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A 13-year-old female patient presented with painless vision loss and proptosis for 18 months. Imaging findings were highly suggestive of a supraorbital aneurysmal bone cyst (ABC) for which she underwent complete surgical excision. Postoperatively, she developed left hemiparesis. Computed tomography angiography (CTA) revealed right complete internal carotid arterial (ICA) thrombosis. This was managed conservatively, and she improved in hemiparesis over the next 3 weeks. Histopathology report revealed osteosarcoma with secondary ABC, for which she was referred for radiotherapy. At 1.5 months follow-up, the patient's left lower limb power improved to 4 + /5. She was walking without support, and her left upper limb power was 4/5. [ABSTRACT FROM AUTHOR]

Purpose: Inter-rater reliability is a critical aspect of stroke image interpretation. This study aims to investigate inter-rater reliability between neurologists and neuro-radiologist when assessing Alberta Stroke Program Early CT Score (ASPECTS) and Intracerebral Haemorrhage (ICH) scores using a mobile application (SMART INDIA App – by neurologists) and the Picture Archiving and Communication System (PACS – by neuro-radiologist). Methods: Adult patients diagnosed with ischemic or haemorrhagic stroke were included in this study. Two Neurologists (R1 and R2) assessed the ASPECTS and ICH scores by viewing the SMART INDIA App. A neuroradiologist expert (R3) assessed the same using PACS. Kappa statistics are presented for agreement between the Raters. Results: 100 consecutive patients each of Acute Ischemic stroke (AIS) and ICH were included. A significant agreement in the total ASPECTS between the Raters 1 and 2 (0.85(95% CI—0.775,0.926)), Raters 1 and 3(0.76(95% CI—0.671,0.857)) and Raters 2 and 3(0.76(95% CI—0.673,0.857)) was noted. A good agreement was ascertained between Rater 1 and 3, even though the devices were different. A similar excellent agreement was also noted in assessing the ICH score. The study's findings indicate that neurologists using different devices and platforms demonstrated good to excellent agreement with the neuro-radiologist (considered the gold standard) when estimating ASPECTS and ICH scores. Conclusion: The interpretations of neurologists using the SMART INDIA App can be deemed valid and reliable from the neuroimaging viewpoint. These results contribute to our understanding of the feasibility and reliability of app-based image evaluation in the assessment of stroke. [ABSTRACT FROM AUTHOR]

Objective : The objective was to describe the clinical presentations, radiologic features, and outcomes of patients with autoimmune encephalitis associated with myelin oligodendrocyte glycoprotein antibody (MOG). Background: During the past decade, the spectrum of the myelin oligodendrocyte glycoprotein antibody-associated diseases (MOGAD) has expanded. Recently, patients with MOG antibody encephalitis (MOG-E) who do not fulfill the criteria for ADEM have been reported. In this study, we aimed to describe the spectrum of MOG-E. Methods: Sixty-four patients with MOGAD were screened for encephalitis-like presentation. We collected the clinical, radiological, laboratory, and outcome data of the patients who presented with encephalitis and compared it with the non-encephalitis group. Results: We identified sixteen patients (nine males and seven females) with MOG-E. The median age of the encephalitis population was significantly lower than the non-encephalitis group (14.5 years (11.75–18) vs. 28 years (19.75–42), p = 0.0004). Twelve out of sixteen patients (75%) had fever at the time of encephalitis. Headache and seizure were present in 9/16 (56.2%) and 7/16 (43.75%) patients, respectively. FLAIR cortical hyperintensity was present in 10/16 (62.5%) patients. Supratentorial deep gray nuclei were involved in 10/16 (62.5%) patients. Three patients had tumefactive demyelination, and one patient had a leukodystrophy-like lesion. Twelve of 16 (75%) patients had a good clinical outcome. Patient with leukodystrophy pattern and other with generalized CNS atrophy showed a chronic progressive course. Conclusion: MOG-E can have heterogeneous radiological presentations. FLAIR cortical hyperintensity, tumefactive demyelination, and leukodystrophy-like presentations are novel radiological presentations associated with MOGAD. Though majority of MOG-E have a good clinical outcome, few patients can have chronic progressive disease even on immunosuppressive therapy. [ABSTRACT FROM AUTHOR]

Background: Introduction of the classification of brain tumours based on DNA methylation profile has significantly changed the diagnostic approach. Due to the paucity of data on the molecular profiling of meningiomas and their clinical implications, no effective therapies and new treatments have been implemented. Methods: DNA methylation profiling, copy number analysis, targeted sequencing and H3K27me3 expression was performed on 35 meningiomas and 5 controls. Results: Unsupervised hierarchical clustering (UHC) analysis revealed four distinct molecular subgroups: Malignant; Intermediate; Benign A, and Benign B. Molecular heterogeneity was observed within the same grade as the Intermediate, Benign A, and Benign B subgroups were composed of WHO grade 1 as well as grade 2 cases. There was association of mutations with distinct methylation subgroups (NF2, AKT1, SMO, TRAF7 and pTERT). Loss of chromosome 22q was observed across all subgroups. 1p/14q co-deletion was seen in 50% of malignant and intermediate while CDKN2A loss was predominantly observed in malignant subgroup (50%). Majority of malignant (75%) and a small proportion of other subgroups (Intermediate: 25%, Benign A: 38.5%, and Benign B: 20%) harboured H3K27me3 loss. 38,734 genes were dysregulated amongst the four subgroups. DKFZ classified 71% cases with acceptable score. On survival analysis, methylation profiling had significant impact on progression-free-survival in WHO grade1 and 2 meningiomas (p = 0.0051). Conclusion: Genome-wide DNA methylation profiling highlights clinically distinct molecular subgroups and heterogeneity within the same grade of meningiomas. Molecular profiling can usher in a paradigm shift in meningioma classification, prognostic prediction, and treatment strategy. [ABSTRACT FROM AUTHOR]

