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An encephalocoele is a spectrum of cranial dysraphism caused due to defective closure of neural tube during early embryonic life, leading to formation of a cerebrospinal fluid (CSF)-filled sac containing brain tissue and meninges, all herniated through a bony defect of the skull. Anterior encephalocoeles, though rare in the Western world, are relatively common in southeast Asia, including some parts of India. Among anterior encephalocoeles, fronto-ethmoidal type is the most common followed by orbital encephalocoeles. Giant encephalocoeles are rare with few published short series, which are mostly located in occipital region. Giant interfrontal encephalocoele through a wide anterior fontanel is the rarest one and is limited to three case reports, including this index case. Here we report a case of giant anterior fontanel encephalocoele in a 20-day-old neonate and discuss various aspects of its management., Competing Interests: There are no conflicts of interest., (Copyright: © 2020 Journal of Pediatric Neurosciences.)

Background: Inflammatory pseudotumor is a histologically proven benign tumor-like lesion of unknown etiopathogenesis. It is less commonly found in central nervous system, and least commonly in the spinal canal. Spinal epidural inflammatory pseudotumor is a rarely reported entity., Case Description: We report a patient who presented with paraplegia, in whom a spinal epidural mass was diagnosed as an inflammatory pseudotumor after surgical excision followed by histopathology., Conclusions: It was a radiologic dilemma; thus, complete excision was required for diagnosis and a better outcome., (Copyright © 2018 Elsevier Inc. All rights reserved.)

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 3 rd 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., Competing Interests: There are no conflicts of interest.

Background: Masson hemangioma is a rare pathologic entity characterized by intravascular papillary endothelial hyperplasia. Although benign, this hemangioma grows to form an expansile compressing mass. This lesion is mostly seen in skin and subcutaneous tissue. Occurrence in the central nervous system is rare. Still rarer is a spinal location with only 5 case reports published to date in the literature., Case Description: A 32-year-old man presented with paraplegia secondary to extradural compression at the T4-5 level. Histopathologic features were consistent with intravascular papillary endothelial hyperplasia, also known as Masson hemangioma. Differential diagnosis, management, and review of literature are discussed in this report., Conclusions: This rare pathology should be in the differential diagnosis when spinal cord compressive myelopathy is encountered., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Subacute subdural hematoma (SASDH) is an entity which is yet to capture the popular imagination among the neurosurgeons. Its management is often equated clinically to that of the chronic subdural hematoma (CSDH). However, their neurological deterioration is usually rapid, which seems to align them with acute subdural hematoma (ASDH). We proceed for their epidemiological evaluation. The advantages of a novel "double barrel technique (DbT)" over the conventional burrhole drainage are also presented., Methods: This retrospective study was conducted on all the patients having clinical and radiological evidence of SASDH, admitted to a tertiary care referral institute, during the period August 2013 to December 2015. Postoperatively, patients were followed-up for 3-24 months., Results: 46.87% of the patients belonged to the 35-54 year age group with a male predominance (3.6:1); 68.7% had a history of alcohol abuse, whereas aspirin users were 25%. 87.5% cases were unilateral, 18.75% were hemispheric, and 46.87% were present on the left side. Altered consciousness (100%) followed by headache (37.5%) were the most common presenting clinical features., Conclusion: SASDH is an uncommon neurosurgical entity (0.89% of traumatic brain injury cases in our study) and mimics both CSDH as well as ASDH. The true incidence of SASDH may have been underestimated due to its clinical imitation with CSDH. This study in a South Asian nation also provides the epidemiological data of this rare neurosurgical entity. Outcome of surgery is good; our retrospective study confirms that "DbT" is an adequate and safe treatment. However, a better designed, randomized control trial will be needed to reinforce our findings.

Spinal schwannomas account for about 25% of primary intradural spinal cord tumors in adult. The prognosis for spinal schwannomas is excellent in most cases. Complete resection is curative. However following subtotal removal, recurrence develops after several years. We describe a case of recurrent spinal schwannoma who had been operated twice before for same disease. The possible cause of recurrence and difficulties in reoperation are discussed.

