Background: Tethered cord syndrome (TCS) is a complex clinicopathologic entity, mostly described in children with limited number of studies describing in adults. This unique and rare subgroup of patients presents with characteristic features of TCS, but unlike children, pain is a predominant clinical symptom. Materials and Methods: Case records of 24 patients aged ≥16 years who had undergone surgery with a diagnosis of TCS between 2001 and 2011 were reviewed. Patients who have underwent surgery earlier for tethered cord or for diastematomyelia/spinal dysraphism and patients who had radiological evidence of tethering elements like lipoma of the cord on magnetic resonance imaging (MRI) were excluded from the study. Results: Low backache was the most common presenting symptom. At the time of final follow-up, 15 (83.3%) patients had shown improvement in backache. Weakness improved by at least one grade in seven (77.8%) patients. Bladder symptoms improved in six (50%) patients. Conclusion: In case of symptomatic patient with low-lying cord, detethering is an advisable option.

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Background: Primary intraventricular hemorrhage (PIVH) is a rare neurological disorder, with bleeding confined to the ventricles only, without recognizable parenchymal or subarachnoid component. Aim: The purpose of this retrospective study was to identify clinical features, predisposing risk factors, etiology, radiological features and yield of diagnostic cerebral angiography in identifying the etiological causes. Settings and Design: Records of patients admitted in neurology division were analyzed in a tertiary care teaching hospital. Materials and Methods: We analyzed the records of 27 patients with PIVH evaluated and treated at our institute from August 2010 to April 2013. PIVH was diagnosed as hemorrhage in the ventricles only, detected by computed tomography scan without evidence of intraparenchymal, subarachnoid hemorrhage or intraventricular hemorrhage associated with trauma. CT angiography (CTA) alone was done in 10 patients (37.03%), digital subtraction angiography (DSA) in 2 patients (7.4%) and both CTA as well as DSA was done in 15 patients (55.5%). Statistical Analysis Used: Categorical and continuous data were analyzed using SPSS version 17. Results: 17 (62.96%) patients were females and 10 (37.03%) were males with ratio of F:M= 1.7:1. Headache was the commonest mode of presentation (85.18%). Hypertension was most common predisposing factor (29.62%) followed by arterio-venous malformations (AVMs) (25.92%), moyamoya disease (MMD) (11.11%), lenticuostriate artery aneurysm (LSA) (11.11%), arterial dissections (7.4%) and dural arteriovenous fistula (dAVF) (3.7%). Conclusions: PIVH is rare and hypertension is important predisposing factor. Yield of cerebral angiography is high in diagnosing the etiology. AVMs and other rare etiological causes like MMD, LSA aneurysm, arterial dissection, and dAVF should be kept in mind with a high index of suspicion and warrants cerebral angiography in them, as some of the causes are potentially treatable.

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Aims: To study the clinical manifestations, illness-related factors and microsurgical management of adolescent-onset idiopathic hemifacial spasm. Materials and Methods: Of the 1221 microvascular decompression procedures performed for idiopathic hemifacial spasm between March 2001 and July 2007, 16 (1.3%) were in adolescent age (≤18). Results: Clinical manifestations in the adolescent-onset were typical, but milder when compared with late-onset. Gender, thickening and adhesion of the arachnoid membrane at the cerebellopontine angle and a small volume of posterior cranial fossa might be important illness-related factors for adolescent-onset idiopathic hemifacial spasm. Arterial compression was the main cause. Typical compression of offending arteries was observed in 14 patients. Compression of petrous bone crest was found in one patient. Immediate effective rate was 100%, and curative rate was 75%. The curative rate, effective rate and delayed resolution rate during the follow-up period (mean: 22.9 months) were 87.5%, 100% and 12.5%, respectively. There was no recurrence. The postoperative complications (2; 12.5%) included: One patient had transient moderate facial palsy and decreased hearing and one patient had transient decreased hearing and tinnitus. Conclusions: This suggests that microvascular decompression is effective in adolescent-onset idiopathic hemifacial spasm.

