In the early 1880s, Joseph Babinski was appointed as Chef de Clinique under Jean-Martin Charcot at Salpétrière, Paris, in France. He appeared for the post of Professeur Agrégé, the pinnacle of academic distinction in France in 1892. Charles Bouchard, the earliest pupil of Charcot, who described the Charcot-Bouchard aneurysm along with his master, was the Chief of the Board of Jury. Charcot and Bouchard did not see eye to eye in the later period, and when German Sée, an external examiner did not join the board of examiners following illness, Bouchard, instead of seeking the assistance of an alternate examiner, employed the special right of Vote of Absence. Babinski and all other pupils of Charcot were unsuccessful, while those of Bouchard, came out with flying colors. An embittered Babinski, along with some of the other unsuccessful candidates, appeared before the Ministry of Public Assistance after a protracted legal battle for nearly 2 years but lost the case. They were even ordered by the court to pay on behalf of the the successful candidates for the legal battle. Babinski never sat for the examination again and this is the story of one of the most ignominious episodes in an examination anywhere.

Approximately 60% of all patients with epilepsy suffer from focal epilepsy syndromes. In approximately 15% of these patients, the seizures are not adequately controlled with anticonvulsive drugs, and such patients are potential candidates for surgical treatment and majority are children. Epilepsy surgery in children, who have been carefully chosen, can result in either seizure freedom or a marked (>90%) reduction in seizures in approximately two-third of children with intractable seizures. In the multimodality presurgical evaluation approach, sufficient concordance should be established among various independent investigations, thus identifying the location and extent of the epileptogenic zone with a high degree of confidence. Early surgery improves the quality of life and cognitive and developmental outcome of the child. Surgically remediable epilepsies in children should be identified early and include temporal lobe epilepsy with focal lesions, lesional extratemporal epilepsies, hemispherical epilepsies, and gelastic epilepsy with hypothalamic hamartoma, and can be treated by resective or disconnection surgery. Palliative procedures include corpus callosotomy and vagal nerve stimulation for children with diffuse and multifocal epilepsies, who are not candidates for resective surgery. Deep brain stimulation in patients with epilepsy is still under evaluation. For children with “surgically remedial epilepsy,” surgery should be offered as a procedure of choice rather than as a treatment of last resort.

Introduction: Hemifacial spasm (HS) is a rare disorder caused by the compression of facial nerve root exit zone (REZ) at the brainstem by a vascular loop. Microvascular decompression (MVD) is a popular treatment modality for HS. Objective: The purpose of this study was to determine the long-term efficacy and safety of MVD for HS by assessing the effect of the procedure from the literature published over the last 25 years. Materials and Methods: A systematic data review from 1992 to 2015 using specific eligibility criteria yielded 27 studies on MVD for HS, the data of which were pooled and subjected to a meta-analysis. Results: The pooled odds ratio (OR) revealed by the meta-analysis showed that anterior inferior cerebellar artery was the most common offending vessel in 37.8% (95% confidence interval [CI]: 27.8–47.7%) of the patients. Complete resolution of HS was seen in 88.5% (95% CI: 86.7–90.4%) of the patients after a long-term follow up. The complication rate was low following MVD, the most common being temporary facial paresis in 5.9% (95% CI: 4.3–7.5%) of patients. Conclusions: MVD is a safe and effective treatment for HS with long-term benefits and a low complication rate.

