Amyotrophic lateral sclerosis (ALS) typically commences in a discrete location in a limb or bulbar territory muscles and then spreads to the adjacent anatomical regions. This pattern is consistent with a contiguous spread of the disease process in motor neuron network resulting in progressive motor weakness. The etiology of ALS onset and the mechanism of the regional ALS spread remain elusive. Over the past 5 years, identification of mutations in two RNA binding proteins, trans active response (TAR) DNA-binding protein (TDP-43) and fused in sarcoma (FUS), in patients with familial ALS has led to a major shift in our understanding of the ALS disease mechanism. In addition to their role in RNA metabolism, TDP-43 and FUS form protein aggregates in the affected neurons. More recent findings demonstrating that both TDP-43 and FUS contain glutamine/asparagine (Q/N) residue-rich prion-like domains have spurred intense research interest. This brief review discusses the prion-related domains in TDP-43 and FUS and their implication in protein aggregate formation and disease spread in ALS.

Aims: To investigate the therapeutic effect of minimally-invasive aspiration and drainage in traumatic epidural hematoma straddling transverse sinus (TEHSTS). Materials and Methods: Fifty-eight patients (39 males and 19 females) with TEHSTS and initial admission Glasgow Coma Scale (GCS) score of 8-10 (mean = 9) were treated with minimally-invasive aspiration and drainage under computed tomography (CT) guidance. Urokinase was used for irrigation and drainage. Post-operatively CT scan was performed at 3 h, 3 days, and 5 days. The volume of hematoma was calculated, and Glasgow outcome scale (GOS) was evaluated 3 months after the operation. Results: The volume of hematoma at 3 h and 3 days post-operation (20 ± 5 ml and 15 ± 2 ml; respectively) were significantly lower than that of pre-operation (45 ± 10 ml; P < 0.05). The hematoma was totally evacuated on 3-5 days post-operation. The GCS was 12 ± 1 on the 5 ^th day after the operation, which was significantly higher than that of pre-operation (8 ± 1; P < 0.05). Three months after operation, 45 (77%) patients had good recovery (GOS: 5) and 9 (15%) patients had moderate disability (GOS: 4). Conclusions: Minimally-invasive aspiration and drainage could be potentially effective in the treatment of TEHSTS with GCS score of equal or greater than 8 points.

Background: The microanatomy features of cerebral arteries may be variable and may be different in different ethnic groups. Aim: To study the anterior cerebral artery (ACA) anatomy in North-West Indian cadavers. Materials and Methods: Microanatomy features of the ACA were studied in 15 formalin fixed human cadaveric brains under microscope. The outer diameter, length, and number of perforating branches with respective anomalies were measured for each of the following vessels: ACA (proximal A1 segment to distal A2 segment), anterior communicating artery (ACoA), Recurrent artery of Heubner (RAH), and callosomarginal artery and photographed for documentation. Results: The mean length and external diameter of right and left A1 segment was 12.09 mm and 12.0 mm and 2.32 mm and 2.36 mm respectively. Narrowing, duplication, and median ACA were seen in 6.6%, 3.3% and 6.6% of the vessels respectively. Complex ACoA type was seen in 40% cadavers. RAH originated at an average point of 0.2 mm distal to ACoA, but in one cadaver it arose 5 mm proximal to ACoA. Double RAH was found in 26.6%. The course of RAH in relation to A1 was superiorly in 60%, in anteriorly 30% and posteriorly in 10% of cadavers. The orbitofrontal artery (OFA) and frontopolar artery (FPA) arose from A2 in 83.3% to 40% respectively. The mean distance of OFA and FPA from ACoA was 4.17 mm and 8.5 mm respectively. After giving rise to central, callosal and cortical branches, pericallosal artery terminated near the splenium of the corpus callosum or on the precuneus as the inferomedial parietal artery. Conclusion: Knowledge of the microvascular anatomy is indispensable and it is mandatory to be aware of the possible variations in the anomalies to minimize morbidity.