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Background: Magnetic resonance imaging (MRI) helps in the diagnosis of neurologic Wilson's disease (WD). The literature regarding MR spectroscopy (MRS) and diffusion-weighted imaging (DWI) in WD is limited. Objectives: To evaluate the clinical features and neuroimaging findings in drug-naοve neurologic WD and to find correlation between clinical stage and disease duration with different imaging findings. Materials and Methods: The study subjects included consecutive and follow-up neurologic WD patients attending movement disorder clinic. The initial clinical and MRI features before commencement of chelation therapy were noted. Of 78 patients, 34 underwent DWI study and MRS was done in 38 patients and in 32 control subjects. Results: Dystonia, dysarthria, tremor, and behavioral abnormality were common presenting features. All patients had MRI abnormality with major affection of basal ganglia. The clinical severity and anatomical extent of MRI abnormalities were positively correlated (P < 0.001; r _s = 0.709). Presence of diffusion restriction was inversely related to duration of disease (P < 0.001; r _s = 0.760). WD patients had reduced N-acetylaspartate/creatine (Cr) and choline (Cho)/Cr ratio (P < 0.001) as compared with control subjects in MRS study. Conclusion: Dystonia, dysarthria and tremor are common neurological features of WD. In this study, MRI abnormalities were positively correlated with disease severity; diffusion restriction was inversely correlated with the duration of the disease process. MRS was also a sensitive tool for diagnosing patient of neurologic WD.

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Objective: Chronic subdural hematoma (CSDH) recurs after surgical evacuation in 5-30% of patients. Inserting subdural drain might reduce the recurrence rate, but is not commonly practiced. There are few prospective studies to evaluate the effect of subdural drains. Materials and Methods: A prospective randomized study to investigate the effect of subdural drains in the on recurrence rates and clinical outcome following burr-hole drainage (BHD) of CSDH was undertaken. During the study period, 246 patients with CSDH were assessed for eligibility. Among 200 patients fulfilling the eligibility criteria, 100 each were assigned to "drain group" (drain inserted into the subdural space following BHD) and "without drain group" (subdural drain was not inserted following BHD) using random allocation software. The primary end point was recurrence needing re-drainage up to a period of 6 months from surgery. Results: Recurrence occurred in 9 of 100 patients with a drain, and 26 of 100 patients in without drain group (P = 0.002). The mortality was 5% in patients with drain and 4% in patients without drain group (P = 0.744). The medical and surgical complications were comparable between the two study groups. Conclusion: Use of a subdural drain after burr-hole evacuation of a CSDH reduces the recurrence rate and is not associated with increased complications.

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To report two cases of traumatic paraplegia who developed Sub-acute Post-Traumatic Ascending Myelopathy (SPAM) following surgical decompression.We hereby report two cases (both 35yr old male) with traumatic paraplegia that developed ascending weakness at 3rd and 5th Post-Op day respectively following surgical decompression. Both the patients experienced remarkable improvement in Neurology after treatment with steroids. The authors conclude by emphasizing on minimum cord handling during surgical decompression of the spinal cord to avoid this potentially life threatening complication.

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Infantile spasm (IS) is an epilepsy syndrome affecting infants and young toddlers and many causes have been reported, including occasional chromosomal abnormalities. We describe a 6-month-oldboy who experienced his first seizure at 5 months of age. The seizures were characterized by brief head nods and forceful flexion of the trunk and limbs. The patient has been developmentally delayed since birth and had deteriorated remarkably in the last month. Interictal electroencephalography showed modified hypsarrhythmia. Magnetic resonance imaging showed delayed myelination and widened brain extracellular space. Chromosomal analysis revealed the karyotype 46, XY, der(9) t(7;9)(p15;p22) pat. His father has the asymptomatic reciprocal translocation t(7;9)(p15;p22). This chromosomal abnormality is probably the etiology for the ISs and severe developmental anomalies in this patient. Chromosomal analysis may be done in patients with IS with no obvious cause.

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There are multiple social, economic, and medical challenges in establishing successful epilepsy surgery programs in India and in other low- and middle-income countries (LAMIC). These can be overcome by reproducing pragmatic and proven epilepsy surgery models throughout the country with a larger aim of developing a national epilepsy surgery program so as to provide affordable and quality surgical care to all the deserving patients. An organized national epilepsy surgery support activity can help interested centers in India and in neighboring countries in developing epilepsy surgery programs.

