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   Table of Contents - Current issue
January-March 2017
Volume 12 | Issue 1
Page Nos. 1-152

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Predictors of visual outcome with transsphenoidal excision of pituitary adenomas having suprasellar extension: A prospective series of 100 cases and brief review of the literature p. 1
Amit Kumar Thotakura, Mohana Rao Patibandla, Manas K Panigrahi, Gokul Chowdary Addagada
Objective: The aim was to assess the factors influencing the visual outcome following trans-sphenoidal excision of pituitary adenomas. Materials and Methods: One hundred consecutive patients of pituitary adenomas with suprasellar extension (SSE) were operated by trans-sphenoidal approach from July 2003 to December 2006. There were 52 male and 48 female patients with a mean age of 42.47 years. The visual impairment score, which was used to evaluate the visual outcome was produced by adding the scores for visual acuity and visual field defects of each patient (from the tables of the German Ophthalmological Society). The mean diameter, the SSE and the parasellar extension of the lesion were noted in the magnetic resonance imaging study. The average follow-up was 43.5 months. Results: The mean diameter of the tumor was 32.97 mm, and the mean SSE was 14.95 mm. The parasellar extension was present in 27 patients. The vision improved in 61 of the 71 patients (85.91%). The shorter the duration of visual symptoms and smaller the size of the lesion resulted in better visual outcome. The age and the preoperative visual impairment did not show any correlation with the visual outcome. Conclusions: Patients with visual symptoms of <1-year duration (P < 0.01) and adenomas of <36.5 mm diameter (P < 0.009) have better visual outcome.
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Perioperative complications in endovascular neurosurgery: Anesthesiologist's perspective p. 6
Megha U Sharma, Pragati Ganjoo, Daljit Singh, Monica S Tandon, Jyotsna Agarwal, Durga P Sharma, Anita Jagetia
Background: Endovascular neurosurgery is known to be associated with potentially serious perioperative complications that can impact the course and outcome of anesthesia. We present here our institutional experience in the anesthetic management of various endovascular neurosurgical procedures and their related complications over a 10-year period. Methods: Data was obtained in 240 patients pertaining to their preoperative status, details of anesthesia and surgery, perioperative course and surgery-related complications. Information regarding hemodynamic alterations, temperature variability, fluid-electrolyte imbalance, coagulation abnormalities and alterations in the anesthesia course was specifically noted. Results: Among the important complications observed were aneurysm rupture (2.5%), vasospasm (6.67%), thromboembolism (4.16%), contrast reactions, hemodynamic alterations, electrolyte abnormalities, hypothermia, delayed emergence from anesthesia, groin hematomas and early postoperative mortality (5.14%). Conclusion: Awareness of the unique challenges of endovascular neurosurgery and prompt and appropriate management of the associated complications by an experienced neuroanesthesiologist is vital to the outcome of these procedures.
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Post ventriculoperitoneal shunt abdominal pseudocyst: Challenges posed in management p. 13
Raashid Hamid, Aejaz A Baba, Nisar A Bhat, Gowhar Mufti, Younis A Mir, Wani Sajad
Background: In patients with hydrocephalus, the abdominal cavity has been used for absorption of cerebrospinal fluid (CSF) since 1905. Ventriculoperitoneal (VP) shunt operation is followed by abdominal complications in about 5-47% cases. Abdominal CSF pseudo cyst is an uncommon, but well described complication. Aim: This survey was conducted to study the clinical profile and management of this entity. We present our experience with cases of CSF pseudo cyst in children. Materials and Methods: Retrospective analysis of 4 cases diagnosed to have abdominal pseudo cyst following VP shunt between 2008 and 2013. All the four cases were suspected clinically and diagnosis was confirmed by abdominal ultrasonography. Results: In three patients, the cyst was multilocular and of varying size. Fourth one had a unilocular cyst at the lower end of VP shunt. All the four patients had features of varying degree raised intracranial pressure and a two patients had abdominal signs also. All the patients needed open exploration. Cyst fluid was drained and partial to complete excision of the cyst was done along with the repositioning of the shunt in abdominal cavity in three patients and exteriorization of shunt in one patient. Patients were followed for any further complication over a period of 1-year. Conclusion: Abdominal pseudo cyst is a rare complication after VP Shunt and could result in shunt malfunction or abdominal symptoms and signs. Whenever suspected it should be confirmed by imaging, followed by open exploration and repositioning of the shunt.
