This review traces the evolution of epilepsy surgery in India from the beginning to the present state. During the last one and half decades, surgical treatment of epilepsies has made resurgence in this country and at present a few centers have very active and sustained epilepsy surgery programs. Within a 14-year period, the R. Madhavan Nayar Center for Comprehensive Epilepsy Care, Trivandrum, has undertaken over 1000 epilepsy surgeries. However, in the whole country, annually, not more than 200 epilepsy surgeries are currently being performed. This number is a miniscule when compared to the number of potential surgical candidates among the vast population of India. The enormous surgical treatment gap can only be minimized by developing many more epilepsy surgery centers in different parts of our country.

Endemic skeletal fluorosis is widely prevalent in India and is a major public health problem. The first ever report of endemic skeletal fluorosis and neurological manifestation was from Prakasam district in Andhra Pradesh in the year 1937. Epidemiological and experimental studies in the endemic areas suggest the role of temperate climate, hard physical labor, nutritional status, presence of abnormal concentrations of trace elements like strontium, uranium, silica in water supplies, high fluoride levels in foods and presence of kidney disease in the development of skeletal fluorosis. Neurological complications of endemic skeletal fluorosis, namely radiculopathy, myelopathy or both are mechanical in nature and till date the evidence for direct neurotoxicity of fluoride is lacking. Prevention of the disease should be the aim, knowing the pathogenesis of fluorosis. Surgery has a limited role in alleviating the neurological disability and should be tailored to the individual based on the imaging findings.

In the October of 1996, Lance Armstrong, celebrated cyclist and one of the greatest athletes the world has ever seen, at the age of 24, was diagnosed with metastatic testicular cancer with disease having already spread to his abdomen, lungs and brain. Lance underwent four cycles of chemotherapy, actually the pretty standard one, pioneered at the Indiana University and not only did he get completely cured of his cancer, he remains extremely well till date, 12 years later. He sure did have a few adverse effects during those cycles of chemotherapy in the form of nausea, vomiting, weakness and fall in blood count but he knew and experienced them only for a short transient time and emerged triumphant and strong. In fact, he went on to win six awe-inspiring and incredible successive Tours de France victories from 1999-2005, one of the most grueling sporting events testing the endurance of the very fittest. After his retirement, he has been so inspired that he has completely devoted himself to educate people about the common myths about cancer, and promised to raise awareness and generate money for furthering research into surgery, radiation therapy and chemotherapy for cancer through his foundation. He says "I am indebted to the doctors, nurses and medicine and would want to pay them back for all their energy and caring." In his successful journey of overcoming cancer, he captures the essence of its treatment so well by declaring "Pain is temporary, it may last a minute, or an hour, or a day, or a year, but eventually, it will subside and something else will take its place. If I quit, however, it will last forever".

Thrombolytic stroke therapy is based on the "re-canalization hypothesis," i.e., that reopening of occluded vessels improves clinical outcome in acute ischemic stroke through regional reperfusion and salvage of threatened tissues. Re-canalization is an important predictor of stroke outcome in all the modalities of thrombolysis. Thrombolysis in acute ischemic stroke evolved from clinical trials with intravenous (IV) tissue plasminogen activator (tPA) to combination treatments with Intra-arterial (IA)/mechanical reperfusion techniques. The combined approach reduces time to initiation of treatment and may increase re-canalization and chances of a good clinical outcome. In IV thrombolysis overall re-canalization rate is 46.2% during the first 6-24 hrs. Intra-arterial thrombolysis has higher early re-canalization rate, 63.2%. The highest re-canalization rate is seen with mechanical thrombolysis, 83.6%. Other factors in addition to re-canalization: stroke severity, older age, systolic hypertension, location of arterial occlusion, collateral blood supply, and time from stroke onset to treatment play an important role in determining the clinical outcome following thrombolysis with tPA. Future trials should look at the interplay of various factors like re-canalization, reperfusion, and collateral flow and clot burden in the evaluation of reperfusion therapies in acute ischemic stroke.

