Molecular cloning, GTP recognition mechanism and tissue-specific expression profiling of myxovirus resistance (Mx) protein in Labeo rohita (Hamilton) after Poly I:C induction.

The myxovirus resistance (Mx) proteins belong to interferon-induced dynamin GTPase and play pivotal role in the inhibition of replication of numerous viruses. These antiviral proteins are released in usual or diseased condition to prevent the viral attack and to carry regular cellular activities like endocytosis and trafficking of nucleoproteins into the nucleus. The invasion of virus up-regulates the expression of Mx transcripts and double-stranded RNA mimic like polyinosinic polycytidyilic acid (Poly I:C). To understand the tissue-specific expression profiling and mechanism of GTP recognition of Mx protein from Labeo rohita (rohu), the full-length gene was cloned, sequenced and characterized through various Bioinformatics tools for the first time. The Mx cDNA was comprised of 2297 bp, and the open reading frame of 1938 bp encodes polypeptide of 631 amino acids. The coding sequence of Mx protein possess the signature motif of dynamin superfamily, LPRG(S/K)GIVTR, the tripartite guanosine-5/triphosphate (GTP)-binding motif (GXXXSGKS/T, DXXG and T/NKXD) and the leucine zipper motifs at the C-terminal end, well conserved in all interferon-induced Mx protein in vertebrates. Western blotting confirmed the molecular weight of Mx protein to be 72 kDa. After the intraperitoneal challenge of L. rohita with a Poly I:C, up-regulation of Mx protein was observed in brain, spleen, liver, kidney, intestine, heart, muscle, and gill. Ontogeny study displayed pronounced expression of Mx protein in all stages of the developmental of Rohu after Poly I:C induction. However a persistent expression of Mx transcript was also observed in Rohu egg as well as milt without induction with Poly I:C. Higher expression of Mx gene was observed on 96 h where it was 6.4 folds higher than the control. The computational modelling of Mx protein portrayed the tripartite N-terminal G-domain that binds to GTP, the bundle-signaling element (BSE) which interconnects the G-domain to the elongated stalk domain and C-terminal helical stalk domain. In agreement with the experimental studies, a series of conserved residues viz., Gln52, Ser53, Ser54, Leu68, Pro69, Gly71, Gly73, Thr76, Asp151, Gly154, Thr220, Lys221, Val251, Cys253, Arg254, and Gly255 were computed to be indispensable for tight anchoring of GTP within binding cavity of G-domain. The binding free energy calculation study depicted that the van der Waals and electrostatic terms contributs significantly to molecular recognition of GTP. Collectively, our study provides mechanistic insights into the tissue-specific expression profiling and GTP binding mechanism of Mx protein from Labeo rohita, which is expected to drive further research on several cellular events including viral resistance and endocytosis in the near future.

Contrecoup Extradural Hematoma with Coronal Suture Diastasis.

Extradural hematoma (EDH) generally occurs in the site of impact, that is, coup injury site. EDH is associated with fracture of skull in many a times due to direct impact. However, EDH in counter coup site is a rare occurrence. Hardly, yet, 12 cases have been reported including this case. Here, we reported a case of a 22-year-old male of contrecoup acute EDH who had sustained head injury due to fall from bike. Physical examination revealed direct impact at the left occipito-parietal region with laceration of scalp and bruise with transient loss of consciousness at the time of injury. There was no evidence of impact on the right side of his head. Computed tomography scan revealed an EDH in the right fronto-temporal region without any bone fracture on bone windows. On intraoperative exploration, it is found that there was coronal suture diastasis with small subgaleal hematoma and right fronto-temporal extradural hematoma. The evacuation of EDH was done. The patient discharged on 3rd postoperative day. It can be concluded that direct impact on head causes exactly opposite hit of brain, and compensatory rebound causes a negative pressure in between the layers causing disruption of vessels and potential accumulation of blood. This mechanism mostly favors for acute subdural hematoma due to compact attachment of dura with cranium. However, in this case, diastasis of coronal suture due to transmitted force is the cause of accumulation of blood extradurally in relatively loosely adhered dura in fronto-temporal region where a potential negative pressure space is created by contrecoup injury.

