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author:("Biswas, atanr")
1.  Clinical profile and genetic correlation of patients with spinocerebellar ataxia: A study from a tertiary care centre in Eastern India 
Background:
Progressive cerebellar ataxia inherited by autosomal dominant transmission is known as Spino Cerebellar Ataxia (SCA).
Aims and Objectives:
To look for various clinical profile and molecular genetics of patients with SCAs and their phenotype-genotype correlation of patients with SCAs.
Materials and Methods:
This was a cross-sectional study conducted at Bangur Institute of Neurosciences, Kolkata from June 2010 to April 2013. We selected patients from the neurogenetic clinic of our institute and performed genetic test for SCA 1, 2, 3, 6 and 12. The diagnosis was based on suggestive clinical features and positive genetic study, done by polymerase chain reaction.
Results:
83 patients were tested for trineucleotide repeats and turned out 45 positive for the mentioned SCAs. We found 13(28.9%) SCA-1, 18(40%) SCA-2, 7(15.6%) SCA-3, 6(13.3) SCA-6 and 1(2.2%) SCA-12 patients. Half of the remaining 38 patients had positive family history. The mean age of onset were 38.46 years in SCA-1, 29.55 years in SCA-2, 38.43 years in SCA-3, 47.33 years in SCA-6. Slow saccades were observed in 7(53.8%) SCA-1, 17(94.4%) SCA-2, 4(57.1%) SCA-3, 3(50%) SCA-6 patients. Hyporeflexia was noticed in 5(27.8%) SCA-2 patients. Pyramidal tract involvement was found in 8(61.5%) SCA-1, 4(22.2%) SCA-2, 4(57.1%) SCA-3 and 1(16.7%) SCA-6 patients.
Conclusion:
Our study showed SCA-2 is the most common variety of SCA and genotypic-phenotypic correlation was observed in SCA-1,2,6 and 12 patients.
doi:10.4103/0972-2327.144005
PMCID: PMC4251010  PMID: 25506158
SCA; SCA genetics; spinocerebellar ataxia; spinocerebellar ataxia genetics
2.  Neuropsychiatric profiles in patients with Alzheimer's disease and vascular dementia 
Background/Aims:
The aim of the following study is to compare the behavioral and psychological symptoms of dementia (BPSD) in patients of Alzheimer disease (AD) and vascular dementia (VaD).
Materials and Methods:
We used National Institute of Neurological and Communicative Disorders and Stroke-Alzheimer's Disease and Related Disorders Association criteria for diagnosing AD and National Institute of Neurological Disorders and Stroke-Association International pour la Recherche et l’Enseignement en Neurosciences Criteria for diagnosing VaD. VaD cohort was further subcategorized into small vessel and large vessel disease. The severity of cognitive impairment and the BPSD were studied by means of the Clinical Dementia Rating Scale (CDR) and the Neuropsychiatric Inventory respectively.
Results:
We studied 50 AD and 50 VaD patients of whom 38 were small vessels and 12 were large vessels VaD. The severity of dementia was comparable in both groups. The agitation/aggression, depression/dysphoria, anxiety, apathy/indifference, irritability, aberrant motor behavior, appetite and eating behavior and night-time behaviors occurred significantly more frequently in patients with VaD than AD. We found a weak positive correlation between the CDR score and the number of neuropsychiatric symptoms per patient in both cohorts. Elation/euphoria, agitation/aggression was significantly more frequent in patients with large vessel in comparison to small vessel VaD.
Conclusions:
BPSD are common in both types of dementia and they are more severe in VaD than AD when the groups have similar levels of cognitive impairment.
doi:10.4103/0972-2327.138520
PMCID: PMC4162022  PMID: 25221405
Alzheimer disease; behavioral and psychological symptoms of dementia; neuropsychiatric inventory; small vessel disease; vascular dementia
3.  Adrenomyeloneuropathy with bulbar palsy: A rare association 
Adrenomyeloneuropathy (AMN) is a variant of adrenoleukodystrophy (ALD), an X-linked recessive peroxisomal disorder associated with accumulation of very long chain fatty acids (VLCFA). Mutations of this gene lead to abnormal peroxisomal β-oxidation, which results in the harmful accumulation of VLCFAs in affected cells. Neurological symptoms occur due to progressive demyelination and destruction of cerebral white matter and primary adrenal insufficiency. Bulbar palsy in a case of AMN is very unusual. We report a case of a 22-year-old male with AMN who developed adrenal insufficiency at the age of 4 years successfully treated by gluco- and mineralocorticoids followed by features of myeloneuropathy with bulbar palsy. AMN with prominent bulbar symptoms emphasizes the diverse clinical manifestation of this disease.
doi:10.4103/0972-2327.138530
PMCID: PMC4162032  PMID: 25221415
Adrenal insufficiency; adrenomyeloneuropathy; bulbar palsy
4.  Raised Serum Proinflammatory Cytokines in Alzheimer’s Disease with Depression 
Aging and Disease  2014;5(3):170-176.