Background: Comparative study of Class I drugs in central poststroke pain (CPSP) is lacking. Objective: To evaluate the safety and efficacy of pregabalin and lamotrigine in patients with CPSP. Setting: Tertiary care teaching referral hospital. Methods: We included consecutive patients with CPSP having ≥50 mm score on Visual Analog Scale (VAS) and randomized them to receive either oral pregabalin or oral lamotrigine. Their clinical findings, serum chemistry, and cranial magnetic resonance imaging findings were noted. The severity of CPSP was assessed by VAS while the allodynia was assessed on a 0–10 scale. The hospital anxiety and depression (HAD) score was also recorded. The primary outcome was >50% reduction in the VAS score while the secondary outcome was >50% reduction in the severity of allodynia and improvement in HAD score at 3 months. Adverse reactions were noted in both the groups. Results: There were 30 patients with CPSP with a median age of 54.5 years (Male:Female = 36:4). While both pregabalin (P < 0.0001) and lamotrigine (P < 0.0001) resulted in significant improvement in the VAS score (63.3% vs. 53.3%), allodynia (66.7% vs. 75%), and HAD score (80% vs. 81.8%) compared to the baseline, the was no significant intergroup difference. Lamotrigine had to be withdrawn in three patients due to skin rash. Conclusions: Both pregabalin and lamotrigine are equally effective in CPSP, but significant side effects leading to drug withdrawal occured with lamotrigine administration.

Introduction: MicroRNAs (miRNAs) are short RNA molecules of approximately 22 nucleotides that function as post-transcriptional regulators of gene expression. They are expressed in a tissue-specific manner and show different expression patterns in development and disease; hence, they can potentially act as disease-specific biomarkers. Several miRNAs have been shown to be deregulated in plasma and skeletal muscles of myotonic dystrophy type 1 (DM1) patients. Methods: We evaluated the expression patterns of 11 candidate miRNAs using quantitative real-time PCR in whole blood (n = 10) and muscle biopsy samples (n = 9) of DM1 patients, and compared them to those of normal control samples (whole blood, n = 10; muscle, n = 9). Results: In DM1 whole blood, miRNA-133a, -29b, and -33a were significantly upregulated, whereas miRNA-1, -133a, and -29c were significantly downregulated in the skeletal muscles compared to controls. Conclusions: Our findings align to those reported in other studies and point towards pathways that potentially contribute toward pathogenesis in DM1. However, the currently available data is not sufficient for these miRNAs to be made DM1-specific biomarkers because they seem to be common to many muscle pathologies. Hence, they lack specificity, but reinforce the need for further exploration of DM1 biomarkers.

Objective: To analyze the mortality and potential risk factors for death in myasthenia gravis (MG) patients. Materials and Methods: A total of 2195 adult patients with MG (aged older than 18 years) diagnosed during the period between 2003 and 2013 were followed-up and retrospectively reviewed. Results: During the 10-year follow-up, 129 patients died and the total mortality rate was 5.88%. The risk factors associated with MG-related deaths were duration of the disease, occurrence of myasthenic crisis, severity of disease that included the Myasthenia Gravis Foundation of America (MGFA) grade III and IV at onset, elevation of acetylcholine receptor antibody (AchR-abs) titers, presence of thymic pathology, and failure of administrating immunosuppressants (P < 0.05). In addition, the non-MG related factors, including the history of preceding strokes, and the presence of chronic obstructive pulmonary disease (COPD), diabetes mellitus, atrial fibrillation, hyperlipidemia, myocardial infarction, and malignant tumors, were closely linked with death in the MG population (the hazard ratios [HRs] were 3.251, 4.173, 3.738, 3.886, 1.945, 2.177, and 14.7, respectively; P< 0.05). Conclusions: The severity of disease at entry, presence of AchRabs, thymic pathology, and duration of the disease predict a higher risk for death. Systemic illnesses including stroke, COPD, diabetes mellitus, atrial fibrillation, hyperlipidemia, myocardial infarction, and malignant tumor, which may also increase the risk of death, should be carefully monitored and managed.