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Neuroendoscopy - Is it safe? p. 17
Manas Panigrahi, Bharat Gupta, Rajesh Reddy
Background: The effect of heat in endoscopic instruments used for laparoscopy and hysteroscopy has been well-studied. Reports of thermal injury from nasal endoscopes have also been reported. However, there are no experimental studies, which have objectively documented and provided recommendations for safe use of endoscopes in neurosurgery. Objectives: To study the heat generated at the tip of the endoscope and the subsequent thermal damage caused to a cotton drape placed in proximity, by varying the intensity of light delivered through different optical cables, ambient (operating room) temperatures and working distances. Materials and Methods: The study was carried out in the operation theater using a 300 watt xenon light source connected to the endoscope with 3.5 mm and 4.8 mm optical cables. A digital thermometer was used to record the heat generated at the tip of the endoscope. Results: The heat generated at the tip of the endoscope reached its peak in the first 6 min and attained a plateau at 15 min after turning on a light source of 60% intensity. Thermal injury to the cotton drape took a longer time with a 3.8 mm cable compared with 4.8 mm cable. The heat generated at the tip of the endoscope, and thereby the thermal injury caused was found to be lower when the ambient temperature was close to 20°C. Conclusions: Complications related to thermal injuries caused by heat generated at the tip of an endoscope can be reduced by using a smaller diameter cable, light intensity of 60%, increasing the working distance (as permissible), reducing the time spent for dissection while keeping the endoscope very close to the target and lowering the ambient temperature to 20°C.
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First report of important causal relationship between the Adamkiewicz artery vasospasm and dorsal root ganglion cell degeneration in spinal subarachnoid hemorrhage: An experimental study using a rabbit model p. 22
Osman N Turkmenoglu, Ayhan Kanat, Coskun Yolas, Mehmet Dumlu Aydin, Naci Ezirmik, Cemal Gundogdu
Background: The blood supply of the lower spinal cord is heavily dependent on the artery of Adamkiewicz. The goal of this study was to elucidate the effects of lumbar subarachnoid hemorrhage (SAH) on the lumbar 4 dorsal root ganglion (L4DRG) cells secondary to Adamkiewicz artery (AKA) vasospasm. Materials and Methods: This study was conducted on 20 rabbits, which were randomly divided into three groups: Spinal SAH (n = 8), serum saline (SS) (SS; n = 6) and control (n = 6) groups. Experimental spinal SAH was performed. After 20 days, volume values of AKA and neuron density of L4DRG were analyzed. Results: The mean alive neuron density of the L4DRG was 15420 ± 1240/mm3 and degenerated neuron density was 1045 ± 260/mm3 in the control group. Whereas, the density of living and degenerated neurons density were 12930 ± 1060/mm3 and 1365 ± 480/mm3 in serum saline (SS), 9845 ± 1028/mm3 and 4560 ± 1340/mm3 in the SAH group. The mean volume of imaginary AKAs was estimated as 1,250 ± 0,310 mm3 in the control group and 1,030 ± 0,240 mm3 in the SF group and 0,910 ± 0,170 mm3 in SAH group. Volume reduction of the AKAs and neuron density L4DRG were significantly different between the SAH and other two groups (P < 0.05). Conclusion: Decreased volume of the lumen of the artery of Adamkiewicz was observed in animals with SAH compared with controls. Increased degeneration the L4 dorsal root ganglion in animals with SAH was also noted. Our findings will aid in the planning of future experimental studies and determining the clinical relevance on such studies.
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Clinical predictors for survival and treatment outcome of high-grade glioma in Prasat Neurological Institute p. 28
Raywat Noiphithak, Kullapat Veerasarn
Objective: The aim was to identify clinical predictors for survival and examine treatment outcome in patients with high-grade glioma (HGG). Materials and Methods: The authors retrospectively reviewed medical records of patients who was diagnosed HGG between January 2007 and December 2009. Demographic data, radiological data and treatment data of patients were reviewed and analyzed. Results: A total of 100 patients were analyzed. There was no difference in demographic data between Grade III and IV glioma. Patients with HGG had median survival time (MST) 18 months, The MST of patients with Grade III and IV glioma were 26 and 13 months, respectively. In this study, only anaplastic oligoastrocytoma and radiotherapy did impact strongly on survival of patients with HGG. In patients with Grade III and IV glioma, radiotherapy found to have influence on survival. Conclusion: Patients with HGG in Prasat Neurological Institute had short survival resemble to other previous study. The clinical predictors for survival of patients were identified on multivariate analysis.
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An evaluation of neuroendocrine dysfunction following acute aneurysmal subarachnoid hemorrhage: A prospective study p. 34
Awadhesh Kumar Jaiswal, Subhash Yadav, Rabi Narayan Sahu, Anant Mehrotra, Sanjay Behari, Ashok Kumar Mahapatra
Objective: The aim was to investigate the incidence and pattern of neuroendocrine changes in cases of acute aneurysmal subarachnoid hemorrhage (SAH). Materials and Methods: Endocrine assessment was performed in 100 consecutive cases of acute aneurysmal SAH presenting within 7 days of ictus. The gonadotropic, somatotrophic, thyrotropic, and corticotrophic axes were evaluated for their possible dysfunction. Results: A total of 100 cases (38 males, 62 females; age range - 17-76 years; mean age - 43.6 years) of acute SAH were studied. The aneurysms were located in the anterior circulation (n = 95) and posterior circulation (n = 5). The most common hormone deficiency was of growth hormone (n = 67), followed by gonadotrophin (n = 50), corticotrophin (n = 49) and thyrotrophin (n = 35). Hyperprolactinemia was noted in 10 cases. One-pituitary hormone axis deficiency was noted in 26 cases while 67 cases had two or more pituitary hormone axes dysfunction. A total of 93 cases had hormonal dysfunction in one or more pituitary hormone axes, and seven cases had no hormonal dysfunction. Conclusions: Endocrine dysfunction occurs in 93% cases of acute SAH and multiple pituitary hormone axes dysfunction occurs in 67% cases. It is suggested that hormonal evaluation should be considered as part of management of acute SAH.