Background: The reported incidence of leptomeningeal carcinomatosis is 3–8% in patients with solid tumours. More commonly, it has been described in the setting of advanced cancers of the lung, breast and malignant melanoma. Case presentation: A 50-year-old diabetic patient with recurrent unresectable squamous cell carcinoma (SCC) of the right retromolar trigone (rT4bN0M0) presented with severe low backache and weakness in bilateral lower limbs 20 days after the completion of concurrent chemoradiotherapy. Contrast-enhanced MRI of the spine showed multiple nodular enhancing leptomeningeal lesions at the lumbar level and an intramedullary T2/FLAIR-hyperintense longitudinal lesion involving the central cord from C2 to C7 vertebral levels, suggestive of leptomeningeal metastases. Cerebrospinal fluid (CSF) analysis revealed pleocytosis, elevated protein and markedly decreased glucose. The CSF cytology revealed scattered large atypical cells, suspicious for metastasis. Non-contrast MRI of the brain showed a T2/FLAIR-hyperintense lesion involving the right caudate nucleus suggestive of either an acute infarct with haemorrhagic transformation or a haemorrhagic brain metastasis. During assessment, he had high-grade fever and was started on empirical intravenous antibiotics (ceftriaxone, vancomycin and subsequently meropenem) in line with the management for acute bacterial meningitis. Gram staining of CSF did not demonstrate the presence of any bacteria and the specimen was sterile on culture. He did not respond to empirical antibiotics, had a progressive downhill course and eventually died due to aspiration pneumonia. Conclusion: This brief report highlights the importance of awareness of leptomeningeal carcinomatosis as a possible cause of backache with sensorimotor deficit and autonomic dysfunction in a previously treated case of head and neck SCC. [ABSTRACT FROM AUTHOR]

Purpose: We planned this prospective study to evaluate PSMA expression in recurrent high-grade gliomas (rHGG), including anaplastic astrocytoma and glioblastoma using Glu-NH-CO-NH-Lys-(Ahx)-[Ga-68 (HBED-CC)]- (Ga-68 PSMA) positron emission tomography (PET), with its theranostic potential in mind. Methods: This was a prospective study enrolling patients with clinical and MRI evidence of rHGG on follow-up. Three treated cases of HGG with RN on MRI were also included as negative controls. Abnormal tracer accumulation in the brain parenchyma, more than the contralateral hemisphere was interpreted as positive study. For semiquantitative analysis, a 3D spherical region of interest (ROI) was drawn around the site of the abnormal Ga-68 PSMA uptake, and the ratio of SUVmax of tumor (T) to SUVmax of the contralateral corresponding area (TBR) was calculated. Each patients' PSMA brain PET was fused to the corresponding MRI and reviewed for concordance. Results: Thirty patients were included in the study, a total of 49 lesions were detected on MRI, and fused PET/MR images showed increased Ga-68 PSMA uptake in all these lesions. Multifocal lesions were better appreciated on fused PET-MR images, and concordance between MRI and PET was 100 % for patient and lesion-wise detection. Recurrent glioma lesions showed SUVmax and SUVmean values (median and IQR) 6.0 (4.4–8.2) and 3.3 (2.8–3.7), respectively. Lesions labeled as radiation necrosis on MRI did not show tracer accumulation. Conclusion: Ga-68 PSMA has potential utility for evaluating recurrence in HGG and its potential for theranostics would encourage its use in the evaluation of these patients. [ABSTRACT FROM AUTHOR]

Magnetic resonance imaging (MRI) of the brain with angiography and vessel wall imaging showed multiple subcortical white matter bleeds in bilateral fronto-parietal, temporal, and cerebellar hemispheres (Fig. Dear sir, Guillain Barre Syndrome (GBS) is an acute polyradiculoneuropathy which is characterized by progressive limb weakness, variable sensory disturbance, absence of deep tendon reflexes, and autonomic dysfunction. [Extracted from the article]

Purpose: H3K27M mutant diffuse midline gliomas (DMGs) are considered grade IV irrespective of histological features and have dismal prognosis. We evaluated clinico-pathologic, radiological, and molecular characteristics of DMGs across all ages. Methods: One twenty-six DMGs were identified over 10 years. Immunohistochemistry was done for H3K27M, ATRX, IDH1, and p53, and Sanger sequencing performed for IDH1 and H3K27M mutation. Patient demographics and clinico-radiologic characteristics were reviewed and survival analysis performed. Results: DMGs comprised 5.3% of all gliomas with 49.2% H3K27M mutant and 50.8% wild types. Majority (75.68%) of pediatric and 38.20% of adults were H3K27M mutant (p = 0.0001). Amongst H3K27M mutants, brainstem (46.43%) was the commonest location in pediatric and thalamus (61.76%) in adults. H3K27M mutation was mutually exclusive with IDH mutation in 93.55%, while p53, ATRX mutation were seen in 56.4% and 30.6% cases respectively. Software-based immunohistochemistry evaluation (H-scoring) showed 99.2% concordance with sequencing for H3K27M mutation. Radiologically, no significant difference in contrast enhancement was seen between mutant and wild types (p = 0.05). The difference in overall survival (OS) was not significant in mutant versus wild types, with age or location. Tumor resection independently and on correlation with H3K27M did not influence OS (p = 0.51 and p = 0.47). Adjuvant therapy impacted survival significantly in adults (p = 0.0009), however, not in pediatric cases (p = 0.06). Conclusions: The study highlights the differences in frequency and location of pediatric and adult DMGs. IHC (H-scoring) for H3K27M mutation is an excellent surrogate for sequencing. Prognosis remains dismal irrespective of age, location, and H3K27M status. Potential therapeutic targets need to be explored. [ABSTRACT FROM AUTHOR]

