Annals of Indian Academy of Neurology
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   2015| April-June  | Volume 18 | Issue 2  
    Online since May 8, 2015

 
 
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REVIEW ARTICLES
Toxic species in amyloid disorders: Oligomers or mature fibrils
Meenakshi Verma, Abhishek Vats, Vibha Taneja
April-June 2015, 18(2):138-145
DOI:10.4103/0972-2327.144284  PMID:26019408
Protein aggregation is the hallmark of several neurodegenerative disorders. These protein aggregation (fibrillization) disorders are also known as amyloid disorders. The mechanism of protein aggregation involves conformation switch of the native protein, oligomer formation leading to protofibrils and finally mature fibrils. Mature fibrils have long been considered as the cause of disease pathogenesis; however, recent evidences suggest oligomeric intermediates formed during fibrillization to be toxic. In this review, we have tried to address the ongoing debate for these toxic amyloid species. We did an extensive literature search and collated information from Pubmed (http://www.ncbi.nlm.nih.gov) and Google search using various permutations and combinations of the following keywords: Neurodegeneration, amyloid disorders, protein aggregation, fibrils, oligomers, toxicity, Alzheimer's Disease, Parkinson's Disease. We describe different instances showing the toxicity of mature fibrils as well as oligomers in Alzheimer's Disease and Parkinson's Disease. Distinct structural framework and morphology of amyloid oligomers suggests difference in toxic effect between oligomers and fibrils. We highlight the difference in structure and proposed toxicity pathways for fibrils and oligomers. We also highlight the evidences indicating that intermediary oligomeric species can act as potential diagnostic biomarker. Since the formation of these toxic species follow a common structural switch among various amyloid disorders, the protein aggregation events can be targeted for developing broad-range therapeutics. The therapeutic trials based on the understanding of different protein conformers (monomers, oligomers, protofibrils and fibrils) in amyloid cascade are also described.
  5,916 120 2
HOW I DO IT
A practical approach to management of focal hand dystonia
Sanjay Pandey
April-June 2015, 18(2):146-153
DOI:10.4103/0972-2327.156563  PMID:26019409
Dystonia can be focal, segmental, multifocal, generalized, or hemidystonia. Focal dystonia is localized to a specific part of the body. Overall upper limb is more commonly involved in focal dystonia than lower limb and since it starts from hand, focal hand dystonia (FHD) is a more accepted terminology. Writer's cramp and musician dystonia are commonest types of FHD. Typically this dystonia is task specific, but in some patients this specificity may be lost over a period of time. Segmental or generalized dystonia may also start as FHD, so a detailed clinical assessment is required, which should be supplemented by relevant investigations. Treatment includes oral medications, injection botulinum toxin, neurosurgery including neurostimulation, and rehabilitation. Role of injection botulinum toxin has been extensively studied in writer's cramp patients and found to be effective; however, selection of muscles and techniques of injection are crucial in getting best results.
  5,325 300 -
CLINICAL SIGN
Lhermitte's Sign: The Current Status
Supreet Khare, Deeksha Seth
April-June 2015, 18(2):154-156
DOI:10.4103/0972-2327.150622  PMID:26019410
Lhermitte's sign was described by Marie and Chatelin and named after Jean Lhermitte. This sign is mostly described as an electric shock like condition by some patients of multiple sclerosis. This sensation occurs when the neck is moved in a wrong way or rather flexed. It can also travel down to the spine, arms, and legs, and sometimes the trunk. Demyelination and hyperexcitability are the main pathophysiological reasons depicted for the Lhermitte's sign. Other causes for Lhermitte's sign include transverse myelitis, behηet's disease, trauma, etc. This article reviews the Lhermitte's sign, its history, and its etiopathophysiology. Very few studies are available on Lermitte's sign and more research need to be done on the same to ensure its sensitivity and specificity.
  2,856 145 -
SHORT COMMUNICATION
Mild serotonin syndrome: A report of 12 cases
Sanjay Prakash, Varsha Patel, Siddharth Kakked, Indravadan Patel, Rahul Yadav
April-June 2015, 18(2):226-230
DOI:10.4103/0972-2327.150612  PMID:26019424
Serotonin syndrome (SS) is an under diagnosed and under reported condition. Mild SS is easily overlooked by physicians. Every patient with mild SS is a potential candidate for developing life-threatening severe SS because of inadvertent overdose or the addition of the second serotonergic drug. Herein, we describe 12 patients with mild SS observed over 12 months in neurology outpatient clinic. It is a retrospective chart review of 12 consecutive patients who had hyperreflexia with tremor (defined as mild SS Hunter's criteria) and had received serotonergic agents in the past 5 weeks. Only four patients (33%) reported tremor as a presenting or main feature. The presenting features in another eight patients were: Dizziness, generalized body pain, headache, and seizure. Five patients responded to the removal of the offending agents and got a complete response in 2-7 days. There were no or minimal responses in another seven patients to the removal of the serotonergic drugs. Cyproheptadine was started in these patients, at the dose of 8 mg three times daily. Response started within 1-3 days of initiation of the drug and the complete responses were noted in 5-14 days. There were no side effects from cyproheptadine in any patient. We suggest that any patient on serotonergic drug developing new symptoms should be examined for the presence of tremor, hypertonia, hyperreflexia, and clonus to look for mild SS. In addition, every patient on any serotonergic drug should be examined for the presence of mild SS before escalating the dose or before adding a new one.
