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Background: Development of different molecular markers has given a new insight in the glioma management. KIAA1549-BRAF gene fusion has a diagnostic and prognostic significance. Aim: The aim of this study was to determine the KIAA1549-BRAF gene fusion in glioma and their correlation with various clinical parameters. Material and Methods: Forty cases of glioma were studied for KIAA1549-BRAF gene fusion by reverse transcription-PCR (RT-PCR). Results: Overall, KIAA1549-BRAF gene fusion was found in 22% (9/40) cases of glioma. Children had higher KIAA1549-BRAF fusion (72%; 8/11) as compared to adults (10%; 3/29) and this difference was statistically significant. Cerebellar location of tumor was significantly associated with KIAA1549-BRAF fusion. KIAA1549-BRAF fusion was highest in pilocytic astrocytoma (89%), and this difference was statistically significant. Statistically significant difference was noted between KIAA1549-BRAF fusion expression and WHO grade I glioma. Conclusion: Overall, KIAA1549-BRAF gene fusion was found in 22% (9/40) cases of glioma. Childhood age, pilocytic astrocytoma histology, cerebellar location and WHO grade I tumor were significantly associated with KIAA1549-BRAF gene fusion.

Objectives Intraparenchymal epidermoid cysts (IECs) are rare lesions. They represent less than 1% of the intracranial epidermoid cysts. The supratentorial IEC is a clinically and prognostically distinct subset. Given the rarity, most of the articles are case reports. We present a series of five cases of supratentorial IEC to characterize their clinical presentation and outcome, with emphasis on the surgical features. Materials and Methods We searched our database for all cases of intracranial epidermoid cysts operated between January 2005 and January 2020. Five patients were identified having IEC from the hospital information system and the neurosurgical operation record book. Standard craniotomy and decompression of the lesion were performed in all these patients. Standard postoperative care includes computed tomography scan of head on the day of surgery and magnetic resonance imaging of brain after 6 weeks to look for the residual lesion, if any. Subsequent follow-up visits in outpatient department to look for resolution of the presurgical symptoms. Results The mean age of the patients in our series was 28.8 years (range: 28–40 years.). All the five patients were male. Four patients had IEC involving frontal lobe and one in parietal lobe with a small occipital lobe extension. Seizure was the most common presenting complaint followed by headache. Complete excision was achieved in all the cases. All the three patients with seizure attained seizure freedom postlesionectomy. Focal neurological deficits resolved gradually in postoperative period. There was no recurrence of lesion during follow-up. Conclusion Supratentorial IEC most commonly affects young males, involve frontal lobe and present clinically with seizure. Complete surgical excision offers best outcome in the form of remission of seizure disorder.

Objective The conventional criteria for defining the basilar invagination (BI) focus on the relationship of odontoid tip to basion and opisthion, landmarks that are intrinsically variable especially in presence of occipitalised atlas. A universal single reference line is proposed that helps in unequivocally establishing the diagnosis of BI, may be relevant in establishing both Goel types A and B BI, as well as in differentiating a ‘very high’ from ‘regular’ BI. Methods Study design – case-control study. In 268 patients (group I with BI [n = 89] including Goel type A BI [n = 66], Goel type B BI [n = 23], and group II controls [n = 179]), the perpendicular distance between odontoid tip and line subtended between posterior tip of hard palate-internal occipital protuberance (P-IOP line) was measured. Logistic regression analysis determined factors influencing the proposed parameter (p < 0.05). Results In patients with a ‘very high’ BI (n = 5), the odontoid tip intersected/or was above the P-IOP line. In patients with a ‘regular’ BI (n = 84), the odontoid tip was 6.56 ± 3.9mm below the P-IOP line; while in controls, this distance was 12.53 ± 4.28 mm (p < 0.01). In Goel type A BI, the distance was 7.01 ± 3.78 mm and in type B BI, it was 5.07 ± 4.19 mm (p = 0.004). Receiver-operating characteristic curve analysis identified 9.0 mm (8.92–9.15 mm) as the cut-point for diagnosing BI using the odontoid tip-P-IOP line distance as reference. Conclusion The odontoid tip either intersecting the P-IOP line (very high BI) or being < 9 mm below the P-IOP line (Goel types A and B BI) is recommended as highly applicable criteria to establish the diagnosis of BI. This parameter may be useful in establishing the diagnosis in all varieties of BI.

Objective Posterior fossa decompression is the treatment of choice in type 1 Chiari malformation (CM-1) without bony instability. Although surgical fixation has been recommended by a few authors recently, comparative studies to evaluate these treatment strategies using objective outcome tools are lacking. Methods Seventy-three patients with pure CM-1 (posterior fossa bony decompression [PFBD], n = 21; posterior fossa bony and dural decompression [PFBDD], n = 40; and posterior fixation [PF], n = 12) underwent a postoperative outcome assessment using Chicago Chiari Outcome Score (CCOS). Logistic regression analysis detected predictors of an unfavorable outcome. Results Minimally symptomatic patients generally underwent a PFBD while most of the clinically severe patients underwent a PFBDD (p = 0.049). The mean CCOS score at discharge was highest in the PF (12.0 ± 1.41) and lowest in PFBDD group (10.98 ± 1.73, p = 0.087). Patients with minimal preoperative clinical disease severity (adjusted odds ratio [AOR], 4.58; 95% confidence interval [CI], 1.29–16.31) and PFBDD (AOR, 7.56; 95% CI, 1.70–33.68) represented risks for an unfavorable short-term postoperative outcome. Though long-term outcomes (CCOS) did not differ among the 3 groups (p = 0.615), PFBD group showed the best long-term improvements (mean follow-up CCOS, 13.71 ± 0.95), PFBDD group improved to a comparable degree despite a poorer short-term outcome while PF had the lowest scores. Late deteriorations (n = 3, 4.1%) occurred in the PFBDD group. Conclusion Minimally symptomatic patients and PFBDD predict a poor short-term postoperative outcome. PFBD appears to be a durable procedure while PFBDD group is marred by complications and late deteriorations. PF does not provide any better results than posterior fossa decompression alone in the long run.

A spectrum of vertebral artery (VA) anomalies have been described with or without an associated congenital craniovertebral junction (CVJ) anomalies. C3 segmental VA, where the VA enters the dura at the level of C2/3 intervertebral foramen is an extremely rare anomaly. We report two cases of congenital CVJ anomaly (irreducible in one with C2/3 fusion and reducible in the other; without any subaxial fusion but with articular agenesis at C2/3 joint on the anomalous artery side). Computed tomographic angiography revealed intraspinal intradural entry of VA through the C2/3 intervertebral foramen on the right side with the contralateral artery found crossing the atlanto-axial joint. Both the patients underwent posterior approach and C2 was spared from instrumentation in both cases. Postoperatively, the patient with irreducible dislocation recovered well while the patient with reducible dislocation expired, possibly secondary to the thrombosis of the dominant VA from C2/3 foraminal encroachment. C3 segmental VA may be advantageous in aggressively exposing the C1/2 joint but instrumentation of C2 or C3 needs caution in view of the possibility of VA injury. Our experience shows that VA may be endangered even while exposing and protecting the artery. For such cases, we recommend posterior decompression of the C2/3 neural foramen during instrumentation in the absence of associated C2/3 fusion, as an abnormal joint morphology of C2/3 indicates a C2/3 instability.