The PFA molecular subgroup of posterior fossa ependymomas (PF-EPNs) shows poor outcome. H3K27me3 (me3) loss by immunohistochemistry (IHC) is a surrogate marker for PFA wherein its loss is attributed to overexpression of Cxorf67/EZH2 inhibitory protein (EZHIP), C17orf96, and ATRX loss. We aimed to subgroup PF-EPNs using me3 IHC and study correlations of the molecular subgroups with other histone related proteins, 1q gain, Tenascin C and outcome. IHC for me3, acetyl-H3K27, H3K27M, ATRX, EZH2, EZHIP, C17orf96, Tenascin-C, and fluorescence in-situ hybridisation for chromosome 1q25 locus were performed on an ambispective PF-EPN cohort (2003–2019). H3K27M-mutant gliomas were included for comparison. Among 69 patients, PFA (me3 loss) constituted 64%. EZHIP overexpression and 1q gain were exclusive to PFA seen in 72% and 19%, respectively. Tenascin C was more frequently positive in PFA (p = 0.02). H3K27M expression and ATRX loss were noted in one case of PFA–EPN each. All H3K27M-mutant gliomas (n = 8) and PFA-EPN (n = 1) were EZHIP negative. C17orf96 and acetyl-H3K27 expression did not correlate with me3 loss. H3K27me3 is a robust surrogate for PF-EPN molecular subgrouping. EZHIP overexpression was exclusive to PFA EPNs and was characteristically absent in midline gliomas and the rare PFA harbouring H3K27M mutations representing mutually exclusive pathways leading to me3 loss. [ABSTRACT FROM AUTHOR]

Introduction: Molecular classification of medulloblastomas (MB) is prognostically and therapeutically relevant and helps in better risk-stratification. Translation of this subgrouping to routine practice still remains a challenge. The most pathologist accessible techniques for molecular subgrouping include immunohistochemistry (IHC), fluorescent in-situ hybridization (FISH) and NanoString. Objectives: (1) Molecular subgrouping of MBs by IHC and FISH, and NanoString assay (2) To compare their efficacy and cost for applicability in resource constrained centers. Methods: Ninety-five cases of MB with adequate tissue were included. Molecular subgrouping was performed by IHC for β-catenin, GAB1 and YAP1; FISH for MYC amplification, and sequencing for CTNNB1, and by NanoString Assay on the same set of MBs. A subset of cases was subjected to 850k DNA methylation array. Results: IHC + FISH classified MBs into 15.8% WNT, 16.8% SHH, and 67.4% non-WNT/non-SHH subgroups; with MYC amplification identified in 20.3% cases of non-WNT/non-SHH. NanoString successfully classified 91.6% MBs into 25.3% WNT, 17.2% SHH, 23% Group 3 and 34.5% Group 4. However, NanoString assay failure was seen in eight cases, all of which were > 8-years-old formalin-fixed paraffin-embedded tissue blocks. Concordant subgroup assignment was noted in 88.5% cases, while subgroup switching was seen in 11.5% cases. Both methods showed prognostic correlation. Methylation profiling performed on discordant cases revealed 1 out of 4 extra WNT identified by NanoString to be WNT, others aligned with IHC subgroups; extra SHH by NanoString turned out to be SHH by methylation. Conclusions: Both IHC supplemented by FISH and NanoString are robust methods for molecular subgrouping, albeit with few disadvantages. IHC cannot differentiate between Groups 3 and 4, while NanoString cannot classify older-archived tumors, and is not available at most centres. Thus, both the methods complement each other and can be used in concert for high confidence allotment of molecular subgroups in clinical practice. [ABSTRACT FROM AUTHOR]

Background: The treatment of primary CNS lymphoma (PCNSL) comprises high dose methotrexate (HDMTX) based chemotherapy followed by whole brain radiotherapy (WBRT), the major drawback of which is long term neurotoxicity. We intended to assess the feasibility of response adapted WBRT in PCNSL in the Indian setting.Methods: We screened 32 patients and enrolled 22 eligible patients with PCNSL from 2015 to 2017 in a prospective phase II trial. The patients underwent five 2-weekly cycles of induction chemotherapy with rituximab, methotrexate, vincristine, procarbazine. Patients with complete response(CR) to induction chemotherapy were given reduced dose WBRT 23.4 Gy/13 fractions/2.5 weeks while those with partial response (PR), stable or progressive disease (SD or PD) were given standard dose WBRT 45 Gy/25 fractions/5 weeks. Thereafter two cycles of consolidation chemotherapy with cytarabine were given. The primary endpoints of the study were assessment of response rate (RR) and progression free survival (PFS). The secondary endpoints of the study were assessment of overall survival (OS), toxicity profile of treatment and serial changes in quality of life and neuropsychological parameters.Results: Out of 19 patients who completed HDMTX based chemotherapy, 10 (52.63%) patients achieved CR, 8 (42.11%) patients had PR and 1 patient had PD. After a median follow-up period of 11.25 months, the estimated median OS was 19 months. The actuarial rates of PFS and OS were respectively 94.1 and 68.2% at 1 year and 50.2 and 48.5% at 2 years. Three patients in reduced dose WBRT arm had recurrence and two of them died of progressive disease, whereas there was no recurrence or disease related death in standard dose WBRT arm. On univariate analysis of PFS, age ≤ 50 years and use of standard dose WBRT (45 Gy) led to significantly improved outcome (p value 0.03 and 0.02 respectively).Conclusion: In patients with PCNSL, reduced dose WBRT after CR to HDMTX based chemotherapy may lead to suboptimal clinical outcome due to higher risk of recurrence, progression and early death. Trial Registration No CTRI/2015/10/006268 [ABSTRACT FROM AUTHOR]