  2,881 95 1
ORIGINAL ARTICLES
Comparison of high-resolution sonography and electrophysiology in the diagnosis of carpal tunnel syndrome
Meena Angamuthu Kanikannan, Demudu Babu Boddu, Umamahesh , Sailaja Sarva, Padmaja Durga, Rupam Borgohain
April-June 2015, 18(2):219-225
DOI:10.4103/0972-2327.150590  PMID:26019423
Background: The diagnostic accuracy of high-resolution ultrasonography (HRUS) in comparison to electro-diagnostic testing (EDX) in carpal tunnel syndrome (CTS) is debatable. Objective: The aim of this study was to compare the diagnostic accuracy of HRUS with EDX in patients with various grades of CTS and CTS associated with peripheral neuropathy (CTS + PNP). Materials and Methods: A prospective cohort of 57 patients with possible CTS was studied along with matched controls. The cross-sectional area (CSA) of the median nerve at the inlet of carpal tunnel was assessed by a sonologist blinded to the clinical and EDX data. Palm wrist distal sensory latency difference (PWDSLD), second lumbrical-interosseus distal motor latency difference (2LIDMLD) and CSA were compared in patients with different grades of severity of CTS and CTS+PNP. Results: Total 92 hands of 57 patients met the clinical criteria for CTS. Mean CSA at the inlet of carpal tunnel was 0.11 ± 0.0275 cm 2 . It had the sensitivity, specificity, positive predictive value and negative predictive values of 76.43%, 72.72%, 89.47% and 68%, respectively (P < 0.0001). Overall, HRUS had good correlation with PWDSLD and 2LIDMLD electro-diagnostic studies in all grades of CTS and CTS + PNP. Conclusion: HRUS can be used as a complementary screening tool to EDX. However, EDX has been found to be more sensitive and specific in mild CTS.
  2,805 87 1
CASE REPORTS
Fasciculations masquerading as minipolymyoclonus in bulbospinal muscular atrophy
Sushanth Bhat, Wei Ma, Elena Kozochonok, Sudhansu Chokroverty
April-June 2015, 18(2):249-251
DOI:10.4103/0972-2327.150624  PMID:26019432
Minipolymyoclonus has been described in both anterior horn cell disorders and central nervous system degenerative conditions. While its etiology remains unclear and speculative, a central generator has been previously proposed. We describe a case of bulbospinal muscular atrophy (Kennedy's disease), where minipolymyoclonus-like movements corresponded to fasciculations in neurophysiological studies. Our novel finding suggests that the etiologies of minipolymyoclonus in central and peripheral nervous system disorders are distinct, despite outward clinical similarity. The term "minipolyfasciculations" may be more reflective of the underlying process causing minipolymyoclonus-like movements in lower motor neuron disorders.
  2,770 63 -
ORIGINAL ARTICLES
Guillain-Barré syndrome complicating pregnancy and correlation with maternal and fetal outcome in North Eastern India: A retrospective study
Shri Ram Sharma, Nalini Sharma, Hussain Masaraf, Santa A Singh
April-June 2015, 18(2):215-218
DOI:10.4103/0972-2327.150608  PMID:26019422
Background: Guillain-Barré syndrome (GBS) is rare in pregnancy with an estimated incidence between 1.2 and 1.9 cases per 100,000 people annually, and it is generally accepted that it carries a high maternal risk. Most reports of GBS with pregnancy are case reports only. Aim: Purpose of this retrospective study was to find the correlation between pregnancy and GBS. Settings and Design: Records of patients admitted in neurology division were analyzed in a tertiary care teaching hospital in the northeastern Indian pregnant female population with GBS between 15-49 years during the period of 2009-2013. Materials and Methods: We analyzed the records of 47 patients with pregnancy and GBS, evaluated and treated in our institute from August 2009 to December 2013. This is retrospective observational study done in North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences (NEIGRIHMS), India. Result: Predominant form of GBS was acute inflammatory demyelinating polyneuropathy (AIDP). The weakness started from the lower limbs in majority of patients. Ten percent of women had bifacial weakness. Most of patients had good maternal and fetal outcome. Two patients received intravenous immunoglobulin (IVIG). Only two patient required ventilator supports and one patient had intrauterine death (IUD) and died due to respiratory failure. Conclusion: Our results indicate that risk of GBS increases in third trimester and first 2 weeks after delivery. Demyelinating variety of GBS was common in our population. GBS natural course during pregnancy is mild and showed quick recovery. Maternal and perinatal outcome was good.