Objectives The spinal dermoid and epidermoid cysts (SDECs) are rare entities comprising less than 1% of pediatric intraspinal tumors. The present study aims to extrapolate the clinicoradiological data, in order to identify the most plausible neural tube closure model in human and provide a retrospective representation from our clinical experience. Materials and Methods We collected the details of all histologically proven, newly diagnosed primary SDECs who underwent excision over the past 20 years. Secondary or recurrent lesions and other spinal cord tumors were excluded. Surgical and follow-up details of these patients as well as those with associated spinal dysraphism were reviewed. Clinical and radiological follow-up revealed the recurrence in these inborn spinal cord disorders. Results A total of 73 patients were included retrospectively, having a mean age of 22.4 ± 13.3 years, and 41 (56.2%) cases fell in the first two decades of life. Twenty-four (32.9%) dermoid and 49 (67.1%) epidermoid cysts comprised the study population and 20 of them had associated spinal dysraphism. The distribution of SDECs was the most common in lumbosacral region (n = 30) which was 10 times more common than in the sacral region (n = 3). Bladder dysfunction 50 (68.5%) and pain 48 (65.7%) were the most common presenting complaints. During follow-up visits, 40/48 (83.3%) cases showed sensory improvement while 11/16 (68.7%) regained normal bowel function. There was no surgical mortality with recurrence seen in eight till the last follow-up. Conclusions The protracted clinical course of the spinal inclusion cysts mandates a long-term follow-up. The results of our study support the multisite closure model and attempt to provide a retrospective reflection of neural tube closure model in humans by using SDECs as the surrogate marker of neural tube closure defect.

Background and Purpose: Multifocality and metastasis from insular glioma are extremely rare. Pathological insights and elaboration of the clinical course of this condition will contribute to their better understanding. Materials and Methods: Among 123 consecutively operated insular gliomas, 5 patients (4.2%) presented with a multifocal tumor. The clinico-radiological, histo-molecular, and treatment outcomes were noted and compared with the unifocal insular glioma cohort. Results: Among the five patients, all were males and involved the right insular lobe. Three patients presented with synchronous tumors, while two patients developed metachronous multifocal tumors. The histology of the insular tumor was Grade I glioma in 1, Grade II astrocytoma with p53 mutation in 2, and anaplastic astrocytoma and glioblastoma in one patient each. Histological confirmation of the second lesion was performed in two patients, showing the same histology of the insular tumor. Interconnection between the tumors was apparent through cerebrospinal fluid pathways in four patients, while no such connection could be established in one patient. Barring the patient of Grade I glioma, the rest of the patients died within months of the diagnosis. Conclusion: Multifocal insular glioma is rare and probably represents a biologically more aggressive tumor. Insular glioma that touches the ventricle appears a common denominator for multifocality. True multicentricity is rare. The prognosis in insular glioma with multifocality is poor in non-Grade I gliomas.

Background Among the patients of drug-resistant epilepsy, a subset which has focal impaired seizures localizes to the temporal lobe region (TLE). A majority of these cases are surgically amenable with anterior-medial temporal lobe resection or “lesionectomy.” Objective In India, there is scarcity of “specialized centers” providing “comprehensive epilepsy care” and this dearth is further worse in populous states. In this article, we share our single center, observational, and retrospective experience of TLE in background of limited resources and utmost requirement. Methodology Our study is a retrospective analysis medically refractory epilepsy patients (2016–2019). Patients with medically refractory epilepsy were selected based upon our noninvasive protocol (clinical semiology, interictal scalp electroencephalography (EEG), long-term video EEG monitoring data, and magnetic resonance injury [MRI]). The follow-up was noted from the last out-patient visit record or through telephonic conversation (International League Against Epilepsy score). Results Of 23 cases of TLE (n = 7, mesial temporal sclerosis; n = 16 temporal lobe like cavernomas, tumors, or arterio-venous malformations). Single photon emission computed tomography/positron emission tomography (SPECT/PET) was performed in five cases (three cases of ictal/interictal SPECT and two cases of PET scan) where there was discordance between EEG/clinical and MRI. The median follow-up was of 19 months with 18 cases being seizure free. Five cases were fully off the antiepileptic drug (AEDs) while in 15 cases, the AEDs dosages or the number were reduced. Average number of AEDs reduced from 2.9 in preoperative period to 1.2 postoperatively. Two cases had quadrantanopia and one case of cerebrospinal fluid leak. Conclusion A multidisciplinary and holistic approach is required for best patient care. The results of our initial surgical experience are encouraging.

Background: Pineal region tumors often present with hydrocephalus. Endoscopic third ventriculostomy (ETV) and simultaneous tumor biopsy remain a minimally invasive procedure offering both diagnostic and therapeutic advantages in the management of these tumors. However, different operative techniques have been described in the literature. Aim: The aim is to study the ETV success rate, diagnostic rate of simultaneous tumor biopsy, complications, and follow-up of patients of pineal region tumors managed with ETV and simultaneous tumor biopsy using the single burr hole technique. Methods: The study was performed by retrospectively reviewing the records of patients of pineal region tumors managed by simultaneous ETV and tumor biopsy using a “single burr hole” technique from January 2012 to December 2019. Results: Thirty-four patients (22 males and 12 females) with a mean age of 28.7 years were analyzed. ETV was successful in relieving hydrocephalus in 29 (87.8%) patients. Three patients needed a ventriculoperitoneal shunt, and one required Ommaya reservoir placement for persistent hydrocephalus. Histological diagnosis was successfully established in 26 (78.8%) patients. There were two procedure-related mortalities. Two patients underwent craniotomy and tumor excision subsequently. Radiotherapy was given to 11 patients, and 9 patients were managed by observation alone. The mean follow-up of our study was 15.8 months. Conclusions: Simultaneous ETV and tumor biopsy using a single burr hole technique is a safe, minimally invasive procedure for the management of pineal region tumors.