Diffuse leptomeningeal glioneuronal tumor (DL-GNT) is a newly introduced tumor entity of uncertain prognosis characterised by a primary diffuse leptomeningeal growth pattern, oligodendroglial-like morphology and dual glial/neuronal differentiation. Predominantly occurring in children, these tumors present as chronic meningitis and mimic infectious/inflammatory diseases. They are surgically challenging tumors with a high incidence of delayed morbidity and mortality despite low-grade histology. Their molecular genetic profile is not fully elucidated and few reports have identified chromosome 1p and 19q deletions, and BRAF alterations. We present a rare instance of a DL-GNT in a 13-year-old female who presented with slowly progressive and sequential neurological deficits over a 12-month duration. Imaging showed leptomeningeal thickening and spinal lesions. Biopsy from the spinal mass showed histomorphological features characteristic of DL-GNT. Further molecular analysis revealed 1p and 19q co-deletion and H3K27M mutation, while no mutation were identified in IDH, TERT, or BRAF genes. Patient died 4 months after diagnosis. Only one previous case of DL-GNT has been reported to harbour H3K27M mutation. Although H3K27M mutations have been described in rare examples of low-grade glial and glioneuronal tumors, whether DL-GNTs with H3K27M represent a rare growth pattern of the aggressive H3K27M-mutant diffuse midline gliomas needs further clarification. [ABSTRACT FROM AUTHOR]

Intracranial aspergillosis (ICA) is very rare in the immunocompetent individuals, usually misdiagnosed as a tumor or an abscess. A high index of clinical suspicion is required in patients who present with focal neurological deficits, headache, or seizures. We report the case of a 25-year-old immunocompetent female, who presented with a 15-month history of headache, seizures, left-sided proptosis and ophthalmoplegia, and right hemiparesis. Recovery from the symptoms and decrease in the lesion size seen on the radiological assessment were achieved through two decompressive craniotomies followed by prolonged combined systemic antifungal therapies. Although the initial neuroimaging suggested a mitotic pathology, the surgical sample confirmed ICA. Now the patient is on single antifungal therapy (Tab. voriconazole, 200 mg twice daily) and doing her daily activities, but with a reduced intelligent quotient. We report a challenging case of ICA where multiple courses of combined antifungal therapies and repeat surgeries paved the way for a good prognosis. [ABSTRACT FROM AUTHOR]

Glioneuronal tumor with neuropil-like islands (GTNI) is a rare, recently described neoplasm, whose pathogenesis has not been studied extensively. The role of ATRX mutations, a class-defining alteration in diffuse astrocytic neoplasms, has not been assessed in GTNIs previously. We therefore aimed to assess the status of ATRX, along with IDH1, 1p/19q and p53, in cases of GTNI in order to evaluate the molecular profile of these tumors. All cases of GTNI diagnosed at our Institute were retrieved and clinicopathological features were reviewed. Immunohistochemistry for ATRX, IDH1 and p53 was performed. We identified four cases of GTNI, majority of which occurred in young adults. Loss of ATRX immunoexpression, a surrogate marker for ATRX mutation, was seen in all four cases. All cases were immunopositive for p53, while IDH1 positivity was seen in all three cases assessed. 1p/19q codeletion was absent in the three cases analyzed. These results indicate that the molecular pathogenesis of GTNIs similar to that of diffuse astrocytic tumors. Further, the loss of ATRX expression is seen in both the glial as well as neuronal components, indicating that both arise from the same tumor stem/progenitor cell and that the latter may be a metaplastic change. Thus, loss of ATRX immunoexpression, shown for the first time in these tumors, along with immunopositivity for p53 and IDH1, indicates that these tumors are molecular astrocytomas, and their clinical behaviour is likely to recapitulate that of ATRX-mutant and IDH-mutant diffuse astrocytomas of the same grade. [ABSTRACT FROM AUTHOR]

Rhabdoid meningioma is a rare meningioma variant, classified as WHO grade III. Although this tumor is known for its aggressive behavior and poor prognosis, extracranial metastasis is rare. We report the rare case of a 31-year-old patient with rhabdoid meningioma which recurred several times despite gross total resection, radiation therapy, and gamma knife radiosurgery, and the last recurrence was associated with metastases to lungs, lymph node and bone. The patient showed no response to paclitaxel-carboplatin, or vincristine-cyclophosphamide-adriamycin chemotherapy, and succumbed to the disease. Metastases from rhabdoid meningioma prove to be a diagnostic challenge, and treatment for metastatic meningiomas is not optimized, thus necessitating documentation and interdisciplinary consensus on management protocols. [ABSTRACT FROM AUTHOR]

Purpose: Papillary glioneuronal tumors (PGNT) have been recently included as a distinct entity in the WHO classification of tumors of the central nervous system. Their molecular pathogenesis is not clear. In the current study, we present the morphological, immunohistochemical, and molecular features of four cases of PGNT reported over the past 11 years. Methods: Over a period of 11 years (January 2000-February 2010), there were four cases of PGNT, which were reviewed for histomorphological features. TP53 and IDH1 mutations were assessed using antibodies against p53 protein and for mutant IDH1 protein, respectively. Immunohistochemistry was also performed for epidermal growth factor receptor (EGFR) protein. Fluorescence in situ hybridization assay was used for analyzing 1p/19q deletion status. Results: All the tumors showed the characteristic biphasic morphology. Rare findings included minigemistocyte-like cells in one, angiomatous areas in three, focal necrosis in one, and a high MIB-1 labeling index of 12 and 13 %, respectively, in two of the cases. All lacked EGFR, IDH1 expression, and 1p/19q deletions. Interestingly, antibody for p53 labeled the tumor cells, mainly those showing glial differentiation, in two cases. At a mean follow-up of 30 months, there was no evidence of disease progression except in one case which recurred after 24 months. Conclusion: PGNT are rare CNS neoplasms. Despite showing focal morphological features reminiscent of oligodendroglial tumors and presence of astrocytic component, they usually lack the common genetic alterations involved in the pathogenesis of gliomas. Multi-institutional pooling of cases may aid in elucidating their oncogenetic pathway. [ABSTRACT FROM AUTHOR]

