Atlanto-axial dislocations (AADs) may be classified into four varieties depending upon the direction and plane of the dislocation i.e. anteroposterior, rotatory, central, and mixed dislocations. However, from the surgical point of view these are divided into two categories i.e. reducible (RAADs) and irreducible (IAADs). Posterior fusion is the treatment of choice for RAAD. Transarticular screw fixation with sub-laminar wiring is the most stable& method of posterior fusion. Often, IAAD is due to inadequate extension in dynamic X-ray study which may also be due to spasm of muscles. If the anatomy at the occipito-atlanto-axial region {O-C1-C2; O: occiput, C1: atlas, C2: axis} is normal on X-ray, the dislocation should be reducible. In case congenital anomalies at O-C1-C2 and IAAD are seen on flexion/extension studies of the cervical spine, the C1-C2 joints should be seen in computerized tomography scan (CT). If the C1-C2 joint facet surfaces are normal, the AAD should be reducible by cervical traction or during surgery by mobilizing the joints. The entity termed "dolichoodontoid" does not exist. It is invariably C2-C3 (C3- third cervical vertebra) fusion which gives an appearance of dolichoodontoid on plain X-ray or on mid-saggital section of magnetic resonance imaging (MRI) or CT scan. The central dislocation and axial invagination should not be confused with basilar invagination. Transoral odontoidectomy alone is never sufficient in cases of congenital IAAD, adequate generous three-dimensional decompression while protecting the underlying neural structures should be achieved. Chronic post-traumatic IAAD are usually Type II odontoid fractures which get malunited or nonunited with pseudoarthrosis in dislocated position. All these dislocations can be reduced by transoral removal of the offending bone, callous and fibrous tissue.

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Aims: To determine the reliability and validity of the National Institute of Health Stroke Scale (NIHSS) with the Hindi and Indian adaptation of items 9 and 10. Materials and Methods: NIHSS items 9 and 10 were modified and culturally adapted at All India Institute of Medical Sciences (AIIMS) and the resulting version was termed as Hindi version (HV-NIHSS). HV-NIHSS was applied by two independent investigators on 107 patients with stroke. Inter-observer agreement and intra-class correlation coefficients were calculated. The predictive validity of the HV-NIHSS was calculated using functional outcome after three months in the form of modified Rankin Scale (mRS) and Barthel Index (BI). Results: The study included 107 patients of stroke recruited from a tertiary referral hospital at Delhi between November 1, 2009, and October 1, 2010; the mean age of these patients was 56.26±13.84 years and 65.4% of them had suffered ischemic stroke. Inter-rater reliability was high between the two examiners, with Pearson's r ranging from 0.72 to 0.99 for the 15 items on the Scale. Intra-class correlation coefficient for the total score was 0.995 (95% CI-0.993-0.997). Concurrent construct validity was established between HV-NIHSS and baseline Glasgow Coma Scale, with a high correlation (Spearman coefficient = -0.863, P<.001). Predictive validity was also established with BI at three months (Spearman's rho: -0.829, P<.001) and with mRS at three months (Spearman's rho: 0.851, P<0.001). Conclusions: This study shows that a Hindi language version of the NIHSS developed at AIIMS appears reliable and valid when applied to a Hindi-speaking population.