Background: Cervical sub-axial dumbbell neurofibromas (NFs) account for nearly 20% of all NFs, with prognosis depending on the extent of excision. When majority of tumor is extra-foraminal (Tomaya's Type IIb and c), certain unconventional anterolateral or posterolateral neck approaches are used for maximum safe excision. In our article, we provide a brief review of the literature regarding various surgical approaches, emphasizing the utility of posterolateral or combined anterior and posterior neck approaches for such giant NF. Methods: We performed a retrospective analysis of prospectively maintained surgical databases, from our hospital discharge codes, for all cervical Type IIb-c dumbbell NF patients, who underwent surgery at our institution between 2015 and 2019. Clinical variables included age at admission, clinical presentation, and surgical difficulties, and the outcome was analyzed. Results: Four patients of age ranging from 22 to 45 years (M:F 3:1) were operated by posterolateral (n = 3) and combined anteroposterior (n = 1) approach. Three patients underwent near-total excision and one patient had total excision. One patient with tumor capsule attached to roots of upper brachial plexus had motor deficit, who was re-admitted for neurotization. There was no intraoperative complication. Conclusion: Posterolateral approach for cervical dumbbell giant NFs is safe, effective, and promises maximum safe excision. The major blood vessels including carotid artery or jugular veins in neck and vertebral artery at foraminal portion are directly under vision and control of surgeon. Despite near-total excision, wherein small part of adhered capsule is left, recurrence rate is low.

Larsen syndrome is chronic debilitating disease that presents with multiple joint dislocations and severely affects the cervical spine in the form of cervical kyphosis and atlantoaxial dislocation. Children usually present in early with a myriad of deficits, compressive myelopathy being the most common. In addition to a bony compression, there is sometimes a soft tissue component, which is seldom addressed. We present here a case of atlantoaxial dislocation with cervical kyphosis due to Larsen syndrome, and along with our previous experience on syndromic atlantoaxial dislocations, we try to define an algorithm for the treatment approach of these onerous challenges. The importance of early intervention is also emphasized with a literature review of similar cases. In addition to the obvious physical damage, early intervention can also avoid the more sinister socioeconomic face of this debilitating disease.

Background Ependymomas are common intramedullary spinal tumors but there are scattered reports of this tumor presenting with exophytic growth patterns masquerading as intradural extramedullary (IDEM) tumors. Such IDEM ependymomas are seldom suspected preoperatively and it is only during surgery that their existence is revealed. Little is known of such rare growth patterns of an otherwise common intramedullary spinal cord tumor, their characteristics, and their management considerations. Methods We present a case of an exophytic dorsal ependymoma with a stalk like attachment to the spinal cord and the surgical management in a 24-year old woman. An extensive literature search was carried out on all prominent databases to find out similar cases reported earlier. We excluded filum/conus ependymoma, purely extradural spinal ependymoma as well as the extraspinal ependymomas. Details of each case reported before were obtained and tabulated. Results IDEM ependymomas have been reported in 54 patients so far, including the present case. Four patterns of growth emerged from the literature review: intramedullary ependymoma with exophytic component (group I, n = 9), exophytic IDEM ependymoma without intramedullary component (group II, n = 6), IDEM ependymoma arising from nerve roots (group III, n = 7), and pure IDEM ependymoma (group IV, n = 32). Except in group I, IDEM ependymoma affects females more frequently, without any specific age predilection. Thoracic spinal cord/canal is the most common location across all groups. Multifocal disease, craniospinal dissemination, and recurrences tend to be maximum in group IV. Conclusions IDEM ependymomas are more common in thoracic segment of the cord and broadly divisible into 4 subgroups. We suggest a subpial origin of group II IDEM ependymomas. We also advocate proliferation index estimation in grade II ependymomas to enable formulation of an optimal management plan.

Morquio syndrome (MS) is an autosomal recessive defect caused by the deficiency of N-acetylgalactosamine-6-sulfatase. Odontoid hypoplasia, periodontoid soft tissue deposition, and cervical stenosis lead to myelopathy and quadriparesis in these patients. Craniovertebral junction instability in MS possesses a surgical challenge as bones are yet to completely ossify. The atlantoaxial dislocation (AAD) is reducible, and the need of transoral decompression for the soft tissue deposition ventral to odontoid is debatable. We present a series of four cases (mean age 4.3 ± 0.4 years) operated through posterior-only approach (n = 2, C1-lateral mass to C2 pars-interarticularis [Goel's technique]; n = 1 sublaminar wiring followed by C1-lateral mass to C2 pars-interarticularis; and n = 1 suboccipital plate with pars-interarticularis of C2 screw and pedicle of C3 and rod fixation). All patients had acceptable outcome and doing well at the last follow-up (12–96-follow-up). None of our patient needed transoral decompression. Patients with MS frequently manifest with spastic quadriparesis at an early age due to reducible AAD. Early surgical fixation with posterior C1–C2 screw and rod technique is recommended for the favorable surgical outcome and long-term stability of the cervical spine.

Introduction: Craniopharyngiomas constitute 2–4% of intracranial neoplasms. Intraventricular craniopharyngiomas (IVCrs) are the rarely encountered varieties of these lesions.Objective: The objective of the study was to study the special features in clinical presentation, imaging, management, and surgical outcome of IVCrs.Materials and Methods: This retrospective analysis included the combined experience from two tertiary care institutions. Medical records of histopathologically proven cases of IVCrs from January 1994 to June 2021 were assessed, and images were analyzed based on the criteria by Migliore et al. for inclusion of solely intraventricular lesion with the third ventricular ependyma demarcating it from the suprasellar cistern.Results: Among the 25 patients included (mean age: 35.4 years), the most common presentation included headache (n = 21, 84%), vomiting and other features of raised ICP (n = 18, 72%), visual complaints (n = 12, 48%), and endocrinopathies (n = 11, 44%). Fifteen had predominantly cystic tumors, two were purely solid, and eight were of mixed consistency. Primary open microsurgical procedures were performed in 18 (72%) patients, of which four (16%) were endoscope-assisted. Seven (28%) underwent a purely endoscopic procedure. One underwent a staged surgery with endoscopic cyst fenestration and intracystic interferon (IFN)-alpha therapy, followed by microsurgical excision. Complete excision was achieved in 10 patients, near-total in nine, and partial excision in six. Four patients underwent a ventriculoperitoneal shunt (one before the definitive procedure). At a median follow-up of 36 months (range:11–147 months), five patients developed a recurrence, and one had a stable small residue. This patient and two others with small cystic recurrences were observed. One patient was managed with radiotherapy alone. Another underwent re-surgery after a trial of radiotherapy, and the last patient developed a local recurrence, which was managed with radiotherapy; he then later developed an intraparenchymal recurrence, which was operated.Conclusion: Purely IVCrs present with raised intracranial pressure, and visual disturbances are less common. Their deep-seated location and limited surgical field-of-view makes minimally invasive endoscopic-assisted surgery most suitable for their excision. The thin-walled cystic lesions may be occasionally adherent to the ependymal wall in close vicinity to the thalamus–hypothalamus complex, making complete excision difficult. Their responsiveness to radiotherapy, often leads to a gratifying long-term outcome.