The frequency and pattern of endocrine abnormalities among patients with traumatic brain injury have been the subject matter of very few studies. This study was intended to assess the pattern of endocrine dysfunction following severe head injury. Severe head injury patients admitted to the Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, from January to December in 1 year formed the study group. Apart from clinical assessment, NCCT of the head was performed on all patients on admission. A complete anterior pituitary hormone analysis was performed within 24 h of injury and was repeated at 2 weeks, 3 months and 6 months amongst patients who survived. A total of 99 patients were included in the study. Forty of our patients succumbed in the hospital. Rest of the patients were followed up for 6 months. Elevations of cortisol followed by prolactin were the most common hormonal derangements at admission. Midline shift on CT scans was inversely related to cortisol elevation and directly related to GH elevation. Infarct on CT scans was inversely related to cortisol and LH elevation. A significant alteration was found in the decreasing trend of the mean T4 values and normalisation or a decreasing trend from initially elevated mean cortisol and GH levels during follow-up (p < 0.05). This study reveals that abnormalities in hormonal profiles appear to be relatively common in severe traumatic brain injury and fluctuate significantly over at least 6 months; there is a correlation with age and radiological findings. Performance of hormonal analysis evaluation should be considered in patients with severe brain injury so that appropriate hormonal replacement can be done to optimise the clinical outcome. [ABSTRACT FROM AUTHOR]

Dysembryoplastic neuroepithelial tumor (DNT) is a relatively newly described entity and is an important cause of intractable epilepsy. We report 32 cases of DNT who were operated and treated in our hospital over a period of 12 years. Immunostaining for various proliferative markers and tumor suppressor gene proteins was done to assess the proliferative potential of these tumors. The most common presentation was partial complex seizures followed by generalized tonic–clonic seizures, focal motor seizures, and myoclonus. The most common location was temporal lobe followed by frontal and in one patient lesion was multifocal. All patients were seizure free at the last postoperative follow-up which varies from 12 to 96 months with mean of 33.7 months. Microscopic examination showed classical histology comprising of intracortical multinodular microcystic lesions with floating neurons. Proliferative indices were very low (<1%) and tumor suppressor gene protein expression was not seen in the present study. Cortical dysplasia of the surrounding brain was observed in 37.3% of cases. [ABSTRACT FROM AUTHOR]

Neurocysticercosis is the commonest CNS parasitic disease worldwide but cysticercal meningitis and intraventricular lesions are relatively rare, especially in Indian patients. We herein report a girl with cysticercal meningitis that remained undiagnosed and the patient later presented with unilateral hydrocephalous due to Foramen of Monroe block by an intraventricular cyst. The need for CSF examination with Wright-Giemsa staining to avoid missing CSF eosinophilia is discussed. [ABSTRACT FROM AUTHOR]

Spinal cord teratomas are uncommon tumours. The diagnosis is rarely suspected before surgery and final diagnosis is invariably made at histopathological examination. We retrospectively analysed our neuropathology records for the last 20 years and all patients diagnosed as spinal teratomas were retrieved. The clinical and radiological features were noted. Slides were reviewed and the diagnosis was confirmed. During a period of 20 years, we found 27 examples of spinal teratomas. There was a wide age range and 37.3% presented in adulthood. Male predominance was observed both in children as well as adults. The dorso-lumbar location was common in children as well as in adults but an intramedullary location, in association with a myelomeningocele and split cord malformation, was more common in children. Twenty one patients had associated features of spinal dysraphism. Of the six who were not associated with this, five were adult patients. Radiologically mixed solid and cystic morphology, fat signal and areas of calcification were seen in one third of patients. All tumours except one showed benign (mature) components. Interestingly, two lesions contained mature renal tissue: one exhibited pulmonary differentiation and one had Wilm’s tumour as a component of the teratoma. In five patients the initial sections showed epidermis with skin adenexal structures and a diagnosis of dermoid cyst was suspected but serial sectioning revealed other teratomatous components. All patients were treated with surgical excision. Although there are no specific features on imaging, mixed solid and cystic morphology, fat signal and areas of calcification are helpful in the pre-operative diagnosis. Most of these lesions are diagnosed on histopathological examination after surgery and therefore thorough sampling and serial sectioning are mandatory to identify all components of the teratoma in order to avoid misdiagnosis. [ABSTRACT FROM AUTHOR]

A case of visual hallucination, headache and left hemiparesis is reported. The patient had a history of recurrent attacks of similar semiology for the previous 15 years. MRI brain revealed a cortical hyperintensity on T2W, FLAIR and diffusion weighted imaging (DWI) in the right cerebral hemisphere with a normal ADC (apparent diffusion coefficient) map and MR angiogram. Detailed workup for MELAS was negative. A diagnosis of sporadic hemiplegic migraine was made and he was managed conservatively. He made a gradual complete recovery over 2 weeks. He was discharged on flunarizine for prophylaxis and has remained asymptomatic over the ensuing 4 months. This interesting condition is reviewed and discussed herein. [ABSTRACT FROM AUTHOR]

25 patients of bronchial asthma with chronic sinusitis were operated with the aim to evaluate the efficacy of functional endoscopic sinus surgery (FESS) on asthma symptoms. The mean postoperative daytime asthma symptom scores, nocturnal asthma symptom scores, mean postoperative nocturnal awakening scores and number of asthma attacks reduced significantly (p<0.001). The mean FEV1 observed postoperatively showed significant increase (p<0.001). 21 patients reported overall significant improvement in the asthma. 6 patients were completely taken off from all sort of anti-asthmatic drugs [inhalational corticosteroids (ICS) + long acting β2FTC agonist (LABA)]. In 3 patients there was significant substantial decrease in the dosage of anti-asthma drugs. It is concluded that FESS is a viable option for improving the control of asthma. [ABSTRACT FROM AUTHOR]