Extensive intracranial calcification of pseudo-TORCH syndrome with features of Dandy-Walker malformation.

Pseudo-TORCH syndrome or congenital infection-like syndrome is a group of conditions which resemble congenital infections such as those caused by toxoplasmosis, rubella, cytomegalovirus (CMV), herpes (TORCH) group of organisms, clinico-radiologically, but serological tests are negative for the organisms. One of the variety shows features such as microcephaly, extensive intracranial calcification showing gross resemblance to congenital CMV infection, making its other name as microcephaly intracranial calcification syndrome (MICS). Dandy-Walker malformation (DWM), in addition to posterior fossa large cyst, cerebellar vermis hypoplasia, and hydrocephalus is often associated with agenesis of the corpus callosum and callosal lipomas, dysplasia of the brainstem, and cerebellar hypoplasia or dysgenesis. But radiological features of DWM with microcephaly and intracranial calcification are very unusual and have been rarely reported in the literature.[1] We report a case of infant showing clinical features suggestive of congenital CMV infection with negative serology and radiological imaging suggestive of DWM with extensive intracranial calcification. Pseudo-TORCH syndrome with radiological features of DWM is a congenital developmental abnormality. Inspite of hydrocephalus, it does not require cerebrospinal fluid (CSF) diversionary procedure due to lack of increased intracranial pressure. Conservative management for seizure disorder is the optimal therapy.

Development of contralateral extradural hematomas after evacuation of primary one.

The occurrence of bilateral extradural hematomas (EDH) is an uncommon consequence of closed head injuries. Incidence of bilateral EDH has been reported in various studies ranging from 2 to 25%. Bilateral EDH may develop simultaneously or second EDH develops few hours after first one. Development of second EDH after evacuation of primary one is rarely seen. We are reporting one such case. Awareness of this entity is required to detect such cases as timely intervention gives an excellent result like an acute EDH.

Transorbital penetrating brain injury by a wooden stick: A neuro-ophthalmologic emergency.

Accidental penetrating brain injury through transorbital route is an unusual occurrence in emergency practice of civilian cases. At time, it could be potentially life threatening. We report an interesting case of transorbital penetrating brain injury with a wooden stick in a 44-year-old male epileptic patient. The stick was removed successfully through transorbital exploration, without any neurovascular complications. The pertinent literature is reviewed and emergent management of such cases is discussed.

Intracerebellar haemorrage in a haemophilia child.

Hemophilia A is an inherited, X-linked, recessive disorder caused by deficiency of clotting factor VIII. Intracranial hemorrhage is the leading cause of morbidity and mortality in these patients. Use of factor replacement products and medications had improved outcome in these patients. But in developing countries many such patients are not able to afford factor replacement products. We report a case of traumatic intracerebellar hemorrage in a hemophilia child. This child presented to us as a case of sub-acute intracerebellar hemorrage, he was managed conservatively with six units of fresh frozen plasma transfusion. He improved clinically and on follow-up investigation hematoma was found to have dissolved spontaneously. Through this report we want to emphasize that those cases of hemophilia presenting with acute or sub-acute intracranial bleed, conservative approach through procoagulant transfusion and intravenous fibrinolysis inhibitors should be tried as first line of management. Before daring for surgery sufficient amount of factor replacement should be kept available.

Spectrum and outcome of traumatic brain injury in children <15 years: A tertiary level experience in India.