  2,577 85 -
Utility of cerebrospinal fluid cortisol level in acute bacterial meningitis
Anish Mehta, Rohan R Mahale, Uchil Sudhir, Mahendra Javali, Rangasetty Srinivasa
April-June 2015, 18(2):210-214
DOI:10.4103/0972-2327.150626  PMID:26019421
Background: Meningitis remains a serious clinical problem in developing as well as developed countries. Delay in diagnosis and treatment results in significant morbidity and mortality. The role and levels of intrathecal endogenous cortisol is not known. Objective: To study the cerebrospinal fluid (CSF) cortisol levels and to evaluate its role as a diagnostic and therapeutic marker in acute bacterial meningitis. Materials and Methods: Thirty patients with acute bacterial meningitis with no prior treatment were evaluated. Cortisol levels were compared with 20 patients with aseptic (viral) meningitis and 25 control subjects. Results: Mean CSF cortisol level was 13.85, 3.47, and 1.05 in bacterial meningitis, aseptic meningitis, and controls, respectively. Mean CSF cortisol level in bacterial meningitis was significantly higher as compared to controls (P < 0.001). There was significant difference in CSFcortisol levels in bacterial and aseptic meningitis (P < 0.001). Conclusions: Cortisol levels in CSF are highly elevated in patients with acute bacterial meningitis. This suggests that intrathecalcortisol may serve as a valuable, rapid, relatively inexpensive diagnostic marker in discriminatingbetween bacterial and aseptic meningitis. This helps in earlier institution of appropriate treatment and thereby decreasing morbidity and mortality.
  2,413 127 -
Factors delaying hospital arrival of patients with acute stroke
VV Ashraf, M Maneesh, R Praveenkumar, K Saifudheen, AS Girija
April-June 2015, 18(2):162-166
DOI:10.4103/0972-2327.150627  PMID:26019412
Background: Low rates of thrombolysis for ischemic stroke in India and other developing countries have been attributed to delays in presentation to the hospital. Materials and Methods: A prospective study was carried out during a 12-month period ending December 2012 in the department of Neurology, Malabar Institute of Medical Sciences, Kerala, India, to look for the factors contributing to delay in hospital arrival of patients with acute stroke. Patients and or their relatives were interviewed within 48 hours of admission using a structured questionnaire. Results: A total of 264 patients attending the emergency department were included. There were 170 men and 94 women. The mean age was 61.5 ± 12.4 years. A total of 67 (25%) patients presented within 4 hours of stroke onset. Factors associated with early arrival (multivariate logistic regression analysis) were distance 15 km or less from hospital (P 0.03, odds ratio (OR) 2.7), directly reaching the stroke department (P < 0.001, OR 9.7), history of coronary artery disease (P 0.001, OR 3.84), higher educational status (P 0.001, OR 3.7), and presence of hemiplegia (P 0.001, OR 5.5). Conclusions: We found a considerable delay in the early arrival of patients to our stroke department. Health promotion strategies to improve community awareness of early symptoms of stroke, education of local physicians about the importance of early referrals to the stroke centers, and wider availability and use of ambulance services are promising methods to help expedite presentation to hospital post stroke and thereby improve the management of stroke in India.
  2,412 121 -
Neuropsychological markers of mild cognitive impairment: A clinic based study from urban India
Ravikesh Tripathi, Keshav Kumar, Rakesh Balachandar, P Marimuthu, Mathew Varghese, Srikala Bharath
April-June 2015, 18(2):177-180
DOI:10.4103/0972-2327.150566  PMID:26019415
Background: Mild cognitive impairment (MCI) is a transitional stage between normal aging and dementia. Persons with MCI are at higher risk to develop dementia. Identifying MCI from normal aging has become a priority area of research. Neuropsychological assessment could help to identify these high risk individuals. Objective: To examine clinical utility and diagnostic accuracy of neuropsychological measures in identifying MCI. Materials and Methods: This is a cross-sectional study of 42 participants (22 patients with MCI and 20 normal controls [NC]) between the age of 60 and 80 years. All participants were screened for dementia and later a detailed neuropsychological assessment was carried out. Results: Persons with MCI performed significantly poorer than NC on word list (immediate and delayed recall), story recall test, stick construction delayed recall, fluency and Go/No-Go test. Measures of episodic memory especially word list delayed recall had the highest discriminating power compared with measures of semantic memory and executive functioning. Conclusion: Word list learning with delayed recall component is a possible candidate for detecting MCI from normal aging.