Nasal obstruction is a common symptom. Rhinomanometry is a tool to objectively assess the nasal airway. A prospective study was undertaken to assess (he nasal airflow and nasal resistance in 25 patients of deviated nasal septum undergoing septoplasty using rhinomanometry preoperatively and postoperatively. Rhinomanometric improvement in nasal airflow and decreased nasal resistance were found in 88%, patients after surgery. [ABSTRACT FROM AUTHOR]

The phytochromes are a family of red/far-red light absorbing photoreceptors that control plant developmental and metabolic processes in response to changes in the light environment. We report here the overexpression of Arabidopsis thaliana PHYTOCHROME A (PHYA) gene in a commercially important indica rice variety ( Oryza sativa L. Pusa Basmati-1). The expression of the transgene was driven by the light-regulated and tissue-specific rice rbcS promoter. Several independent homozygous sixth generation (T5) transgenic lines were characterized and shown to accumulate relatively high levels of PHYA protein in the light. Under both far-red and red light, PHYA-overexpressing lines showed inhibition of the coleoptile extension in comparison to non-transgenic seedlings. Furthermore, compared with non-transgenic rice plants, mature transgenic plants showed significant reduction in plant height, internode length and internode diameter (including differences in cell size and number), and produced an increased number of panicles per plant. Under greenhouse conditions, rice grain yield was 6–21% higher in three PHYA-overexpressing lines than in non-transgenic plants. These results demonstrate the potential of manipulating light signal-transduction pathways to minimize the problems of lodging in basmati/aromatic rice and to enhance grain productivity. [ABSTRACT FROM AUTHOR]

Malignant rhabdoid tumor (MRT) most commonly occurs in kidney. In the central nervous system, cerebellum is the most common site of occurrence. CNS rhabdoid tumors typically occur in small children, do not respond favorably to treatment and are usually fatal within 1-year. Here is reported a 4-year-old child who presented with features of raised intracranial pressure. Apart from papillodema, there were no neurological signs. Imaging revealed a left lateral ventricular heterogeneous mass abutting the foramen of monro, with mild irregular contrast enhancement and hydrocephalus. The child underwent right ventriculo-peritoneal shunt followed by craniotomy and gross total tumor resection. He was discharged 10-days after surgery without any neurological deficits. Histopathology revealed features compatible with rhabdoid tumor. Despite radiotherapy and chemotherapy, the child died of progressive disease 10-months after surgery. The highly malignant nature of this tumor makes early diagnosis essential for aggressive management and prognostication. [ABSTRACT FROM AUTHOR]

The authors report seven uncommon cases of paraganglioma of the cauda equina region with reference to their clinical, radiological, and pathological findings. The common presenting symptoms in all the patients were low backache with radiation to both thighs, sensory and motor deficits in four patients, and urinary retention in three patients. One patient presented with ataxia and superficial sidrosis of the brain. Magnetic resonance imaging (MRI) revealed well-circumscribed lesions that were isointense on T1- and T2-weighted images with flow voids. All the tumors were well circumscribed and could be excised completely; however, one recurred. Histological examination revealed a classic “zellballen” pattern in four tumors. Two mimicked ependymoma but were confirmed as paraganglioma by immunohistochemistry. MIB-1 LI was low in all the tumors. Thus, there were no identifying clinical or radiological features that helped in differentiating paraganglioma from other common tumors, such as ependymoma or neurinoma, in this region, and preoperative diagnosis was not possible in any of the cases. Morphologic features and immunohistochemical analysis proved to be the mainstay of arriving at a correct diagnosis. This report highlights the significance of important morphologic features and immunohistochemistry in the diagnosis of paraganglioma at this unusual site. [ABSTRACT FROM AUTHOR]

The present case study focuses on indoor radon dispersion in an experimental room made of unfired mud brick by employing CFD modeling, active measurement, and passive measurement in closed and open room conditions. Radon flux for walls and floor surfaces of the room was measured experimentally. A CFD model was designed to simulate spatial distribution pattern of indoor radon. The CFD simulation results are validated through a newly developed RnDuo monitor and single-entry pinhole-based twin-cup dosimeter. The mean indoor radon concentration decreases by approximately one-fourth level in an open room as compared to a closed room. [ABSTRACT FROM AUTHOR]