Background: Intramedullary spinal cord metastases (IMSCM) are typically drop lesions from intracranial metastases and are a rare manifestation of systemic malignancy (8.5% of central nervous system metastases). They arise from primaries such as the lungs, breast, kidney, melanoma, or lymphoma. On the other hand, they arise very rarely from papillary thyroid carcinoma (PTC), even though it is the most common type of primary thyroid malignancy., Case Description: A 54-year-old male presented with pain in the lower back along with tingling, numbness, and weakness in the bilateral lower limbs. This was associated with urine incontinence for 1½ months. In the previous month, he developed a left-sided solitary thyroid nodule. Fine needle aspiration cytology and ultrasonography were suggestive of metastasis. Furthermore, the thoracolumbar magnetic resonance imaging showed T1-hypo and T2-hyper-intense D11-D12 level intramedullary lesion, with intense enhancement, which was consistent with an intramedullary lesion involving the conus. At surgery, a firm, brownish yellow, friable, vascular tumour was removed en toto. Upon discharge, the patient was neurologically intact except for residual bladder incontinence., Conclusion: In an extensive literature review (pubmed), IMSCM metastasis from PTC primary is confirmed as a rarity and this may be the fourth documented case. Moreover, this may be the first report of a case of PTC metastatic neurological deterioration "even before the treatment of the primary was undertaken." Early diagnosis and microsurgical resection can result in improvement of neurological deficits and in the quality of life of patients with IMSCM.

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.

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.

Epidermoid cysts involving the brainstem are extremely rare, with only 18 reported cases in the literature and only five purely intrinsic epidermoid cysts within this group. "White epidermoids", a rare entity, have high protein content and show reversed signal intensity on magnetic resonance images. In contrast to the classical variety, these cysts show high signal intensity on T1-weighted images and low signal intensity on T2-weighted images. Here, we report an interesting case of intrinsic brainstem "white epidermoid cyst" in a 15-year-old girl and discuss its clinical characteristics, radiological features, and surgical treatment. The girl presented with a one-year history of progressive quadriparesis, and features of multiple cranial nerve involvement. Because the cyst was purely intrinsic and had altered signal intensity, the diagnosis was initially unclear until definitive neuroimaging was performed using diffusion-weighted magnetic resonance imaging (DW-MRI) sequences.

Multiple myeloma is a malignant neoplasm of bone marrow affecting plasma cells. It is usually detected in skull bone with characteristic features of multiple punched-out lesions. Its presentation as a solitary scalp swelling with underlying skull bone erosion and intracranial extension is very rare. A 35-year-old female presented to us with complains of rapidly growing left-side scalp swelling with right-side paresis and simple partial seizure of right upper limb. Local examination, X-ray skull, CT scan, and MRI of brain were suggestive of a malignant lesion. Near total excision of lesion was done. Histopathological study was suggestive of plasmacytoma of skull. Bone marrow study further confirmed it as a case of multiple myeloma. Cases presenting with solitary osteolytic skull lesions, possibility of plasmacytoma, or multiple myeloma should be kept in mind.

Malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma. Most arise in association with major nerve trunks. Their most common anatomical sites are the proximal portions of the upper and lower extremities and the trunk. MPNSTs have rarely been reported in literature to occur in other unusual body parts. We review all such cases reported till now in terms of site of origin, surgical treatment, adjuvant therapy and outcome and shortly describe our experience with two of these cases. Both of our case presented with lump at unusual sites resembling neurofibroma, one at orbitotemporal area and other in the paraspinal region with characteristic feature of neurofibroma with the exception that both had very short history of progression. They underwent gross total removal of the tumor with adjuvant radiotherapy postoperatively. At 6-month follow-up both are doing well with no evidence of recurrence.

The symptoms of tethered cord syndrome (TCS) cases mostly appear during infancy and childhood. Though the adolescent presentation of TCS is well-recognized, it continues to pose significant diagnostic and management controversies. In this report, we describe two cases of adolescent onset TCS associated with two different etiologies. Our first case, an 18-year-old girl who presented due to overflow incontinence in association with TCS was diagnosed to have lumbar meningocele. The second case, a 19-year-girl presenting with perianal anesthesia and bowel and bladder incontinence had lipomyelomeningocele as the cause of TCS. Both of them underwent untethering surgery. The clinical charts and follow-up data were studied in respect to the clinical manifestation, surgical intervention and outcome with a brief review of pertinent literature.