Purpose: To detect the expressions of Fas/DcR3 and to investigate the cytotoxic effects of RGD-FasL on pituitary adenoma cells. Materials and Methods: Fas/DcR3 mRNAs were detected by Reverse transcription polymerase chain reaction (RT-PCR) and their surface expressions were measured by flow cytometry. Cytotoxicities exerted by FasL and newly-constructed RGD-FasL on tumor cells were measured with 3-(4,5-Dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide (MTT) assay. The apoptotic cells were examined by electron microscopy and the induced apoptosis was determined by agarose gel electrophoresis. The cell cycle was assessed by flow cytometry with ANNEXIN V FITC/PI. The expressions of caspases, Bcl-2, RANKL and JNK2 were detected by Western blotting. Results: Fas/DcR3 was expressed in GH3/MMQ/AtT20 cells. The cytotoxic effects of RGD-FasL on tumor cells were seen in a dose-dependent manner. These cells showed the same sensitivity to RGD-FasL as to FasL. RGD-FasL induced apoptosis and G1/G0 arrest. The expressions of caspase-8/9/3, RANKL, JNK2 were increased while that of Bcl-2 was decreased with treatment of RGD-FasL. Conclusions: Fas can be a novel target for the treatment of pituitary adenomas. RGD-FasL induces apoptosis of pituitary adenoma cells through caspase activation.

Background: C ₁ and C ₂ nerve sheath tumors are to be considered as a separate clinical entity because of their unique clinical presentation, relation to the adjacent structures and surgical approaches when compared to their counterparts in other regions. Aim: To present the clinical characteristics, radiological findings, operative approaches, and outcomes. Setting and Design: Of the 32 patients, the study was retrospective in 22 patients and in 10 it was prospective. Materials and Methods: Patients' case records were analyzed. Diagnosis was established by magnetic resonance imaging of craniovertebral junction. Patients were evaluated by Yasargil grading and operative procedure was done accordingly. Results: In this series the tumors were multiple and had extradural extension and hourglass expansion. Surgical results were excellent in most patients when procedure was done early in the course of the disease. Best results in terms of complete removal and good neurological outcome were achieved with posterior or posterolaterlly located lesion. Of the 20 patients who had surgery by posterior approach, 19 (95%) patients had improvement postoperatively, while one (5%) patient remained unchanged. While of the 8 patients who had surgery by posterolateral approach, 6 (75%) had improvement postoperatively, one had no change and the other had neurological deterioration. Conclusion: C ₁ and C ₂ nerve sheath tumors are to be considered as a separate clinical entity. Operative results and recovery of patients are excellent when tumor is located posterior or posterolaterally and surgical procedure is done early in the disease course.

Background: Primary central nervous system lymphoma (PCNSL) occurs in both immunocompetent and immunosuppressed individuals. The role of Epstein-Barr virus (EBV) has been implicated in immunosuppressed individuals but its role is not established in immunocompetent individuals. Aims: To study the possible role of EBV in PCNSL in immunocompetent individuals.Setting and Design: Retrospective study. Materials and Methods: Thirty patients with PCNSL were studied immunohistochemically with antibodies to CD45, CD20, CD3 and EBV latent membrane protein-1 (EBV LMP-1). In situ hybridization was done in 19 patients where enough tissue was available using a specific oligonucleotide probe for EBV-Early RNA (EBER). Results: All the patients were immunocompetent and mean age was 41.6 years. Histologically they were diffuse large cell lymphoma: 25 (83.3%) were B cell, 1(3.3%) was T cell and 4 (13.3%) were unclassified. EBV LMP-1 showed variable membrane and cytoplasmic positivity in 24 (80%) patients. In situ hybridization for EBER was negative in all the 19 patients studied. Conclusion: In this region of the world probably EBV has no etiologic role in PCNSL in immunocompetent individuals.

Background: Ullrich congenital muscular dystrophy (UCMD) is a unique congenital disorder characterized clinically by generalized muscle weakness, contractures of the proximal joints and hyperextensibility of the distal joints and begins from birth or early infancy. Materials and Methods: We prospectively evaluated nine cases of classical UCMD and recorded the clinical phenotypic characteristics and the histopathological findings. Results: There were eight boys and one girl child with classical features of severe muscle weakness, prominent proximal contractures, distal hyperlaxity and prominent calcanei. Immunohistochemistry for Collagen VI A1 done on seven cases showed total absence of labeling in six while sarcolemmal-specific deficiency was noted in one case confirming the diagnosis of UCMD. Interestingly, all our patients were noted to have near total absence of major palmar and plantar creases, and instead there were fine mesh-like lines in addition to the soft velvety skin on the palms and soles suggestive of altered collagen arrangements in the skin. Hitherto, this clinical finding has not been described in UCMD in the English literature.