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Outcome after operative intervention for traumatic brain injuries in the elderly p. 37
Lai-Fung Li, Wai-Man Lui, Heidi Hay-Tai Wong, Wai-Kei Yuen, Gilberto Ka-Kit Leung
Introduction: The management of traumatic brain injuries in the elderly (age ≥ 65 years) is a constant dilemma. The aim of this study is to investigate for factors that may predict outcome of operative treatment in this group of patients. Materials and Methods: A retrospective analysis was conducted on 68 elderly patients who had been operated in a designated center from 2006 to 2010. Patients' age, Glasgow Coma score (GCS), pupillary responses, imaging findings, medical conditions, and the use of anticoagulant/antiplatelet agents on patient outcomes were studied. Results: The overall mortality rate was 55.9%. Older age, abnormal pupillary response, low GCS, the presence of midline shift and cistern obliteration on computerized tomography were associated with poor survival. Patient aged 75-84 with normal bilateral pupillary response still had an overall survival rate of 52.6% and good outcomes (Glasgow outcome score: 4 or 5) in 36.8% of patients. Abnormal pupillary response in at least one eye and preoperative GCS ≤ 12 were associated with very poor prognosis. Conclusions: More advanced age was found to be associated with progressively worse outcome. A subgroup patients aged below 85 would survive and could achieve good clinical outcome. The prognosis of those aged over 85 with moderate or severe head injuries was extremely poor.
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Unusual site of Rathke's cleft cyst: A case report and review of literature p. 44
Vivek Agarwal, Deepak Palande, Vernon Velho, Rachana S Binayke
We report an eight years female child of symptomatic giant Rathke's cleft cyst extending from right parasellar, paraventricular region to right fronto-temporo-parietal cortical surface which is an unusual site for Rathke's cyst. MRI Brain scans of patient who presented with diminution of vision in right eye with headache and vomiting revealed a large cystic mass in right parasellar, paraventricular region with peripheral enhancement and midline shift. In this case, we performed the right fronto-temporo-parietal craniotomy with drainage and marsupilization of cyst wall. Histopathologically, it was inflamed Rathke's cleft cyst with focal epithelial metaplasia.
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Spontaneous extradural and subgaleal hematoma: A rare neurosurgical crisis of sickle cell disease p. 47
Sudhansu S Mishra, Satya B Senapati, Amiya K Gouda, Sanjay K Behera, Ashis Patnaik
Extradural hematoma (EDH) in absence of trauma is a rare entity with only few cases reported in literature. The various causes reported include: Vascular malformation of dura, coagulopathies, sinus infection, middle ear or orbital infection, and tumor. Occurrence of spontaneous EDH as a complication of sickle cell disease is even much rarer. We report a case with sickle cell disease who presented with spontaneous extradural and subgaleal hematomas following an episode of vaso-oclusive crisis. He was managed successfully with surgery. The association of epidural hematomas in sickling hemoglobinopathies is reviewed. In all cases, we noticed one episode of sickle cell crisis just before the occurrence of spontaneous EDH. Perhaps this crisis puts an extra demand over the hematopoietic skull tissue disrupting inner and outer skull margins leading to spontaneous EDH and subgaleal hematoma.
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Persistent L5 lumbosacral radiculopathy caused by lumbosacral trunk schwannoma p. 51
Guive Sharifi, Amin Jahanbakhshi
Schwannomais, usually, benign tumor of nerve sheath that occurs evenly along the spinal cord. Intra-pelvic schwannoma is very rare entity that may arise from lumbosacral nerve roots or from sciatic nerve. Radicular pain of the lower limb as a presenting symptom of pelvic schwannoma is extremely rare. In the current report, the patient is presented with a right sided L5 radicular pain typical of lumbar discopathy. Interestingly, a herniated lumbar disc was noted on lumbosacral magnetic resonance imaging (MRI). In pre-operative studies a large pelvic mass was detected in the right pre-sacral area with solid and cystic components consistent with schwannoma. The patient underwent a low midline laparotomy to evacuate the retroperitoneal mass. Uniquely, we found the tumor to be arisen from lumbosacral trunk not from a root or peripheral nerve. Most cases with intra-pelvic schwannoma present so late with vague abdominal and pelvic discomfort or pain, low back pain, urinary and bowel symptoms because of compressive effect of the tumor, or incidentally following gynecologic work-ups; So, these patients are mostly referred to gynecologists and urologists. A neurosurgeon should have a high degree of suspicion to diagnose such an entity among his or her patients presented with pains typical for discopathy.