Background Hospital-based cancer registries can provide information on the magnitude and distribution of cancers in a given hospital. Hospital-based brain tumor registry data, focusing on glioma, from a tertiary care rural neurological center is lacking in the scientific literature. This data can be useful in understanding the need for research and funding required for these specific brain tumors. Data of patients operated for glioma, at our institute, was collected between January 2004 and December 2015. Patients’ clinical details and histopathological diagnosis were recorded. Data were analyzed and compared with that of previously published literature, and inferences were drawn on patterns of reporting and epidemiology. Results A total of 1450 cases of glioma, with a mean age of 39.3 (± 17.36 SD) years with males (66.6%) comprising more population as compared to females. Majority of patients 70.8% (n = 1027) belong to the economically active age group of country (18–60 years). Majority of cases (41.4%) were glioblastoma with the next common tumor (22.8%) being diffuse astrocytoma (n = 331) followed by pilocytic astrocytoma (6.2%) and oligodendroglioma (4.5%) in that order. While our data followed similar trends with other Indian data the average age of glioma was a decade younger to what is quoted earlier in Indian and international studies. Conclusion This data for glioma gives a glimpse of the prevalence of this tumor in a primarily rural population and highlights the need for a National Brain Tumor Registry with the need for the development of evidence-based policymaking and enhanced research into this particular ailment.

Background and Introduction: Clipping an aneurysm on an elongated and tented V4 segment near the origin of the posterior inferior cerebellar artery (high-riding VA-PICA junction aneurysm) can be challenging. Objective: We demonstrate the microsurgical clipping technique of such an aneurysm using a modified retromastoid approach (MRMA) and glossopharyngeal-cochlear triangle (GCT). Surgical Technique: A 50-year-old female with a ruptured high-riding left VA-PICA junction aneurysm underwent an MRMA. Using segmental vessel isolation with proximal and distal temporary clips, this aneurysm was occluded through the GCT by applying a tandem clipping technique while preserving the PICA. Results: The procedure was uneventful. Apart from transient ataxia, she recovered completely and maintains a good status at follow-up. Conclusion: In high-riding VA-PICA junction aneurysms, a conventional far lateral approach may create awkward viewing and working angles. An MRMA with a horizontal trajectory through the GCT may be a more appropriate strategy.

Brachial plexus repair forms an unmet need in terms of posttraumatic rehabilitation, especially the young population, wherein the incidence of accidents is high. This leads to decrease in the number of functionally active years after the accident. We encountered an interesting case of posttraumatic posterior cord injury predominantly affecting the shoulder abduction beyond 15°. An electrodiagnostic study showed a complete lack of conduction within the axillary nerve with reduced conduction velocity in the radial nerve. We took the patient up for the long head of the triceps transfer to the anterior division of the axillary nerve transfer. Intraoperatively, we found that the long head branch was originating from the axillary nerve at the point of division. As it could not be used for neurotization, we transferred the medial head branch of the radial nerve to the axillary nerve. The patient started to show electroclinical improvement after 3 months of the surgery. A few similar cases have been published, as a cadaveric finding. We report this case to highlight the possibility and need for a high clinical suspicion and also to provide a possible treatment option, in such aberrant anatomy.

Background Vascular loop compression remains the most accepted theory for trigeminal neuralgia (TN). Apart from the normal adjoining vascular loops, certain unusual vascular loops incriminated in TN such as vertebrobasilar dolichoectasia and pure venous compressions do not truly fit into the traditional classification. Moreover, vascular diseases such as cavernoma and aneurysms causing TN are considered as secondary TNs. There is a lack of unified classification for TN with underlying vascular offenders, otherwise amenable to microvascular decompression. Methods We classified vascular offenders in TN (n = 53) into the usual offenders such as superior cerebellar artery or anterior inferior cerebellar artery loop with (n = 4) or without (n = 34) superior petrosal vein loop (n = 38, group I). The unusual vascular offenders (n = 15, group II) comprised unusual arterial loops (n = 4, IIa), pure venous compressions (n = 8, IIb) and vascular diseases (n = 3, IIc). The clinical symptoms, pain severity scores, and surgical outcomes were compared. Results A right-sided preference and male predominance typified the unusual group. The incidence of atypical pain and sensory impairment was higher in group II. Group II also showed a less favorable immediate pain outcome, particularly the patients in group IIb. Group IIb also showed a higher incidence of postoperative hemorrhagic complications. However, long-term outcomes did not differ significantly. Conclusions Unusual vascular offenders in TN do constitute a significant population. They differ from the usual group with respect to the type of pain, gender, and side of involvement and tend to have more complications with similar pain outcomes after microvascular decompression.

Background Paraganglioma in cavernous sinus is a rare entity often misdiagnosed as meningioma or schwannoma on radiology. The embryological origin, from cells of neural crest, does not explain the location per se. Because of its highly vascular nature and close proximity to the carotid artery, surgical excision is a challenge. We herein report the first case of cavernous sinus paraganglioma, without the characteristic “salt and pepper appearance,” excised near completely by the subtemporal approach. Case Description A 30-year-old woman without a history of any comorbid conditions, particularly hypertension, presented with a 6-month history of progressive headache and double vision. She exhibited upward gaze palsy and hypoesthesia over the right half of her face. There was no papilledema and proptosis. Radiologic imaging revealed an extra-axial T1-weighted isointense, T2-weighted hyperintense lesion with homogenous intense contrast uptake in right parasellar region encasing cavernous internal carotid artery. Furthermore, the tumor was extending along the superior orbital fissure. Suspecting a cavernous sinus meningioma, we performed near-total excision via a subtemporal approach. Postoperative recovery was uneventful, and patient was discharged on fifth postoperative day. The histopathology and immunohistochemistry were consistent with paraganglioma. The patient underwent adjuvant radiotherapy and is under our outpatient follow-up. Conclusions Paraganglioma must be considered in the differential diagnosis of parasellar tumors, even when secretory symptoms are absent. The radiologic findings are obscure, and it is difficult to differentiate it from more common tumors of this region such as meningioma, schwannoma, and pituitary adenoma. Surgical excision followed by radiotherapy is the mainstay of management.

Background Proatlas segmentation anomalies represent a rare subset of congenital craniovertebral junction anomalies. In this condition, the structures originating from the proatlas, such as the clivus, occipital condyles, foramen magnum rim, and odontoid tip, may demonstrate congenital anomalies, usually without any spinal instability. Elongated clivus, as a result of nonsegmentation of the odontoid tip from the rest of the proatlas, has been reported before to cause ventral spinal cord compression. We report such a case with certain unreported other associations and explore the pathoembryology and management options of such complex anomalies. Case Description An 8-year old girl presented with a 2-year history of progressive spastic quadriparesis. On neuroimaging, the anterior arch of the atlas was deficient, the odontoid process was foreshortened, and the clivus was elongated, encroaching into the spinal canal leading to ventral spinal cord compression. Additionally, there was rotatory posterior dislocation of the occipital condyles onto the posterior atlantal arch and vertebral artery anomaly. This patient underwent transoral decompression followed by occipitocervical fusion using rods and screws with satisfactory results. Conclusions Proatlas anomalies are rare, varied, and often subtle enough to go unrecognized. Knowledge of the embryology and its aberrations is necessary to understand these anomalies. Our case describes a rare form of bony anomalies pertaining to the fate of the proatlas with accompanying atlanto-occipital dislocation.

