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Background Mucormycosis of the central nervous system is an uncommon infection caused by saprophytic or parasitic fungi of the subphylum Mucormycotina and order Mucorales viz. Rhizopus, Mucor, and Rhizomucor. Isolated, chronic involvement of the central nervous system is a rare occurrence. To the best of our knowledge, isolated chronic ventricular involvement in an infant has not been reported previously. Isolated intracerebral mucormycosis is a disease of the immunocompromised patient, and to date only 6 cases have been reported in immunocompetent patients, including 2 pediatric cases. Case Description We present the case of an immunocompetent infant presenting with features of increased intracranial tension. He underwent cerebrospinal fluid diversion and was found to harbor mucormycosis on histopathologic examination of intraventricular debris. We also present a brief review of the relevant literature. Conclusions Although mucormycosis is an acute fulminant infection, chronic isolated cerebral cases are known in the immunocompetent patient. Patients also may present with isolated hydrocephalus, and hence fungal infection must be ruled out in all, especially if a shunt is warranted.

Background A majority of patients with glioneuronal tumors present with seizures. Although a number of studies have shown that greater extent of resection improves overall patient survival, few studies have focused on post-operative seizure outcome after resection of these tumors. We aim to characterize seizure control rates in patients undergoing glioneuronal tumor resection and evaluate the association between poor seizure outcome and tumor recurrence or progression. Methods The study population included patients who had undergone resection of glioneuronal tumors between 2014 and 2019 at our institution. Seizure outcome was assessed using Engel grading. Preoperative seizure characteristics, tumor characteristics, surgical factors, and postoperative seizure outcomes were reviewed. Results 26 patients (n=16, temporal lobe; n=6, frontal lobe; n=4, parietal lobe) with mean seizures duration of 56.9-months, were assessed. Histopathologically, n=15 dysembryoplastic neurepithelial tumour, n=7 ganglioglioma and n=4 Diffuse lepto-meningeal neuroepithelial tumour. There were 2 cases of complex DNET and one case of DLMNT had associated cortical dysplasia. At mean follow-up of 49.7 months, n=20 Engel 1, n=4 Engel 2 and n=2 had Engel 3 outcome. n=20 underwent gross total excision (n=18 Engel 1 and n=2 Engel 2) and n=6 sub-total excision. Among the 4 patients who needed resurgery, two were in Engel 2 and another two were in Engel 3. Conclusions Good seizure-outcome is likely associated with extent of resection. Younger age of patient, less than one-year of seizure duration and absence of generalization of seizure are good prognostic indicators. The best seizure-control can be achieved by early surgical intervention.

Background: Mesial temporal lobe epilepsy attributed to low-grade glioma is known for intractable seizures and choice of surgery range from lesionectomy (Lo) to lesionectomy with anteromesial temporal resection (L0 + AMTR) is still debatable. We intend to analyze the seizure outcome after lesionectomy alone or with AMTR. Subjects and Methods: Retrospective analyses of patients operated for medial low-grade temporal lobe tumors with seizures were included in the study. Preoperative records include video-electroencephalographic, magnetic resonance imaging (epilepsy protocol), and neuropsychological evaluation for language, memory, and dominance were assessed. Two groups (Lo [Group I] and Lo + AMTR [Group II]) were assessed after surgery by the international league against epilepsy (ILAE) seizure outcome scale. Results: A total of 39 patients underwent Lo (n = 20) and Lo + AMTR (n = 19) with a mean age of 26.92 ± 12.96 months, and mean duration of seizures was 36.87 46.76 months. A total of 23 patients had long-term intractable seizures for >1 year despite >2 drugs(Group I [n = 10], Group II [n = 13]); remaining 16 had frequent seizures of

Background Tumors presenting with drug-resistant seizures are termed as long-term epilepsy-associated tumors (LEATs). LEATs are more common in the temporal lobe, occur predominantly in pediatric age, and focal neurological deficits are rare. In this article, we aim to highlight our surgical experience in terms of seizure outcome among LEATs and discuss the factors affecting outcome. Methodology We have retrospectively analyzed all the operated cases of intra-axial brain tumors with seizures (2015–2019). The clinical and radiographic data were collected from the hospital record system. For comparison, 2 groups were made (group 1 with good seizure control, i.e., Engel 1; and group 2 poor seizure outcome, i.e., Engel 2 and 3). Results A total of 51 cases were included; the temporal lobe was the most common location (n = 27); 23 patients had seizure frequency of “more than 1 seizure per week.” Focal unaware seizures/complex partial seizures were the most common type of seizures encountered (n = 28). At a mean follow-up of 39.60 months, 38 patients had Engel 1 (78.5%) outcome (35 cases [71.05%] had the seizure duration of ≤2 years). The median duration of symptoms (group 1, 25 months vs. group 2, 65 months) was significantly different (P = 0.002). On comparing patients with seizure duration, we found a statistically significant difference (P Conclusion A shorter duration of symptoms, younger age of the patient, partial/focal seizures, and gross total excision were predictors of a good seizure outcome. Histopathology of the tumor does not affect the outcome when one compares glioneuronal tumors with non–glioneuronal tumors.