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Introduction: Sleep-related disorders (SRDs) though frequent, are under-reported and their implications are often neglected. Objective: To estimate SRDs in an apparently healthy South Indian population. Materials and Methods: Data was collected by administering a questionnaire including Sleep Disorders Proforma, Epworth Sleepiness Scale, and Pittsburgh Sleep Quality Index (PSQI) to 1050 apparently healthy attendants/relatives of patients attending a tertiary healthcare institution. Results: The mean age of the respondents was 35.1±8.7 years with even gender distribution (male: female; 29:21), work hours were 7.8±1.33 h and had regional representation from the southern Indian states. The majority of the respondents did not report any significant medical/psychiatric co-morbidities, hypertension was noted in 42.6%, in one-fourth, the body mass index (BMI) was >25, and in 7.7% the neck size was >40 cm. Daily tea (70.3%) and coffee (17.9%) consumption was common and 22.2% used tobacco. Average time-to-fall-asleep was 22 min (range: 5-90 min), average duration-of-actual-sleep was 7 h (range: 3.5-9.1 h) with the majority (93.8%) reporting good-quality sleep (global PSQI ≤5). The reported rates of SRDs varied between 20.0% and 34.2% depending on the instrument used in the questionnaire. Insomnia, sleep-related breathing disorders (SRBD), narcolepsy, and restless legs syndrome (RLS) were reported by 18.6%, 18.4%, 1.04% and 2.9%, respectively. Obesity was not strongly associated with SRBD. in 51.8% of subjects with SRBD BMI was <25 kg/m ² . Of the respondents with insomnia, 18% had difficulty in initiating sleep, 18% in maintaining sleep and 7.9% had early morning awakening. Respondents attributed insomnia to depression (11.7%) or anxiety (2.5%). Insomnia was marginally high in females when compared to males (10.3% vs. 8.3%) and depression was the major reason. RLS, which was maximal at night, was responsible for delayed sleep onset (74.2%). Other SRDs included night terrors (0.6%), nightmares (1.5%), somnambulism (0.6%), and sleep-talking (2.6%). Family history of SRDs was present in 31.4% respondents. While, only 2.2% of the respondents self-reported and acknowledged having SRD, health-seeking was extremely low (0.3%). Conclusion: SRDs are widely prevalent in India. Considering the health implications and poor awareness, there is a need to sensitize physicians and increase awareness among the public.

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Background: While operating intracranial meningiomas, neurosurgeons commonly drill the hyperostotic bone and put the bone flap back. Objective: To determine whether bony changes seen in meningioma are due to tumor invasion or reactionary changes. Materials and Methods: This prospective study, conducted over 10 months (October 2010- July 2011) included consecutive patients with intracranial meningiomas. Preoperatively, computed tomography (CT) was done in all patients and reviewed by two neurosurgeons for associated bony hyperostosis. During surgery, a piece of bone showing hyperostosis was taken for histopathological evaluation for tumor invasion. In absence of hyperostosis, the bone sample was taken from the bone in contact with the dural attachment of the tumor. Results: This study included 40 consecutive patients who underwent resection for intracranial meningiomas. Radiological evidence of hyperostosis was present in 30 (75%) patients. On histopathological examination, tumor invasion of the bone was seen in eight (20%) patients. These included seven patients who demonstrated hyperostosis and one patient without hyperostosis. Convexity meningiomas (n=12) showed the highest rate of bony invasion (33.3%). Conclusions: A significant number of patients with radiological hyperostosis have tumor invasion of the bone. The authors recommend that one should remove the bone (flap) whenever possible in order to achieve total excision of the tumor and use synthetic material to cover the defect.

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Background: Meningeal tuberculosis (TB) has higher mortality compared to other forms of central nervous system TB. However, data on predictors of mortality is limited. Aims: To determine the predictors of mortality in patients with meningeal TB. Materials and Methods: This study retrospectively analyzed the data of patients admitted with a diagnosis of meningeal TB between January 2006 and December 2008. Thwaites' index score of four or less was used for the diagnosis of meningeal TB which is a weighted diagnostic index score for dichotomised clinical variables. Predictors of mortality were analyzed separately for both patients with human immunodeficiency virus (HIV) infection and without. Statistical Analysis: Univariate analysis and multinomial logistic regression was done. Results: Univariate analysis showed age >40 years, Glasgow Coma Scale (GCS) score <8, absence of headache, cerebrospinal fluid (CSF) protein ≤60 mg% and Medical Research Council (MRC) Stage III at presentation to predict in-hospital mortality. In multinomial logistic regression age >40 years was a risk factor for mortality when HIV patients were included (P=0.049) as well as when they were excluded (P=0.048). CSF protein ͳ 60 mg% was found to be a significant risk factor when both HIV seropositive persons (P=0.011) as well as seronegative persons (P=0.004) were included. HIV seropositivity, steroid treatment or delay in treatment did not affect mortality. Conclusions: Identification of factors predictive of in-hospital mortality will help to prognosticate patients with meningeal TB at the time of admission.