Background and Objectives: The endoscopic transsphenoidal approach is commonly used surgical approach for pituitary adenomas. However, adenomas with dumbbell configuration, pure suprasellar location, and fibrous consistency are difficult to remove by this approach. Recently, the extended endoscopic endonasal approach (EEEA) has been utilized to excise this subgroup of pituitary adenomas successfully. Materials and Methods: Between January 2009 and December 2011, 13 patients with pituitary macroadenomas were treated with EEEA. The tumor subgroups included: Dumbbell tumor configuration (4), pure suprasellar tumor location (2), and large suprasellar tumors with subfrontal extension (2). Five patients had fibrous/recurrent tumors and required addition of transtubercular-transplanum extension to the standard endoscopic endonasal exposure for radical resection. Results: The tumor removal was gross total in 8 (61.5%) patients, subtotal in 4 (30.7%), and partial in 1 (7.7%) patient. Clinical improvement was observed in almost all patients, immediate relief in headaches in 88% and normalization of vision in 90% of patients with pre-operative visual disturbances. Three patients with secreting adenomas, two with growth hormone-secreting adenomas and one with prolactin-secreting adenoma, had normalization of hormonal status. Three patients developed temporary diabetes insipidus two patients suffered transient ischemic attacks and one patient with a recurrent giant pituitary adenoma experienced a serious injury to the perforating artery. Four patients (30.7%) developed post-operative cerebrospinal rhinorrhea and two patients required surgical repair. Conclusions: Our early experience suggests that the EEEA offers a potentially viable treatment option in certain pituitary tumors which are difficult to remove by the standard endoscopic approaches. However, longer follow-up and larger series are needed to establish the efficacy of this approach.

Background: The effectiveness of antiepileptic prophylaxis in patients with newly diagnosed high-grade glioma is debated. Craniotomy, surgical manipulation and bleeding are believed to favor the onset of seizures and, therefore, perioperative antiepileptic drugs (AEDs) are generally used. Nevertheless, evidence to initiate preoperative AED prophylaxis are weak. Aim: Aim of this paper was to evaluate the need for AED prophylaxis in surgically-treated malignant glioma patients without history of seizures. Materials and Methods: We conducted a retrospective, two-center cohort study to assess the effectiveness of preoperative AED prophylaxis. Patients were divided in two groups: one with AED preoperative administration and the other without. Because of its non-hepatic metabolism, levetiracetam (LEV) was chosen. Logistic regression models were used to investigate the odds ratio for each group. The explanatory variables included the treatment received, sex, age, and site of lesion. The outcome measure of successful LEV prophylaxis was seizure vs. no seizure post-operatively, at three and six months after surgery. Results: Our results showed that LEV prophylaxis was not a significant predictor of seizure occurrence, although the regression coefficient indicated a slight reduction in seizure risk following LEV administration. Patient's age was a significant predictor of seizure occurrence. Younger patients had a higher risk of seizure in the six months post-surgery. Conclusions: We conclude that AEDs prophylaxis does not provide a substantial benefit to surgically treated high-grade glioma patients and should not be administered routinely. Further investigations are required to detect subgroups of patients at higher risk of developing seizures in order to selectively administer AED.

Background: Though, loss of heterozygosity (LOH) at chromosome 22q is considered to be the most likely initiating event in the formation of meningiomas, LOH at other chromosomes (1, 3, 6, 9, 10, 11, 14.17, and 18) have been implicated in its progression. The aim of this study was to analyze microsatellite markers on a select set of chromosomes including, 22q, 10q, 14q, and 17p for LOH in patients with meningiomas. Materials and Methods: Tumor tissue and its corresponding blood sample were collected from 27 patients with meningioma. Four polymorphic microsatellite markers (D10S520, D17S1289, D14S555, and D22S417) were characterized for LOH analysis. Results: There were 14 World Health Organization (WHO) grade I, 12 WHO grade II and 1 WHO grade III meningiomas. LOH was seen most often at D22S417 with an equal distribution between the grades (33% of informative samples in each grade). Though, LOH at D14S555 was seen in 50% of informative WHO grade II tumors, compared to 11.1% of informative WHO grade I tumors it did not reach statistical significance. However, allelic imbalance (AI) at D14S555 was significantly associated with atypia (P = 0.05). LOH at D17S1289 was seen only in one tumor sample, and none of the informative samples displayed LOH at D10S520. Conclusion: The frequency and equal distribution of LOH at chromosome 22 supports the hypothesis that it is an early event in the tumorigenesis of meningiomas. The association of AI at D14S555 in WHO grade II meningiomas needs to be investigated on a larger set of samples.

Background: Resting state (task independent) Functional Magnetic Resonance Imaging (fMRI) has opened a new avenue in cognitive studies and has found practical clinical applications. Materials and Methods: Resting fMRI analysis was performed in six patients with brain tumor in the motor cortex. For comparison, task-related mapping of the motor cortex was done. Connectivity analysis to study the connections and strength of the connections between the primary motor cortex, premotor cortex, and primary somatosensory cortex on the affected side was also performed and compared with the contralateral normal side and the controls. Results: Resting fMRI in patients with brain tumor in the motor cortex mapped the motor cortex in a task-free state and the results were comparable to the motor task paradigm. Decreased connectivity on the tumor-affected side was observed, as compared to the unaffected side. Conclusion: Resting fMRI and connectivity analysis are useful in the presurgical evaluation of patients with brain tumors and may help in uncooperative or pediatric patients. They can also prognosticate the postoperative outcome. This method also has significant applications due to the ease of image acquisition.