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Malignant solitary fibrous tumour of orbit p. 55
Hrishikesh Chakrabartty, Shashi Singhvi, Devendra Purohit, Radhey Shyam Mittal
Solitary fibrous tumor (SFT) is a rare neoplasm that is thought to be of mesenchymal origin. Occurrence of such a tumor in the orbit is rare, more so in its malignant form. Histopathologically, it can mimic several other tumors of the orbit and can be differentiated by CD34 positivity. We report a case of malignant transformation of an SFT of the orbit that recurred after 15 years. The differentiating histopathological features with special stress on the importance of CD34 positivity and principles of management are outlined. The need for long-term follow-up to detect recurrence and malignancy is stressed.
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Third ventricular tuberculoma mimicking as a tumor: Report of a very rare case p. 58
Gopal R Sharma, Prashant Kaushal, Bivek Vaidya, Pawan Kumar
Intracranial tuberculoma is a common neurosurgical problem in developing countries; however, intraventricular tuberculoma is a rare entity. Here, we report a rare case of third ventricular tuberculoma in a 21-year-old girl who presented with features of raised intracranial pressure. Radiological findings and management of third ventricular tuberculoma would be discussed and literature regarding such lesions will be reviewed.
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Primary cerebellopontine angle glioblastoma in an adult p. 62
Souvagya Panigrahi, Sudhansu S Mishra, Srikant Das
Supratentorial glioblastoma is the most frequent primary intracranial tumor in adults. Its localization in the cerebellum is extremely rare and such tumor presenting as cerebellopontine angle (CPA) mass in an adult is even rarer. We report the case of primary cerebellar glioblastoma in a 52-year-old woman who presented clinically and radiologically as CPA mass. Its clinical presentation, radiological findings, and treatment instituted are discussed in view of pertinent literature.
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Progressive hemiparesis in a young man: Hemicerebral atrophy as the initial manifestation of basal ganglia germinoma p. 65
Peter Yat Ming Woo, Alberto Chi Ho Chu, Kwong Yau Chan, John Ching Kwong Kwok
Basal ganglia germinomas are rare and patients are classically young Oriental males presenting with gradually progressive hemiparesis. Although early treatment with chemo-radiotherapy can be curative and significantly improve the quality of life, medical attention is often delayed. A young Chinese male experienced a 6-month history of right hemiparesis with magnetic resonance imaging findings of hemicerebral atrophy and lentiform nucleus microhemorrhage, highly suggestive and early signs of basal ganglia germinomas. No further imaging was performed until 2 years later when he was admitted for acute neurological deterioration and a repeat scan revealed a large infiltrative tumor pathologically confirmed to be a pure germinoma.
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Primary CNS vasculitis masquerading as glioblastoma: A case report and review p. 69
Pelluru Pavan Kumar, Alugolu Rajesh, Rukmini Mrudula Kandadai, Aniruddh Kumar Purohit, Challa Sundaram
Isolated angitis of the central nervous system (IACNS)/primary angitis of central nervous system vasculitis (PACNS) is an uncommon vascular disease, sparingly presenting as an isolated inflammatory lesion on magnetic resonance imaging (MRI). The disease usually manifests as a long-drawn and progressive ischemic event. Delay in diagnosis due to focal nature of the lesion also contributes to the poor prognosis as the dismal natural history and immunosuppressive therapy. To date, only a few cases with tumor-like isolated angitis of CNS have been reported with clear and definitive diagnostic workup.
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A rare case of congenital glioblastoma with atypical presentation in an eleven month-old infant: Case report with review of literature p. 72
Vangala Bramha Prasad, Alugolu Rajesh, Aniruddh Kumar Purohit, Megha Shantveer Uppin
Glioblastoma multiforme (GBM) is the most common and malignant primary brain tumor in adults. The occurrence of this entity in infants is a rarity and portends a uniform dismal prognosis and survival in spite of all the latest available management options. The authors herein report a case of a GBM in an 11 month-old infant with tumor involving predominantly the left temporal and frontal regions who expired 10 weeks after tumor decompression. Literature and available management options have been reviewed in the context of the presented case.
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Intraventricular glioblastoma multiforme mimicking meningioma and review of the literature p. 75
Ashis Patnaik, Sudhansu S Mishra, Satya B Senapati
Glioblastoma multiforme (GBM) is the most common primary brain tumor and is usually found in aged persons in the cerebral hemispheres particularly the frontotemporal region. But intraventricular GBM is rare and only few cases have been reported in the literature. We report a case of a 27-year-old man who presented with headache, vision loss in both eyes, and other signs and symptoms of increased intracranial pressure. Computed tomography and magnetic resonance imaging showed an intraventricular, well-circumscribed lesion with homogeneous enhancement of contrast, suggestive of meningioma that is more common than GBM in this location. The patient underwent surgical removal through transcortical route. The final pathologic diagnosis was GBM. We present the clinical features, radiological findings, and surgical management of this case and discuss the pathogenesis and review of the literature of intraventricular GBM.
