Objective: Guillain-Barre Syndrome (GBS) is an acute inflammatory polyradiculoneuropathy causing limb weaknesses. The mortality is around 8%, with about 20% of patients remaining disabled. The purpose of this study is to examine the performance of Indian Medical Scientists in the area of GBS over a period of 40 years. India's contribution to GBS has been examined based on the global publication output and share, growth rate, citation impact, publication share in various field, highly cited papers and their impact, most productive authors and their citation impact and collaboration of research on GBS with the international community. Materials and Methods: The data for this study has been taken from Scopus, a multidisciplinary database using keywords GBS and Miller Fisher Syndrome. The contribution of India on GBS has been compared with different countries for its publication and citation count during a period of 40 years (1973-2012). Results: It is found that there were 10,633 publications available from global medical research covering the various aspects of GBS. India's share is 2.92% of global output and holds 10 ^th rank in overall publication output on GBS, whereas United States topped the rank with the highest number of publications. Sanjay Gandhi Post-graduate Institute of Medical Research, Lucknow is the most productive institutions while Tally AB from the National Institute of Mental Health and Neurosciences, Bangalore is the most productive author who has contributed the highest number of papers on GBS. Conclusion: India is far behind in GBS research as compared to other countries like USA, as there might be a low case report of GBS in Indian population. Still the research focusing this disease is quite prominent and needs a careful medical attention through research capacity building in order to look for better treatment of the disease in Indian cases. This study shall be helpful in enhancing the awareness of the GBS by Indian scientists.

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Background: Olfactory dysfunction in Parkinson's Disease (PD) has been recognized for a long time and a number of studies have been performed in various parts of the world, using culturally appropriate smell identification tests. Objective: In this study, for the first time, olfactory function has been assessed in the Indian Idiopathic Parkinson's Disease (IPD) patients using an indigenously developed smell test. Materials and Methods: Olfaction was assessed in 53 IPD patients and 50 healthy controls using SniffIn-sticks ^® test and Indian Smell Identification test (INSIT). In both these tests, the subjects were asked to identify the smell from a set of choices and were scored out of 10 and 12 for INSIT and SniffIn-sticks ^® test, respectively. Results: Both SniffIn-sticks ^® test and INSIT showed significant impairment in olfaction in IPD patients (P < 0.001). There was no significant correlation of the scores of both tests with Hoehn and Yahr (H and Y) stage, duration of illness and Levodopa Equivalent Daily Dose (LEDD). The tests had a high correlation, r = 0.75 (P < 0.001) and the area under the Receiver Operating Characteristic (ROC) curves did not differ significantly. Using a cut off value of 4 (values ≤ 4 indicating disease), INSIT showed a sensitivity of 79.2% and specificity of 78%. Conclusion: INSIT, being cheap, convenient and more acceptable in the Indian population, can be considered as a better alternative for SniffIn-sticks ^® test in the evaluation of olfaction in Indian PD subjects.

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Background: Astrocytes are abundantly present as glial cells in the brain and play an important role in the regenerative processes. The possible role of stem cell derived astrocytes in the spinal cord injuries is possible related to their influence at the synaptic junctions. Aim: The present study is focused on in vitro differentiation of cultured human CD34+ cells into astrocytes. Materials and Methods: Granulocyte-colony stimulating factor mobilized human CD34+ cells were isolated from peripheral blood using apheresis method from a donor. These cells were further purified by fluorescence-activated cell sorting and cultured in Dulbecco's modified eagle's medium. Thus, cultured cells were induced with astrocyte defined medium (ADM) and in the differentiated astrocytes serine/threonine protein kinases (STPK) and glutamine synthetase (GLUL) activities were estimated. The expression of glial fibrillary acidic protein (GFAP) and GLUL were confirmed by reverse transcriptase-polymerase chain reaction (RT-PCR). Results: The cultured human CD34+ cells differentiated into astrocytes after 11 h of incubation in ADM. The RT-PCR experiment showed the expression of GLUL (1.5 kb) and GFAP (2.9 kb) in differentiated astrocytes. The high enzyme activities of GLUL and STPK in differentiated astrocytes compared with cultured human CD34+ cells confirmed astrocyte formation. Conclusion: In the present study, in vitro differentiation of stem cells with retinoic acid induction may result in the formation of astrocytes.

