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ABSTRACT: Dysembryoplastic neuroepithelial tumor (DNT) is a relatively newly described entity and is an important cause of intractable
epilepsy. We report 32 cases of DNT who were operated and treated in our hospital over a period of 12years. Immunostaining
for various proliferative markers and tumor suppressor gene proteins was done to assess the proliferative potential of these
tumors. The most common presentation was partial complex seizures followed by generalized tonic–clonic seizures, focal motor
seizures, and myoclonus. The most common location was temporal lobe followed by frontal and in one patient lesion was multifocal.
All patients were seizure free at the last postoperative follow-up which varies from 12 to 96months with mean of 33.7months.
Microscopic examination showed classical histology comprising of intracortical multinodular microcystic lesions with floating
neurons. Proliferative indices were very low (<1%) and tumor suppressor gene protein expression was not seen in the present
study. Cortical dysplasia of the surrounding brain was observed in 37.3% of cases.
Neurosurgical Review 04/2012; 32(2):161-170. · 2.04 Impact Factor
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Acta Neurochirurgica 02/2012; 154(4):665-6. · 1.52 Impact Factor
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ABSTRACT: Relatively few studies have been performed on molecular properties of pediatric glioblastoma multiforme (GBM). Methylation of DNA repair gene O(6)-methylguanine-DNA methyltransferase (MGMT) promoter region has been associated with favorable prognosis and prolonged survival in adult GBM patients treated with temozolomide (TMZ). We explored the frequency of MGMT gene promoter methylation in pediatric glioblastomas and compared it with the known molecular alterations in p53.
Twenty pediatric GBM cases were selected. MGMT promoter methylation was assessed by methylation specific PCR. p53 expression was determined by immunohistochemistry.
MGMT gene promoter methylation was observed in 50% of pediatric glioblastomas. p53 protein expression was detected in 60% of cases. Seventy percent of cases with methylated MGMT promoter were p53 immunopositive.
The frequency of MGMT gene promoter methylation in pediatric GBMs was similar to adult GBM patients. The pediatric GBMs should also be investigated for MGMT promoter methylation to identify a subset of patients likely to benefit from TMZ therapy. p53 protein overexpression was more common in pediatric primary GBMs. To the best of our knowledge this is only the second study on MGMT gene promoter methylation status in pediatric GBMs.
Child s Nervous System 11/2010; 26(11):1613-8. · 1.54 Impact Factor
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Prerana Jha,
Shipra Agarwal,
Pankaj Pathak,
Arti Srivastava,
Vaishali Suri,
Mehar Chand Sharma,
Kunzang Chosdol,
Tapasya Srivastava,
Deepak Gupta, Aditya Gupta,
Ashish Suri,
Chitra Sarkar
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ABSTRACT: There are few reports of loss of heterozygosity (LOH) of 1p and 19q in astrocytic tumors, especially glioblastoma multiforme (GBM). We evaluated 1p and 19q (either or both) heterozygosity status in 71 astrocytomas, including 6 pediatric cases: 20 diffuse astrocytomas (DA), 9 anaplastic astrocytomas (AA), and 42 GBM. In the GBMs, p53 protein expression was assessed by immunohistochemistry and epidermal growth factor receptor (EGFR) gene amplification by fluorescence in situ hybridization; TP53 sequencing was done in 15 of the GBMs. In adults, LOH of 1p or 19q was detected in 16% of DAs and 50% of GBMs; none of the AAs showed this alteration. In GBMs, LOH of 19q was most common (26%), followed by combined 1p and 19q LOH (13%) and 1p LOH (10%). Pediatric GBMs also harbored isolated 1p and 19q LOH (50%). Notably, LOH of 1p or 19q LOH was more frequent in p53 immunopositive secondary GBMs (61%) than in primary GBMs (17%). This suggests that LOH of 1p and 19q may be acquired during progression to secondary GBMs. Thus, 1p and 19q LOH can occur in astrocytic tumors, most commonly in secondary GBMs without morphological correlation with an oligodendroglial histology. The clinical significance of recognition of this subset of GBMs is based on several recent reports of association with better prognosis, although long-term follow-up studies are required.