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Clival giant cell tumor - A rare case report and review of literature with respect to current line of management p. 78
Mohana Rao Patibandla, Amit Kumar Thotakura, Marabathina Nageswara Rao, Gokul Chowdary Addagada, Manisha Chowdary Nukavarapu, Manas Kumar Panigrahi, Shantiveer Uppin, Sundaram Challa, Srinivas Dandamudi
Giant-cell tumor (GCT) involving the skull base is rare. Sphenoid bone is the most commonly involved bone followed by petrous temporal bone. Histopathology and radiological features of these lesions are similar to GCT involving bone elsewhere. Unlike other sites, skull base is not an ideal site for the radical surgery. Hence adjuvant treatment has pivotal role. Radiation therapy with intensity-modulated radiation therapy, stereotactic radiosurgery or chemotherapy with adriamycin are promising as described in some case reports. Bisphosphonates showed good control in local recurrence. In vitro studies with Zolendronate loaded bone cement and phase 2 trials of Denosumab showed hopeful results, may be useful in future.
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Lateral ventricular gliosarcoma with attachment to septum pellucidum p. 82
Pravin Salunke, Harnarayan Singh, Kim Vaiphei
Gliosarcomas are lesions with both glial and sarcomatous elements and are usually seen abutting the dura. Intraventricular location is extremely rare. Such an unusual intraventricular gliosarcoma is being reported.
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Primary intracranial choriocarcinoma presenting as a ring-enhancing lesion: A case report and review of literature p. 85
Mayur Sharma, Vernon Velho, Rachana Binayake, Chandranath Tiwari
We report here a rare case of primary intracranial choriocarcinoma without evidence of tumor elsewhere, presenting as a ring-enhancing lesion managed successfully in our institute (Grant Medical College and Sir J. J. Group of Hospitals, Mumbai, Maharashtra, India). A 22-year-old, right-handed housewife presented with complaints of headache, vomiting, generalized tonic–clonic seizures, and right upper limb weakness of short duration. On neurological examination, the patient was conscious, cooperative, well-oriented in time, place, and person. Visual acuity was 6/9 in both eyes. Fundus was showing grade 1 papilledema. Tone was increased on the right side. Power was decreased in the right upper limb as compared to the left. Reflexes were brisk in the right upper limb. Imaging was suggestive of a thick ring-enhancing lesion in the left parietal region suggestive of glioma or tuberculosis. Complete excision of the lesion was achieved. The diagnosis was confirmed by histopathologic examination of the lesion and β-human chorionic gonadotropin (β-HCG) levels. The patient recovered well postoperatively without neurological deficit. She was relieved of headache completely and there were no further episodes of vomiting or seizures. The right upper limb weakness improved to 4+/5. Choriocarcinoma is the most malignant lesion of all the gestational trophoblastic diseases. Primary brain choriocarcinoma presenting as a ring-enhancing lesion has not been reported in the literature. This diagnosis should be kept in mind, especially in young females of child-bearing age group. Complete surgical excision with adjunctive treatment is required to achieve prolonged remissions.
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Abdominal schwannoma in a case of neurofibromatosis type 2: A report of a rare combination p. 89
Khandkar Ali Kawsar, Md Raziul Haque, Forhad Hossain Chowdhury
Neurofibromatosis-2 (NF2) is an autosomal-dominant disease, which is characterized by vestibular schwannomas (VSs) (acoustic neurinoma) as well as tumours of the peripheral and central nervous system, demonstrating a variety of expression. A 12-year-old girl presented to us with headache and ataxia for four months. We examined and found a lump in the right side of her abdomen. On magnetic resonance imaging (MRI) of brain, a bilateral VS at the cerebellopontine (CP) angle was detected, and on computerized tomography (CT) scan and ultrasonography of her abdomen a large retroperitoneal schwannoma was revealed in the right side of her abdomen. At first, the right-sided CP angle tumour and two months later, the left-sided lesion was operated. After some days, she became mute and incontinent, and was found to have hydrocephalus on CT scan. We introduced a ventriculoperitoneal shunt. Then we operated the abdominal lump, which was histologically proven as schwannoma. The association of these three tumours is rare and untiring surgical approaches made her better. The patient recovered well except bilateral mild facial and vestibulocochlear deficit.
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Bilateral cervico-dorsal gossypiboma presenting as discharging sinus p. 92
Sushil Kumar, Pooja Abbey, Amit Handa
Retained surgical sponge is an uncommon complication after laminectomy. A 67-year-old male presented with a discharging sinus in the cervical region following cervical laminectomy. Magnetic resonance imaging (MRI) revealed bilateral symmetrical lesions in the paraspinal area. Re-exploration revealed gauze pieces on either side under the muscle. Persistent discharging wound after surgery should arouse the possibility of a foreign body. Because of the rarity of bilateral lesions, the present case is being reported.