Background: Reports on therapeutic plasma exchange (TPE) with the standard hemodialysis equipment are scarce, particularly from developing countries. Materials and Methods: A retrospective analysis of safety and efficacy of membrane-based TPE with a standard hemodialysis equipment for the treatment of severe Guillain–Barré syndrome (GBS) was conducted. Results: A total of 120 TPE sessions were performed in 31 GBS patients over a period of 5½ years. Each patient underwent a mean of 3.8 ± 1.5 TPE sessions. One (3.2%) patient died. Thirty (96.8%) patients survived and recovered. At 2 weeks, there was a significant improvement in the grade of power in both the upper and lower extremities (P = 0.001) and a significant decrease in the GBS disability grade (P = 0.001). Twenty four (77.4%) patients were able to walk unaided. Complications observed were: hypotension in 12 (10%), accelerated hypertension in 3 (2.5%), chills and rigors in 5 (4.2%), bleeding in 5 (4.2%), and filter clotting in 6 (5%) sessions. One patient experienced an anaphylactoid reaction and 1 patient survived a cardiorespiratory arrest. Two patients developed aspiration pneumonia and 1 patient developed catheter site infection. Conclusions: Membrane-based TPE can be conveniently delivered with the standard hemodialysis equipment. It is a safe, effective, and comparatively less expensive treatment option for GBS.

Objective: To study the frequency, types, phenomenology, and effectiveness of sensory tricks in patients with primary blepharospasm and idiopathic cervical dystonia. Background: Sensory tricks are maneuvers seen in patients with dystonia, which are used to temporarily reduce the severity of dystonic postures. In different types of dystonia, the frequency of sensory tricks have been described to range from 17–89%. Materials and Methods: In this cross-sectional observational study, we analyzed the frequency, types, phenomenology, and effectiveness of sensory tricks in 20 patients with primary blepharospasm and 20 patients with idiopathic cervical dystonia, respectively. Patients underwent a clinical examination, and the severity of dystonia was measured using the Burke Fahn Marsden dystonia rating scale (eyes and neck components).A questionnaire regarding the sensory tricks was administered to all the patients. Results: Eighteen patients with blepharospasm and 7 patients with cervical dystonia used sensory tricks to alleviate their dystonic movements (P < 0.05). The age group of the patients with cervical dystonia presenting with sensory tricks was significantly lower than that of those having blepharospasm (P < 0.05). Sensory tricks, when used, were effective every time in 72.2% of patients with blepharospasm and in 85.8% of patients with cervical dystonia. However, majority of the patients with blepharospasm (61.1%) and cervical dystonia (42.9%) had only partial benefit (<50%) with these maneuvers. Conclusions: In this study, sensory tricks were more common in patients with primary blepharospasm than in patients with idiopathic cervical dystonia.Age of the patients with cervical dystonia was significantly lesser than those suffering from blepharospasm.We did not find any correlation between the presence of sensory tricks, the severity of dystonia and the duration of the disease.Majority of the patients derived benefit with sensory tricks every time; however, the benefit was only partial.

Background: The clinicoradiological outcome of endoscopic fenestration of intracranial cysts and predictors of an unfavorable outcome, including age, are under reported in the neurosurgical literature. In this cohort, our experience in the endoscopic fenestration of intracranial cysts is reviewed. Materials and Methods: Thirty consecutive patients treated with endoscopic fenestration for intracranial cysts were identified and analyzed. The study population in our series was followed clinically and radiographically. Results: In this series, the overall resolution of clinical symptoms such as headache, seizures, and neurological deficits was 83%, P= 0.0001. The percentage of clinical resolution after endoscopic intervention was significantly higher (85% vs. 76%, P= 0.001) in arachnoid cysts compared to other cyst types. The reduction of arachnoid cyst size was significantly higher in adults with obstructive hydrocephalus compared to the children group (P = 0.037). In addition, requirement of a cystoperitoneal shunt placement (P = 0.0001) and its subsequent revision (P = 0.0001) was significantly lower in adults compared to children. Adults (P = 0.041), presence of an arachnoid cyst (P = 0.026), female gender (P = 0.016), and presence of communicative hydrocephalus (P = 0.015) were significant predictors for improvement in the symptoms of intracranial pressure. Lastly, adults (P = 0.028), presence of arachnoid cyst (P = 0.046), and presence of communicative hydrocephalus (P = 0.012) were significant positive predictors for shunt revision. Conclusions: This study revealed that endoscopic fenestration is an effective neurosurgical procedure for the management of intracranial cysts both in adults and children. Moreover, endoscopic fenestration is more beneficial in adults and patients with an arachnoid cyst compared to that in children and other cyst types, respectively.