BACKGROUND: Though, traumatic brain injury (TBI) has been documented as the single most common cause of morbidity and mortality in infancy and childhood, the exact incidence is unavailable in India. Moreover, modes of injury, mechanisms of damage, and management differ significantly from that of an adult. AIMS AND OBJECTIVES: To analyze the epidemiological factors, the spectrum of TBI, modes of injury, types of injury, and the outcome in the children <15 years with TBI. MATERIALS AND METHODS: This is a retrospective study from August 2012 to May 2013 at Department of Neurosurgery, S.C.B. Medical College, Cuttack, Odisha, India. All the pertinent details from case records of hundred and forty-seven children <15 years with TBI were analyzed. Follow-up was done for 6 months at outpatients department. RESULTS: Age wise, incidence and severity of TBI is more common in 10-15 years. Males outnumber females with a male: female ratio 2.19:1. Overall, road traffic accident (RTA) is the commonest mode of injury. Assault is not uncommon (7.48% cases). Falls is common in <5 years while RTA is common in 5-15 years. The extradural hematoma was the most common injury pattern; however, surgical consideration was maximal for fracture skull. Overall mortality was 7.48%. Diffuse axonal injury has the maximum individual potential for mortality. We noticed excellent recovery in 68.7%, disabilities in 17.68%, and persistent vegetative state in 5.45% cases. CONCLUSION: TBI in children carries good outcome, if resuscitated and referred early to a neurotrauma center, and managed subsequently on an individualized basis with a well-organized team approach. Severe TBI in children has a poor outcome.

Congenital Malrotation of the Atlas with Unilateral Hypertrophy of the Atlanto-occipital Joint-A Rare Anomaly of the Craniovertebral Junction and Its Management.

BACKGROUND: Congenital anomalies are commonly encountered in the craniovertebral junction because of its unique embryologic development. The craniovertebral junction usually comprises the occiput, atlas, and axis. However, malrotation of the atlas (C1) in between the occiput (C0) and axis (C2) with Chiari I malformation as well as unilateral hypertrophy of the atlanto-occipital joint and a lateral mass manifesting features of high cervical myelopathy is a rarely reported anomaly. CASE DESCRIPTION: A 22-year-old woman presented to us with high cervical compressive myelopathy. Imaging revealed rotation of the C1 vertebra approximately 20° toward the left side with right atlanto-occipital joint dislocation and hypertrophy. Imaging also revealed hypertrophy of a right lateral mass of C1, Chiari I malformation, and right atlantoaxial dislocation exposing the right C2 superior facet. She underwent a right extreme far lateral approach comprising a right C1 posterior arch excision and medialization of the right vertebral artery with excision of the hypertrophied atlanto-occipital joint extradurally and posteromedial excision of the superior articular facet of C1. The patient's compressive myelopathy features improved postoperatively. CONCLUSIONS: We report the successful management of this rare congenital anomaly and review the literature.

Intradural Spinal Arachnoid Cyst: A Long-Term Postlaminectomy Complication: A Case Report and Review of the Literature.

BACKGROUND: Spinal arachnoid cysts are a rare cause of spinal cord compression. Intradural arachnoid cysts are rarer than extradural arachnoid cysts. Spinal arachnoid cysts are mostly congenital in origin. Arachnoid cysts due to trauma, lumbar puncture, or surgery are rarely reported. Most arachnoid cysts are located posterior to the spinal cord in the thoracic regions. The ideal treatment is laminectomy or laminoplasty with puncture, marsupialization, or excision. But the development of a cervico-thoracic spinal intradural extramedullary arachnoid cyst anteriorly located 28 years after laminectomy is a recognizable complication of laminectomy. CASE DESCRIPTION: We report here a case of a 45-year-old man who underwent C6-T1 laminectomy at the age of 17 years for cervical intervertebral disc prolapse (C6/7, C7/T1) and compressive myelopathy. Twenty-eight years after laminectomy, he developed spastic quadriparesis and was diagnosed with a spinal intradural extramedullary anterior arachnoid cyst at the laminectomy site with compressive myelopathy. CONCLUSIONS: So, although laminectomy with excision is usually practiced to treat spinal arachnoid cysts, laminectomy itself is a cause of development of intradural arachnoid cysts.

Supratentorial extradural hematoma in children: An institutional clinical experience of 65 cases.