  2,396 128 -
IMAGES IN NEUROLOGY
Mineralizing angiopathy with basal ganglia stroke in an infant
Puneet Jain, Praveen Kishore, Jasjit Singh Bhasin, Subhash Chand Arya
April-June 2015, 18(2):233-234
DOI:10.4103/0972-2327.150619  PMID:26019426
Basal ganglia stroke is known following trivial head trauma. Recently a distinct clinic-radiological entity termed 'mineralizing angiopathy' was described. We report an infant who developed basal ganglia stroke following trivial fall. His clinic-radiological features are described.
  2,400 85 -
CASE REPORTS
Utilization behavior
Sanjay Pandey, Neelav Sarma
April-June 2015, 18(2):235-237
DOI:10.4103/0972-2327.150613  PMID:26019427
Utilisation Behaviour (UB) denotes the appropriate usage of an object by a patient, however at an inappropriate situation. Patients are compelled by the visual, visual-tactile presentation to grasp and use the object kept in front of them. UB is observed in patients having unilateral or bilateral frontal lesions. It is considered to be an extension of magnetic apraxia, which is defined as bilateral manual grasping behavior. The proposed hypothesis is that in frontal lesions there is suppression of the inhibitory effect of frontal lobe on parietal lobe. Neurological models to explain this phenomenon to occur as a result of loss of inhibitory biological system within the brain. A disturbance of balance between the patient's dependence on and independence from the outside world is believed to be present in such cases. From a cognitive point of view, three models have been described. Since its first description by Lhermitte in 1983, UB remains to be an interesting neurological phenomenon. We present a case of UB in a patient of fronto-temporal dementia and also summarise the neurological and cognitive models that explain this behaviour.
  2,299 77 -
REVIEW ARTICLES
Association between myasthenia gravis and cognitive function: A systematic review and meta-analysis
Zhifeng Mao, Junjie Yin, Zhengqi Lu, Xueqiang Hu
April-June 2015, 18(2):131-137
DOI:10.4103/0972-2327.156560  PMID:26019407
The course of myasthenia gravis (MG) is complicated by increased reports of cognitive defects in both human and animal models, which suggests potential central nervous system (CNS) damage. We conducted a systematic review of the relationships between MG and cognitive function. This systematic review followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Major databases were searched to examine the neuropsychological studies of adults with MG. Weighted effect sizes were pooled by cognitive domain. Eight studies representing 300 subjects were included. Eight cognitive domain categories were identified: (i) Mini-Mental State Examination (MMSE), (ii) language, (iii) processing speed, (iv) verbal learning and memory, (v) visual learning and memory, (vi) attention span, (vii) response fluency, and (viii) motor performance. Nine (cognitive domain categories, MMSE, language, processing speed, verbal learning and memory (except for delayed recall memory), and motor performance) of 16 cognitive tasks revealed significant moderate effect sizes. Verbal logical-delayed memory, finger tapping with the preferred hand, and the Symbol Digit Modalities Test showed a greater magnitude relationship to cognitive function than did other specific cognitive domains. Verbal learning and memory seems to be the most significant affected according to cognitive domain categories. For MG, the ability of attention, response fluency, visual learning, and memory seems to be reserved. The MG patients seem to perform significantly worse than the non-MG controls in a range of cognitive domains. Our findings should be interpreted with caution because of the clinical and methodological heterogeneity of included studies.
  2,166 97 -
HISTORY OF MEDICINE
Famous people with Tourette's syndrome: Dr. Samuel Johnson (yes) & Wolfgang Amadeus Mozart (may be): Victims of Tourette's syndrome?
Kalyan B Bhattacharyya, Saurabh Rai
April-June 2015, 18(2):157-161
DOI:10.4103/0972-2327.145288  PMID:26019411
Tourette's syndrome is a clinical condition characterized by multiple motor tics and vocal tics which occurs in the age range 5-25 years and the intensity of the symptoms changes with time. It is felt that at least two remarkable personalities namely, Dr. Samuel Johnson from England, a man of letters and the compiler of the first ever English dictionary, and Wolfgang Amadeus Mozart from Austria, one of the greatest musical genius of all time, possibly suffered from this condition. Tourette's syndrome is often described as the classical borderzone between neurology and psychiatry and every neurologist wonders at the curious and fascinating clinical features of this condition. It seems that at least two remarkable personalities, Dr. Samuel Johnson, a man of letters and the first person to compile an English dictionary, and Wolfgang Amadeus Mozart, arguably the most creative musical composer of all time, were possibly afflicted with this condition.