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Background: Minocycline is a semisynthetic derivative of the tetracycline group of antibiotics, which have neuroprotective effects. In animal stroke models, minocycline had shown promising evidence to improve clinical and functional outcomes. Objective: To analyze the effect of oral minocycline in acute ischemic stroke patients. Materials and Methods: This was a randomized single-blinded open-label study. The study group received oral minocycline 200 mg/day for 5 days and the control group received oral vitamin B capsules. Baseline assessment included the following: National Institute of Health Stroke Scale (NIHSS) score, modified Barthel Index (mBI), modified Rankin Scale (mRS) score, Magnetic Resonance Imaging (MRI) of brain including Diffusion Weighted Imaging (DWI), chest X-ray, and routine laboratory investigations. The clinical scales were repeated at days 1, 7, and 30. The end point was outcomes at 3 months (90 days). Statistical analysis was done with SPSS 11.5 (P<0.05). Paired t-test and multiple-measures Analysis Of Variance (ANOVA) were used. Results: Fifty patients with acute ischemic stroke were included in the study. Of these, 23 patients received minocycline and 27 patients received placebo i.e., vitamin B capsules. NIHSS score in patients receiving minocycline had shown statistically significant improvement at day 30 and 90 as compared with the controls. Similarly, mRS scores and BI showed significant improvement in patients receiving minocycline at three months as compared to the control group. No mortality, myocardial infarctions, recurrent strokes, and hemorrhagic transformations were noted in both groups. Conclusions: Patients with acute ischemic stroke had significantly better outcome with minocycline treatment as compared with those administered placebo. The above findings suggest that minocycline can be helpful in reducing the clinical deficits after acute ischemic stroke.

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Embryonal tumor with multilayered rosettes (ETMR) is a well-recognized histopathological variant of primitive neurectodermal tumors of the central nervous system. This tumor depicts histopathological features that are common to both ependymoblastoma (EBL) and neuroblastoma. Here we report two pediatric cases of ETMR, one exhibiting dominant neuronal differentiation and the other with dominant glial differentiation, thereby expanding the previously known pathologic spectrum. The varying histological features, common morphologic diagnostic difficulties as well as variable postsurgical survival of this entity compared to similar embryonal tumors are highlighted.

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The clinical manifestations of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes syndrome (MELAS syndrome) are nonspecific and can easily be misdiagnosed. Magnetic resonance spectroscopy (MRS)-based detection of lactate in the brain has been found to be of diagnostic help in MELAS syndrome, however, the issue of whether MRS features vary by stage remains unresolved. We assessed the causative mutation and radiological features of a family of MELAS. Four of the family members harbored the A3243G mutation, probably of maternal inheritance. However, the clinical phenotypic expression was different in these patients. MRS showed a lactate peak, decreased N-acetylaspartate, choline, and creatine, which became more pronounced with progression of the disease, demonstrating that brain-MRS-based detection of lactate may be a suitable way to monitor the progression and treatment of MELAS.