Background: Pediatric thoracolumbar (TL) spinal injuries are uncommon entities with an incidence of 5–34% of all pediatric spinal injuries. There is a scarcity of studies done on the pediatric population in the developing countries like India. This study aims to review our experience with TL spine injuries in children over a 12-year period. Materials and Methods: We retrospectively reviewed the epidemiology, risk factors, mechanisms, patterns of injury and management of pediatric TL injuries in our population. Results: There were 90 children with TL spine injuries comprising 2% of all the spine injuries treated in our institute. The mean age was 15.9 ± 3.2 years (range: 2–18 years) with a male predominance (3:1). The most common mode of injury was fall from height. 64/90 (71.1%) children sustained injury due to fall from height, 18/90 (20%) children sustained injury following motor vehicle accident and rest of the children sustained injury due to the fall of a heavy object over the neck. Most of the patients (27.8%) sustained Grade A injury. Lumbar spine was the most common spinal level injured (53.3%), and fractures were the most common type of injury (93.3%). Surgical fixation was performed in 18/90 (20%) children. Follow-up was available for 21 children of which 13 (62%) were ambulant at follow-up. Conclusion: TL injuries are rare and are most common in children older than 10 years and mainly involve the lumbar region. When indicated, surgical fusion of the involved vertebrae is safe and effective.

Background: Peripheral nerve injuries (PNIs) remain an important health problem often leading to severe motor disabilities predominantly in the younger population. Objective: To analyze our experience of clinical and electrodiagnostic evaluation (EDX) of PNIs over a 26-year period. Materials and Methods: Between 1989 and 2014, 1124 consecutive patients with 1418 PNIs were referred for clinical as well as EDX evaluation. These PNIs involved upper and lower limbs as well as the facial nerves. Patients with iatrogenic lesions and spinal cord/spinal root lesions were excluded from this analysis. Brachial plexus (BP) injuries with associated or not with root avulsions were considered as one particular nerve and was include in the study as BP. The etiological categories of the sustained trauma included vehicular accidents, penetrating injuries, falls, gunshot wounds, car accidents involving pedestrians, sports injuries, and miscellaneous injuries. Results: The mean age of our patients was 34.2 years and most were males (76.7%). Majority (80.9%) of the PNIs were isolated injuries. Combined lesions most commonly involved the ulnar and median nerves. Upper-limb PNIs accounted for 72.6% of our patients. The ulnar nerve was injured most often, either singly or in combination. Vehicular accidents were the most common causes of injury (46.4%), affecting the brachial BP or the radial, fibular, or sciatic nerves. Penetrating trauma (23.9%) commonly affected the ulnar and the median nerves. Falls and gunshot wounds frequently affected the ulnar, radial, and median nerves. Sports injuries, mostly soccer related, affected predominantly the fibular nerves. BP injuries were considerably more common in accidents involving motorcycles than those involving cars (46.1% vs. 17.1%), and root avulsions was more frequently associated in these cases. Conclusions: Most PNIs were caused by vehicular accidents and penetrating trauma, and affected young men. Overall, ulnar nerve, primary BP, and median nerve PNIs were the most prevalent lesions.

Tumefactive demyelinating lesions are tumour-like presentations of acute demyelinating lesions. They have been described with multiple sclerosis only and not with other varieties of acquired demyelination like acute disseminated encephalomyelitis (ADEM). The uncertainty about the diagnosis at the onset of the disease in tumefactive ADEM makes it important that the physicians should be aware of this entity. Various radiological similarities with more sinister lesions like central nervous system gliomas or lymphomas may lead to this confusion. Appropriate supportive treatment with steroids and follow up is required in these cases to avoid unnecessary interventions.