Cancer genetics and cytogenetics 04/2010; 198(2):126-34. · 1.54 Impact Factor
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ABSTRACT: Oculomotor nerve palsy (ONP) can be the presenting feature of ruptured or an unruptured posterior communicating artery (PcomA) aneurysm. Etiopathogenesis and recovery of ONP following treatment of PcomA aneurysm has been a subject of controversy. Case records of thirteen patients (mean age 42 years (range 19-65 years), M:F: 3:10) with PcomA aneurysm and ONP who underwent surgery over a period of eight years were analyzed. Twelve patients presented with subarchnoid hemorrhage and one had unruptured aneurysm. The interval between the onset of symptoms and surgery ranged between 4 and 70 days (mean 16.7 days). All the four patients with partial ONP had complete recovery and of the nine patients with complete ONP, six had complete recovery and three had partial recovery. The recovery of the ONP is influenced by the degree of preoperative deficit. Although clipping of the aneurysm probably expedites the recovery of the third nerve palsy, age, sex and timing of surgery may not have any influence on the recovery patterns.
Neurology India. 01/2010;
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Pediatric Neurosurgery 01/2010; 46(4):324-5. · 0.70 Impact Factor
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ABSTRACT: Ophthalmic segment aneurysms account for about 5% of all intracranial aneurysms. Anatomical complexity of the paraclinoid region makes surgical management of aneurysms arising from the ophthalmic segment challenging. This study was carried out to assess the presenting features, complications and outcomes after surgical treatment of ophthalmic segment aneurysms. The authors retrospectively analysed the clinical records of patients with ophthalmic aneurysms treated at our Institute from January 2001 to September 2008, which constituted about 9% (78/850) of all intracranial aneurysms. Of the 78 ophthalmic segment aneurysms, six patients (8%) had giant aneurysms and 19 (24%) patients had multiple aneurysms. Fifty-six patients underwent microsurgery, with direct clipping in most. The mean age was 42 years (range 12-75 years) and the mean follow-up was 8 months (range, 2-93 months). A good outcome was achieved in 46 (83%) patients (Glasgow Outcome Scale [GOS] score 4-5) and 17% had a poor outcome (GOS score 1-3) at last follow-up. The overall complication rate was 21% (12/56), most of which were transient complications, with 3.5% (2/56) mortality. Direct microsurgical clipping remains our preferred treatment approach, whenever possible, for ophthalmic segment aneurysms. This surgery has an acceptable complication rate and leads to a good outcome in more than 80% of patients with ophthalmic aneurysms. Use of modern microsurgical instrumentation and endovascular adjuncts can further reduce the surgical morbidity associated with these vascular lesions.
Journal of Clinical Neuroscience 12/2009; 17(1):38-42. · 1.25 Impact Factor
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ABSTRACT: The objective of this retrospective study was to study the outcome in patients with basal ganglia, thalamus and brainstem (central/deep) arteriovenous malformations (AVMs) treated with gamma knife radiosurgery (GKS) and to compare the results with that for AVMs at other intracranial locations.
The results of 53 patients with central AVMs and 255 patients with AVMs at other locations treated with GKS at our center between April 1997 and March 2005 with minimum follow-up of 1 year were analyzed. CENTRAL AVMS: Forty of these 53 AVMs were Spetzler-Martin grade III, 11 were grade IV, and 2 were grade V. The mean AVM volume was 4.3 cm(3) (range 0.1-36.6 cm(3)). The mean marginal dose given was 23.3 Gy (range 16-25 Gy). The mean follow-up was 28 months (range 12-96 months). Check angiograms were advised at 2 years after GKS and yearly thereafter in the presence of residual AVM till 4 years. Presence of a residual AVM on an angiogram at 4 years after radiosurgery was considered as radiosurgical failure. Complete obliteration of the AVM was documented in 14 (74%) of the 19 patients with complete angiographic follow-up. Significantly lower obliteration rates (37% vs. 100%) were seen in larger AVMs (>3 cm(3)) and AVMs of higher (IV and V) Spetzler-Martin grades (28% vs. 100%). The 3- and 4-year actuarial rates of nidus obliteration were 68% and 74%, respectively. Eight patients (15%) developed radiation edema with a statistically significantly higher incidence in patients with AVM volume >3 cm(3) and in patients with Spetzler-Martin grade IV and V AVMs. Five patients (9.4%) had hemorrhage in the period of latency. COMPARISON OF RESULTS WITH AVMS AT OTHER LOCATIONS: Patients with central AVMs presented at a younger age (mean age 22.7 years vs. 29 years), with a very high proportion (81% vs. 63%) presenting with hemorrhage. Significantly higher incidence of radiation edema (15% vs. 5%) and lower obliteration rates (74% vs. 93%) were seen in patients with central AVMs.