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Background and Purpose: The aim of this study was to evaluate the anatomical changes and investigate the prevalence in intracranial aneurysm with fenestrations using magnetic resonance angiography (MRA). Materials and Methods: Between June 2008 and October 2010, 4652 patients (aged 23-73 years) with suspected intracranial aneurysm or other cerebrovascular diseases underwent MRA examination. MRA was performed using a three-dimensional time-of-flight technique (3D-TOF) with volume rendering (VR) and maximum intensity projection reconstruction methods. The presence and location of fenestrations and aneurysms was reviewed. When fenestrations were present in combination with aneurysms, we noted the relationship of the locations. The classification of fenestration accompanied by intracranial aneurysm was divided into three types according to the anatomical relationship as follows: Type I, aneurysm adjacent to but not on a fenestration; Type II, aneurysm located on the fenestration; type III, aneurysm located at a position remote from a fenestration. Results: Among the 4652 patients examined, 409 patients were defined with 412 intracranial aneurysms, and the prevalence of aneurysms was 8.8%. One hundred and forty-one patients were identified with fenestrations; 24 of these patients were confirmed with intracranial aneurysms. Seven cases were classified as type I, three as type II and 14 as type III. The prevalence of intracranial aneurysm with fenestrations was 17.0%, with significant statistical difference compared with aneurysms unaccompanied with fenestrations (P=0.0064). Conclusion: The anatomical relationship between fenestrations and intracranial aneurysms was visualized by MRA with VR, which displayed pathologies with sufficient clarity to enable diagnosis. Furthermore, the results of this study suggest that physicians should be alerted to the occurrence of intracranial aneurysm following the detection of fenestrations by MRA.

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Narcolepsy is a common sleep disorder with a prevalence of about 0.02%. However, it may remain largely unrecognized in the Indian population owing to the perceived low prevalence. To the best of our knowledge there is only one case of narcolepsy reported from India so far. We present a case of narcolepsy with cataplexy with classical clinical and polysomnographic findings of narcolepsy.

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Intracerebral hemorrhage (ICH) is a heterogeneous disease with genetic factors playing an important role. Association studies on a wide range of candidate pathways suggest a weak but significant effect for several alleles with ICH risk. Among the most widely investigated genes are those involved in the renin-angiotensin-aldosterone system (e.g., angiotensin-converting enzyme), coagulation pathway (e.g., Factor XIII, Factor VII, platelet-activating factor acetylhydrolase, Factor V Leiden, and beta1-tubulin), lipid metabolism (e.g., apolipoproteins (Apo)E, Apo(a), ApoH), homocysteine metabolism (e.g., methylenetetrahydrofolate reductase), inflammation (e.g., interleukin-6 and tumor necrosis-alpha) and other candidate pathways. To identify the robustness of the above associations with ICH, a search of Pubmed (1988 through December 2011) was performed, with searches limited to English-language studies conducted among adult human subjects. This article presents a review of the examined literature on the genetics of ICH.

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Context : There are currently few published studies that compare hippocampal volume (HCV) in children with temporal lobe epilepsy (TLE). Aims : T0 o compare HCVs in children with TLE and in relation to normal controls (NC), and to analyze HCV change in the acute phase in pediatric subjects using magnetic resonance imaging (MRI). Setting and Design : T0 he case group (n=24) was matched in gender and age range with NC subjects (n=24). Subjects were divided into three groups according to age: 2-5.9 years, 6-8.9 years and 9-13 years. Materials and Methods : M0 anual measurements were used to obtain HCVs on oblique coronary MRI images obtained by three-dimensional magnetization-prepared rapid gradient-echo (3D-MPRAGE) sequence. Statistical Analyses : T0 he HCV was calculated and normalized. Paired t-tests were used to compare the right and left HCVs within each study group. Analysis of covariance (ANCOVA) was used to consider the impact of age and gender on HCVs. Results : T0 wenty-two patients were diagnosed with definite/probable TLE, and two were non-syndromic focal epilepsy which was distributed in the 2-5.9 years group. The range of left to right mean HCVs of the case and NC group aged 2-13 years was 2014.14 ± 54.32 mm ³ to 2165.31 ± 80.99 mm ³ and 2015.46 ± 26.97 mm ³ to 2100.93 ± 57.33 mm ³ respectively. There were age-related differences in left and right HCV, but no effect of gender. Relative to NC subjects, cases group aged 2-5.9 years had significantly different HCVs, while no significant difference was found in the other two groups. There was significant difference in the right to left HCVs in the case subjects aged 2-5.9 years, but not in the other age groups. Conclusions : T0 he heterogeneity in the 2-5.9 years age cohort may relate to the increased HCVs. The HCV data from NC subjects may be used as a reference to assess hippocampal abnormalities in clinical practice.