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Solitary plasmacytoma of temporal bone: A rare case report p. 95
Ritesh Kumar, Narendra Kumar, Sandeep Mohindro, BD Radotra
Solitary plasmacytoma of base of skull is a rare disease. It presents with headache, swelling, and other vague symptoms. Systemic work-up to rule out multiple myeloma is necessary. Radiotherapy is the treatment of choice. We, here, present a case of solitary plasmacytoma of base of skull in right temporal bone in a 50-year-old female treated with surgery and radiotherapy.
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Supratentorial intermediate grade meningeal melanocytoma with intratumoral bleed in the background of neurocutaneous melanosis: Report of an unusual case and review of literature p. 98
Kuntal Das, Anup Nair, Sushila Jaiswal, Rabi Sahu, Arun Srivastava, Raj Kumar, Anant Mehrotra
Primary melanocytic tumours of the central nervous system (CNS) are rare. According to the WHO classification (2007), these tumours include diffuse leptomeningeal melanosis, melanomatosis, melanocytoma, and primary CNS melanoma. Meningeal melanocytoma, most commonly seen in the infratentorial compartment and cervical spinal cord, is a benign primary melanocytic neoplasm. Primary CNS melanoma, on the other hand, represents the malignant end of the spectrum. Intermediate grade melanocytoma is a rare histological subtype of primary meningeal tumours and is characterised by the clinicopathological features between the two extremes. Neurocutaneous melanosis (NCM) is a rare phacomatosis characterised by melanotic lesions on the skin and leptomeninges. Leptomeningeal manifestation in NCM may be observed either in the form of diffuse leptomeningeal melanosis or primary CNS melanoma. Melanocytomas are focal lesions and their association with NCM is extremely rare. In this report, we present an unusual case of NCM accompanied by right frontal intermediate grade melanocytoma with intratumoral bleeding in a 17-year-old boy. A brief literature review is also presented.
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An interesting case of wrongly diagnosed optic neuritis p. 103
Vivek Tandon, Kanwaljeet Garg, Ashok K Mahapatra
Optic neuritis (ON) may rarely mimic optic nerve tumor, index of suspicion should be kept high. A 34-year-old woman presented to a major academic institute with a history of right-sided ocular pain and progressive visual loss in the same eye. Her magnetic resonance imaging showed markedly thickened optic nerve; her workup for inflammatory pathology was negative; she was diagnosed as a case of optic nerve tumor and was planned for surgery. Patient for second opinion came to a tertiary care institute where on proper history taking and evaluation she was diagnosed and treated on the lines of ON and she improved. The diagnosis of ON is a clinical one, it may mimic optic nerve tumor in rare cases.
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Challenges in a case of ophthalmic artery aneurysm associated with abnormal internal carotid arteries p. 106
Eduard B Dinca, Felix Brehar, Andrei Giovani, Alexandru V Ciurea
Ophthalmic artery aneurysms account for 5% of all cerebral aneurysms and are an important cause of morbidity and mortality related to subarachnoid hemorrhage. The diagnosis is often made only when the aneurysm is large enough to become symptomatic. They remain technically challenging for both neurosurgeon and interventional radiologist. We present the case of a 62-year-old woman admitted for transient loss of consciousness, followed by generalized tonic-clonic seizures. Computed tomography (CT) showed a subarachnoid hemorrhage (SAH), clinically graded as Hunt and Hess III. Magnetic resonance imaging (angioMR) and the four-vessel digital subtraction angiography (DSA) identified a ruptured, 8 mm left ophthalmic artery aneurysm. Embolization was the first therapeutic choice. Nevertheless, the attempt had to be aborted due to a combination of a hypoplastic right internal carotid artery (ICA) and an irregular atheromatous plaque on the left ICA, rendering the procedure unduly hazardous. Therefore, microsurgical clipping of the aneurysm became the procedure of choice. Postoperatively, the patient was in good condition, with no visual and neurological deficits. At 6 months follow up, she was assigned maximum scores of 5 and 8 on the Glasgow Outcome Scale (GOS) and Extended GOS (GOS-E), respectively. Aneurysm rupture represents a neurosurgical emergency and an early intervention (less than 48 h) is recommended to maximize the chances of deficit-free survival. The peculiarities of this case consisted in the combination between the size and the location of the aneurysm, abrupt presentation, and the impossibility of embolization due to bilateral ICA abnormalities, congenital (hypoplastic right ICA) and acquired (extensively atherosclerotic left ICA).
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Primary intracranial rhabdomyosarcoma of the cerebellopontine angle mimicking a vestibular schwannoma in a child p. 109
Prakash Nair, Kuntal Kanti Das, Arun K Srivastava, RN Sahu, Raj Kumar, Kamlesh Yadava, Rakesh Pandey
Primary intracranial rhabdomyosarcoma (PIRMS) is a rare neoplasm, which affects infants and young children. We report a rare case of a primary embryonal rhabdomyosarcoma of the cerebellopontine angle in a 7-year-old boy with clinical and radiological features mimicking a vestibular schwannoma. The patient underwent definitive surgery and radiotherapy and is recurrence free at 6 months. PIRMS commonly occur in parameningeal locations or metastasize to the brain from an extracranial primary. Gross total tumor resection followed by adjuvant radiotherapy remains the current standard of treatment; however, chemotherapy has also been tried with favorable results. The overall prognosis continues to remain poor.