GKS is an effective modality of treatment for central AVMs, though relatively lower obliteration rates and higher complication rates are seen compared to AVMs at other locations.
Acta Neurochirurgica 05/2009; 151(12):1575-82. · 1.52 Impact Factor
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ABSTRACT: Although the effects of Gamma Knife surgery (GKS) on the risk of hemorrhage are poorly understood, a certain subset of patients does suffer bleeding after GKS. This study was undertaken to analyze the outcome of patients sustaining hemorrhage after GKS; it is the most feared complication of radiosurgical management of cerebral arteriovenous malformations (AVMs).
Between May 1997 and June 2006, 494 cerebral AVMs in 489 patients were treated using a Leksell Gamma Knife Model B, and follow-up evaluations were conducted until June 2007 at the All India Institute of Medical Sciences in New Delhi. Fourteen patients who sustained a hemorrhage after GKS formed the study group. In most of these patients conservative management was chosen.
The mortality rate was 0% and there was a 7% risk of sustaining a severe deficit following rebleeding after GKS. None of the patients sustained rebleeding after complete obliteration. Patients with Spetzler-Martin Grade III or less had increased chances of hemorrhage after GKS (p < 0.002). The presence of deep venous drainage, aneurysm, venous hypertension, or periventricular location on angiography was common in patients with hemorrhage after GKS.
The risk of hemorrhage that remains following GKS for cerebral AVMs is highest in the 1st year after treatment. The present study showed a relatively good outcome even in cases with hemorrhage following GKS, with no deaths and minimal morbidity, further substantiating the safety and efficacy of the procedure.
Journal of Neurosurgery 01/2009; 110(5):1003-9. · 2.96 Impact Factor
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ABSTRACT: The natural history of giant intracranial aneurysms is generally morbid. Mortality and morbidity associated with giant aneurysms is also higher than for smaller aneurysms. This study was carried out to assess the demographic profile, presenting features, complications, and outcome after surgical treatment of giant intracranial aneurysms.
A retrospective review of the medical records of all patients with giant intracranial aneurysms treated in the Department of Neurosurgery, Neurosciences Centre, All India Institute of Medical Sciences, New Delhi, from January 1995 through June 2007 was performed. The demographic profiles, presenting features, radiological findings, surgical treatments, and outcomes were assessed.
A total of 1412 patients harboring 1675 aneurysms were treated. Out of these, 222 patients had 229 (13.7%) giant aneurysms, and of those, 181 aneurysms in 177 patients were managed surgically while 48 were treated with endovascular therapy. In the patients treated with surgery, common clinical presentations included subarachnoid hemorrhage (SAH) in 110 (62%) cases followed by mass effect in 57 (32%) cases. In patients who presented with SAH, the Hunt and Hess SAH grading was: grade I in 43 (39%), grade II in 40 (36%), grade III in 23 (21%), grade IV in two (2%), and grade V in 2 (2%) patients. One hundred and seven aneurysms (in 103 patients) were treated using direct surgical clipping. Forty-six patients with good collateral circulation were treated by gradual occlusion and ligation of the internal carotid artery (ICA) in the neck with a Silverstone clamp. Another nine patients with good collateral circulation, but persisting symptoms after ICA ligation, required trapping for obliteration of the aneurysm. Eleven patients with poor collateral circulation required extracranial-intracranial (EC-IC) bypass before proximal ICA ligation. A post-operative digital subtraction angiography (DSA) was performed in 118 patients and revealed well-obliterated aneurysm in 106 patients. The total treatment mortality rate was 9%. In the last 5 years, 117 patients were operated on with four operative deaths. Overall, the outcome was excellent in 131 (74.0%), good in 22 (12.4%), and poor in eight (4.5%) cases.