Background Poor sanitation, poor hygiene, and archaic cooking practices have led to neurocysticercosis (NCC) being endemic in India. Apart from a cortical location that leads to seizures, intraventricular NCC can present with hydrocephalus and sudden deterioration in sensorium. Consequently, endoscopic excision plays an important role in its treatment because a dilated ventricle offers a minimally invasive and less traumatic route to the pathology. Methods All endoscopically excised intraventricular NCC cases operated were retrospectively analyzed from 2014 to 2017, discussing surgical nuances and postoperative outcome. Results Twelve such cases were found (mean age, 21.9 ± 8.36 years; 9 men and 3 women). The mean follow-up period was 21.17 ± 13.96 months (range, 2–40 months). The most common site was the aqueduct and fourth ventricle. Conclusions An endoscopic approach is a feasible and safe tool for treating this disease. Technical nuances such as entry point and trajectory of endoscope need to be kept in mind while operating.

Localized proliferation of atypical plasma cells, either at bony or extramedullary, forms a rare subset of multiple myeloma (MM) disorders. The patients usually present with intractable pain and pathological fractures and respond well to radiotherapy. The clinical presentation is variable and radiologically is nonspecific. The spinal location is rare, and the solitary plasmacytoma in the intradural extramedullary (IDEM) region is unusual. Herein, we report the second case of solitary plasmacytoma at the lumbar IDEM region. A 54-year-male patient was referred to our institute with complaints of radicular pain in bilateral lower limbs along L5 dermatome for the past 3 months. The neurological examination was normal with power 5/5 and reflexes 2+, except for bilateral straight leg raising test restricted at 30. Magnetic resonance imaging of spine showed a well-defined eccentrically placed (left Side) spherical lesion at the level of L2 vertebrae. The lesion had foraminal extension and showed minimal contrast enhancement. The underlying ventral vertebral body was irregular and hyperosteotic. Our radiological impression was primarily neurofibroma, but the features were slightly atypical. The patient underwent L1-L2 laminectomy and excision of IDEM tumor. The histopathological features were consistent with plasmacytoma. The histopathology was a surprise to us. We further evaluated the patient for MM. He received adjuvant radiotherapy and is currently asymptomatic. An index of suspicion for SP must be kept among differentials of intradural lesions, especially when adjacent bony changes are present. The diagnosis of plasmacytoma warrants further workup according to the recommendations of the International Myeloma Working Group.

Background Densely packed neurovascular structures, often times inseparable capsular adhesions and sometimes a multicompartmental tumor extension, make surgical excision of cerebellopontine angle epidermoids (CPEs) a challenging task. A simultaneous or an exclusive endoscopic visualization has added a new dimension to the classical microscopic approaches to these tumors recently. Methods Eighty-six patients (age: 31.6 ± 11.7 years, M:F = 1:1) were included. Nineteen patients (22.1%) had a multicompartmental tumor. Tumor extension was classified into five subtypes. Sixty-two patients underwent a pure microscopic approach (72%) out of which 10 patients (16%) underwent an endoscope-assisted surgery (11.6%) and 24 patients (28%) underwent an endoscope-controlled excision. Surgical outcomes were retrospectively analyzed. Results Headache (53.4%), hearing loss (46.5%), and trigeminal neuralgia (41.8%) were the leading symptoms. Interestingly, 21% of the patients had at least one preexisting cranial nerve deficit. Endoscopic assistance helped in removing an unseen tumor lobule in 3 of 10 patients (30%). Pure endoscopic approach significantly reduced the hospital stay from 9.2 to 7.3 days (p = 0.012), and had a statistically insignificant yet a clearly noticeable lesser incidence of subtotal tumor excision (0 vs. 10%, p = 0.18) with comparable cranial nerve deficits but with a higher postoperative cerebrospinal fluid (CSF) leak rate (29% vs. 4.8%, p = 0.004). Conclusion Endoscope assistance in CPE surgery is a useful addition to conventional microscopic retromastoid approach. Pure endoscopic excision in CPE is feasible, associated with a lesser duration of hospital stay, better extent of excision in selected cases, and it has a comparable cranial nerve morbidity profile albeit with a higher rate of CSF leak.

Insular gliomas are unique, challenging and evoke a lot of interest amongst neurosurgeons. Publications on insular glioma generally focus on the surgical intricacies and extent of resection pertaining to the low-grade gliomas. Insular glioblastomas (iGBM) have not been analysed separately before.Histologically proven WHO grade IV gliomas involving the insula over a 9-year period were studied. Their clinical presentation, radiological features, surgical findings and survival outcomes were assessed. Statistical methods were used to determine the favourable predictors of survival.Out of 27 patients (M:F = 2.9:1), 18 (66%) patients had a tumour extension beyond the insula, 10 (37%) of whom had basal ganglia involvement. Total, near total and subtotal excisions were performed in 7 (26%), 9 (33%) and 11 (40.7%) patients, respectively. Twenty-three patients had glioblastoma, while four had gliosarcoma. IDH mutation was negative in six of the seven patients where it was done. Median overall survival was 5 months. Multivariate analysis showed that a female gender (Insular glioblastomas have a poor prognosis. Insular location and certain tumour characteristics often limit the extent of resection of iGBMs. Moreover, postoperative complications sometimes negate the advantages of a radical resection. A female gender, presentation with seizures and completion of adjuvant chemoradiotherapy appear to be good prognostic factors.

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.

Objective Spinal epidural arteriovenous fistulas (SEAVFs) are the rarest variety of spinal vascular malformation and are often misdiagnosed as type 1 spinal dural fistula. This retrospective study highlights the salient anatomic differentiating points of these entities and also highlights the importance of a planned endovascular treatment approach using different routes of access. Efficacy of the endovascular treatment at 3 months follow-up was also studied. Methods We retrospectively reviewed 11 treated patients with SEAVF. Existence of epidural arteriovenous fistula in all these patients was confirmed by spinal angiography. The Aminoff-Logue Scale score was assigned both before and after the procedure. The statistical results were expressed as percentages, and the preprocedure scale was compared with the postprocedure scale at 3 months by using a nonparametric Wilcoxon signed-rank test. Results The patients ranged in age from 7 to 53 years, with male predominance. Paraparesis was the commonest symptom, and 1 patient had congestive cardiac failure caused by a large fistula. Location was mostly dorsolumbar with intradural venous reflux into the perimedullary venous system (Castilla type A) noted in 3/11(27%) patients, and the remaining 73% patients had Castilla type B1 with an enlarged epidural venous sac. Therapeutic embolization was performed from arterial, venous, or combined routes. The 3-month postprocedure clinical assessment showed statistically significant (P Conclusions The differentiating points between SEAVF compared with type 1 spinal dural fistula are emphasized. The study also highlights the importance of a good angiographic assessment to best access the fistula by arterial, venous, percutaneous, or combined routes. Endovascular treatment resulted in statistically significant clinical improvement at 3 months follow-up.