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Objective: The aim of this study is to compare the efficacy of cerebrospinal fluid (CSF) repair with and without fibrin glue in pediatric patients with CSF rhinorrhea treated endoscopically. Materials and Methods: This was a retrospective study carried out in the Departments of Otolaryngology and Neurosurgery at a tertiary care center between December 2005 and July 2010. Results: The 27 patients with CSF rhinorrhea included in the study were divided into Group A: 13 patients who underwent endoscopic endonasal CSF repair using fibrin glue (Tisseel, Baxter, Vienna, Austria) by an endoscopic rhinologist and Group B: 14 patients who underwent endonasal CSF repair without fibrin glue. There was no statistically significant difference in endoscopic CSF repairs with or without fibrin glue (P = 0.48) in all the variables studied. CSF repair without fibrin glue appeared more cost saving as compared to repair with glue. Conclusion: CSF repairs with and without fibrin glue are equally effective and CSF repair without fibrin is costs saving.

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Background: GNE myopathy is a clinicopathologically distinct distal myopathy with autosomal-recessive inheritance. The GNE gene mutations are known to cause this form of distal myopathy Materials and Methods: Over the last 6 years, a total of 54 patients from 48 families were diagnosed to have GNE myopathy based on the clinical and histopathological findings. We have reported on 23 cases earlier and from this cohort 12 patients from 11 families underwent genetic testing for GNE mutation. Results: Nine patients belonging to eight families were confirmed as GNE myopathy by genetic analysis. There were six women and three men. Mean age of onset was 26.7 ± 5.47 years (20-36 years) and mean age at clinical examination was 32.3 ± 4.2 years (28-39 years). Mean duration of the illness was 5.7 ± 4.7 years (1-14 years). All had characteristic clinical features of progressive weakness and wasting of the anterior part of leg muscles, adductors of thighs and hamstrings with relative sparing of the quadriceps muscles. Biopsy from the tibialis anterior muscles revealed the presence of rimmed vacuoles. Mutation analysis of the GNE gene revealed that c. 2086G > A (p.Val696Met) change was common in our series like Thailand and six of eight families carried this mutation, heterozygously. Conclusion: These results show the presence of a common mutation in GNE gene in Southeast Asia.

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This study reports the clinical, laboratory profile and outcome in seven patients with biotinidase deficiency. The serum biotinidase activity was assayed using spectrophotometric analysis. The age at presentation varied from day 1 of life to the 5 ^th month. Seizures were the presenting complaint in six patients and clonic seizures were the predominant seizure type. Sparse hair was seen in four patients, while three did not have any cutaneous manifestation. None of the patients had acidosis or hyperammonemia. The clinical response to biotin was dramatic with seizure control in all patients. One patient had neurological deficit at follow-up, while none had optic atrophy or sensorineural hearing loss. Biotinidase deficiency, a potentially treatable condition, should be thought of in any child presenting with neurological symptoms, especially seizures, even in the absence of cutaneous or laboratory manifestations.