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Background: Myasthenic crisis can be occasionally a complication after surgery for thymomatous myasthenia gravis (T-MG). Aims: The purpose of this study was to investigate the influence of thymectomy on the subsequent clinical course of T-MG. Study Design: Retrospective study. Materials and Methods: Only T-MG patients with at least 36 months of follow-up after transsternal thymectomy for thymoma was screened, and those with successfully weaned from a ventilator after surgery were included in the study. Results: Forty-eight T-MG patients were enrolled during the study period. Myasthenic crisis after thymectomy (MCAT) occurred in 12 (25%) patients with T-MG. Eight (67%) patients with MCAT experienced respiratory failure within the first 1 and 2 years after disease onset. The ratio of measured forced vital capacity (mFVC) to the predicted FVC (pFVC) preoperatively was the only independent factor affecting the occurrence of MCAT (odds ratio, 0.916; 95% confidence interval [CI], 0.867-0.967; P = 0.002). The area under the curve of the receiver operating characteristic for mFVC/pFVC was 0.881 (95% CI 0.754-0.956, P < 0.001), with sensitivity, specificity, and positive and negative predictive values of 58.3%, 97.2%, 87.5%, and 90%, respectively, at a threshold of ≤65% of mFVC/pFVC. Conclusions: MCAT may occur in patients with T-MG after thymectomy especially within 2 years after disease onset. Preoperative mFVC/pFVC is strongly associated with the occurrence of MCAT postoperatively.

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Objective: This meta-analysis was conducted to assess the relationship between interleukin-10-1082 G/A single nucleotide polymorphism with atherosclerosis (AS) risk. Materials and Methods: The databases of PubMed, EMBASE, Chinese National Knowledge Infrastructure and Wan-Fang were searched from January 2000 to January 2014. 16 studies (involving 7779 cases and 7271 controls) were finally included. Each eligible study was scored for quality assessment. We adopted the most probably appropriate genetic model (recessive model) after carefully calculation. Between study heterogeneity was explored by subgroup analysis and publication bias was estimated by Begg's funnel plot and Egger's regression test. Results: Statistically significant association was observed between AA genotype with overall AS risk, being mainly in coronary heart disease and stroke subgroups among Asian population, and peripheral artery disease (PAD) subgroup among Caucasians. Conclusions: Interleukin-10-1082 AA genotype is associated with increased overall AS risk. AA carriers of Asians seem to be more susceptible to coronary artery disease and stroke, and Caucasians are more susceptible to PAD.

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Background: Parkinson's disease (PD) is a disabling neurological disorder characterized by progressive degeneration of dopaminergic neurons. Mutations analysis within the α-synuclein gene (SNCA) on chromosome 4 has been reported in the last decade. Objective: To elucidate the possible role of SNCA gene in the pathogenesis of PD in Indian population specifically in north Karnataka. Materials and Methods: The study subjects included 100 clinically diagnosed PD patients and 100 ethnically matched healthy controls. Isolated deoxyribonucleic acid (DNA) samples from both were subjected to exon-specific polymerase chain reaction (PCR) amplification and amplicons were subjected to capillary-based direct DNA sequencing. Result: No mutations were observed in SNCA gene of PD samples in comparison with control samples. Conclusion: These findings support the hypothesis that the SNCA gene mutations might be population specific and may not be playing role in causing PD in all the populations.