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Objective: To determine the pattern of muscle involvement in patients with ulnar neuropathy at the elbow. Materials and Methods: This study evaluated all patients referred for upper limb electrodiagnostic study (EDX) during 2007-2011 and included. patients with clinical signs and symptoms of ulnar neuropathy at the elbow. All patients had nerve conduction studies (NCS) for ulnar neuropathy. Needle electromyography (EMG) of four ulnar innervated muscles, flexor carpi ulnaris (FCU), flexor digitrom profoundus (FDP), first dorsal interosseous (FDI) and abductor digiti minimi (ADM)) was evaluated. Results: During the study period 34 (23 males and 11 females) patients were diagnosed with ulnar neuropathy at the elbow and three of them had bilateral involvement. Muscle involvement by EMG was as follows: FDI: 91.9%, ADM: 91.3%, FCU: 64.9% and FDP: 56.8%. Conclusion: In this study, EMG abnormalities of nerve damage were presented more commonly in the FCU muscle than in the FDP in patients with ulnar nerve lesion at the elbow.

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Intracranial germ cell tumors constitute a unique group of tumors, more often reported from the Asian region. Amongst them, the non-germinomatous variety occurs with a lesser frequency than the germinomatous variety. We report two children with mixed germ cell tumors with unusual clinical presentations: Central diabetes insipidus and recent-onset oculomotor palsy mimicking pituitary apoplexy. Unlike in adults, suprasellar lesions with a pituitary apoplexy-like picture in the pediatric age group may suggest a possibility of a mixed germ cell tumor.

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Background: Narcolepsy is not an uncommon sleep disorder in the West. There is, however, only one reported case in literature from India. In this study, we report characteristics of patients with narcolepsy over a seven-year period. Materials and Methods: Details of all patients with narcolepsy seen at a Sleep Disorders Clinic over seven years were analyzed. Diagnosis had been established by clinical history and two or more Sleep Onset Rapid Eye Movement Periods (SOREMPs) on Multiple Sleep Latency Test (MSLT) following an overnight Polysomnography (PSG), using the International Classification of Sleep Disorders - 2 (ICSD-2) criteria. Patients fulfilling the criteria, but suffering from other disorders were excluded. Results: Data of 20 patients were analyzed and 4 patients were excluded, as they had other associated conditions. Mean age at onset of symptoms was 25±10 years; 12 (60%) patients had narcolepsy with cataplexy, 4 (20%) patients presented with all cardinal symptoms of narcolepsy, 8 (40%) with 3 symptoms, while 8 (40%) presented with 2 symptoms. History of Excessive Day-Time Sleepiness (EDS) was present in all patients. Three patients reported accidents due to sleep attacks, one being life-threatening. On PSG, mean sleep efficiency was 79.4±12.40%. Mean sleep latency during MSLT was 1.30 minute (range: 0.30-2.30 minute) and mean REM latency was 2.58±0.64 minute. Conclusion: Narcolepsy with and without cataplexy is infrequently seen in the North Indian population; however, clinical and polysomnographic features are similar to those observed in Western and other Asian populations.

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The clinical characteristics of aggressive craniocervical junction dural arteriovenous fistula (CCJDAVF) and effect treatment was studied in four patients and all four patients were treated endovascularly with liquid embolic agents. Post-embolization angiography documented complete obliteration in one patient and partial obliteration in the other three patients. All patients had good recovery without any procedure-related complications. Endovascular embolization can be used to reduce the rate of flow through CCJDAVFs or as a definitive treatment when the fistula is accessible endovascularly.