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Background: There is a lack of data from India on the impact of migraine on health-related quality of life (HRQoL) and the extent of psychiatric co-morbidities in migraine. Objective: The objectives of the study were to quantify the impairment in HRQoL in migraine patients compared to healthy controls, to compare the prevalence of clinically significant anxiety and depressive symptoms in these groups, and to identify patient and headache characteristics that may predict health-related quality of life. Materials and Methods: We interviewed 71 consecutive newly diagnosed migraine patients seen in the headache clinic of a tertiary referral center between September and December 2008. Age- and sex-matched healthy subjects (n = 71) were used as controls. Short Form-36, Migraine Disability Assessment Score, and Hospital Anxiety and Depression Scale were administered. Predictors of HRQoL were identified using regression analysis. Results: Migraineurs were significantly impaired in all subscales of the SF-36 compared to controls, with greatest impairments in role physical, general health, and role emotional subscales. Prevalence of clinically significant anxiety (48%) and depressive (41%) symptoms in patients was higher than in healthy controls. Female gender, headache-related disability, and severity of anxiety predicted worse Physical Component Summary scores, while severity of both anxiety and depressive symptoms predicted worse Mental Component Summary scores. Conclusion: HRQoL is significantly reduced in Indian migraine patients compared to healthy controls. Incidence of clinically significant anxiety and depressive symptoms is also much higher in these patients. These findings corroborate well with studies from other parts of the world and suggest that cultural differences do not significantly alter the subjective impact of migraine on quality of life.

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Deep brain stimulation (DBS) is a major advancement in the field of functional neurosurgery in the last century. This treatment option is now utilized for many hyperkinetic and hypokinetic movement disorders and certain disorders in the field of psychiatry. The basis of treatment is a by-product of excellent advancement made in the field of basic neuroscience and technology. Due to the improvement in the field of neuroimaging, brain structures are now better localized leading to a better outcome. Newer sites of stimulations are being recognized, which may further improve the clinical outcome in patients. However, it is very important to stick to stringent inclusion and exclusion criteria while selecting patients for DBS to get the best results.

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Background: Spinal dural arteriovenous fistula (SDAVF) is a common type of spinal vascular lesion. However, there has not been any published study on its clinico-radiological characteristics or surgical outcome from India. Aim: The aim of this study was to determine the clinico-radiological features of patients with SDAVF, outcomes following surgical ligation of the fistula and the various factors involved. Materials and Methods: Patients who were operated for SDAVF were studied for demographic details, symptoms, clinical severity, radiological features and neurological outcome in the form of improvement in gait disability grades. Appropriate statistical tests were performed. Results: There were 22 (19 males, 3 females) patients of SDAVF who underwent surgical ligation with a mean age of 55 years. The mean duration of symptoms at presentation was 15 months. Three patients had acute onset while the rest had insidious onset of symptoms. Out of the 22 patients, 11 (50%) had motor weakness as the first symptom, 13 (59%) were bedridden and 19 (86.4%) had bladder involvement at presentation. Thirteen patients had fistulae in thoracic spine, whereas eight had fistulae in the lumbar spine. All had a favorable outcome in the form of at least non-progression of gait disability (14 had improvement while 8 had stabilized). The improvement was non-significantly associated with younger age, acute onset, ambulant status and fistula below T9. It was inversely associated with pain as the first symptom and fluctuant clinical course. Conclusion: Surgical occlusion of SDAVF is usually associated with either improvement or stabilization of motor weakness.

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Objective: To investigate the value of susceptibility weighted imaging (SWI) in evaluating the histopathologic grade of cerebral astrocytomas and compare the relative value of SWI and conventional magnetic resonance imaging (MRI) sequences. Materials and Methods: This is an analysis of 26 untreated patients with pathologically confirmed astrocytomas. The tumors were classified as low grade (grade I-II: 12 cases) or high grade (grade III-IV: 14 cases). Imaging was performed with a 3.0 T MRI scanner. Conventional sequences [T1-weighted imaging (T1WI), contrast enhanced T1WI (CE-T1WI), T2-weighted imaging (T2WI), and T2 FLuid Attenuated Inversion Recovery (T2FLAIR)] and SWI sequence (including CE-SWI) were done. The number of small vessels and the amount of blood products in the tumors were determined for each sequence. Differences between the two groups were analyzed statistically. Results: SWI was more sensitive than conventional sequences (T1WI, CE-T1WI, T2WI, and T2FLAIR) in visualizing small vessels and microhemorrhages in cerebral astrocytomas (P < 0.01). CE-SWI was better than CE-T1WI sequences for visualizing tumor small vessels and microhemorrhages. SWI visualized greater numbers of small vessels and areas of microhemorrhages in high-grade tumors than in low-grade tumors (P < 0.01). This was especially true after contrast administration (P < 0.01). Conclusion: SWI plays an important role in astrocytoma grading, especially for enhanced astrocytomas after contrast injection. CE-SWI was better than CE-T1WI in visualizing tumor architecture.