Background: Intracranial mesenchymal chondrosarcoma (IMC) is a rare malignant tumor in pediatric population. IMC can present as extra- or intra-axial lesion in pediatric patients, though the former is commoner causing raised intracranial pressure (ICP). Radiological diagnosis is a challenge in these cases, as is it difficult to differentiate these from other extra-axial neoplasms due to the wide differential diagnosis in pediatric population. We aim to systematically review the literature and present a rare case of extraskeletal intracranial mesenchymal chondrosarcoma treated with safe maximal resection.A systematic review of literature was conducted in accordance with PRISMA guidelines. PubMed and Scopus databases were queried using the search terms, “primary intracranial chondrosarcoma”, “extraskeletal mesenchymal chondrosarcoma”, “mesenchymal chondrosarcoma” and “pediatric”. Presentation, surgical management and outcome of a 15-year-old male with an extraskeletal IMC are also described.The search yielded 25 articles which met the inclusion criteria. These published records consisted of 33 IMC cases with mean age at presentation of 9.81 ± 5.2 years (range 2 months to 18 years). Frontal region was the commonest locations (11, 33.3%). Most common presentation was headache (14, 42.4%). All patients underwent surgical intervention: gross total resection (20, 60.6%), subtotal resection (9, 27.3%) and no extent mentioned (4, 12.1%). No adjuvant therapy was received in 15 patients (45.5%). On latest follow-up, 11 patients (33.3%) are on remission, 5 patients (15.2%) are symptom free, 3 patients (9.1%) had recurrence, 2 patients (6.1%) had metastasis and 9 patients (27.3%) expired.IMC is a rare entity in pediatric population with imaging findings which are non-characteristic leading to its diagnostic challenge. It can masquerade as other extra-axial intracranial neoplasm (meningioma or hemangiopericytoma). Combination of clinico-radiological and pathological examination can help in accurate diagnosis.  Safe Maximal resection followed by radiotherapy is the preferred treatment strategy.Methods: Intracranial mesenchymal chondrosarcoma (IMC) is a rare malignant tumor in pediatric population. IMC can present as extra- or intra-axial lesion in pediatric patients, though the former is commoner causing raised intracranial pressure (ICP). Radiological diagnosis is a challenge in these cases, as is it difficult to differentiate these from other extra-axial neoplasms due to the wide differential diagnosis in pediatric population. We aim to systematically review the literature and present a rare case of extraskeletal intracranial mesenchymal chondrosarcoma treated with safe maximal resection.A systematic review of literature was conducted in accordance with PRISMA guidelines. PubMed and Scopus databases were queried using the search terms, “primary intracranial chondrosarcoma”, “extraskeletal mesenchymal chondrosarcoma”, “mesenchymal chondrosarcoma” and “pediatric”. Presentation, surgical management and outcome of a 15-year-old male with an extraskeletal IMC are also described.The search yielded 25 articles which met the inclusion criteria. These published records consisted of 33 IMC cases with mean age at presentation of 9.81 ± 5.2 years (range 2 months to 18 years). Frontal region was the commonest locations (11, 33.3%). Most common presentation was headache (14, 42.4%). All patients underwent surgical intervention: gross total resection (20, 60.6%), subtotal resection (9, 27.3%) and no extent mentioned (4, 12.1%). No adjuvant therapy was received in 15 patients (45.5%). On latest follow-up, 11 patients (33.3%) are on remission, 5 patients (15.2%) are symptom free, 3 patients (9.1%) had recurrence, 2 patients (6.1%) had metastasis and 9 patients (27.3%) expired.IMC is a rare entity in pediatric population with imaging findings which are non-characteristic leading to its diagnostic challenge. It can masquerade as other extra-axial intracranial neoplasm (meningioma or hemangiopericytoma). Combination of clinico-radiological and pathological examination can help in accurate diagnosis.  Safe Maximal resection followed by radiotherapy is the preferred treatment strategy.Results: Intracranial mesenchymal chondrosarcoma (IMC) is a rare malignant tumor in pediatric population. IMC can present as extra- or intra-axial lesion in pediatric patients, though the former is commoner causing raised intracranial pressure (ICP). Radiological diagnosis is a challenge in these cases, as is it difficult to differentiate these from other extra-axial neoplasms due to the wide differential diagnosis in pediatric population. We aim to systematically review the literature and present a rare case of extraskeletal intracranial mesenchymal chondrosarcoma treated with safe maximal resection.A systematic review of literature was conducted in accordance with PRISMA guidelines. PubMed and Scopus databases were queried using the search terms, “primary intracranial chondrosarcoma”, “extraskeletal mesenchymal chondrosarcoma”, “mesenchymal chondrosarcoma” and “pediatric”. Presentation, surgical management and outcome of a 15-year-old male with an extraskeletal IMC are also described.The search yielded 25 articles which met the inclusion criteria. These published records consisted of 33 IMC cases with mean age at presentation of 9.81 ± 5.2 years (range 2 months to 18 years). Frontal region was the commonest locations (11, 33.3%). Most common presentation was headache (14, 42.4%). All patients underwent surgical intervention: gross total resection (20, 60.6%), subtotal resection (9, 27.3%) and no extent mentioned (4, 12.1%). No adjuvant therapy was received in 15 patients (45.5%). On latest follow-up, 11 patients (33.3%) are on remission, 5 patients (15.2%) are symptom free, 3 patients (9.1%) had recurrence, 2 patients (6.1%) had metastasis and 9 patients (27.3%) expired.IMC is a rare entity in pediatric population with imaging findings which are non-characteristic leading to its diagnostic challenge. It can masquerade as other extra-axial intracranial neoplasm (meningioma or hemangiopericytoma). Combination of clinico-radiological and pathological examination can help in accurate diagnosis.  Safe Maximal resection followed by radiotherapy is the preferred treatment strategy.Conclusion: Intracranial mesenchymal chondrosarcoma (IMC) is a rare malignant tumor in pediatric population. IMC can present as extra- or intra-axial lesion in pediatric patients, though the former is commoner causing raised intracranial pressure (ICP). Radiological diagnosis is a challenge in these cases, as is it difficult to differentiate these from other extra-axial neoplasms due to the wide differential diagnosis in pediatric population. We aim to systematically review the literature and present a rare case of extraskeletal intracranial mesenchymal chondrosarcoma treated with safe maximal resection.A systematic review of literature was conducted in accordance with PRISMA guidelines. PubMed and Scopus databases were queried using the search terms, “primary intracranial chondrosarcoma”, “extraskeletal mesenchymal chondrosarcoma”, “mesenchymal chondrosarcoma” and “pediatric”. Presentation, surgical management and outcome of a 15-year-old male with an extraskeletal IMC are also described.The search yielded 25 articles which met the inclusion criteria. These published records consisted of 33 IMC cases with mean age at presentation of 9.81 ± 5.2 years (range 2 months to 18 years). Frontal region was the commonest locations (11, 33.3%). Most common presentation was headache (14, 42.4%). All patients underwent surgical intervention: gross total resection (20, 60.6%), subtotal resection (9, 27.3%) and no extent mentioned (4, 12.1%). No adjuvant therapy was received in 15 patients (45.5%). On latest follow-up, 11 patients (33.3%) are on remission, 5 patients (15.2%) are symptom free, 3 patients (9.1%) had recurrence, 2 patients (6.1%) had metastasis and 9 patients (27.3%) expired.IMC is a rare entity in pediatric population with imaging findings which are non-characteristic leading to its diagnostic challenge. It can masquerade as other extra-axial intracranial neoplasm (meningioma or hemangiopericytoma). Combination of clinico-radiological and pathological examination can help in accurate diagnosis.  Safe Maximal resection followed by radiotherapy is the preferred treatment strategy. [ABSTRACT FROM AUTHOR]