  2,145 62 -
CASE REPORTS
Mirror movements in progressive hemifacial atrophy
Rajesh Verma, Puneet Kumar Dixit, Rakesh Lalla, Babita Singh
April-June 2015, 18(2):246-248
DOI:10.4103/0972-2327.150606  PMID:26019431
Mirror movements are simultaneous, involuntary, identical movements occurring during contralateral voluntary movements. These movements are considered as soft neurologic signs seen uncommonly in clinical practice. The mirror movements are described in various neurological disorders which include parkinsonism, cranio veretebral junction anamolies, and hemiplegic cerebral palsy. These movements are intriguing and can pose significant disability. However, no such observation regarding mirror movements in progressive hemifacial atrophy have been reported previously. We are reporting a teenage girl suffering from progressive hemifacial atrophy and epilepsy with demonstrable mirror movements in hand.
  2,118 74 2
ORIGINAL ARTICLES
Neurological associations in auditory neuropathy spectrum disorder: Results from a tertiary hospital in South India
Anjali Lepcha, Reni K Chandran, Mathew Alexander, Ann Mary Agustine, K Thenmozhi, Achamma Balraj
April-June 2015, 18(2):171-180
DOI:10.4103/0972-2327.150578  PMID:26019414
Aims: To find out the prevalence and types of neurological abnormalities associated in auditory neuropathy spectrum disorder in a large tertiary referral center. Settings and Design: A prospective clinical study was conducted on all patients diagnosed with auditory neuropathy spectrum disorder in the ear, nose, and throat (ENT) and neurology departments during a 17-month period. Patients with neurological abnormalities on history and examination were further assessed by a neurologist to determine the type of disorder present. Results: The frequency of auditory neuropathy spectrum disorder was 1.12%. Sixty percent were found to have neurological involvement. This included cerebral palsy in children, peripheral neuropathy (PN), spinocerebellar ataxia, hereditary motor-sensory neuropathy, spastic paresis, and ponto-bulbar palsy. Neurological lesions did not present simultaneously with hearing loss in most patients. Sixty-six percent of patients with auditory neuropathy spectrum disorder were born of consanguineous marriages. Conclusions: There is a high prevalence of neurological lesions in auditory neuropathy spectrum disorder which has to be kept in mind while evaluating such patients. Follow-up and counselling regarding the appearance of neuropathies is therefore important in such patients. A hereditary etiology is indicated in a majority of cases of auditory neuropathy spectrum disorder.
  2,098 79 -
CASE REPORTS
Polyneuritis cranialis with generalized hyperreflexia as a presenting manifestation of thyrotoxicosis
Gaurav M Kasundra, Amita Narendra Bhargava, Bharat Bhushan, Khichar Shubhakaran, Isha Sood
April-June 2015, 18(2):240-242
DOI:10.4103/0972-2327.150625  PMID:26019429
A 22-year-old male student with no past medical illness, presented with acute onset dysarthria, binocular diplopia, and dysphagia over 10 hours. On examination, he had tachycardia, hypertension, generalized hyper-reflexia, and bilateral pupil sparing oculomotor, troclear, abducens, trigeminal, facial, glossopharyngeal, and vagus nerve palsy. Rest examination was unremarkable. Facial nerve conduction study (NCS) showed decreased amplitude bilaterally and neurogenic pattern on electromyography. Limb NCS, repetitive nerve stimulation, neostigmine test, brain magnetic resonance imaging, cerebrospinal fluid, and biochemical tests were normal. Only positive tests were low thyroid-stimulating hormone (TSH) (<0.01), high free T3 (19.2 pmol/L), and high free T4 (39.2 pmol/L). Thyroid ultrasonography, anti-thyroid peroxidase, and anti-thyroglobulin antibody were normal. Patient was treated with anti-thyroid drugs, with which he completely recovered in 2 months. Though many cases with thyrotoxic myopathy have been reported, only few mention neuropathic cause of dysphagia or polyneuritis cranialis. Getting done thyroid function tests may be helpful in patients with polyneuritis cranialis of uncertain etiology.
