex (P = 0.011), but no difference was seen between the latter two modalities. Qualitative imaging characteristics of the cysticercus on MRI and CT scan are described. All cysts were round in shape, with an average diameter of <10 mm, and maximum diameter of 19 mm. Conclusion: In the present prospective series, contrast CT was nearly as sensitive as MRI in detecting solitary cerebral cysticerci. Thus, in highly resource-limited settings contrast CT may be sufficient for the diagnosis and management of neurocysticercosis.

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Objective: In this study, we investigated matrix metalloproteinases (MMPs) and tissue inhibitor of metalloproteinase (TIMPs) in cerebrospinal fluid (CSF) and plasma of traumatic brain injury (TBI) patients. Patients and Methods: A total of 30 patients with moderate and severe TBI and 15 age-matched controls were enrolled in this study. Plasma and CSF samples were collected within 24 h (as the initial value), at 72 and 120 h post injury. CSF and plasma MMP-9, MMP-2, TIMP-1 and TIMP-2 were estimated using ELISA. Different levels of these indexes were compared in the two groups and further investigated the correlation between each other. Results: There was a significant elevation in the levels of the initial MMP-9 in the CSF (P < 0.05), which lasted for 72 h post injury. TIMP-1 kept increasing within 120 h post injury and it was different compared with TIMP-1 at 24 and 72 h post injury. Plasma levels of MMP-9, MMP-2, TIMP-1 and TIMP-2 in TBI patients were also significantly different from those in controls. Furthermore the CSF MMP-9 in patients with severe TBI was higher than that in patients with moderate TBI. In addition, there was a positive relationship between the initial MMP-9 and TIMP-1 at 120 h post injury (r = 0.614, P < 0.01). Conclusion: MMPs and TIMPs are increased in both CSF and plasma of TBI patients. TIMP-1 has a positive correlation with MMP-9 and the initial MMP-9 is associated with the neurological outcomes.

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Background: In inflammatory myopathies muscle biopsy is a crucial diagnostic test. Misinterpretation between inflammatory myopathies and muscular dystrophies with inflammation is known. Materials and Methods: Thirty-one patients clinically and pathologically diagnosed to have polymyositis and dermatomyositis and 16 patients of muscular dystrophy with inflammation were studied for MHC-I, MHC-II, CD4 and CD8 expression in skeletal muscle tissue. Results: MHC-I upregulation was noted in all samples of PM and DM. Interstitial and perivascular inflammation in PM were predominantly CD8+ cells, in dermatomyositis, interstitial and perimysial perivascular inflammatory cells were CD4+ T cells and CD8+ T cells were seen around endomysial vessels. Interestingly MHC-I upregulation was seen in all 16 cases of muscular dystrophy with presence of inflammation. Conclusion: The pattern of MHC-I and II expression appeared to be similar in both inflammatory myopathies as well as in muscular dystrophies with inflammation and hence differentiating them on MHC - I expression was difficult.

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Background: Previous studies have shown that the cost of hospitalization due to stroke is significantly associated with the length of stay, stroke severity and other clinical characteristics, as well as various socio-demographic factors. However, these studies have been rather inconsistent with regard to the influence of stroke subtypes on costs. Aims: This study was examined and compared hospital charges of in-patients with acute ischemic stroke according to the Trial of Org 10172 in Acute Stroke Treatment classification. Materials and Methods: The costs of case of 749 patients with first ever ischemic stroke who were admitted to an academic medical center between January 2006 and December 2008 were analyzed. The hospital charges were compared among the stroke subtypes using Analysis of Variance. Multiple regression analyses were further performed to test the significance of the impact of the stroke subtype after controlling for other variables. Results: The stroke subtype turned out to be a statistically significant factor influencing both the total charge and several categorized charges even after controlling for other contributing factors such as hospital length of stay and stroke severity. Conclusions: This study concludes that the stroke subtype should be included when considering in-patient medical expenses of acute ischemic stroke.

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We describe surgical management of herniated coil using indocyanine green video angiography (ICGA) following failure of endovascular retrieval methods in a patient who had endovascular treatment for anterior communicating artery aneurysm. The interdisciplinary cooperation between the endovascular and neurosurgical teams proved to be crucial for obtainment of a desirable outcome in this case. ICGA technology applied in such difficult cases may improve the surgical outcome.

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