Intracranial peripheral primitive neuroectodermal tumors (pPNETs) are rare lesions, accounting for only 1% of all pPNETs, and usually arise from bone and soft tissue. These tumors primarily affect young patients (30 years old) and rarely affect older patients. The common intracranial locations of these lesions are frontal dura and tentorium. These lesions rarely may involve the cerebellopontine angle, cavernous sinus, and jugular foramen. Involvement of clivus has not been reported to date. Intracranial metastasis from primary intracranial pPNET is a further rare occurrence.A 74-year-old woman presented with gradually progressive painless diminution of vision in both eyes alongwith headache for 6 months duration. Radiology demonstrated a clival mass as well as a suspected intracranial metastasis. Histopathology was suggestive of pPNET. We also present a brief review of the literature on pPNETs.pPNETs of the clival region are exceptionally rare. In elderly patients with clival mass lesions, the rare possibility of pPNET should be kept in mind.

Reactive oxygen species (ROS) plays an important role in senescence, which was studied for cotyledon senescence in mung bean (Vigna radiata) seeds following germination in the present work. Morphology and chlorophyll content analysis revealed that cotyledons undergo a short developmental stage followed by senescence when attached with axis while detachment prolonged such developmental changes and delayed senescence. Histolocalization and spectrophotometric analysis of ROS revealed a positive correlation between the total chlorophyll content and extracellular superoxide (O2•−) level that accumulated around chlorophyllous regions, whereas hydrogen peroxide (H2O2) was mainly localized in the peripheral cells, wherefrom storage mobilization started for both intact and isolated cotyledons. Among the antioxidant enzymes, catalase (CAT, EC 1.11.1.6) and ascorbate peroxidase (APX, EC 1.11.1.11) activity declines when peroxidase (POX, EC 1.11.1.7) and superoxide dismutase (SOD, EC 1.15.1.1) activity was maintained during cotyledon senescence. POX activity, which was mainly localized in the cell wall, showed a positive correlation with total chlorophyll content for both intact and isolated cotyledons, whereas CAT activity showed positive and negative correlation with total chlorophyll content for intact and isolated cotyledons, respectively. On the other hand, a clear negative correlation was shown by APX activity of both intact and isolated cotyledons, whereas a highly positive correlation was established for SOD activity with total chlorophyll content of intact cotyledons only. Possible association of O2•− with cotyledon development and H2O2 with storage mobilization followed by senescence has been proposed.

Background: The Ewing sarcoma peripheral PNET (ES-pPNET) is very rare small round cell tumour that involves the CNS as either a primary dural neoplasm or by direct extension from contiguous bone or soft tissue. Materials and Methods: Biopsy proven cases of intracranial ES/pPNET with orbital involvement operated during Jan 2010-Jan 2014 were retrospectively included and their clinical data, operative and histological findings were reviewed from institutional oncology register. Results: seven patients (4 males; 3 female) were studied with mean age at presentation of 13 years. Six patients had orbital involvement in one or other form. Surgical excision was gross total in five, near total in one, and subtotal in one patient. All patients received adjuvant therapy, only chemotherapy in 2, only Radiotherapy in four, both in one. MRI characteristics were studied in six patients. Four patients died with average survival of 33.2 months and three patients are having Progression free survival of average 23.3 months. Conclusions: The EWS-pPNET is very rare tumour and very poorly described in literature. These tumours are showing special predilection for the frontotemporal dura and erode through the flat bone of cranium like orbital roof and lateral wall of the orbit. These tumours are aggressive, multi compartmental, vascular and very rapidly growing, so missing or overlooking the primary symptoms of dural stretching/bony involvement leads to delay in management and poor outcome.

Pleomorphic xanthoastrocytomas are rare tumors of glial origin comprising1% of all astrocytic tumors of brain. These tumors are rare in the infratentorial compartment and were not known to arise from the middle cerebellar peduncle.We discuss the case of a 16-year-boy who presented with altered sensorium and features of increased intracranial pressure and was found to have a 4 × 4 × 3 cm mass arising from the middle cerebellar peduncle.We also discuss the histopathologic features of these tumors suggesting their resemblance to other maldevelopmental tumors, the rarity of pure World Health Organization grade II pleomorphic xanthoastrocytoma in the infratentorial compartment, as well as the appropriate management.

Angiocentric glioma (AG) is a relatively uncommon clinico-pathological entity that presents in childhood. Angiocentric glioma displays various histopathological features which resemble cortical ependymoma, astroblastoma, and pilomyxoid astrocytoma and schwannoma. The astrocytes in angiocentric glioma appear peculiarly elongated, bipolar in shape, and characteristically arranged around blood vessels. They resemble radial glia and tanycytes morphologically. Unlike ependymomas, AG is a diffusely infiltrating lesion and perivascular processes are often much thicker than those in classic ependymomas. AG usually present clinically as seizures, often as medically intractable epilepsy. In the indexed case, apart from unusual presentation with features of raised intra-cranial tension, an unusual histological picture of a more cellular oligodendroglioma like component was also seen. The appropriate diagnosis is critical as AG is usually slowly growing and treatable by surgical excision alone.

Background Cerebellar liponeurocytoma (cLNC) is a rare benign glioneuronal tumor with only ~60 cases reported since its first description in 1978. They have occurred largely as sporadic cases; however, familial cases with possible autosomal dominant inheritance have also been reported. Surgical excision has been considered the main modality of treatment, even for recurrent lesions. Uncertainties exist regarding the natural history, long-term outcomes, and optimal postoperative management and follow-up protocols for cLNC in general and multifocal cLNC in particular. Multifocality is exceedingly rare, with only 4 cases reported previously. Case Description We present a case of multifocal cLNC in a young woman who had presented with progressive cerebellar symptoms of 5 months’ duration. Her diagnostic neuroimaging evaluations revealed 2 asymmetric mass lesions (1 on each cerebellar hemisphere). We excised the larger lesion to relieve the mass effect and decided to monitor the smaller lesion radiologically. However, she developed a new lesion during the follow-up period and required adjuvant radiotherapy. Conclusion Surgical excision forms the cornerstone of management of cLNCs. However, it is necessary to vigilantly monitor these patients owing to the high recurrence rates of cLNC despite the innocuous histologic features.