We report the clinical, radiological, biochemical, muscle histology, and electron microscopic features of two members of a family with combined Ehlers–Danlos syndrome (EDS) [classic and vascular type] and progressive myopathy as the primary manifestation. A 35-year old lady presented with severe gluteal and thigh muscle pain and easy fatigability for 5 years. She developed weakness and wasting of pelvic and pectoral girdles and thighs for 3 years and severe neck flexor and truncal weakness for 6 months. She had a history of recurrent jaw dislocation, easy bruising with hyperpigmentation, hyperextensibility of joints, translucent skin, and papyraceous scars. She had high myopia with astigmatism. She had wasting of temporalis, masseters, sternocleidomastoids and trapezius. There was moderate weakness of temporalis, masseters, and facial muscles. Muscle power was Medical Research Council (MRC) grade 4 at shoulders and arms, and grade 3+ at pelvis and thighs. Serum homocysteine level was normal, and creatine kinase (CK) was 275 IU. Two dimensional echocardiogram (2D Echo) showed myxomatous degeneration of mitral valves. Electromyography (EMG) was suggestive of a myopathic pattern. Muscle magnetic resonance imaging (MR) revealed severe fatty infiltration of paraspinal muscles, gluteus maximus and medius, quadriceps, hamstrings, and gastrocnemius. Electron microscopy showed an occasional distorted fibril with mild increase in oxytalan fibers and variation in thickness of blood vessel basement membrane. Her 15-year old daughter had exertion-induced myalgias, right hemifacial hypoplasia, myopia, hyperextensible joints, hyperelastic skin, and neck muscle weakness. However, her CK and 2D Echo were normal. This report presents the rare combination of classic and vascular type of EDS primarily presenting as muscle weakness and associated with facial and trigeminal motor weakness.

Management of giant intracranial aneurysms presents unique challenges to the neurosurgical team. Various techniques such as adenosine-induced hypotension, rapid ventricular pacing, and inducing deep hypothermia are described in the literature to effect circulatory arrest for the successful obliteration of giant aneurysms. We describe a novel technique of induced hypotension for clipping a giant aneurysm by using an inflatable balloon across the main pulmonary artery with a successful outcome. This technique has not been described earlier in the literature.

Background: Papillary craniopharyngioma (PCP) are uncommon variants of craniopharyngiomas (CP), which are benign epithelial neoplasms of the sellar and suprasellar region. Histologically, PCPs are typically composed of well-differentiated stratified squamous epithelium; however, focal variations are not uncommon. A distinction from other lesions of the region, despite being difficult to achieve due to the overlapping radiological and clinical features, is important for adequate treatment to be administered. Objective: Our aim was to study the clinical and histological features of PCP with emphasis on features that are helpful in its distinction from other lesions that are similar in appearance. Materials and Methods: We reviewed 13 cases of PCP diagnosed in our institution between January 2010 and December 2015. Results: The mean age at presentation was 30.76 years. Two of the patients belonged to the pediatric age group. Male-to-female ratio was 2.25:1. Suprasellar location (either alone or in combination with sellar region) was the most common tumor site. Microscopically, all of the cases showed stratified squamous epithelium with frequent pseudopapillae formation. Focal adamantinomatous epithelium and columnar epithelium with variable cilia and goblet cells were seen in 4 (30.7%) cases. Brain invasion was observed in 3 (23%) cases. Four patients died of their disease; 2 of the 7 patients with an available follow up, developed recurrences; and, 5 experienced severe postoperative morbidity. Conclusion: Majority of the PCPs exhibited typical features with minor variations. Knowledge of the variations in histologic features helps in reaching the correct diagnosis. These tumors can behave aggressively with a high recurrence rate and decreased overall survival.