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

Objective: Larsen syndrome (LS) is characterized by osteo-chondrodysplasia, multiple joint dislocations, and craniofacial abnormalities. Symptomatic myelopathy is attributed to C1–C2 instability and sub-axial cervical kyphosis. In this article, we have analyzed the surgical outcome after posterior fixation in LS with craniovertebral junction instability. Methods: Ten symptomatic pediatric patients, operated between 2011 and 2019, were included, and the clinical outcome was assessed by Nurick grade, neurological improvement, and complications. The requirement of anti-spasticity drugs, the degree of bony fusion, and restriction of neck movement were also noted. At last follow-up, patient satisfaction score (PSS) and back to school status were studied. We also reviewed the literature and categorized two types of presentation of reported LS patients and discussed the pattern of disease progression among both. Results: Ten patients, age range 1.5–16 years, underwent 12 surgeries (6 C1–C2 fixation, 4 long-segment posterior cervical fixation, and 2 trans-oral decompressions as the second stage); the mean follow-up was 23 (range, 6–86 months). All the ten patients in our study had the characteristic “dish-” like face and nine patients had acral anomalies. The median Nurick grade improved from preoperative (median = 4) to follow-up (median = 3). The requirement of anti-spasticity drugs decreased in seven patients and the neck-pain improved in nine patients. The median satisfaction at follow-up was good (median PSS = 2); five patients were going back to school. Conclusion: Craniovertebral junction instability in LS is rare and surgically challenging. Early posterior fixation showed a promising outcome with a halt in the disease progression.

Introduction: Severe acute respiratory syndrome, coronavirus 2 (SARS-COV 2) has inexplicably and irreversibly changed the way of neurosurgery practice. There has been a substantial reduction in neurosurgical operations during the period of lockdown. The lockdown might be the most effective measure to curtail viral transmission. Once we return to the normalization of the lifestyle, there will be a backlog of unoperated pending cases along with the possibility of further spread of the coronavirus. Methods: We reviewed the available literature and protocols for neurosurgical practice in different geographic locations. We drafted a consensus statement based on the literature and protocols suggested by the World Health Organization (WHO) and various professional societies to prevent the spread of SARS-COV2 while streamlining the neurosurgical practice. Results: The consensus statement suggests the patient triage, workflow, resource distribution, and operational efficacy for care providers at different stages of management. The priority is set at personal protection while ensuring patients' safety, timely management, and capacity building. We performed a detailed subsection analysis for the management of trauma and set up for COVID-free hospitals for simultaneous management of routine neurosurgical indications. In this time of medicolegal upheaval, special consent from the patients should be taken in view of the chances of delay in management and the added risk of corona infection. The consensus statements are applicable to neurosurgical setups of all capacities. Conclusion: Along with the glaring problem of infection, there is another threat of neurosurgery emergency building up. This wave may overwhelm the already stretched systems to the hilt. We need to flatten this curve while avoiding contagion. These measures may guide neurosurgery practitioners to effectively manage patients ensuring the safety of caregivers and care seekers both.

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.

Objective Tumors with seizures as primary mode of presentation are collectively called Long-term epilepsy associated tumors (LEATs or Epileptomas). The overall survival is good so ‘seizure outcome’ becomes the primary goal rather than neuro-oncological outcome. Methods A retrospective analysis of our surgical database (2015−19) was done to find operated patients of intra-axial brain tumors with age less than 25-years and who had presented with seizures. Results The mean age at presentation was 16.44 years (SD + 6.82 years). Complex partial seizures/focal unaware seizures were the most common type of seizures encountered (n = 22) with mean duration of seizures was 49.50 months (SD + 31.04 months). The most common pathology was glioneuronal tumors (GNTs) (n = 17). Gross total resection (GTR) group had a significantly better seizure outcome as compared with the Subtotal resection (STR) group (p = 0.006). Presence of focal or partial seizure was a significant factor pointing towards a better seizure control (p = 0.005). Conclusion The shorter duration of symptoms, partial/focal seizures and gross total excision were predictors of a good seizure-outcome. Age of the patient and the histopathology of the tumor does not affect seizure-outcome on comparing GNTs with non GNTs.

Background: The COVID-19 infection outbreak has aroused increasing attention and affected thousands of people nationwide. The long incubation period, high infectious rate, varied manifestation, and absence of effective treatment make it difficult to manage the disease transmission. Objective: The intended goals are to encourage efficient management of neurological and neurosurgical patients, resource utilization, and protecting the healthcare provider during the COVID-19 epidemic. Herein, we present a consensus statement from various centers in India Methodology: In addition to the literature review, recommendations were included from neurologists and neurosurgeons from various centers in India. Results: Every patient presenting for treatment should be treated as a potential asymptomatic infected case. Patients should be categorized based upon the priority as acute (require immediate treatment/surgery within 24 h), sub-acute (requiring treatment within a maximum of 7–10 days), or chronic (requiring treatment within a month). Non-essential elective surgeries and outpatient clinics should be avoided after informing the patient(s). There is a high risk of aerosol dispersion during intubation and certain neurosurgical procedures particularly those involving drills and endoscopes. These procedures should be performed wearing full personal protective equipment. The workflow of the operating rooms should also be modified significantly. Minor modifications in personal and professional lifestyles and routine training to use the PPE will ensure efficient management of resources. Conclusion: These recommendations could be used to mitigate the risks and reduce exposure to other patients, public, and healthcare staff.