  2,112 62 -
ORIGINAL ARTICLES
Preservative solution for skeletal muscle biopsy samples
Yasemin Gulcan Kurt, Bulent Kurt, Omer Ozcan, Turgut Topal, Abdullah Kilic, Tuba Muftuoglu, Cengizhan Acikel, Kenan Sener, Fatih Sahiner, Nuri Yigit, Ibrahim Aydin, Semih Alay, Safak Ekinci
April-June 2015, 18(2):187-193
DOI:10.4103/0972-2327.150601  PMID:26019417
Context : Muscle biopsy samples must be frozen with liquid nitrogen immediately after excision and maintained at -80 o C until analysis. Because of this requirement for tissue processing, patients with neuromuscular diseases often have to travel to centers with on-site muscle pathology laboratories for muscle biopsy sample excision to ensure that samples are properly preserved. Aim: Here, we developed a preservative solution and examined its protectiveness on striated muscle tissues for a minimum of the length of time that would be required to reach a specific muscle pathology laboratory. Materials and Methods: A preservative solution called Kurt-Ozcan (KO) solution was prepared. Eight healthy Sprague-Dawley rats were sacrificed; striated muscle tissue samples were collected and divided into six different groups. Muscle tissue samples were separated into groups for morphological, enzyme histochemical, molecular, and biochemical analysis. Statistical method used: Chi-square and Kruskal Wallis tests. Results: Samples kept in the KO and University of Wisconsin (UW) solutions exhibited very good morphological scores at 3, 6, and 18 hours, but artificial changes were observed at 24 hours. Similar findings were observed for the evaluated enzyme activities. There were no differences between the control group and the samples kept in the KO or UW solution at 3, 6, and 18 hours for morphological, enzyme histochemical, and biochemical features. The messenger ribonucleic acid (mRNA) of β-actin gene was protected up to 6 hours in the KO and UW solutions. Conclusion: The KO solution protects the morphological, enzyme histochemical, and biochemical features of striated muscle tissue of healthy rats for 18 hours and preserves the mRNA for 6 hours.
  2,047 54 -
CASE REPORTS
Cerebral fat embolism: Use of MR spectroscopy for accurate diagnosis
Laxmi Kokatnur, Mohan Rudrappa, Khaled R Khasawneh
April-June 2015, 18(2):252-255
DOI:10.4103/0972-2327.150604  PMID:26019433
Cerebral fat embolism (CFE) is an uncommon but serious complication following orthopedic procedures. It usually presents with altered mental status, and can be a part of fat embolism syndrome (FES) if associated with cutaneous and respiratory manifestations. Because of the presence of other common factors affecting the mental status, particularly in the postoperative period, the diagnosis of CFE can be challenging. Magnetic resonance imaging (MRI) of brain typically shows multiple lesions distributed predominantly in the subcortical region, which appear as hyperintense lesions on T2 and diffusion weighted images. Although the location offers a clue, the MRI findings are not specific for CFE. Watershed infarcts, hypoxic encephalopathy, disseminated infections, demyelinating disorders, diffuse axonal injury can also show similar changes on MRI of brain. The presence of fat in these hyperintense lesions, identified by MR spectroscopy as raised lipid peaks will help in accurate diagnosis of CFE. Normal brain tissue or conditions producing similar MRI changes will not show any lipid peak on MR spectroscopy. We present a case of CFE initially misdiagnosed as brain stem stroke based on clinical presentation and cranial computed tomography (CT) scan, and later, MR spectroscopy elucidated the accurate diagnosis.
  2,018 55 1
LETTERS TO THE EDITOR
Early detection of adult onset Susac's syndrome in a South Indian female
George Kovoor, Krishnan Parameswaran, Rajeev Rajasekharan, Kamble J Harsha, Akash K George
April-June 2015, 18(2):257-259
DOI:10.4103/0972-2327.150609  PMID:26019436
  1,984 81 -
ORIGINAL ARTICLES
Clinico pathological study of adult dermatomyositis: Importance of muscle histology in the diagnosis
Sudhir Babu Karri, Meena Anga Muthu Kannan, Liza Rajashekhar, Megha S Uppin, Sundaram Challa
April-June 2015, 18(2):194-199
DOI:10.4103/0972-2327.150603  PMID:26019418
Aims: To study the histological features on muscle biopsy and correlate them with clinical features, other laboratory data in adult patients to make a diagnosis of dermatomyositis (DM), applying the European Neuromuscular center (ENMC) criteria. Materials and Methods: Adult patients who fulfilled clinical, laboratory, and muscle biopsy findings according to ENMC criteria for DM during the period 2010-2013 were included in the study. Cryostat sections of muscle biopsy were reviewed with emphasis on Perifascicular atrophy (PFA), perivascular/endomysial inflammation. Muscular dystrophies and metabolic myopathies were excluded by appropriate immunohistochemistry and special stains. Results: The diagnosis of adult DM was made in 45 patients out of 170 clinically suspected idiopathic inflammatory myopathies. These included 33 definite, 4 probable, 7 possible sine dermatitis, and 1 amyopathic DM. All patients with definite DM had typical rash and proximal muscle weakness and muscle biopsy showed PFA with or without inflammation. Thirteen patients had quadriparesis, neck muscle weakness, dysphagia/dysphonia at presentation. Patients with probable DM had rash and showed perivascular/endomysial inflammation with no PFA. Possible DM sine dermatitis showed PFA with perivascular/endomysial infiltrates. One patient of amyopathic DM had typical heliotrope rash and characteristic skin biopsy. Conclusions: Histological features are important for the diagnosis of DM. Relying on PFA for diagnosis of definite DM underestimates the true frequency of DM.