Background: The degree of contribution from the vertebro-basilar and carotid systems to the origin of the posterior cerebral artery (PCA) is of clinico-anatomical importance. Three configurations in the bifurcation of the posterior communicating artery (PcomA) have been described, foetal, transitional and adult. Objective: To examine the extent of contribution from the vertebro-basilar and carotid systems to the origin of the PCA in adult autopsy brains in subjects who had died of causes unrelated to the brain. Materials and Methods: The external diameter of the PcomA, pre communicating part (P1) and the post communicating part (P2) of PCA in 225 normal Sri Lankan adult cadaver brains was measured using a calibrated grid. Results: The adult configuration was present in 220 (93.3%); (bilaterally in 200 (88.8%), and unilaterally in 20 (8.8%) ten on each side. Foetal configuration was seen in 17 (4.4%); bilaterally in 3 (1.3%), and unilaterally in 14 (6%) 8 on the left and 6 on the right. Transitional configuration was seen in 8 (2.2%); bilaterally in 2 (0.9%), and unilaterally in 6 (2.5%) 2 on the left and 4 on the right. Conclusion: This study reveals that the internal carotid artery (ICA) provided the major supply to the PCA in 4.4% of foetal and 2.2% of transitional configurations. The adult configuration was present in 220 (93.3%), the highest recorded in the literature. This finding may be of relevance to vertebrobasilar ischaemia and infarcts in the territory of the PCA.

Background: Estimations of D-dimer correlate with deep vein thrombosis and pulmonary thromboembolism and may serve as a marker of cortical venous sinus thrombosis (CVST). Aim: To study the usefulness of D-dimer in the diagnosis of CVST. Materials and Methods: A total of 26 patients with CVST were subjected to a detailed medical history and neurological evaluation. A cranial MRI was carried out on a 1.5T scanner using T1, T2, and DWI sequences and two-dimensional time of flight MR venography. D-dimer was estimated using a rapid latex agglutination slide test using monoclonal antibodies. Results: The age of patients ranged between 16 and 70 years old (median 31 years old); 8 were males and were examined after a mean duration of 22 days of symptoms. Cortical venous thrombosis was attributed to puerperium in 4 patients, infection in 5 patients, and pregnancy, dehydration, insect bite, and migraine in 1 patient each. Superior sagittal sinus was involved in 15 patients, transverse sinus in 16 patients, straight sinus in 3 patients, sigmoid sinus in 7 patients, and cavernous and deep system in 1 patient each. A total of 12 patients had more than one sinus involvement. D-dimer was positive in 20 patients and correlated with the duration of symptoms but not with the extent of sinus thrombosis and the outcome. Conclusion: D-dimer is useful in patients suspected of CVST and patients with positive test results should be urgently sent for MR imaging.

We report a young girl who developed Henoch-Schoenlein purpura at the age of 11 years. Two weeks later she developed severe asymmetric choreic movements and behavioral disturbances. Sydenham´s chorea was diagnosed based on the laboratory evidence and she was given intravenous methylprednisolone for five consecutive days. Both behavioral and movement disorder rapidly resolved. She was asymptomatic at three years of follow-up. The rapid resolution of choreic movements and behavioral disturbances in our patients suggests, intravenous corticosteroids may be an option in the treatment of Sydenham's chorea, more so when the movements are disabling.

Frontotemporal dementia is an important neurodegenerative disorder accounting for a significant proportion of dementia cases with onset before 60 years of age. Apart from the well recognized behavioral changes the disease has many other distinctive features like predominant language involvement alone or associated features of motor neuron disease or parkinsonism etc. which at times may be the presenting manifestation itself. In the following article we describe a rare presenting manifestation; prosopagnosia, in the setting of frontotemporal degeneration

Giant cell arteritis (GCA), the most common form of systemic granulomatous vasculitis in adults, preferentially involves large and medium-sized arteries. Scalp necrosis is a rare complication and can be the presenting feature. This report presents two patients of GCA with severe scalp necrosis as the presenting symptoms. Both the patients were treated successfully with selective debridement, local wound care, antimicrobial and systemic steroid treatment. Skin necrosis is a potentially treatable complication of GCA and needs a multidisciplinary approach.

Reversible cerebral vasoconstriction syndromes (RCVS) are a group of disorders that have in common an acute presentation with headache, reversible vasoconstriction of cerebral arteries, with or without neurological signs and symptoms. In contrast to primary central nervous system vasculitis, they have a relatively benign course. We describe here a patient who was diagnosed with RCVS.