An 11-year-old neutropenic female child with acute lymphoblastic leukemia (ALL) developed a large right frontal mass a month following the induction of chemotherapy. A well encapsulated mass on surgical excision turned out to be aspergilloma with metastatic infiltration in frontal lobe. A genetic defect in form of microsatellite instability was also demonstrated in frontal mass. A possibility of fungal granuloma in a neutropenic child treated for ALL (on chemotherapy) remained strong on clinico-radiological evaluation. However, the cranial involvement in ALL also amounts to be 50 to 80% in untreated children. The child under discussion had a rare manifestation of both leukemic infiltration and fungal granuloma formation. Though the microsatellite instability was demonstrated in the mass, but further genetic studies would be required to establish the role of genetic defect in evolution of such cerebral masses/leukemic deposits. (J Pediatr Neurol 2004; 2(1): 39-43).

Context: Multiple glioblastomas (GBMs) have a reported incidence of 2-20%. Aims: We intend to study these subsets of GBMs to know whether these are similar to their solitary counterparts. Setting and Design: A retrospective study. Materials and Methods: We analyzed 7 cases of biopsy-proven multiple GBMs. Multiple GBMs were described if there were >1 lesion which was at least 1 cm apart. The clinical data, radiological features, histopathological and immunohistochemical analysis and follow-up were recorded. Results: The mean age was 45 years (range 17-69 years). All cases presented with features of raised intracranial pressure (ICP). Totally, 3 cases presented with hemiparesis and 2 cases with altered sensorium and generalized tonic clonic seizures each. The median Karnofsky performance status (KPS) was 50. Mean duration of symptoms was 40 days. All lesions were contrast enhancing (2 with homogenous enhancement and 5 had ring enhancement). Total excision of the lesion causing mass effect was done in all cases. Histopathologically, small cells were significantly present in 4 cases, and satellitosis was seen in 5 cases. Glial fibrillary acidic protein (GFAP) was absent in all cases in which small cells were significant. In these 4 cases, the proliferation index ranged from 40% to 95%. Totally, 3 patients died within 2 months of surgery, whereas remaining 4 patients underwent chemo-radiotherapy. Conclusions: We conclude that the cases usually present with features of raised ICP and poor KPS. Histopathologically these lesions show significant small cell population, satellitosis, and GFAP negativity.

We present a case of brain abscess in an immunocompetent child due to the dematiaceous fungus Microascus cinereus, an organism commonly found in soil and stored grains. The etiologic agent was demonstrated by direct microscopy and culture. The patient responded well to surgical excision of abscess along with a course of amphotericin B and voriconazole. Microascus species have emerged as significant invasive pathogens especially in the immunocompromised patients. To the best of our knowledge, this is the first reported case of brain abscess caused by M. cinereus in an immunocompetent individual with no underlying risk factors.

Context The mechanism behind hypophosphatemia in the setting of neurofibromatosis type 1 (NF1) is not known. We describe a possible role of fibroblast growth factor-23 (FGF23) in the pathophysiology of hypophosphatemia in a patient with NF1. Case description A 34-year woman with NF1 presented with severe hypophosphatemia, osteomalacia, and elevated plasma FGF23. The patient had considerable improvement on replacement of oral phosphate. Two Ga68 DOTANOC PET-CT scans over a period of 2 years failed to detect any localized uptake. Immuno-staining for FGF23 was absent in the neural-derived tumour cells of the neurofibromas in the proband. Conclusion The patient with NF1 had elevated circulating FGF23. Tumour cells in the neurofibroma tissues did not stain for FGF23 on IHC. It is unlikely for neurofibromas to contribute to high circulating FGF23 levels in the proband.

Posterior fossa hemangioblastomas are WHO grade I benign lesions with a surprisingly high recurrence rate. This study determines the factors responsible for recurrence and the clinico-radiological and histopathological differences between primary (group A; n = 60) and recurrent/symptomatic residual (group B; n = 24) tumors. Radiologically, tumors were differentiated into cystic, cystic with a mural nodule, solid-cystic/microcystic and solid. Surgery was undertaken via a midline or lateral suboccipital approach. Histopathology differentiated them into reticular, cellular or mixed subtypes. Truncal-appendicular ataxia in group A and von Hippel-Lindau (VHL) disease and raised intracranial pressure in group B were the predominant presentations. VHL patients in group B had a longer duration of symptoms (median 72 months) than those with non-VHL recurrences (median: 36 months). Multicentric mural nodules (n = 9/24, 37.50%, P = 0.0001) and bilateral cerebellar hemispheric involvement (n = 6/24, 25%, P = 0.0003) were exclusively seen in the preoperative radiology of group B tumors. Brainstem involvement was seen in the tumors of ten (16.67%) patients in group A and six (25.00%) patients in group B. One subset of patients required several resurgeries for repeated recurrences. Abnormal vascular proliferation and papillary projections into adjacent brain, and highly cellular stroma were unique histological features at recurrence. Total resection was achieved in 45 group A and 19 group B patients. The outcome based on the Karnofsky performance scale (KPS) was assessed at follow-up at 6 weeks (A: n = 60, B: n = 24): KPS0: A = 4, B = 1; KPS10–40 (dependent): A: 4, B = 5; KPS50–70 (independent for daily needs): A = 36, B = 16; KPS80–100 (fully independent): A = 16; B = 2. In recurrent/residual tumors, the radiological as well as histopathological features showed a distinctive change toward a more aggressive nature. Higher incidences of multiple mural nodules, bilateral spread, remote recurrence and VHL disease at preoperative radiology; as well as proliferative angioarchitecture, an irregular brain tumor interface and highly cellular stroma at histology were found in these patients compared to their primary counterparts.