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A 43-year-old man presented with the symptoms of recurrent lower abdominal pain, malaise and loss of appetite of 3-week duration, followed by acute onset of generalized paresthesias, fever and headache which progressed over few days to quadriparesis, altered sensorium, urinary and fecal incontinence. He had consumed raw tongue, liver, gall bladder and testicles of monitor lizard (Varanus bengalensis). Blood picture showed eosinophilia and cerebrospinal fluid (CSF) analysis revealed elevated protein and eosinophilia. Serum and CSF serology was positive for angiostrongyliasis. Magnetic resonance imaging showed focal hyperintense lesions in the corpus callosum and brainstem and an enhancing lesion in the cerebellum. Post-contrast T1-weighted axial images of spine showed evidence of cervical cord hyperintense lesions and root enhancement. Susceptibility weighted images/phase images showed unusual feature of multiple hemorrhagic lesions in the posterior fossa and supratentorial areas. Diffusion showed no restriction of corpus callosal lesions. Patient was treated with the high dose parenteral steroids with albendazole and at 6-month follow-up and had a remarkable recovery.

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Compartment syndrome is a rare complication of spectrum of purple glove syndrome, seen as a side effect of intravenous phenytoin. This involves rapid discolouration of the involved limb along with edema and sometimes blistering of the skin also. Treatment is usually conservative and rarely requires surgery. We present a case of compartment syndrome following intravenous phenytoin administration and review of literature related to the case.

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Background and Aim: Intracranial arachnoid cysts (IAC) are benign congenital cystic lesions filled with cerebrospinal fluid (CSF). This study evaluated microsurgical craniotomy and endoscopy in the surgical treatment of IAC. Materials and Methods: Eight-one consecutive pediatric patients with IAC were surgically treated between January 2004 and January 2011. The surgical procedures included microsurgical craniotomy and endoscopy. Symptoms at presentation, location of IAC, surgical treatment options, and effectiveness were evaluated. Results: There were 43 males and 38 females and the mean age was 8.7 years (range between 1 month and 14 years) at the time of surgery. The cyst location was supratentorial in 72 patients and infratentorial in 9 patients, arachnoid cyst were identified. Follow-up period ranged between 2 and 8 years. Of the 49 patients with headache 83.67% of patients had cure and 10.2% had significant improvement. Of the eight patients with hydrocephalus and gait disturbances, six (75%) had complete total relief of symptoms and two (25%) patients had significant improvement. Four of the six patients with cognitive decline and weakness showed improvement. Of the 18 patients with epilepsy seizure freedom was: Engle class I grade I in 14 (77.78%) patients; class II in 2 (11.11%) patients; and class III in 2 (11.11%) patients. Follow-up studies from 2 to 8 years showed that headache was cured in 41 of the 49 cases (83.67%), significantly improved in 5 cases (10.20%), and showed no variation in 3 cases (6.12%). Hydrocephalus and gait disturbances were controlled in six of the eight cases (75.00%) and significantly improved in two cases (25.00%). Cognitive decline and weakness were obviously improved in four of the six cases (66.67%) and exhibited no variation in two cases (33.33%). According to the Engle standard, the following results were obtained from 18 patients with epilepsy: Grade I in 14 cases (77.78%); grade II in 2 cases (11.11%); and grade III in 2 cases (11.11%). Eleven cases with local or general enlarged skull exhibited no further progression. On follow-up computed tomography (CT) scan, there was variable alleviation of mass effect in all the 81 patients. Cystic size was significantly reduced in 65 patients with supratentorial arachnoid cysts and in 9 patients with infratentorial archnoid cysts. Twenty-one patients who had decreased skull thickness, had no further progression. Four patients who had cranioplasty had good outcome. Conclusion: The endoscopic approach was highly effective for most cases of IAC, particularly for cysts in the suprasellar and quadrigeminal regions as well as in the posterior fossa. Microsurgical craniotomy was recommended for IAC in the extracerebral convexity and intracerebrum. Local skull cranioplasty is needed for patients, or patients with preoperative diagnosis showed signs of cystic tumor and cyst-related epilepsy.