  1,952 72 -
Cognitive profiles in Mild Cognitive Impairment (MCI) patients associated with Parkinson's disease and cognitive disorders
Michele Pistacchi, Manuela Gioulis, Franco Contin, Flavio Sanson, Sandro Zambito Marsala
April-June 2015, 18(2):200-205
DOI:10.4103/0972-2327.150611  PMID:26019419
Background: Mild cognitive impairment (MCI) is rapidly becoming one of the most common clinical manifestations affecting the elderly and represents an heterogeneous clinical syndrome that can be ascribed to different etiologies; the construct of MCI in Parkinson's disease (PD) (MCI-PD) is more recent but the range of deficits is still variable. Early recognition and accurate classification of MCI-PD could offer opportunities for novel therapeutic interventions to improve the natural pathologic course. Objective: To investigate the clinical phenotype of amnestic mild cognitive impairment (aMCI) and in patients with PD and MCI (MCI-PD). Materials and Methods: Seventy-three patients with aMCI and in 38 patients with MCI-PD were enrolled. They all underwent Mini-mental State Examination (MMSE), the Rey auditory-verbal learning test and the immediate visual memory (IVM) item of the Mental Deterioration Battery, the Rey auditory-verbal learning test included the Rey-immediate (Rey-I), and the delayed recall of the word list (Rey test deferred, Rey-D). The Geriatric Depression Scale (GDS) was used for mood assessment. Results: The results of the Rey-I and Rey-D and of the IVM item showed statistically significant differences between the aMCI and the MCI-PD group. The mean Rey-I and Rey-D score was significantly lower as well as the IVM score was higher in patients with aMCI than in those with MCI-PD, aMCI patients showed greater impairment in long-term memory, whereas more aMCI than MCI-PD patients had preserved attention, computation, praxis, and conceptualization. Conclusions: Our findings demonstrate that the cognitive deficit profile is specific for each of the two disorders: Memory impairment was a typical feature in aMCI patients while MCI-PD patients suffered from executive functions and visuospatial attention deficits.
  1,923 55 -
Methotrexate-induced chemical meningitis in patients with acute lymphoblastic leukemia/lymphoma
Linu A Jacob, Aparna Sreevatsa, Lakshmaiah K Chinnagiriyappa, Lokanatha Dasappa, TM Suresh, Govind Babu
April-June 2015, 18(2):206-209
DOI:10.4103/0972-2327.150586  PMID:26019420
Background: Intrathecal methotrexate (ITMTX) is an important component in the treatment as well as prophylaxis of leukemia/lymphoma. ITMTX can cause chemical meningitis characterized by vomiting, headache, and fever lasting 2-5 days with spontaneous resolution of symptoms which differentiates this syndrome from bacterial meningitis. Objective: This prospective observational study was carried out to determine incidence of post-ITMTX syndrome in patients receiving prophylactic ITMTX as part of Berlin-Frankfurt-Munster (BFM) protocol. Materials and Methods: Patients aged 15-50 years receiving BFM 90 or BFM 95 protocol for acute lymphoblastic leukemia or lymphoblastic lymphoma were followed up for post-ITMTX syndrome, defined as vomiting, headache and fever between 38 o and 39 o C following ITMTX. Results: Thirty-three patients received a total of 297 courses of ITMTX. Of the 297 doses of ITMTX, 20 episodes (6.7%) of post-ITMTX syndrome were observed. The incidence of post-ITMTX syndrome was highest after the second dose of ITMTX (24%). The most common symptom of post-ITMTX syndrome was headache which was seen in 17 (85%) patients. Seventeen (85%) patients had vomiting, 10 (50%) patients had fever, and 4 (20%) patients had backache. Meningeal signs were present in 2 (10%) patients. Conclusions: Post-ITMTX syndrome is not uncommon in adult patients receiving prophylactic ITMTX for treatment of acute lymphoblastic leukemia and lymphoblastic lymphoma. Patients develop a toxic syndrome closely mimicking acute bacterial meningitis but spontaneous recovery is seen without any neurological sequelae.