Purpose Qualitative (assignment of lipid components) and quantitative (quantification of lipid components) analysis of lipid components were performed in skeletal muscle tissue of patients with muscular dystrophy in early phase of the disease as compared to control/normal subjects. Methods Proton nuclear magnetic resonance (NMR) spectroscopy based experiment was performed on the lipid extract of skeletal muscle tissue of patients with muscular dystrophy in early phase of the disease and normal individuals for the analysis of lipid components [triglycerides, phospholipids, total cholesterol and unsaturated fatty acids (arachidonic, linolenic and linoleic acid)]. Specimens of muscle tissue were obtained from patients with Duchenne muscular dystrophy (DMD) [ n = 11; Age, Mean ± SD; 9.2 ± 1.4 years; all were males], Becker muscular dystrophy (BMD) [ n = 12; Age, Mean ± SD; 21.4 ± 5.0 years; all were males], facioscapulohumeral muscular dystrophy (FSHD) [ n = 11; Age, Mean ± SD; 23.7 ± 7.5 years; all were males] and limb girdle muscular dystrophy-2B (LGMD-2B) [ n = 18; Age, Mean ± SD; 24.2 ± 4.1 years; all were males]. Muscle specimens were also obtained from [ n = 30; Mean age ± SD 23.1 ± 6.0 years; all were males] normal/control subjects. Results Assigned lipid components in skeletal muscle tissue were triglycerides (TG), phospholipids (PL), total cholesterol (CHOL) and unsaturated fatty acids (arachidonic, linolenic and linoleic acid)]. Quantity of lipid components was observed in skeletal muscle tissue of DMD, BMD, FSHD and LGMD-2B patients as compared to control/normal subjects. TG was significantly elevated in muscle tissue of DMD, BMD and LGMD-2B patients. Increase level of CHOL was found only in muscle of DMD patients. Level of PL was found insignificant for DMD, BMD and LGMD-2B patients. Quantity of TG, PL and CHOL was unaltered in the muscle of patients with FSHD as compared to control/normal subjects. Linoleic acids were significantly reduced in muscle tissue of DMD, BMD, FSHD and LGMD-2B as compared to normal/control individuals. Conclusions Results clearly indicate alteration of lipid metabolism in patients with muscular dystrophy in early phase of the disease. Moreover, further evaluation is required to understand whether these changes are primary or secondary to muscular dystrophy. In future, these findings may prove an additional and improved approach for the diagnosis of different forms of muscular dystrophy.

Isolated mitochondrial myopathy is characterized by slowly progressive limb-girdle muscle weakness and resembles other muscle disorders like muscular dystrophy or inflammatory myopathy on clinical grounds. Identification of abnormal mitochondria in the muscle tissue is required for the diagnosis of isolated mitochondrial myopathy. Therefore, this study was done with aim to identify patients with isolated mitochondrial myopathy among those with limb-girdle muscle syndromes of undefined cause. Forty-eight consecutive patients with limb-girdle muscle disease from 2008 to 2010 were screened for Duchenne/Becker muscular dystrophy gene deletion, metabolic myopathy, and drug-induced and endocrine causes. Twenty patients without an identifiable cause were subjected to muscle biopsy for hematoxylin and eosin staining and enzyme histochemistry. Clinical, biochemical, and electrophysiological features in all these patients with limb-girdle muscle disease were nonspecific, and no conclusion regarding the underlying cause could be drawn from these investigations. On hematoxylin and eosin staining, 12 patients were diagnosed as muscular dystrophy, inflammatory myopathy with characteristic appearance of polymyositis was diagnosed in 4 patients, and 3 patients had normal muscle histology. After enzyme histochemistry, one patient was identified having mitochondrial myopathy. A brief case summary of the only patient diagnosed as isolated mitochondrial myopathy in our study is presented.

Background The simultaneous presence of Lhermitte-Duclos disease (LDD) with focal areas of nodular dysembryoplastic neuroepithelial tumor (DNET) differentiation in the cerebellar hemisphere is reported in a patient who showed the characteristic magnetic resonance imaging (MRI) appearance of “tiger striping.” Case Report A 25-year-old man presented with a 7-month history of holocranial headache, progressive vision diminution, and right-sided cerebellar signs. Computed tomography (CT) scan revealed a hypodense, nonenhancing right cerebellar lesion effacing the fourth ventricle. There were thin hyperdense, linear striations across the tumor with hypodense streaks between them. The third and lateral ventricles were dilated with periventricular lucency. MRI showed the hypointense-to-isointense right cerebellar lesion with linear striations on T1-weighted images. The lesion was hyperintense on T2-weighted images with areas of linear hypointensity streaks running throughout the lesion characteristic of the “tiger striping” effect of LDD. Sagittal MRI revealed tonsillar herniation. Gross total removal of the tumor was accomplished with right paramedian suboccipital craniectomy. The postoperative course was unremarkable, and the patient remained symptom-free at 3-month follow-up examination. Histology revealed the simultaneous presence of LDD with DNET. Conclusions To the best of the authors' knowledge, the simultaneous presence of cerebellar LDD with DNET has never been reported before. This case report may point to their common genesis with cortical dysplasia and neuronal migrational abnormalities playing an important role. LDD and DNET may represent different points in the spectrum of the lesion—from being purely hamartomatous to having a greater proliferative potential. The simultaneous presence of LDD and DNET was perhaps responsible for the unusually rapid neurologic deterioration in this patient before surgical intervention.