In the original publication of the article, the middle name was missing in corresponding author's name. [ABSTRACT FROM AUTHOR]

considerable controversy surrounds the advisability and efficacy of middle turbinate resection in endoscopic sinus surgery. Some are in favor of middle turbinate preservation considering it to be important anatomic and physiological structure and others advocate middle turbinate resection as it improves nasal air flow and decreases synechiae formation. So a study was undertaken to evaluate the efficacy of middle turbinate resection in patients of chronic sinusitis undergoing Functional Endoscopic Sinus Surgery (FESS). 40 patients of chronic sinusitis were selected and divided into 2 groups, group I undergoing FESS with middle turbinate preservation and group II undergoing FESS along with middle turbinate resection. Final results were recorded 6 months after the operative procedure. 50% patients had relief in nasal obstruction in group I as compared to 88% in group II. Postoperative synechiae formation was seen in five patients in group I and in one patient in group II. [ABSTRACT FROM AUTHOR]

Ingestion of a foreign body is a problem seen in nearly all otolaryngologie practices. Ingested foreign bodies which migrate extraluminally are rare occurrences If untreated, they may result in life threatening complications. We are reporting a case of sharp metallic pin which migrated extraluminally in the hypopharynx and became lodged in the soft tissue. It was successfully removed by direct laryngoscopy. [ABSTRACT FROM AUTHOR]

A study was conducted to assess the merits and demerits of endoscopic septoplasty. Fifty patients having symptomatic DNS were randomly divided into two groups of 25 patients each. One group underwent endoscopic septoplasty and other group underwent conventional septoplasty. The groups were compared regarding the complaints with pack in postoperative period, relief of symptoms after surgery and complications. The symptoms complained by the patients with pack in postoperative period and complications after surgery were significantly, less in endoscopic septoplasty group. [ABSTRACT FROM AUTHOR]

A 6-year-old girl who presented with developmental delay and non-progressive ataxia is described. MRI of brain showed agenesis of cerebellar vermis with fusion of cerebellar hemispheres and dentate nuclei. MRI findings were characteristic of Rhombencephalosynapsis. Partial agenesis of corpus callosum and absent septum pellucidum were also seen. The child had also been noted to have a single umbilical artery at birth: a hitherto undescribed association. [ABSTRACT FROM AUTHOR]

Lissencephaly (LIS) is a brain malformation manifested by a smooth cerebral surface, thickened cortical mantle and microscopic evidence of incomplete neuronal migration, excluding polymicrogyria and other cortical dysplasias. It is important to consider LIS in the diagnosis of developmental delay as many patients may be diagnosed as cerebral palsy. It may have familial occurrence and can occur in sibs of same family often leading to a diagnostic problem. Several lissencephaly syndromes have been described. Here a familial syndrome of lissencephaly is reported. Autosomal recessive inheritance is suggested by recurrence in sibs within the same family, but germ cell mosaicism for a dominant mutation cannot be excluded. [ABSTRACT FROM AUTHOR]

Following trauma, the commonly used radiological investigations, plain radiographs and computed tomography (CT) studies do not rule out injury to the spinal cord. This is especially true for children, as an entity known by the acronym SCIWORA (spinal cord injury without radiological abnormality) exists and the changes may be picked up only on magnetic resonance imaging (MRI). Early treatment (within 6 hours) with high dose methylprednisolone improves the outcome. Spinal trauma being common it is possible that the burden of neurological handicap following this can be reduced by increasing awareness and early treatment with steroids. In the community, pediatricians are often the first medical contact after spinal trauma and awareness of the lacune of conventional imaging techniques is important especially if clinical symptoms pertaining to the spine are present. The community pediatrician is hereby made aware of the need to investigate spinal trauma with a MRI for possible SCIWORA situation as it generates a possibility for therapeutic intervention to alter the outcome positively. [ABSTRACT FROM AUTHOR]

Gliomatosis cerebri is a rare and typically fatal tumor of neuroepithelial origin causing a diffuse infiltration of brain structures with mild or negligible destruction of parenchymal architecture. Very few cases have been reported in children. A 6-year-old boy who presented with worsening hemiplegia, behaviour problems and seizures after an episode of encephalitis-like illness is reported. MRI revealed diffuse signal change and swelling of the left cerebral hemisphere. The diagnosis of gliomatosis cerebri was confirmed by brain biopsy. Parents refused radiotherapy and the child worsened and died 6 months after diagnosis. [ABSTRACT FROM AUTHOR]

Cricopharyngeal achalasia is a rare clinical entity in which upper esophagael sphincter (cricopharyngeus) does not open adequately during swallowing leading to dysphagia. Barium swallow reveals a smooth posterior impression cricopharyngeus at C6 level. We report cricopharyngeal achalasia in a 68 year male who was managed with dilatation. [ABSTRACT FROM AUTHOR]

The article focuses on the Tessier cleft No. 5 type of oblique facial cleft. It describes oblique facial cleft as an extremely rare and disfiguring congenital abnormality of the face in which Tessier clefts affect eyebrows, eyelids, maxilla, nose and lip. Tessier cleft No. 5 is cited as the least frequently found among all facial clefts. Under such cleft condition, the cleft passes lateral to infraorbital foramen yet the pyriform aperture is not affected.