The purpose of the present study was to identify the changes in the levels of proinflammatory cytokines like IL-1β, IL-6 and TNF-α in peripheral circulation in Alzheimer’s disease (AD) subjects and to correlate these with associated depression and cognitive deficit. Fifty five AD subjects and thirty seven age and sex matched controls were included in the study. The AD patients were grouped as AD with depression (n= 31) and AD without depression (n= 24). The serum levels of IL-1β, IL-6 and TNF-α were determined by immunoassay by commercially available kits. The serum levels of IL-6 and TNF-α were elevated in AD patients with depression compared to control (p<0.001) or AD without depression (p<0.001). The serum level of IL-1β was higher in AD patients with or without depression as compared to controls. Furthermore, a strong inverse correlation was observed between the MMSE scores and serum levels of IL-6 or TNF-α in AD subjects with depression. The study highlights the important role of peripheral IL-6 and TNF-α in AD associated depression and cognitive deficits.
doi:10.14336/AD.2014.0500170
PMCID: PMC4037308  PMID: 24900939
Alzheimer’s disease; IL-6; IL-1β; TNF-α; Depression; Cognitive deficit
5.  A comparative study of pre-operative oral clonidine and pregabalin on post-operative analgesia after spinal anesthesia 
Objectives:
Pregabalin and clonidine have anti-nociceptive properties. This study assesses their efficacy in prolonging the analgesic effect of spinal anesthesia and post-operative analgesic requirement in patients undergoing vaginal hysterectomy.
Materials and Methods:
A total of 90 females in the age group of 30-60 years were randomly allocated in to three groups of 30 each, to receive either oral clonidine (150 μg) or oral pregabalin (150 mg) or oral multivitamin as placebo 1.5 h before spinal anesthesia with 3ml (15 mg) of 0.5% hyperbaric bupivacaine. Intensity of pain was measured on a visual analog scale (VAS) at the end of operation (0 h) then at 1,2,4,6,12 and 24 h thereafter. Diclofenac sodium intramuscularly 1 mg/kg was provided when the VASscore was >4 in the study period. Sedation was defined by Ramsay sedation scale at 0,6,12 and 24 h. Side-effects such as nausea and vomiting, respiratory depression and dryness of mouth were noted.
Results:
The VAS scores were significantly less in the pregabalin group compared with the clonidine group at 6,12 and 24 h post-operatively with a P < 0.0001. More sedation was seen in the clonidine group than in the pregabalin group (P < 0.05). Analgesic consumption and VAS scores were lower in clonidine and pregabalin group compared with the placebo group (P < 0.05).
Conclusion:
Oral pregabalin (150 mg) prolongs the post-operative pain relief after spinal anesthesia but produces less sedation compared with oral clonidine (150 μg).
doi:10.4103/0259-1162.128907
PMCID: PMC4173598
Clonidine; post-operative analgesia; pregabalin; Ramsay sedation scale; vaginal hysterectomy; visual analog scale score
6.  Case series of probable sporadic Creutzfeldt-Jakob disease from Eastern India 
Background:
Creutzfeldt-Jakob disease is a rapidly progressive, fatal, transmissible neurodegenerative disorder caused by prion protein. It is still considered rare in countries like India. This is probably due to nonavailability of autopsy studies in majority of the center. The recent European diagnostic criterion for sporadic CJD (sCJD) is useful for making an early diagnosis.
Objective:
To report a series of patients of probable sCJD from a neurology institute of eastern India.
Materials and Methods:
Patients of rapidly developing dementia fulfilling the diagnostic criteria for sCJD were included. All were investigated in detail to find out any possible treatable cause including electroencephalography (EEG), magnetic resonance imaging (MRI) of brain, and cerebrospinal fluid analysis.
Results:
A total 10 patients of probable sCJD diagnosed using the European diagnostic criterion between December 2011 and January 2013. The clinical features are consistent with other reported series. While 60% of patients had the classical EEG findings, 100% had typical MRI features. Eight patients died within a mean duration of 4.56 months from the disease onset.