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Background: Endovascular therapy of complicated ruptured anterior communicating artery (ACoA) aneurysms is difficult due to their small size and unfavorable shape. Aim: Based on the anatomical features of the ACoA complex, we investigated the feasibility and efficacy of different coil embolism strategies for complicated ACoA aneurysms. Materials and Methods: Sixteen patients with complicated ruptured ACoA aneurysms received endovascular treatment. Aneurysm sac plus ACoA embolism or ACoA coil embolism were performed if the bilateral A1 segment was normally developed or unilateral A1 segment dysplasia (≥1/2 normal contralateral diameter) was present. Where unilateral A1 segment dysplasia (<1/2 normal contralateral diameter) or aplasia was present, sac embolism alone was performed. Follow-up angiography was performed, and clinical follow-up data were categorized as fully recovered, improved, unchanged or worsened. Results: Aneurysm sac plus ACoA (n=5) or ACoA alone (n=2) coil embolism was performed in seven patients with normal bilateral A1 segments (n=5) or dysplasia (n=2). Sac coil embolism was performed in nine patients with unilateral A1 segment dysplasia (n=1) or aplasia (n=8). Immediate angiography indicated total/near-total occlusion was achieved in 14 patients. Final angiographic (mean 11.9 ± 5.1 months) and clinical (mean 17.7 ± 5.9 months) follow-up confirmed total/near-total occlusion in 12 patients, one partial occlusion, two enlarged residual sacs and one reopened aneurysm. Clinical symptoms fully recovered in 10 patients, improved in four, were unchanged in one and worsened in one patient. Conclusion: This small middle-term follow-up study demonstrates coil embolism endovascular treatment of complicated ruptured ACoA aneurysms, based on the anatomical features of the ACoA complex, is feasible and effective.

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Numerous Single-Nucleotide Polymorphisms (SNPs) of the Diacylglycerol Kinase Delta (DGKD) isoform 1 gene have been associated with Parkinson Disease (PD) in the genome-wide association studies of Caucasian population. This association has not been proven in the Han Chinese PD patients. This study included 376 unrelated Han Chinese PD patients from West China and 273 unrelated healthy controls from the same region. Five SNPs (rs2971859, rs1550532, rs2305539, rs2034762, and rs2242102) were genotyped using the Sequenom iPLEX Assay technology. No significant differences were observed in genotype frequencies and in the Minor Allele Frequency (MAF) in the five SNPs between PD patients and controls, early-onset PD and controls, late-onset PD and controls, and between early-onset and late-onset PD patients. The present study is the first to report on the lack of association of DGKD SNPs with PD in the Han Chinese population. More related studies involving larger numbers of participants are necessary to confirm the present finding.

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Background: There is significant inter-observer variation amongst the neuro-pathologists in the typing, subtyping, and grading of glial neoplasms for diagnosis. Centralized pathology review has been proposed to minimize this inter-observer variation and is now almost mandatory for accrual into multicentric trials. We sought to assess the concordance between neuro-pathologists on histopathological diagnosis of glioblastoma. Materials and Methods: Comparison of local, institutional, and central neuro-oncopathology reporting in a cohort of 34 patients with newly diagnosed supratentorial glioblastoma accrued consecutively at a tertiary-care institution on a prospective trial testing the addition of a new agent to standard chemo-radiation regimen. Results: Concordance was sub-optimal between local histological diagnosis and central review, fair between local diagnosis and institutional review, and good between institutional and central review, with respect to histological typing/subtyping. Twelve (39%) of 31 patients with local histological diagnosis had identical tumor type, subtype and grade on central review. Overall agreement was modestly better (52%) between local diagnosis and institutional review. In contrast, 28 (83%) of 34 patients had completely concordant histopathologic diagnosis between institutional and central review. The inter-observer reliability test showed poor agreement between local and central review (kappa statistic=0.12, 95% confidence interval (CI): -0.03-0.32, P=0.043), but moderate agreement between institutional and central review (kappa statistic=0.51, 95%CI: 0.17-0.84, P=0.00003). Agreement between local diagnosis and institutional review was fair. Conclusions: There exists significant inter-observer variation regarding histopathological diagnosis of glioblastoma with significant implications for clinical research and practice. There is a need for more objective, quantitative, robust, and reproducible criteria for better subtyping for accurate diagnosis.

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