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Alveolar soft part sarcoma with brain metastases p. 112
Vinodh Vayara Perumall, Rahmat Harun, Pulivendhan Sellamuthu, Mohd Shariman Md Shah
Metastatic tumors are the most common mass lesions in the brain. This case reports a rare form of sarcoma with metastasis to the brain. The appropriate management of a patient with metastatic alveolar soft part sarcoma to the brain is discussed. Author describes a 32-year-old gentleman diagnosed with primary tumor at gluteus and distant metastases at lower lobe of right lung and the brain. Histopathology proves diagnosis as alveolar soft part sarcoma. Craniotomy with excision of brain lesion was done. Repeated magnetic resonance imaging of the brain after 2 months showed rapidly growing new lesions. The next step of management was made by the oncology team as recurrence rate was high and due to multi-systemic involvement. Patient was planned for palliative chemotherapy and to be reassessed later. This case report discusses the appropriate approach to any form of brain metastases and the role of early follow-up especially after surgery for better outcome and choice of post operative management such as radiotherapy or chemotherapy or both for malignant tumors. Based on this report, it was concluded that every brain tumor patient should be frequently monitored even in the outpatient setting as most of them are metastatic and rapidly spreading. The patient should be considered for radiotherapy or chemotherapy or both after surgery if the histopathology result is suggestive of malignancy.
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Trigeminocardiac reflex preceding development of postoperative superior orbital fissure syndrome p. 116
Rudrashish Haldar, Prakhar Gyanesh, Arun Srivastava, Guru Prasad Bettaswamy
The superior orbital fissure syndrome (SOFS) is a rare condition presenting as painful unilateral opthalmoplegia, ptosis, pupillary dilatation and anesthesia of the upper eyelid and forehead. It has been described after facial trauma. Other etiologies include infections, tumors or spontaneous hemorrhages in the retro-orbital space. Occurrence of SOFS after surgery in the skull base has not been described before. We recently encountered a case where a young female developed SOFS following surgery for cerebrospinal fluid (CSF) rhinorrhea. The occurrence of SOFS was preceded by episodes of trigemino-cardiac reflex (TCR) during the surgery. This is the first case report associating TCR with SOFS. We managed the case report with megadose methylprednisolone and the patient showed improvement in the deficit over time.
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Excision of hypoglossal neurinoma by condyle sparing suboccipital keyhole approach p. 120
Harjinder Bhatoe
Hypoglossal neurinomas are rare tumors that present with gradually worsening unilateral wasting of the tongue. The tumors often attain large size prior to detection and often extend into the hypoglossal canal. Large tumors can distort the brainstem and cerebellum. Preoperative diagnosis can often be made by magnetic resonance imaging (MRI) with contrast enhancement. Traditionally, surgery for these tumors has entailed large transcondylar approaches, often necessitating occipitocervical stabilization. We managed one 23-year-old female patient with a large hypoglossal neurinoma. The tumor could be excised completely by a condyle-sparing minimally invasive suboccipital keyhole approach.
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Confounding features of ectopic craniopharyngioma: A differential for tumors showing diffusion restriction p. 123
Sudha Karnan, Vanitha Krishnamoorthy, Kailasanathan Natarajan, Babu Peter Sathyanathan
Craniopharyngiomas comprise approximately 1-3% of all intracranial tumors and arise from squamous epithelial rests along remnants of Rathke's cleft. They have bimodal age distribution. The ectopic sites reported are the nasopharynx, sphenoid bone, pineal region, cerebellopontine angle and third ventricle. We report a case of ectopic craniopharyngioma in the Foramen of Monro with restricted diffusion, which have is an unusual feature, not commonly reported and is a potential pitfall.
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Dissecting intracranial aneurysm in pregnancy: A rare association p. 127
Manasi Mehrotra, Anant Mehrotra, Anup Nair, Arun Srivastava, Rabi Narayan Sahu, Mandakini Pradhan, Raj Kumar
We report a case of dissecting aneurysm of the right posterior cerebral artery presenting with sudden onset headache and altered behavior during labor. A 26-year-old P1001 with uncomplicated antenatal period, except history of headache off and on since 32 weeks of pregnancy, developed sudden onset headache and altered behavior during 3rd stage of labor. She had vaginal delivery and a live born male baby was delivered with good Apgar score. The CT was suggestive of subarachnoid hemorrhage and DSA was suggestive of dissecting aneurysm of the right P2 segment with hypoplasia of right P1 segment and A1 segment of posterior and anterior cerebral artery, respectively. The patient was managed conservatively. At 4-month follow-up, the patient was doing well. We discuss about the rare association of a dissecting aneurysm and pregnancy, especially dissection of the posterior cerebral artery.