There are significant differences in the prevalence and behavior of differentiated thyroid cancers (DTC) in the iodine-deficient areas (IDA) and iodine-sufficient areas (ISA) of the world. The sodium iodide symporter (NIS), mediates active transport of iodide across the basolateral aspect of the thyroid follicular cell. However, no study had specifically addressed the issue of expression of sodium iodide symporter (NIS) in thyroid cancer specimens from IDA. The aim of the present study was to find an expression pattern of NIS in DTC in an iodine-deficient population, and to correlate it with histological subtypes, i.e., papillary carcinoma (PTC), follicular carcinoma (FTC), poorly differentiated carcinoma (PDTC), as well as with clinicopathological risk factors and iodine ((131)I) uptake by distant metastases.Immunohistochemistry was carried out in 39 cases of thyroid cancer (41 samples) including PTC (15), FTC (10), PDTC (9), anaplastic cancer (5), and resected metastases (2). Expression was correlated with the patient's age, sex, tumor size, presence or absence of extrathyroidal invasion, distant and lymph node metastases, and whole body radioiodine scan.Overall, 61.8% of DTC patients showed NIS expression. There was no significant difference in expression rate between PTC (73.3%) and FTC (70.0%). However, expression was significantly less in PDTC (33.3%). There was no correlation between NIS expression and any clinicopathological risk factor (p.05). The results of NIS expression were not concordant with (131)I uptake by metastases in 4 of 10 cases. (131)I uptake was absent in one case despite the finding that a metastatic site itself showed NIS expression in that case, whereas in the remaining 9 cases (131)I uptake was present although three cases did not show NIS expression.In our experience, overall expression of NIS was comparable to other studies from ISA. We conclude that expression may not accurately predict radioactive iodine (RAI) uptake by metastases.

Primary spinal primitive neuroectodermal tumor (PSPNET) is extremely rare and only 25 cases have been reported in the world literature so far. Three patients of 8, 9 and 18 years of age, who presented with variable grades of neurological deficit were diagnosed as having a dorsal intramedullary lesion, a holocord lesion and cervical extradural tumor with extraspinal extension, respectively, and were operated at our institute. The histopathology of all 3 children revealed PNET. The clinical course, image characteristics and outcome of the 3 children are described, and the relevant literature is reviewed. The following conclusions were drawn from the present study and review of the literature. PNET may manifest itself as a primary lesion of the spine unlike the more common drop metastases from an intracranial lesion. PSPNET may be intramedullary, intradural and extradural with variable extraspinal extension. PSPNET may present as holocord intramedullary lesion, an entity which has not been described earlier. These lesions have a short history, significant neurological deficits and rapid course of illness. PSPNET, though an established entity, did not find a place in the WHO 2000 classification of CNS tumors. Hence its status has to be defined.

Rasmussen's encephalitis (RE) is a form of chronic encephalitis presenting with intractable seizures and progressive neurological deficits in children. The occurrence of anaplastic ependymoma in a diagnosed case of RE has not been reported in the literature.We report an eight and a half year-old boy, a diagnosed case of RE, suffering from intractable right-sided focal seizures, who developed features of raised intracranial pressure during follow-up. Repeat imaging revealed left fronto-temporoparietal mass for which near-total decompression was done. Histopathology of the mass revealed anaplastic ependymoma.The etiology of such a co-existence or development of malignancy in a pre-existing RE is yet to be completely understood. Both these entities could have a common etiology of viral- or autoimmune-mediated process, but no definite conclusion can be drawn with all the literature available at the moment.

Endometriosis involving the neural axis is extremely rare. A twenty-five-year-old woman presented with acute onset paraparesis with bladder involvement of five-day duration. We missed her history of cyclical back-ache related to menses at the emergency room. Magnetic resonance imaging (MRI) of spinal cord showed an intramedullary tumour with bleeding at conus–epiconus level. She was operated in the emergency department with laminectomy–durotomy and tumour decompression. Histopathology of the tumour was suggested endometriosis.

A 25-year-old male presented with off-and-on vertigo of 10-year duration. He had left-sided cerebellar signs, left vocal cord paresis, and minimal left-sided hearing impairment. Computed tomography of the head revealed significant hyperostosis of squamous occipital, mastoid, and petrous temporal bone with no adjacent soft tissue mass. On excision, it turned out to be paraganglioma. Paraganglioma as a pure bony mass is not reported in the literature. The site of origin of such a tumor could not be ascertained, even on generous screening. The tumor remained nonsecretory on clinical and biochemical investigations.

Sodium iodide symporter (NIS) is a molecule involved in active accumulation of iodine in thyroid gland for the biosynthesis of thyroid hormone. Its expression has also been demonstrated in extra-thyroidal tissues including lactating mice mammary gland and also in human breast cancers. Iodide transport in thyroid cells through NIS is the basis for using radioiodine for diagnosis and treatment of differentiated thyroid carcinoma. The similar approach may prove beneficial for the diagnosis and treatment of breast cancer if iodine uptake, its retention and NIS expression can be shown unequivocally in malignant tumors. The aim of the present study was to investigate NIS expression, in vivo iodine transport ability and fate of iodine in human breast tumors. Women (age 33-58 years) with infiltrating duct carcinoma confirmed by FNAC and subsequent histopathology were the subject of this study. Expression of NIS RNA and protein was confirmed by RNAase protection assay, western blot and immunohistochemistry respectively in surgically excised breast tumor tissue. Iodine transport ability and its nature was assessed both in vivo and in vitro. We report high NIS expression at both transcriptional and translational level and its ability to transport iodine in human breast tumors. The in vivo iodine transport ability was confirmed by scintigraphy. Unlike thyroid, perchlorate and thiocyanate do not inhibit iodine transport in breast tumors. The presence of iodinated proteins suggests the longer retention time. The unequivocal demonstration of NIS expression, its functionality and retention of iodine by organification further provides supportive evidence for use of radioiodine as an additional treatment modality of human breast carcinoma.