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Background: Guillain Barré Syndrome (GBS) has a variable clinical course. The influence of season on the rate of recovery has not been evaluated previously, despite documentation of seasonal variation in the occurrence of GBS. This study evaluated the influence of season on the rate of recovery from GBS. Materials and Methods: Records of 184 patients with GBS over a 10-year period were reviewed. Patients were divided into four groups depending on the date of admission: Q1 (March-May), Q2 (June-August), Q3 (September-November), and Q4 (December-February). Demographic characteristics and recovery characteristics (duration of mechanical ventilation, ICU and hospital stay, and time for recovery from the time of initiation of definitive therapy) were compared across the four quarters. Results: There was no significant difference in age, antecedent illnesses, treatment received, electrophysiological findings, and muscle power at admission across the four groups. Significant differences among various seasons were found with respect to duration of mechanical ventilation (23 ± 20, 36 ± 34, 27 ± 22, and 38 ± 28 days for Q1-Q4, respectively; P = 0.05), ICU stay (27 ± 22, 40 ± 37, 31 ± 23, and 43 ± 30 days for Q1-Q4, respectively; P = 0.05), hospital stay (42 ± 28, 55 ± 44, 47 ± 34, and 72 ± 54 days for Q1-Q4, respectively; P = 0.02), and time for recovery from treatment (15 ± 14, 29 ± 34, 18 ± 14, and 29 ± 20 days for Q1-Q4, respectively; P = 0.02). Conclusions: This study demonstrates a seasonal variation in the recovery of patients with GBS requiring mechanical ventilation. Patients admitted in Q1 have the fastest recovery and those in Q4 have the slowest recovery.

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Background and Objective: Previous genetic studies in Parkinson's disease (PD) have provided conclusive evidence for association of genes with strong biological rationale for PD. Recently several studies in different populations have found a strong association between idiopathic PD and the single-nucleotide polymorphism (SNP) rs2736990, located within an intron of the α-synuclein (SNCA) gene. In this study, we aimed to verify these findings and to explore the characteristic of the association in a subset of Chinese Han PD patients. Materials and Methods: A total of 515 unrelated patients with sporadic PD and 450 healthy ethnically matched control subjects were recruited consecutively for the study. Patients and healthy controls were genotyped for SNCA rs2736990 variant by polymerase chain reaction - ligase detection reaction. Results: Our data showed a significant association between the rs2736990 polymorphism and PD, the frequency of the allele C in PD patients was significantly higher than that in controls (P = 0.017, OR = 1.26, 95% confident intervals (CI) =1.04-1.51). The distribution of C > T genotypes was different between patients and controls (P = 0.027). Furthermore, allele C of SNP rs2736990 in early-onset PD was significantly more frequent than that in healthy controls (P = 0.007, odds ratio = 1.60, 95% CI = 1.13-2.26). Conclusions: Our study demonstrated that SNCA rs2736990 C > T polymorphism was associated with susceptibility to PD in Chinese Han population. Further studies are needed to replicate the association we found.

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