Background: This prospective study analyzes the factors responsible for pre and postoperative persistent tinnitus following vestibular schwannoma (VS) surgery and discusses the possible etiopathogenetic mechanisms. Materials and Methods: Sixty-seven consecutive patients with unilateral VS operated via the retrosigmoid–suboccipital approach were included in the study. The Cochlear nerve, often unidentifiable from the tumor capsule, was resected during the surgery. Tinnitus Handicap Inventory (THI) score assessed the severity of pre and postoperative tinnitus. Result: Twenty-eight (41%) patients had preoperative tinnitus. Out of those 28 patients, 24(85%) had significantly improvement in postoperative THI score. In 15 of the 24 patients, tinnitus subsided completely. In 3 of the 28 (10%) patients, THI scores were unaltered, and in 1 of the 28 (3.5%) patients, THI scores worsened. In 39 (58.2%) patients without preoperative tinnitus, 4 (10%) developed a new-onset postoperative tinnitus. Patients with severe sensory neural hearing loss (SNHL) had significantly higher incidence of postoperative persistent tinnitus (PPT) (P = 0.00) compared to those with mild-to-moderate SNHL. Patients with profound SNHL, however, had a much lower incidence of PPT (P = 0.007; odds ratio = 0. 0.077; 95% CI: 0.009–0.637). Large (P = 0.07) and giant schwannomas (P = 0.03) VS had an increased risk of PPT. Patients with PPT further analyzed with brain stem auditory evoked response (BAER) showed normal contralateral waveform. Conclusion: Assessment of tinnitus is mandatory during the management of VS as there are high chances (nearly 46%) of PPT. Preoperative tinnitus, linked to the degree of SNHL (higher incidence in severe SNHL compared to mild-to-moderate/profound SNHL), is dependent on an intact cochlear nerve functioning. However, PPT is dependent on other mechanisms (brain stem/ipsilateral cochlear nuclei compression, and cortical reorganization) as it persists despite cochlear nerve resection.

Background: Progressive vision loss is a dismal sequelae of idiopathic intracranial hypertension (IIH) and secondary intracranial hypertension with cerebro-venous sinus thrombosis (CVST). The initial management revolves around weight loss, acetazolamide, steroids, and diuretics. A subset of unfortunate patients, refractory to medical therapy, need surgical intervention in the form of CSF diversion or optic nerve decompression (OND). The ONDd is an emerging alternative with encouraging early results. Aim: In our study, we share our experience of ONDd by endoscopic endonasal corridor, highlighting the technical nuances of procedure and discuss the indications of the same in the era of advanced technology. Materials and Methods: A retrospective, noncomparative review of the medical records of all the patients of IIH (ICHD-III criteria) with severe vision loss, refractory to medical treatment, and with established objective evidence of papilledema was done. All the patients were operated in our department by endoscopic endonasal sheath fenestration. Results: Nine patients (M:F 3:6) underwent endoscopic endonasal optic nerve decompression (2016–2019) approach for medically refractive IIH (n = 6) and CVST (n = 3). The mean age of population was 21.44 ± 5.14 years; 6 patients had improvement in headache and 6 had improvement in visual acuity. The visual acuity deteriorated in two patients (n = 1 IIH and n = 1 CVST with dural AVF). One patient needed postoperative lumbar drain for CSF leak, while none had meningitis. Conclusion: Endoscopic optic nerve sheath fenestration is minimally invasive and effective alternative with promising outcome in the management of medical refractory IIH or CVST.

Background: Insular high-grade gliomas are uncommon and constitute approximately 10% of all intracranial high-grade gliomas. Several publications in the recent years have thrown substantial light in the understanding of insular low-grade gliomas. However, there is a paucity of information concerning the spectrum of high-grade lesions affecting the insula, the mode of presentation vis-à -vis low-grade gliomas, and the survival rates to modern therapy. Aims and Objectives: We aim to highlight various clinical patterns, histo-pathological spectrum and the survival rates in patients with high-grade insular lesions. Also, we explore the factors that govern favourable outcomes. Materials and Methods: A retrospective study of 41 patients operated for high-grade insular tumors at our institute between March 2010 to December 2018 was done to evaluate the clinico-radiological features, surgical nuances, survival rates and seizure outcomes. Results: Raised intracranial pressure was the most frequent clinical presentation (n=28/41, 68.3%). Nearly 60% of the patients (n=25) had involvement of all four Berger-Sanai zones. The high-grade tumors encountered in our series were: glioblastoma (n=15), gliosarcoma (n=3), and embryonal tumor, not otherwise specified in 3 patients, while 21 patients had grade 3 astrocytoma. 33 out of 41 patients (80.5%) in our study showed excellent seizure control (ILAE grade 1A) at follow-up. Clinical presentation with seizures (P = 0.01, HR=0.3), WHO grade IV histopathology (P = 0.04, HR=3.7) and development of recurrence (P = 0.05, HR=5.5) were found to be independent predictors of OS. Conclusion: Insular high-grade gliomas are commoner than thought and nearly half of these are grade IV tumors (51%). A presentation with seizures may indicate precursor low-grade gliomas and portend a better survival. A maximum “safe” surgical resection, keeping the postoperative quality of life in mind, should be the goal.

Background: Postoperative pain and cerebrospinal fluid (CSF) leak are common known complications of spinal surgery, both having a synergistic effect on each other. Thus, both need to be dealt simultaneously. Double drug impregnated autologous coagulum patch (DDIAC) is a novel method which reduced both incidences of postoperative CSF leaks as well as pain. Methodology: Twenty-seven patients undergoing lumbar disc surgery without instrumentation were included and randomized into DDIAC and single drug impregnated autologous coagulum patch (SDIAC) group. The patients were assessed postoperatively with visual analog scale (VAS). Results: There were 21 males and 6 females in the study. Seventeen (63%) patients got randomized for DDIAC patch application and other 10 (37%) patients entered the control arm group (SDIAC) patch use. Preoperative VAS was 5 in both the groups. The average postoperative VAS was 3.01 in DDIAC arm and 4.29 in control arm. The average analgesic shot required in the DDIAC group was 0.41 in 24 h and SDIAC group was 4.1 in 24 h. In DDIAC group, none of these patients had CSF leak from the surgical wound till discharge from the hospital. In the SDIAC group, one (10%, n = 10) patient had CSF leak. Conclusions: DDIAC patch was effective in controlling pain in the postoperative period; however, few patients may require analgesic shots for pain management. SDIAC patch may control some pain in the immediate postoperative period; however, this was not sufficient for a longer postoperative period.

Genetic or developmental intrauterine insults before the 10th week of intrauterine life subsequently result in one or all of osseous and neural anomalies like congenital atlanto-axial dislocation (CAAD), platybasia, occipitalized atlas, Klippel–Feil anomaly, basilar invagination (BI), Arnold Chiari malformation, and various vascular anomalies. More than 90% of patients with BI present with irreducible AAD as well as occipitalized atlas, and about 30% of them have associated Chiari malformation and syringomyelia. Development and progression of anomalous bony changes in patients with cranio-vertebral junction (CVJ) anomalies are phasic, in which mechanical displacement of facet joints occurs in the background of its primary osseous dysgenesis. Although it can present at any age, it is commonly seen in the second or third decade of life. The clinical presentation varies from asymptomatic to spastic weakness of all four extremities. The treatment protocol has shifted from the conventional concepts of decompression and bone removal to cranio-vertebral realignment and fixation-arthrodesis in the recent era. This chapter is an attempt to describe the various aspects of CAAD (that often results in a high cervical myelopathy and is an important cause of morbidity and mortality), with the overall focus being on its embryological basis, anatomy, clinical features, and nuances of surgical management. By a review of the relevant literature, we also attempt to summarize the outcome of surgical treatment of CVJ anomalies by different methods and approaches. The percentage of improvement in the neurological deficits in the postoperative period utilizing various methodologies has also been described. The algorithm of management of this complex disease must be individualized based on the facet joint anatomy, presence or absence of instability, as well as the surgeon’s personal experience and choice.