Balò's concentric sclerosis (BCS) is a rare primary demyelinating disease of central nervous system (CNS) and is considered to be a variant of multiple sclerosis (MS). It is characterized by a severe, rapidly evolving course with CNS lesions consisting of concentric rings of demyelination alternating with myelination in the white matter. We report a patient with BCS from mainland China diagnosed on magnetic resonance imaging (MRI) findings. In addition to two contrast enhanced open ring lesions. MRI also showed multiple small white matter lesions in both the hemispheres. The patient had a good response to corticosteroids and had a benign prognosis during a follow-up period of two years.

The authors report two cases of hypoglycemic coma in which both diffusion-weighted imaging (DWI) and single photon emission computed tomography (SPECT) were used. A 74-year-old woman (case 1) presented with deep coma associated with hypoglycemia (blood glucose level: 20 mg/dl), and DWI on admission revealed extensive hyperintensity lesions. SPECT obtained on the second hospital day revealed diffuse hyperperfusion and a follow-up SPECT on the eighth hospital day showed relative hypoperfusion. An 89- year- old woman (case 2) presented with coma associated with hypoglycemia (blood glucose level: 45 mg/dl), and DWI showed diffuse hyperintensity lesions in both hemispheres. SPECT obtained on the ninth hospital day showed relative hypoperfusion compared to the first scan obtained on the third hospital day. Although the blood glucose level was rapidly corrected, the level of consciousness remained unchanged in both cases. Our results suggest that cerebral blood flow may increase even after glucose correction and that relative hypoperfusion could occur in the subacute stage of hypoglycemic coma.

We report a 57-year-old man who presented one month after sustaining a traumatic right temporal intracerebral hematoma with history of headache, left hemiparesis and altered sensorium of two days duration. A diagnosis of right temporal resolving hematoma was made on computed tomography scan. However, his sensorium progressively deteriorated and he underwent craniotomy and partial excision of an abscess. He was treated with appropriate antibiotics for six weeks despite of which he did not improve and died nine months later. We conclude that there should be a high index of suspicion for brain abscess in patients with traumatic intracerebral hemorrhage if the clinical and radiological picture is different from the expected course of a resolving hematoma.

Olfactory ensheathing cells (OECs) are found in the olfactory bulb and olfactory nasal mucosa. They resemble Schwann cells on light and electron microscopy, however, immunohistochemical staining can distinguish between the two. There are less than 30 cases of olfactory groove schwannomas reported in the literature while there is only one reported case of OEC tumor. We report an OEC tumor in a 42-year-old male and discuss the pathology and origin of this rare tumor.

An elderly male was evaluated for subarachnoid hemorrhage and found to have a persistent primitive hypoglossal artery with its associated abnormal posterior fossa circulation and a lower basilar artery aneurysm. Angiography findings are described and are corroborated with computed tomography and magnetic resonance imaging. Its surgical and clinical relevance are discussed.

Bilateral cavernous sinus and optic nerve metastases is extremely rare. We report an extremely rare case of metastatic adenocarcinoma of bilateral cavernous sinus and optic nerve with unknown primary presenting as orbital pseudotumor. She underwent bilateral optic nerve decompression and the diagnosis was established by biopsy.

Anterior communicating artery (Acom) complex and anterior cerebral artery (ACA) are the frequent sites of arterial anomalies. Aneurysms associated with triplicate ACA are rare. Blood blister-like aneurysm (BBA) of the Acom is also very rare. This report presents these two rare types of aneurysms in a 63-year old man who presented with subarchnoid hemorrhage secondary to accessory ACA aneurysmal rupture. During surgery, a BBA of the Acom was also found. Both the aneurysms were treated simultaneously. The BBA bled intraprocedurally but was repaired successfully by neck reconstruction and clipping with the preservation of the parent and the branching arteries. The ruptured accessory ACA aneurysm was clipped together with the artery which had multiple aneurysms distally. For the treatment of a complex aneurysm like this, accurate understanding of the anatomic variations, proper imaging study and extremely careful technique are essential.

A case of pure epidural cavernous angioma involving the dorsal region of the spine is presented. The clinical picture was of a slowly progressive compressive myelopathy. Imaging studies only showed a dorsally located epidural mass lesion. Histology confirmed the diagnosis of this rare type of lesion. The patient improved after excision of the lesion.