It is concluded that 14% of all intracranial aneurysms are giant. The most common clinical presentation is SAH followed by features of an intracranial mass lesion. The cavernous ICA is the most common portion of the ICA affected. Direct surgical clipping is a safe and effective method of treatment and should be considered the first line of treatment whenever possible. With proper case selection, optimal radiological evaluation, and appropriate surgical strategy, it is possible to achieve a favorable outcome in almost 90% of the cases.
Clinical Neurology and Neurosurgery 08/2008; 110(7):674-81. · 1.58 Impact Factor
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ABSTRACT: Seizures and psychosis coexist in a large number of patients with epilepsy, and a significant amount of research on their relationship has been published. There are several reports and reviews on postictal and interictal psychosis in patients with epilepsy. We describe three patients with refractory temporal lobe epilepsy, each of whom presented with a history of episodic psychosis that preceded almost all habitual seizures and, thus, served as a useful warning symptom. All three patients had intractable left complex partial seizures; two had right mesial temporal sclerosis, and the third had a gliotic area in the right frontotemporal region on MRI. This is the first report of psychosis preceding seizures. The literature on seizure anticipation, as well as on the complex relationship between seizures and psychosis, is also reviewed.
Epilepsy & Behavior 08/2008; 13(1):252-5. · 2.34 Impact Factor
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ABSTRACT: Radiosurgery has been widely adopted for the treatment of cerebral AVMs. However radiosurgical treatment of patients with hemorrhagic presentation is fraught with risk of rebleed during latency period. The present study intends to analyze the obliteration rate, time to obliteration and chances of rebleed in patients with hemorrhagic versus non-hemorrhagic clinical presentation in cerebral arteriovenous malformations (AVMs) treated with gamma knife radiosurgery (GKS).
Of all the patients with cerebral AVMs treated from May 1997 to June 2006, 157 patients with neuroimaging follow up with digital subtraction angiography harboring 160 AVM nidii formed the study group. The mean age of presentation was 28 years (range, 6-58 years); mean nidus volume being 3.64 cm3 (range, 0.011-36.6 cm3). The mean follow up period was 70 months (range, 13-121 months). All the patients were treated predominantly by primary GKS with use of adjunctive pre-GKS embolization in selected patients.
A total of 103 (64%) patients presented with hemorrhage. There was no difference in the obliteration rate (69% versus 67%, p=0.672), mean latency period to obliteration (30 months versus 32 months, p=0.1989) and chances of hemorrhage (4.8% versus 3.5%, p=0.690) in patients with hemorrhagic as compared to non-hemorrhagic presentation.
Prior hemorrhage does not affect the outcome after GKS in terms of obliteration rate, latency to obliteration as well as chances of hemorrhage during latency period. Gamma knife appears equally efficacious irrespective of the mode of clinical presentation in the management of cerebral AVMs; a concomitant use of pre-GKS embolization/surgery may be needed in patients with hemorrhagic presentation in selected cases, however.
Clinical Neurology and Neurosurgery 07/2008; 110(8):804-9. · 1.58 Impact Factor
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ABSTRACT: Myxopapillary ependymoma rarely presents as a primary intra-sacral lesion and extensive bony destruction is unusual. Radiological features do not help in distinction from other commoner sacral tumors, like chordoma. Hence, histopathology serves as the best diagnostic modality for this differentiation. We report the case of a 42-year-old man with a primary intra-sacral myxopapillary ependymoma causing extensive osteolysis.