Background: Inflammatory myopathy (IM) as a manifestation of paraneoplastic syndrome has been well-documented. However, the prevalence of malignancies reported varies across the studies. There are very few studies reported from Asia, only one from India. Aim: The aim of this analysis was to study the prevalence of malignancy in biopsy-proven cases of IM in India and to study the difference between malignant and non-malignant groups. Materials and Methods: The study was a retrospective review of case records of patients with a biopsy-proven IM attending Tertiary Care University Hospital. Results: Of the total 86 patients with biopsy-proven IM, 22 patients were polymyositis, 63 patients had dermatomyositis (DM) and one was with an inclusion body myositis, not included for further analysis. Associated malignancy was diagnosed in 6 (7%) patients, and five of them were females. Diagnosis of associated malignancy was identified at the time of diagnosis of IM in four (66.7%) patients. All the six patients with an associated malignancy had DM. Only one patient died within 1 year of diagnosis. Creatinine kinase was much lower in patients with malignancy associated IM than in patients with no malignancy (P < 0.0001). Conclusion: The prevalence of malignancy was very low in our cohort as compared to the studies from other countries. Breast cancer was the most common malignancy associated with DM. The type of associated malignancy was quite variable.

Background: Epilepsia partialis continua (EPC), is a subtype of status epilepticus, have a varied spectrum of etiology and the out-come depends on the etiology. Aims and Objectives: The present study is aimed to analyze the clinical characteristics and outcome. Materials and Methods: This is a prospective analysis of 17 patients admitted to our center between August 2010 and April 2012. EPC was defined as regular or irregular clonic muscular twitches affecting a limited part of the body, occurring for a minimum of 1 h, and recurring at intervals of no more than 10 s. The data collected included etiology, radiological findings, electroencephalogram (EEG) abnormalities, associated comorbid conditions, and outcome. Results: The mean age at presentation was 44.26 ± 13.77 years and the mean duration was 2.7 ± 1.5 days. There were ten patients with diabetic non-ketotic hyperosmolar state and one patient each of oligodendroglioma, varicella zoster vasculitis, central nervous demyelination, ischemic stroke, post traumatic seizure, arteriovenous malformation, and in one patient no cause could be established. Imaging showed abnormality only in five patients and EEG was abnormal in four patients. The EPC was controlled by one antiepileptic drug (AED) in eight patients, with two AEDs in seven patients and two patients required three AEDs. Conclusion: EPC is a rare type of focal motor status epilepticus. Treatment of the underlying cause in addition to controlling EPC is essential to achieve the good outcomes.

Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL) is a recently described disorder with an autosomal recessive mode of inheritance. We report a case of rare adult-onset LBSL with typical magnetic resonance imaging (MRI) features. The DARS2 gene mutation analysis has identified a c. 228-20_21delTTinsC (p.R76SfsX5) mutation and a c. 850G > A (p. 284E > K) mutation. With glucocorticosteroid treatment the patient has had improvement in bladder symptoms. This is the first reported adult-onset LBSL case in the Chinese Han population. A review of the literature suggests that brain lactate elevation in adult-onset LBSL is lower than early-onset cases (P < 0.01), and early-onset cases show mild intelligence and cognition decline. These observations suggest that age of onset and brain lactate levels probably influence the prognosis of LBSL.

Airplane travel headache is rare and has recently been described as a new form of headache associated with a specific situation. Of the 1,208 patients with primary headaches attending a tertiary care neurology hospital, two (0.16%) patients satisfied the criteria for headache related to airplane travel. Both the patients fulfilled the proposed diagnostic criteria for airplane travel headache. This unique headache had a mean duration of 24 minutes, localized to the medial supraorbital region described as having an intense jabbing or stabbing character that occurred exclusively and maximally during aircraft landing or take-off, following which pain intensity subsided . This rare headache felt on aircraft descent is probably due to the squeeze effect on the frontal sinus wall, when air trapped inside it contracts producing a negative pressure leading to mucosal edema, transudation and intense pain. Use of nasal decongestants either alone or in combination with naproxen sodium prior to ascent and descent abated the headache episodes. Awareness about this unique entity is essential to provide proper treatment and avoid patient suffering.