Purpose The purpose of the current study was to prospectively evaluate the role of corrected gradient echo phase imaging in differentiation of calcified granuloma from chronic hemorrhage. Method Eighty-five patients with single/multiple calcifications and hemorrhages irrespective of their location were studied with corrected gradient echo phase imaging. In all the cases, CT was used as the gold standard for the presence/absence of calcification. Results All calcified lesions showed positive phase, whereas chronic hemorrhages showed negative phase in all cases. Five calcified lesions showed no phase shift at TE =15 ms and positive shift at TE = 35 ms. Heterogeneous phase shift was observed in three calcified lesions at TE = 35 ms; all three lesions showed positive phase shift at TE = 15 ms. There was no site-specific problem in differentiation of calcification from chronic hemorrhage including in the basal ganglia. Conclusion We conclude that calcified granuloma can be easily differentiated from chronic hemorrhage with corrected gradient echo phase imaging, which may obviate the need for CT for its confirmation.

An unusual case of cerebellar neurocytoma with rhabdomyomatous differentiation in a 6-year-old boy is reported. Immunocytochemical and ultrastructural features of the tumour were studied. Abortive synapse formation, the presence of clear vesicles and synaptophysin immunoreactivity of the tumour cells indicated its intermediate neuronal differentiation, while the presence of myoblasts and myotubes and immunolabelling by desmin confirmed the rhabdomyomatous differentiation. In addition, the mesenchymal cells variably expressed neurofilament protein and glial fibrillary acidic protein, suggesting inductive interaction between the neuroectodermal and ectomesenchymal elements and persistence of the pleuripotential nature of the cells along the rhombic area of the brain stem.

We describe a stillborn female with joint contractures, subcutaneous oedema, ectropion, a severely flattened nose, an 'O' shaped open mouth and extensive peeling of skin. The head circumference was normal. She was born at 33 weeks of gestation to consanguineous parents, who had one previous offspring affected with non-immune hydrops fetalis. Pathological, radiological and prenatal findings are reported. The features of the present case are compared with those of Neu-Laxova syndrome, restrictive dermopathy and harlequin fetus.

Human sodium iodide symporter (hNIS), responsible for the active transport of iodine is an integral plasma membrane glycoprotein present in the thyroid cells and extrathyroid tissues like breast and salivary glands. If its functional form is unequivocally shown in benign or malignant breast tissues, then it may serve as a basis for diagnosis and treatment using radioactive iodine. With an aim to analyze the hNIS expression in a distinct benign breast condition of fibroadenoma, biopsy proven fibroadenoma tissues, normal non-lactating breast tissue and biopsy proven infiltrating duct carcinoma tissues were examined for hNIS expression using immunohistochemistry. Out of 20 biopsy proven fibroadenoma tissues, 19 (95%) showed positivity for hNIS protein and only one was negative. Of these 10% were mildly positive, 50% cases were moderately positive and 35% showed intense positivity. None of the control tissue obtained from reduction mammoplasty specimens or normal breast tissues samples (5 cms away from the tumor) were positive, hNIS was also intensely positive in 9 out of 10 (90%) infiltrating duct carcinoma tissues and moderately positive in one case. These preliminary results show that hNIS was present in high frequency as demonstrated by immunohistochemistry in fibroadenoma breast.

Lipomatous meningiomas are an uncommon subtype of meningiomas. In the present report, the clinical characteristics, neuroimaing and pathological features of 5 patients (4 males, 1 female; age range, 17-45 years; mean age, 35.2 years) with lipomatous meningioma were analyzed. The neuro-pathological reevaluation of lipomatous meningioma involved assessment of histological features proposed by the current WHO classification. The presence or absence of high whorls, fascicles, solid growth, glial invasion, necrosis and nuclear pleomorphism was assessed. The most common presenting symptom was seizures. Location of tumor was frontal (3 cases), parietal (1 case) and fronto-temporal (1 case). All cases showed radiological features of conventional meningioma. Complete excision of tumor along with involved dura was performed in all the patients. Three tumors were meningothelial and 2 were transitional. Tumoral lipid content was variable from 10% to 30%. In addition, there were microcystic features (2 cases) and psammoma bodies (1 case). No recurrence was noted in any of our cases till the last follow-up. Lipomatous meningioma represents a rare distinctive type of meningioma, with a good prognosis with complete removal.

Amyloidosis associated with Langerhans cell histiocytosis (LCH) is extremely rare. We here present the 18F-FDG PET/CT images of a 48-year-old male patient with multifocal skeletal involvement of LCH. In addition, he had a left lung upper lobe 18F-FDG avid mass that was misinterpreted as pulmonary involvement of LCH on PET/CT. Biopsy from the mass showed amyloidosis. Therefore, amyloidosis should be kept as a differential for 18F-FDG pulmonary avid nodule.