Journal of Neuro-Oncology 03/2008; 86(3):349-52. · 3.21 Impact Factor
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ABSTRACT: This retrospective study was designed to study the outcome in children with intracranial arteriovenous malformations (AVMs) treated with Gamma Knife surgery (GKS).
One hundred and forty-two children were treated with GKS at the authors' institution between April 1997 and March 2006; of these, 103 patients with a mean follow-up of 26.4 months (range 6-96 months) were included. The mean age at presentation was 13.9 years (range 3-18 years). Eighty-six (83%) patients presented with hemorrhage. In 57 children the AVMs were Spetzler-Martin Grade I or II, and in 46 the AVMs were Grades III, IV, or V. The mean volume of the AVMs was 2.4 ml (range 0.04-23.3 ml). The mean marginal dose administered was 24.4 Gy (range 15-27 Gy). Follow-up angiography was advised at 2 years after GKS and yearly thereafter. In patients with residual AVMs, follow-up angiography was advised yearly until 4 years after GKS. If residual AVM was present, even on a follow-up angiogram obtained 4 years postsurgery, the GKS was considered a failure.
Complete obliteration of the AVM was documented in 34 (87%) of the 39 patients with complete angiographic follow-up. The 3- and 4-year actuarial rates of nidus obliteration were 66 and 86% respectively. Three patients (2.9%) experienced bleeding during the latency period, and symptomatic radiation-induced edema was noted in four patients (3.8%). A significantly higher incidence of radiation edema was noted in patients with AVM volumes greater than 3 ml and in patients with Spetzler-Martin Grade IV and V AVMs.
Gamma Knife radiosurgery is an effective modality for the treatment of intracranial AVMs in children, yielding high obliteration rates and low complication rates.
Journal of Neurosurgery 01/2008; 107(6 Suppl):479-84. · 2.96 Impact Factor
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Manjari Tripathi,
Mahendra Singh,
M Padma,
Shailesh Gaikwad,
C Bal,
Madhavi Tripathi,
C Sarkar, Aditya Gupta,
Garima Shukla,
V Singh,
Satish Jain,
Bhawani Sharma,
P Chandra
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ABSTRACT: Background: There has been sparse description of cortical dysplasias (CDs) causing intractable epilepsy from India. Aim: Clinical retrospective study of CDs causing intractable epilepsy that underwent surgery. Materials and Methods: Fifty-seven cases of CDs reviewed (1995 till July 2006) are presented. All patients had intractable epilepsy, and underwent a complete epilepsy surgery workup (inter ictal electroencephalography (EEG), video EEG, MRI as per epilepsy protocol, SPECT {interictal, ictal with subtraction and co-registration when required}, and PET when necessary). Surgical treatment included a wide exposure of the pathology with a detailed electrocorticography under optimal anesthetic conditions. Mapping of the sensori-motor area was performed where indicated. Procedures included resection either alone or combined with multiple subpial transactions when extending into the eloquent areas. Results: Our study had 28 (49.12%) cases of isolated focal CDs, and 29 (50.67%) with dual pathology. Average age at the time of onset of seizures in our series was 7.04 years (three months to 24 years), and average age at the time of surgery was 10.97 years (eight months to 45 years). Among coexistent pathologies, one had associated MTS, 16 had coexistent gangliogliomas and 12 (dysembryonic neuroepithelial tumor) DNTs. At an average follow-up of 3.035 years (range 5-10 years), three patients were lost to follow-up. Fifty-one per cent (29/57) patients had a good outcome (Engel Grade I) and 26%(15/57) had a Grade II outcome. Conclusion: Cortical dysplasias have a good outcome if evaluated and managed with concordant electrical and imaging modalities.
Neurology India. 01/2008;
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ABSTRACT: Meningeal melanocytomas are uncommon intracranial tumors and their occurrence at the cerebellopontine angle (CPA) is extremely rare. The authors describe the case of a 58-year-old woman who presented with a left CPA tumor; on the basis of histopathological studies after resection, a diagnosis of meningeal melanocytoma was reached. The relevant literature is reviewed.