AIM AND OBJECTIVE: To survey the epidemiology, management, and severity of extradural hematoma (EDH) in children. MATERIALS AND METHODS: All patients of EDH (n = 65) in the age group of 0-16 years admitted to our department during the period of August 13 and July 14 were analyzed retrospectively from the hospital records. In all patients, age, sex, mode of injury, clinical presentation, site of EDH, management, duration of hospitalization, and outcome were evaluated. OBSERVATION AND RESULTS: Of 65 patients, males were 70.76% with a male to female ratio of 2.4:1, most of the victims (47.69%) were in the age group of 11-16 years. Mean duration of hospitalization was 4.32 days. The most common mode of injury was fall from height in 29 cases (44.61%) followed by road traffic accident (RTA) in 23 cases (35.35%). Temporoparietal EDH was the most common computed tomography finding present in 22 (33.84%) patients. 67.69% patients presented to casualty with minor head injury having Glasgow coma scale (GCS) between 14 and 15. Most common presenting feature was vomiting in 52 cases (80%) and next to it was altered sensorium. The mortality rate was 7.69% (n = 5). CONCLUSION: Extradural hematoma is a life-threatening entity encountered in pediatric head injury. Timely intervention and diagnosis decrease mortality to a great degree. Most of the mortality is encountered in patients who presented late at the neurosurgical unit with low GCS.

Isolated thoracic (D5) intramedullary epidermoid cyst without spinal dysraphism: A rare case report.

Spinal epidermoid cyst, congenital or acquired, is mainly congenital associated with spinal dysraphism, rarely in isolation. Intramedullary epidermoid cysts (IECs) are rare with less than 60 cases reported so far; isolated variety (i.e., without spinal dysraphism) is still rarer. Complete microsurgical excision is the dictum of surgical treatment. A 14-year-old boy presented with 4-month history of upper backache accompanied with progressive descending paresthesia with paraparesis with early bladder and bowel involvement. His condition deteriorated rapidly making him bedridden. Neurological examination revealed upper thoracic myeloradiculopathy probably of neoplastic origin with sensory localization to D5 spinal level. Digital X-ray revealed no feature suggestive of spinal dysraphism. Contrast magnetic resonance imaging (MRI) characteristics clinched the presumptive diagnosis. Near-total microsurgical excision was done leaving behind a small part of the calcified capsule densely adhered to cord. Histopathological features were confirmative of an epidermoid cyst. Postoperatively, he improved significantly with a gain of motor power sufficient to walk without support within a span of 6 months. Spinal IECs, without any specific clinical presentation, are often diagnosed based upon intraoperative and histopathological findings, however early diagnosis is possible on complete MRI valuation. Complete microsurgical excision, resulting in cessation of clinical progression and remission of symptoms, has to be limited to sub-total or near-total excision if cyst is adherent to cord or its confines.

Spinal cord compression due to primary intramedullary tuberculoma of the spinal cord presenting as paraplegia: A case report and literature review.

BACKGROUND: Spinal cord compression can be due to various causes but spinal intramedullary tuberculoma is a rare cause. We report a case that had an intramedullary spinal cord tuberculomas in which the diagnosis was made histologically, without evidence of symptoms of systemic tuberculosis. This lesion, located in the thoracic region, mimicked as an intramedullary tumor radiologically. CASE DESCRIPTION: The patient was a 25-year-old male who presented with a history of progressive paraparesis. Initial diagnosis was made as an intramedullary tumor by magnetic resonance imaging (MRI). The treatment of the patient involved is complete surgical excision of intramedullary lesion followed by appropriate antituberculous therapy. Postoperatively, his neurological symptoms were dramatically improved. With combination of both surgical and medical treatments, excellent clinical outcome was obtained. CONCLUSION: This case illustrates the risk of misdiagnosis and the importance of histological confirmation of a pathological lesion as spinal cord tuberculoma prior to surgical therapy, which should be kept in mind as a differential diagnosis of the intramedullary spinal cord tumors.

Management of superior sagittal sinus injury encountered in traumatic head injury patients: Analysis of 15 cases.