In vestibular schwannomas (VS), the tumour size, as well as the size of the cystic component, have a considerable bearing on the outcome. This study addresses the differences between the cystic and solid variants of giant vestibular schwannomas. The study included 62 patients with giant VSs, of which 40 were solid and 22 were cystic (those in which cystic component greater or equal to 30% of the total tumour volume). The cystic tumour group was further divided into type A (31-60% volume of the cyst within tumour), type B (61-90% intra-tumoural cyst volume) and type C (more than 90% volume of the cyst). The clinicoradiological features, operative findings, histopathological characteristics and outcome of surgery of the two groups were compared. The mean duration of symptoms for the solid and cystic tumours were 21.1 and 26.2 months, respectively. However, six patients with cystic tumours showed recent and rapid neurological deterioration after a protracted existence. Papilloedema, lower cranial nerve involvement, facial paraesthesias and preoperative hydrocephalus were significantly more in cystic tumours. Total excision was achieved in 38 of the solid and 18 of the cystic tumours. VIIth nerve preservation was higher in the cystic lesions [solid 33/40 (82.5%), cystic 21/22 (95.4%)]. Myxoid degeneration, lobular growth patterns and cellular atypia were more prominent in the cystic variants. The giant vestibular schwannomas were associated with a higher incidence of cystic degeneration than has been reported for smaller tumours in literature. In cystic lesions, VIIth nerve preservation was higher due to early decompression of the lesion that facilitated in early identification of the VIIth nerve, except in patients with type C cystic tumour.

This paper highlights the etiology, diagnosis, management and outcome in nine unusual cases of extrahepatic biliary obstruction in children. Extrahepatic biliary atresia and choledochal cyst constituted 127 out of 136 (93%) cases of all pediatric surgical biliary disorders managed between March 2000 and February 2007 at the reporting centre. However, nine children (aged 1.5-15 years) presented with uncommon causes like (1) idiopathic benign non-traumatic inflammatory stricture (n = 3), (2) idiopathic fibrosing chronic pancreatitis (n = 2), (3) post-cholecystectomy type 4 benign biliary stricture (n = 1), (4) post-acute pancreatitis pseudo-cyst of pancreas (n = 1), (5) non-Hodgkin's lymphoma (NHL) with extramural common bile duct compression and gall bladder perforation (n = 1), and (6) Langerhan cell histiocytosis (LCH, n = 1). The clinical features and the diagnostic work up of each group are discussed. A preoperative endoscopic/percutaneous biliary drainage was required in four children because of cholangitis at presentation. A biliary-enteric anastomosis was performed for all seven children in groups (1)-(4). The patients with NHL and LCH were referred for chemotherapy after establishing tissue diagnosis at laparotomy. With a follow-up period of 3 months to 7 years, seven children (with the exception of patients with NHL and LCH) are currently anicteric. This paper draws attention to some infrequently discussed causes of extrahepatic biliary obstruction in children. The management entails a carefully planned combination of endoscopic interventions, interventional radiology and surgery. The outcome in benign cases is usually satisfactory.

Neuropathy in rheumatoid arthritis (RA) may result secondary to entrapment, vasculitis, and drug toxicity. We aimed to study clinical and electrophysiological neuropathy and pathological changes in sural nerve in patients with RA. One hundred eight patients of RA, fulfilling American College of Rheumatology 1987 criteria (mean age, 45.83 years; M/F 1:3, 80.3% seropositive) were examined clinically and electrophysiologically for evidence of peripheral neuropathy. Sural nerve biopsies were performed in the involved cases. In all RA patient medications, disease activity, results of blood tests, and X-rays of affected joints were recorded. Twenty-three patients complained of paresthesias in the extremities. Vibration sensations were decreased in 9, and tendon reflexes were decreased or absent in 28 patients. Sixty-two (57.4%) patients had electrophysiologic evidence of neuropathy. Of these 53 (85.5%) patients had pure sensory or sensory motor axonal neuropathy (mononeuritis multiplex, n = 7), while 9 (14.5%) had demyelinating neuropathy (chronic inflammatory demyelinating polyneuropathy, n = 1). Carpal tunnel syndrome was seen in 11 (10.1%) patients (associated with neuropathy in 6). Of 23 sural nerve biopsies available, perineurial thickening (n = 5, amyloid deposits n = 4), perivascular lymphomononuclear cell infiltrate (n = 4), loss of myelin fibers (n = 2), and necrotizing vasculitis (n = 1) were found. Clinically, however, seven patients had evidence of cutaneous vasculitis. Comparing the clinical characteristics of the patients with or without electrophysiological neuropathy, absence of deep tendon jerks (p < 0.005) and presence of extra articular manifestations (p < 0.01) were conspicuous in the neuropathic group. There was no relation of neuropathy with the duration of RA, seropositivity, joint erosions, joint deformities, prior disease-modifying anti-rheumatic drugs or glucocorticoid intake, and 28-joint disease activity score. Neuropathy in RA was mostly subclinical and predominantly axonal. Pathologically, neuropathy secondary to amyloid infiltration was second only to vasculitic neuropathy. Absence of deep tendon jerks and presence of vasculitis were more commonly observed in patients with neuropathy.

Gliomatosis cerebri(GC) is a highly aggressive, rare form of neuroepithelial tumor. Due to the diffuse nature of the tumor, its pathological grade is not conclusively established unless a biopsy is carried out from multiple sites. The tumor typically infiltrates the cortex in the subpial plane and also insinuates in the perineuronal space and around the Virchow-Robin spaces with relative preservation of the underlying brain parenchyma. Most of the previously reported cases of GC are astrocytic in nature. In this study, we report 2 pediatric patients of GC with oligodendroglial morphology on histopathological examination. In view of the rarity of the oligodendroglial phenotype of gliomatosis cerebri as well as the rare occurrence in children, we report these 2 cases.