Journal of Neurosurgery 07/2007; 106(6):1094-7. · 2.96 Impact Factor
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ABSTRACT: Intracranial aneurysms are rare in children and have different epidemiological, clinical, and morphological characteristics and outcome from those in adults.
We analyzed demographic, clinical, radiologic features, treatment, and outcome in 55 patients <18 years of age, treated from Jan 1995 through December 2005.
Intracranial aneurysms in children below 18 years constituted 4% of all intracranial aneurysms. Internal Carotid artery (ICA) bifurcation was the commonest location. About half of the aneurysms were complex. Three-fourth of the patients required surgical treatment. Two patients died, constituting 5% mortality. Two patients (5%) had poor outcome, whereas the majority (90%) had a favorable outcome.
Pediatric aneurysms have male predominance, higher incidence of clinical features of mass effect or seizures, high incidence of large, traumatic/mycotic aneurysms, associated illnesses and ICH/IVH and hydrocephalus, better Hunt and Hess grades at presentation, ICA bifurcation as the commonest site, and better outcome than their adult counterparts.
Child s Nervous System 03/2007; 23(3):327-33. · 1.54 Impact Factor
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Chitra Sarkar,
Mehar Chand Sharma,
Prabal Deb,
V P Singh,
P Sarat Chandra, Aditya Gupta,
Manjari Tripathi,
Manvir Bhatia,
Shailesh Gaikwad,
C S Bal,
Satish Jain
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ABSTRACT: Surgical management of intractable epilepsies is currently an established mode of therapy in various clinical settings.
To retrospectively evaluate the neuropathological findings in both temporal and extratemporal lobe resections in such patients.
The study included resected specimens from patients with intractable epilepsy managed at a tertiary care hospital of India, during a 10-year period (1995-2004).
A total of 153 patients, with mean age of 19.4 years and male predominance (73.2%) were included in the study. Overall, there was a predilection for the temporal lobe (73.2%), while 41cases were extratemporal in location. On histopathology, mesial temporal sclerosis (MTS) (24.8%) was the commonest lesion, followed by tumors (19.6%) and isolated focal cortical dysplasia (FCD - 15.11%). Other less common findings included Rasmussen encephalitis, non-specific gliosis and vascular malformations. In addition, 20.9% (32 cases) had dual lesions, majority of which included FCD with ganglioglioma (15 cases) or with dysembryoplastic neuroepithelial tumor (12 cases). In the temporal lobe, neoplasms and dual lesions formed the majority (apart from MTS), unlike dual lesions followed by neoplasms and FCD, in the extratemporal location.
This series demonstrates that most patients with chronic intractable epilepsy have significant histopathological findings and highlights the neuropathological spectrum of such lesions, in the Indian context. This was similar to that reported from the West, but different from the single Indian series available in the literature. Further, the overall profile of temporal lobe lesions was not different from the extratemporal ones.
Neurology India 07/2006; 54(2):144-50; discussion 150-1. · 0.96 Impact Factor
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Journal of Neuro-Oncology 06/2006; 78(1):47-8. · 3.21 Impact Factor
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ABSTRACT: Meningioangiomatosis (MA) is a rare lesion, probably of malformative origin, consisting of meningovascular proliferation and leptomeningeal calcification. Patients with MA usually present with seizures or persistent headaches. Neurofibromatosis may be associated in a variable proportion of patients, while in others it may be sporadic. Surgical treatment is usually recommended, and is gratifying in most cases. Rarely, MA has been described coexisting with meningiomas, arteriovenous malformations, encephaloceles, oligodendrogliomas, meningeal haemangiopericytomas and orbital erosion. Among these, meningiomatosis with meningioma is the most frequent combination.
We report a case of MA with meningioma in an 18-month-old girl, who presented with recurrent seizures.
In these situations, it is extremely important for the pathologist to be aware of this entity and to distinguish it from other lesions, like cortical invasion by a meningioma, intraparenchymal meningioma and intracerebral schwannoma, which it may mimic.
Child s Nervous System 02/2006; 22(1):78-83. · 1.54 Impact Factor