BACKGROUND: Significant dural sinus injury occurs in 1.5- 5% of all head injuries and injury to superior sagittal sinus accounts for 70-80% of these. Its management is also a challenging issue to every neurosurgeon. MATERIALS AND METHODS: In a period of one year (Aug 2011 to july2012) out of 549 cases of head injuries operated in our department analysis of 15 cases (2.73%) shown to have superior sagittal sinus injury. Three dimensional reconstruction of skull bone during CT scan helped us to diagnose possible dural sinus injury pre-operatively. RESULTS: SSS injury was due to direct impact of fracture segment (5 cases), extension of fracture line over sinus area (4 cases) or due to coronal suture diastases (6 cases). Injury to anterior third of sagittal sinus occurred in 12 cases (80%) and injury to middle third occurred in 3 cases (20%). Small tear over superior sagittal sinus was found in 7 cases (46.66%) and was managed with Gelfoam(®) compression only and in two cases (13.33%) were large tear, which was managed with sinoraphy. In four cases (36.34%) hitch stitches over Gelfoam(®) to adjacent bone given and in rest two cases (13.33%) a strip of bone was left over the sinus area and bilateral hitch stitches were applied. CONCLUSION: Injury to SSS in traumatic head injury patient though rarely encountered during surgery, the management is challenging to every neurosurgeon. Preoperative suspicion and combination of surgical techniques rather than one single technique may be effective in decreasing the sinus related mortality rate.

Large vertex meningoencephalocele with schizencephaly: An interesting case with neurosurgical challenge.

An encephalocele is a developmental disorder of neural tube closure, where as schizencephaly is a developmental disorder of neuronal migration. There are only few case reports suggesting association of schizencephaly with meningoencephalocele. We report a case of 6-year-old child who was brought by her parent with complaints of progressive swelling over her vertex since birth, left sided hemiparesis and intermittent generalized tonic clonic convulsion for last 3 years. Clinical examination, computed tomography (CT) and magnetic resonance imaging (MRI) of brain revealed a large calvarial defect with meningoencephalocele and associated schizencephaly. Initially, ventriculoperitoneal (VP) shunt was carried out, followed by successful repairing of the defect. This case is interesting for late presentation, management difficulties and associated anomalies "schizencephaly", which is a rare developmental disorder.

Shunt site chronic calcified extradural hematoma: An avoidable complication.

Extradural hematoma (EDH) after ventriculoperitoneal (VP) shunt procedure is a rare, dangerous but easily avoidable and manageable complication. It is more common in children and young adults presumably due to relatively lax adhesion of dura to calvarium. We report a case of an 18-year-old male with acqueductal stenosis who underwent VP shunt procedure. Three months later, a computed tomography (CT) scan was done for the complaints of intractable headache and altered sensorium which showed chronic calcified EDH near shunt site. The ventricular catheter was in position and the ventricles were decompressed. After surgical decompression of EDH his symptoms improved. We discuss the factors leading to formation of EDH, with stress on proper technique to prevent or minimize such an avoidable complication.

Giant aneurysmal bone cyst of cervical spine: Surgical management and circumferential spinal fusion in a 13-year-old girl.

Aneurysmal bone cysts (ABCs) of the cervical spine are rare occurrences accounting for about 2% of all cases. Having a propensity to develop in the pediatric population, it generally involves the posterior elements with occasional extension to the facets and body of one vertebral level. Surgical treatment of high cervical ABC in pediatric patients is challenging. Where as complete tumor resection should be attempted for the best chance for cure, spinal fusion should be added to correct the defects created by resection to minimize the risk of postoperative instability. This paper presents a multi-level giant expansile ABC of the high cervical spine involving all three spinal columns in a 13-year-old girl and discuss the surgical management. Surgical treatment in this patient included complete tumor excision and circumferential fusion in two stages without any neurovascular injury. The safety and efficacy of this method, especially in the pediatric population is discussed in view of pertinent literature.

Cerebellar giant cell glioblastoma multiforme in an adult.

Cerebellar glioblastoma multiforme (GBM) is a rare tumor that accounts for only 1% of all cases of GBM and its giant cell variant is even much rarely encountered in adults. A case of cerebellar giant cell GBM managed at our institution reporting its clinical presentation, radiological and histological findings, and treatment instituted is described. In conjunction, a literature review, including particular issues, clinical data, advances in imaging studies, pathological characteristics, treatment options, and the behavior of such malignant tumor is presented. It is very important for the neurosurgeon to make the differential diagnosis between the cerebellar GBM, and other diseases such as metastasis, anaplastic astrocytomas, and cerebellar infarct because their treatment modalities, prognosis, and outcome are different.