Clinically relevant pituitary tumors presenting with altered hormonal secretion or mass effect represent a significant proportion of patients in endocrinology clinics. However, in recent years, these patients are also referred to clinical genetic services due to possible germline mutations causing syndromic or isolated pituitary adenomas. While somatic mutations have been identified in GNAS, USB8, PIK3CA, GPR101 and rarely in RAS, germline mutations have been identified in MEN1, cyclin dependent kinase inhibitor genes, AIP, DICER1, PRKAR1A, PRKACA, SDH genes and GPR101. In this review, we present a short overview of pituitary adenoma classifications, pituitary development and somatic and germline genetic changes identified in these adenomas.

Background: Superior hypophyseal artery (SHA) aneurysms form a unique subgroup of paraclinoid aneurysms having a propensity to grow to a large size in the suprasellar region resulting in compression of the optic nerve, chiasma, and/or tract. Aim: A new classification of SHA aneurysms is proposed that helps in identifying the surgical issues encountered during surgical clipping of these medially directed aneurysms located at different segments of the medial surface of the internal carotid artery (ICA). Settings and Design: This descriptive study was conducted at a tertiary care university hospital. Material and Methods: In 14 patients operated for a SHA (mean age: 49.43 ± 11.28 years; presenting either with subarachnoid hemorrhage (n = 11; 78.57%) or mass effect (n = 3; 21.42%), 4 parameters having a bearing on surgery [a. size: (small <1 cm n = 5, large 1–2.5 cm n = 7, giant >2.5 cm n = 2); b. origin of SHA aneurysmal neck and direction of its fundus; c. relationship of the aneurysm to important neurovascular structures; and, d. whether the aneurysms were saccular or fusiform] were used to divide SHA aneurysms into 7 categories a. Antero-supero-medial (n = 2); b. Antero-infero-medial (n = 1); c. Supero-medial (n = 3); d. Infero-medial (n = 3); e. Postero-medial (n = 2); f. Fusiform (n = 1); and, g. Giant (n = 2). Modified Rankin Scale (MRS) score was utilized to assess outcome [favorable: mRS 0-2; unfavorable: mRS 3–6] at discharge and follow up. Results: Modified Hunt and Hess grade at admission was 0 = 3; I = 3; II = 3; III = 2; IV = 3 (favorable mRS: 10; 71.42%; unfavorable mRS: 4; 28.57%); and, Fisher grade was I = 3; II = 6; III = 2; IV = 3. Twelve patients required anterior clinoid process drilling/carotid collar opening to facilitate optic nerve mobilization, for proximal ICA control, and to assess the proximal part of the neck of aneurysm for aneurysmal clipping (n = 13) or wrapping (n = 1). Fenestrated clip was applied in 5 patients. In the supero-medial group (antero-supero-medial, supero-medial, and postero-supero-medial), the aneurysmal fundus was found directly below the ipsilateral optic apparatus, elevating it; in the postero-supero-medial group, the fundus often lay in close proximity to A1 artery, Heubner's recurrent artery, ICA bifurcation, or dorsum sellae. Optic pathway, hypothalamic, and medial lenticulostriate perforators also required careful separation. Antero-infero-medial SHA aneurysm was hidden from view, embedded in the anterior wall of sella below tuberculum sellae. At a median follow-up of 17.5 ± 26.78 months (range: 8–84 months), 9 (64.28%) patients had a favorable mRS and 5 (35.71%) an unfavorable one. Conclusion: Systematically classifying SHA aneurysms and anticipating the surgical risk based upon their anatomical variations helps in achieving a good surgical outcome.

Neurosurgery at Bombay Hospital started in 1953 when Dr. Ram Ginde joined as the Honorary Neurosurgeon. Over the years, the Department of Neurosurgery has earned the reputation of providing contemporary medical services as well as of imparting excellent training to the neurosurgical residents. Professor S. N. Bhagwati started the prestigious annual 'Ginde oration' in 1991, that has been given by several illustrious orators from across the world and has become a precursor of various medical activities held thereafter. This article retraces the history and achievements of the Department of Neurosurgery, Bombay Hospital Institute of Medical Sciences, Mumbai, and also highlights the immense contributions made by its members in the field of Neurosurgery in India