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Totally cystic intradural schwannoma in thoracic region p. 131
Sushil Kumar, Raghavendra Gupta, Amit Handa, Rohan Sinha
Spinal schwannomas are benign intradural extramedullary tumors arising from spinal nerve root sheath. They are usually solid or heterogeneously solid. Totally cystic schwannomas are rare entities. Herein, we report a 60-year-old male presenting with backache radiating along the chest wall and weakness of both lower limbs. He had spastic paraparesis. Magnetic resonance imaging revealed a cystic mass in the thoracic region. At operation, the cystic mass was seen to be attached to D4 dorsal rootlets. It was excised in toto and histopathology confirmed it to be a schwannoma. The relevant literature is reviewed.
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C2 Primary leiomyoma in an immunocompetent woman: A case report and review of literature p. 134
Mohana Rao Patibandla, Madhukar T Nayak, AK Purohit, Megha Uppin, Sundaram Challa, Gokul Chowdary Addagada, Manisha Nukavarapu
Clinical case report and review of the literature. This is the first case of primary leiomyoma in an immunocompetent woman without previous history of uterine leiomyoma being reported in the literature to the best of our knowledge.Leiomyoma, a type of smooth muscle cell tumor, involving the vertebra is extremely rare. There were very few primary leiomyoma in patients with AIDS or in the immune-suppressed patients.This 48-year-old female came with H/o neck pain, weakness and bladder retention. On examination, tone increased in all four limbs, power on the right side of the limbs 4/5, power on the left upper limb 0/5, lower limb 3/5, left plantar was up going, decreased sensation over the left second cervical vertebra (C2) dermatome and all modalities decreased below C2. X-ray and magnetic resonance imaging (MRI) of the cervical spine showed kyphosis of the cervical spine with destruction of the C2 vertebral body along with pathological fracture. The patient underwent decompression of the C2 lesion through the C2 right pedicle with occipito-C1-C3 lateral mass screws fixation. Lesion anterior to the cord was reached by a transpedicular approach and decompression was performed.The lesion was pinkish grey, firm and moderately vascular and was destroying the C2 vertebral body. The patient improved symptomatically in power in the left upper limb and lower limb over the next 1 week duration from 0/5 to 4+/5. Histopathology revealed primary leiomyoma. The patient was evaluated with ultrasound abdomen and contrast tomogram of the chest, abdomen and pelvis to rule out other possible lesions in the lung, intestines and uterus.We suggest that leiomyoma should be included in the differential diagnosis of destructive lytic lesions involving the C2 vertebra. Histopathological examination with immunohistochemistry is necessary for the definitive diagnosis. Treatment of choice is surgery with complete removal.
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Choroid plexus papilloma p. 139
Divya Sethi, Rashmi Arora, Ketan Garg, Parul Tanwar
Choroid plexus tumors are rare intracranial tumors which account for only 0.4-0.6% of all brain tumors. These are intraventricular papillary neoplasms derived from choroid plexus epithelium and range from choroid plexus papillomas (World Health Organisation (WHO) grade I) to choroid plexus carcinomas (WHO grade III). It is an important albeit rare cause of hydrocephalous. We present to you the case of a 1-year-old child who presented with the signs and symptoms of hydrocephalous and was diagnosed as choroid plexus papilloma on histopathology.
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Intracranial hemorrhage from giant aneurysm in pregnancy: A rare association p. 142
Manasi Mehrotra, Anant Mehrotra, Anup Nair, Arun Srivastava, Rabi Narayan Sahu, Mandakini Pradhan, Raj Kumar
We report a case of giant aneurysm causing subarachnoid hemorrhage in a pregnant female. A 25-year-old female presented with sudden onset of severe headache and vomiting with altered sensorium and right hemiplegia. On investigation, she had a giant supraclinoid segment internal carotid artery (ICA) aneurysm. She was planned for digital substraction angiography, but during the procedure she deteriorated neurologically and went into spontaneous labor. The baby was a male child with weight of 1.1 kg. She was taken up for surgery and aneurysm was clipped. We discuss the rare occurrence of intracranial hemorrhage in pregnancy due to a giant ICA aneurysm.
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Occipital condyle syndrome as the first sign of skull metastasis from lung cancer p. 145
Satoru Takeuchi, Hideo Osada, Kimihiro Nagatani, Katsuji Shima
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Economic and outcome following severe head injury p. 147
Somsri Wiwanitkit, Viroj Wiwanitkit
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Pneumocephalus is not a serious outcome after surgery for chronic SDH p. 148
Pratyush Chaudhuri
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Severe hypotension in transforaminal lumbar interbody fusion surgery: Is it vasovagal or? p. 149
Tumul Chowdhury, Harsh Sapra, Sudhir Dubey
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Extensive traumatic thalamic contusions in a child p. 151
Amit Agrawal, Amit Mittal, GB Kohali, Sunil Sampley, Satendra Singh
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