Central nervous system aspergillosis is rare with reported mortality rate of 13–50% involving foci in paranasal sinuses and even higher mortality rates (80–100%) in patients of immune-compromised status. Modern day imaging offers an opportunity for early diagnosis, but findings are minimal. A typical finding is that of a space occupying mass lesion with iso-hypointense signal on T1-weighted sequences and extreme hypointense appearance on T2-weighted. This results from the concentration of ferromagnetic substances (iron/zinc/magnesium/manganese) within the lesion. Gadolinium enhancement pattern varies from homogeneous to peripheral ring enhancement. The immune-competent hosts present with homogeneous enhancement while those with immune compromise bear more variable radiological appearance. Due to such radiological appearance, meningioma or tuberculoma is considered in differential earlier than fungal granuloma, if not for the hypointense appearance on T2-weighted sequences which strongly points to fungal granuloma. Through this case report, we attempt to highlight the unusual radiological presentation of the entity.

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.

Germ cell tumors (GCTs) are rare intracranial tumors with a strong predilection for children. Commonly, these tumors arise either in the suprasellar or the pineal region. The basal ganglia-thalamus complex represents a rare site of nonmidline intracranial GCTs. Such basal ganglionic GCTs have been reported to produce certain interesting clinico-radiological features, the knowledge of which may provide important diagnostic clues preoperatively. We present the case of a 9.5-year-old boy who presented with right hemiparesis and precocious puberty. Imaging revealed a heterogeneously enhancing mass involving the left capsuloganglionic region, sparing the thalamus. There was little perilesional edema and midline shift. Because of the deep location of the mass and a lack of mass effect, a neuronavigation-guided tumor biopsy was performed which unraveled a pure germinoma. The child was referred for adjuvant radiotherapy following an uneventful postoperative course. At the time of writing the report, the child was on radiotherapy and doing well.

Cranial nerve schwannomas usually arise from sensory nerve and the occurrence of schwannoma in a motor nerve is rare, especially in sporadic cases. Oculomotor nerve schwannomas (ONS) are rare and they are unique as they arise from motor nerve. ONS palsy may or may not be the presenting feature of oculomotor schwannoma. We present the case of a young male with ONS, presenting with oculomotor nerve palsy along with features of raised intracranial pressure. Oculomotor schwannoma is described in literature only as case reports, and oculomotor nerve is also a rare site for schwannoma as being a motor nerve. In this article, we describe a case of cisternal ONS with review of pertinent literature.

Trans -sellar trans-sphenoidal encephalocele is an extremely rare entity. We present the case of an 18-month old boy who presented with a trans-sellar, trans-sphenoidal encephalocele associated with cleft lip, cleft palate and microphthalmia. This patient was treated successfully by a trans-cranial extra-dural route. In this paper, we discuss the clinico-radiological findings as well as various surgical options in managing these rare lesions and briefly review the literature.

Background: Primary extramedullary tumors involving multiple compartments around the spine are a technically demanding group of tumors whose extent traverses beyond the normal confines of those anatomical regions which fall in the common domain of neurosurgeons. In the following series, we present 12 patients who were diagnosed with primary spinal extramedullary tumors with multicompartmental extension, and whose surgical management was facilitated by a combined multidisciplinary approach involving surgeons of other superspecialties. This multidisciplinary assistance from the inception to the culmination of surgical management helped in achieving a better surgical removal, thereby resulting in better surgical outcomes. Materials and Methods: Twelve consecutive patients who fulfilled the inclusion criteria in the 5-year period (January 2010 to January 2015) were included in the series based on the radiological and pathological characteristics of the tumor. Depending on the site of the spine involved by the lesion, radiologists and surgeons were involved from the planning phase of the surgical management, and their assistance in procedures such as preoperative embolization/ureteric stenting was sought whenever was deemed necessary. The extent of resection and total blood loss was recorded meticulously. Regular follow-up (3, 6, and 12 months and 2 and 5 years) of the patients was done after the initial follow-up at 6 weeks and their disability scores were recorded. Results: Of the 12 cases (6 males and 6 females), sacrum was the most common location of the tumors (6). Histopathologically, giant-cell tumors, schwannomas, and chondrosarcomas (3 each) were most common followed by Ewing's sarcoma (2) and malignant peripheral nerve sheath tumor (1). Eight patients had functional status of McCormick scale 1 and two patients had a functional status of 2. One patient was lost to follow-up and one patient died during surgery. Conclusion: Judicious involvement of access surgeons and adjunct therapies along with careful preoperative planning can help in improving surgical outcome in multicompartmental spinal tumors.

Background: Skull base lesions are not uncommon, but their management has been challenging for surgeons. There is large no of bony tumors at the skull base which has not been studied in detail as a group. These tumors are difficult not only because of their location but also due to their variability in the involvement of important local structure. Through this retrospective analysis from a Tertiary Care Centre, we are summarizing the details of skull base bony lesions and its management nuances. Materials and Methods: The histopathologically, radiologically, and surgically proven cases of skull base bony tumors or lesions involving bone were analyzed from the neurosurgery, neuropathology record of our Tertiary Care Institute from January 2009 to January 2014. All available preoperative and postoperative details were noted from their case files. The extent of excision was ascertained from operation records and postoperative magnetic resonance imaging if available. Results: We have surgically managed 41 cases of skull base bony tumors. It includes 11 patients of anterior skull base, 13 middle skull base, and 17 posterior skull base bony tumors. The most common bony tumor was chordoma 15 (36.6%), followed by fibrous dysplasia 5 (12.2%), chondrosarcoma (12.2%), and ewings sarcoma-peripheral primitive neuroectodermal tumor (EWS-pPNET) five cases (12.2%) each. There were more malignant lesions (n = 29, 70.7%) at skull base than benign (n = 12, 29.3%) lesions. The surgical approach employed depended on location of tumor and pathology. Total mortality was 8 (20%) of whom 5 patients were of histological proven EWS-pPNET. Conclusions: Bony skull base lesion consists of wide variety of lesions, and requires multispecialty management. The complex lesions required tailored approaches surgery of these lesions. With the advent of microsurgical and endoscopic techniques, and use of navigation better outcomes are being seen, but these lesions require further study for development of proper management plan.

Brainstem gliomas are relatively rare in adults (