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Background: Despite advances in vestibular schwannoma (VS) surgery and intraoperative electrophysiological monitoring, immediate and delayed facial nerve outcomes are difficult to accurately predict consistently. Objective: To determine the utility of proximal to distal facial nerve amplitude and latency ratios in predicting the long-term postoperative facial nerve function in patients undergoing excision of VS. Materials and Methods: One hundred consecutive patients undergoing surgery for VS with intraoperative facial nerve monitoring were included. Clinical, radiological, electrophysiological, and postoperative outcome data were prospectively entered into a database. Other parameters such as brainstem distance, size of the porus acousticus, and facial nerve length were also analyzed. Results: Of the 100 patients, 53 were women. The mean age was 42.5 ± 14.1 years (range, 14-71 years) and the average tumor size was 4.1 ± 0.8 cm (range, 2.4-6.5 cm). Total excision was done in 89% of patients. Intraoperatively, the facial nerve was anatomically preserved in 86 patients, but electrophysiological responses were obtained from the root entry zone (REZ) in only 77 patients at the end of surgery, 75% of which had good facial function at long-term follow-up. In nine patients where no responses were obtained but the facial nerve was anatomically intact, 50% had good facial function at long-term follow-up. Proximal and distal amplitude and latency ratios, size or consistency of the tumor, brainstem distance, size of the porus acousticus, and length of the facial nerve were not useful in predicting long-term functional outcome. Conclusions: While a positive response to facial nerve stimulation at the end of VS surgery is a good predictor of long-term postoperative function, the absence of responses in an anatomically intact nerve does not preclude good function in the long term. Proximal to-distal amplitude and latency ratios did not correlate with the final facial function.

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Purpose: Although seizures are the most common presentation of neurocysticercosis (NCC), the electroclinical characteristics of the seizures have not been adequately studied. Materials and Methods: The study cohort included 308 consecutive patients presenting with a new-onset seizure, both acute symptomatic or unprovoked. They were divided into two groups: Group 1 (NCC group), those in whom imaging studies revealed active or inactive NCC (N = 184 (59.7%)) and group 2 (non-NCC group), those in whom either imaging was normal or revealed abnormalities other than NCC or the diagnosis was idiopathic generalized epilepsy (N = 124 (40.3%)). Results: Clinical features significantly associated with group 1 included focal seizures (specifically, aphasic seizures (P < 0.05)), seizure clusters (P < 0.0001), postictal Todd's paresis (P < 0.05), and peri-ictal headaches (P < 0.008). In addition, somatosensory and visual seizures, and focal-clonic and focal-tonic were more frequent in this group. Generalized clonic seizures (P < 0.05) were significantly more common in the group 2. The findings of regional epileptiform abnormalities (P = 0.0001) and primary generalized epilepsy (P < 0.0001) on electroencephalography were significantly more common in group 2. Significance: In patients presenting with new-onset seizure, aphasic seizures, seizure clusters, postictal Todd's palsy and peri-ictal headaches might augur the finding of NCC, both active and inactive lesions, on imaging.

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Background: Stroke is a multifactorial, polygenic disorder, influenced by both environmental and genetic factors. Aim: The purpose of this study was to determine the association of beta-1 and beta-2 adrenergic receptor (AR) polymorphisms with intracerebral hemorrhagic stroke (ICH) in a North Indian population. Materials and Methods: One hundred and six patients with intracerebral hemorrhage (ICH) and 106 age- and sex- matched controls were recruited. Genotyping was performed by using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) method. RFLP results of three samples of each genotype were confirmed by DNA sequencing. Results: Mean age of cases and controls were 53.47 ± 14 and 52.92 ± 13.4, respectively. Significant association between Ser49Gly polymorphism of beta-1 AR and ICH in patients who had onset of disease at a later stage of life (>50 years) under a dominant model of inheritance (OR, 3.6; 95% 1.4 to 9.7) was observed. Under the dominant model of inheritance, Gln27Glu polymorphism of beta-2 AR was associated with risk of ICH (OR, 3.2; 95% CI, 1.7 to 5.8) and significant association persisted even after adjustment for demographic and other risk factor variables (OR, 2.9; 95% CI, 1.04 to 8; P = 0.04). Age-stratified analysis showed an independent significant association of Gln27Glu polymorphism of beta-2 AR with risk of ICH in patients those had onset of disease at young age (<50 years) under a dominant model of inheritance (OR, 3.5; 95% CI, 1.1 to 11). Conclusion: The present study provides the first preliminary evidence that Gln27Glu polymorphism of beta-2 AR may contribute modest effect in increase in risk of ICH in North Indian Population. Large prospective studies with large sample size are required to confirm these findings.

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