The molecular basis of hepatic dysfunction in thyrotoxicosis is not fully understood. Here, we investigated the effect of altered thyroidal status on death receptor pathways including p75 neurotrophin receptor (p75NTR), a member of tumor necrosis factor (TNF) receptor superfamily, in rat liver.Hyperthyroidism was induced in Sprague-Dawley rats by daily injections of triiodothyronine in a dose of 12.5 microg/100 g body weight for 10 days.Terminal deoxynucleotide-transferase-mediated dUTP nick end labeling assay and caspase-3 activation data confirmed apoptosis in hyperthyroid rat liver. We observed the elevated levels of death ligands, TNF-alpha, Fas ligand and their cognate receptors, TNF-receptor-1 and Fas, and 8-fold increase in caspase-8 activation in hyperthyroid rat liver (p0.001). We demonstrated for the first time that hyperthyroidism elevates p75NTR levels and its ligands, pro-nerve growth factor and pro-brain-derived neurotrophic factor, in rat liver. Further we showed that most of the apoptotic cells in hyperthyroid liver express p75NTR. We also demonstrated that triiodothyronine administration to rats causes NF-kappaB activation, but persistent exposure (10 days) to triiodothyronine deactivates NF-kappaB leading to sustained c-Jun N-terminal kinase (JNK) activation.This study showed that hyperthyroidism-induced apoptosis in rat liver involves the activation of death receptor-mediated pathways, including p75NTR.

Thyroid hormone insufficiency adversely affects cortical development; however, its effect on apoptosis modulation during cerebral cortex development is not understood. We investigated the effect of perinatal hypothyroidism on apoptosis and its mechanisms during rat cerebral cortex development. Primary hypothyroidism was induced by feeding methimazole (0.025% wt/vol) in the drinking water to pregnant and lactating rats and continued until the animals were killed (hypothyroid group). Cerebral cortices from pups were harvested at different postnatal ages (postnatal d 0, 8, 16, and 24 and adult), and apoptosis was quantitated by terminal deoxynucleotide transferase-mediated dUTP nick end labeling and cleaved caspase-3 immunoreactivity. Compared with the euthyroid, primary somatosensory cortex (S1) in the hypothyroid group exhibited enhanced apoptosis. In S1 of euthyroid rats, apoptotic cells were mostly found in cortical layers I–III and the proportion of apoptotic cells enhanced significantly in the hypothyroid group (P < 0.001). Most of the apoptotic cells were neurons, as assessed by double immunolabeling. A significantly increased activation of caspase-3 and -7, decreased levels of antiapoptotic proteins Bcl-2 and Bcl-xL, and increased levels of proapoptotic protein Bax was observed in the developing cerebral cortex of hypothyroid rats, compared with the euthyroid (P < 0.001). In addition, hypothyroidism significantly elevated the levels of 53-kDa pro-nerve growth factor (P < 0.001) and p75 neurotrophin receptor (P < 0.001) and decreased TrkA expression. Taken together, we provide evidence for the possible contribution of pro-nerve growth factor/p75 neurotrophin receptor pathway in hypothyroidism-enhanced apoptosis during rat cortical development. Thus, the present study may help in explaining the mechanism of the deleterious effect of thyroid hormone deficiency on cerebral cortex development in children.

A rare case of an advanced primary broad ligament carcinoma is discussed, with a review of the literature regarding its incidence, presentation and management. This patient showed a complete response to adjuvant cisplatin-based chemotherapy following panhysterectomy and is presently without any evidence of disease, 15 months after completion of her treatment.

Purpose: To examine whether all the histopathologically seen features of cysticercus cysts excised from brain of swine naturally infected with neurocysticercosis during its evolution are actually visible on magnetic resonance imaging (MRI). Materials and Methods: Five swine naturally infected with cerebral cysticercosis were subjected to fast spin-echo (SE) T2, SE T1, fluid attenuated inversion recovery imaging, T1-weighted magnetization transfer (MT), and postcontrast T1-weighted MT sequences on MRI. These animals were sacrificed after imaging and ex vivo imaging of the intact excised brain using the same imaging protocol was also performed. Grossing of these brains was done similar to the ex vivo imaging planes. Numeral density and external appearance of each cyst and scolex were evaluated on each pulse sequence. Amount of pericystic edema, if present, was also assessed. On histopathology, cellular characteristics, inflammatory response, and the extent of edema, if present, in the brain parenchyma around the cysts were graded. Cysts were categorized into viable, early, and late degenerated on histopathology. The MRI features of each cyst were correlated with their histopathologic findings. Results: Out of 31 cysts, eight were found to be viable, 13 early degenerated, and 10 late degenerated on histopathology. T2-weighted imaging demonstrated all the cysts while T1-weighted imaging showed 97% of the cysts. Scolex was seen in 90.3% and 93.5% of the cysts on T2- and T1weighted images, respectively. Minimal edema (grade I) and inflammation in degenerating cysts present on histopathology was not visible on MRI. All but one of eight degenerated cysts, which showed enhancement on postcontrast MRI, had edema on imaging as well as on histopathology. Conclusion: T2-weighted MRI demonstrated all the cysts that were visible on histopathology. Non-enhancement of some of the degenerated cysts along with absence of edema on MRI is likely to underestimate the staging of neurocysticercosis evolution, and these early degenerating cysts may be misdiagnosed as in viable stage.