Conclusions:
The clinical features are similar to other reported series. Our observation raises question about the prevalence of this disease in India which needs more elaborate studies.
doi:10.4103/0972-2327.120486
PMCID: PMC3841621  PMID: 24339600
Creutzfeldt-Jakob disease; electroencephalography; magnetic resonance imaging
7.  Comparison between different tests and their combination for prediction of difficult intubation: An analytical study 
Context:
There is an impelling need for accurate tests to predict difficult intubation, as failure to achieve endotracheal intubation causes significant morbidity and mortality in anesthetic practice.
Aim:
To calculate the validity of the different tests along with their combination and agreement when compared with endotracheal intubation in predicting difficult intubation.
Settings and Design:
Operation theaters, analytical study.
Materials and Methods:
Three hundred patients aged between 16 and 60 years of American society of anesthesiologist (ASA) physical status I and II, scheduled for elective surgical procedures requiring endotracheal intubation were studied during January-July 2012. Mallampati grade (MP), sternomental distance (SMD), thyromental distance (TMD), and Delilkan and Calder test were recorded for every patient. Endotracheal intubation was performed by an experienced anesthesiologist blinded to the measurements and recorded grading of intubation. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), likelihood ratio (LR), odds ratio (OR), and kappa coefficient of tests individually and in combination were calculated.
Statistical Analysis Used:
IBM SPSS software (version 16.0) and Epi-info software (version 3.2).
Results:
Difficult and failed intubation was 13.3% and 0.6%, respectively. Difficult intubation increased with age. TMD and Calder test showed highest sensitivity individually and Dellilkan's test showed least sensitivity. Among the combination of tests, MP with SMD and MP with Calder test had the highest sensitivity.
Conclusion:
Among individual test TMD and Calder are better predictive tests in terms of sensitivity. Combination of tests increases the chance of prediction of difficult intubation.
doi:10.4103/0259-1162.114014
PMCID: PMC4173503
Delilkan and Calder test; difficult intubation; Mallampati grade; sternomental and thyromental distance
8.  Seizure, spinal schwannoma, peripheral neuropathy and pulmonary stenosis – A rare combination in a patient of Neurofibromatosis 1 
Neurofibromatosis 1 (NF1) is the most common neurocutaneous syndrome. It is estimated to occur in approximately 1 out of every 3300 infants. The manifestations of this condition are diverse and can arise from almost any system in the body. The neurofibroma is the hallmark lesion of NF1 that develops from peripheral nerves. Here, we are reporting an 18-year-old girl with NF1. Clinical diagnosis was made according to the diagnostic criteria established by the National Institutes of Health Consensus Development Conference in 1987. She presented with quadriparesis due to dumbbell-shaped spinal schwannoma in the cervical region. She had history of recurrent seizures in the past, with poor scholastic performance. There were clinical and electrophysiological features of peripheral neuropathy and clinical and echocardiographical features of pulmonary stenosis. These are uncommon features of NF 1. The presence of all these features in a single patient makes it a unique case.
doi:10.4103/0972-2327.93281
PMCID: PMC3299073  PMID: 22412275
Epilepsy; neurofibromatosis 1; neuropathy; pulmonary stenosis; spinal tumor
9.  Use of Sugar on the Healing of Diabetic Ulcers: A Review 
With the advent of several innovative wound care management tools, the choice of products and treatment modalities available to clinicians continues to expand. High costs associated with wound care, especially diabetic foot wounds, make it important for clinician scientists to research alternative therapies and optimally incorporate them into wound care protocols appropriately. This article reviews using sugar as a treatment option in diabetic foot care and provides a guide to its appropriate use in healing foot ulcers. In addition to a clinical case study, the physiological significance and advantages of sugar are discussed.
PMCID: PMC2956799  PMID: 20920433
diabetic foot ulcers; sugar; wound healing
10.  The Micrograft Concept for Wound Healing: Strategies and Applications 
The standard of care for wound coverage is to use an autologous skin graft. However, large or chronic wounds become an exceptionally challenging problem especially when donor sites are limited. It is important that the clinician be aware of various treatment modalities for wound care and incorporate those methods appropriately in the proper clinical context. This report reviews an alternative to traditional meshed skin grafting for wound coverage: micrografting. The physiological concept of micrografting, along with historical context, and the evolution of the technique are discussed, as well as studies needed for micrograft characterization and future applications of the technique.
PMCID: PMC2909510  PMID: 20663442
diabetic foot ulcers; micrografting; wound healing

Results 1-10 (10)