  1,914 56 -
Ambulation following spinal cord injury and its correlates
Nitin Menon, Anupam Gupta, Meeka Khanna, Arun B Taly
April-June 2015, 18(2):167-170
DOI:10.4103/0972-2327.150605  PMID:26019413
Objectives: To assess walking ability of spinal cord injury (SCI) patients and observe its correlation with functional and neurological outcomes. Patients and Methods: The present prospective, observational study was conducted in a tertiary research hospital in India with 66 patients (46 males) between January 2012 and December 2013. Mean age was 32.62 ± 11.85 years (range 16-65 years), mean duration of injury was 85.3 ± 97.6 days (range 14-365 days) and mean length of stay in the rehabilitation unit was 38.08 ± 21.66 days (range 14-97 days) in the study. Walking Index for spinal cord injury (WISCI II) was used to assess ambulation of the SCI patients. Functional recovery was assessed using Barthel Index (BI) and Spinal Cord Independence Measures (SCIM). Neurological recovery was assessed using ASIA impairment scale (AIS). We tried to correlate ambulatory ability of the patients with functional and neurological recovery. Results: Ambulatory ability of the patients improved significantly using WISCI II (P < 0.001) when admission and discharge scores were compared (1.4 ± 3.5 vs 7.6 ± 6.03). Similarly, functional (BI: 31.7 ± 20.5 vs 58.4 ± 23.7 and SCIM: 29.9 ± 15.1 vs 56.2 ± 20.6) and neurological recovery were found to be very significant (P < 0.001) when admission vs discharge scores were compared. Improvement in WISCI II scores was significantly correlated with improvement in neurological (using AIS scores) and functional status (using BI and SCIM scores) (P < 0.001). Conclusions: Significant improvement was seen in WISCI II, BI, and SCIM scores after in-patient rehabilitation. Improvement in WISCI II scores also significantly correlated with functional and neurological recovery.
  1,866 64 -
CASE REPORTS
Encephalitis due to antibodies to voltage gated potassium channel (VGKC) with cerebellar involvement in a teenager
Megan M Langille, Jay Desai
April-June 2015, 18(2):238-239
DOI:10.4103/0972-2327.150623  PMID:26019428
Encephalitis due to antibodies to voltage gated potassium channel (VGKC) typically presents with limbic encephalitis and medial temporal lobe involvement on neuroimaging. We describe a case of 13 year girl female with encephalitis due to antibodies to VGKC with signal changes in the cerebellar dentate nuclei bilaterally and clinical features that suggested predominant cerebellar involvement. These have never been reported previously in the literature. Our case expands the phenotypic spectrum of this rare condition.
  1,816 79 1
Limbic encephalitis with antibodies to glutamic acid decarboxylase presenting with brainstem symptoms
Faruk Incecik, Ozlem M Hergüner, Dincer Yildizdas, Ozden Horoz, Seyda Besen
April-June 2015, 18(2):243-245
DOI:10.4103/0972-2327.150628  PMID:26019430
Limbic encephalitis (LE) is a neurological syndrome that may present in association with cancer, infection, or as an isolate clinical condition often accompanying autoimmune disorders. LE associated with glutamic acid decarboxylase antibodies (anti-GAD) is rare in children. Here, we characterized the clinical and laboratory features of a patient presenting with brainstem involvement with non-paraneoplastic LE associated with anti-GAD antibodies. In our patient, after plasma exchange, we determined a dramatic improvement of the neurological deficits.
  1,801 64 -
LETTERS TO THE EDITOR
Refractory status epilepticus from NMDA receptor encephalitis successfully treated with an adjunctive ketogenic diet
Syed Amer, Parth Shah, Vishnu Kommineni
April-June 2015, 18(2):256-257
DOI:10.4103/0972-2327.150620  PMID:26019434
  1,725 48 -
ORIGINAL ARTICLES
External validation of the SEDAN score: The real world practice of a single center
Sombat Muengtaweepongsa, Pornpoj Prapa-Anantachai, Pornpatr A Dharmasaroja, Pattarawit Rukkul, Pornchai Yodvisitsak
April-June 2015, 18(2):181-186
DOI:10.4103/0972-2327.150592  PMID:26019416
Background and Purpose: Symptomatic intracranial hemorrhage (sICH) is the most serious adverse event in stroke patients who received i.v. rt-PA and is usually associated with poor outcomes. The SEDAN score is built up to predict sICH. We aim to externally validate the SEDAN score in Thai patients from single center in the real world practice. Methods: The SEDAN score of stroke patients treated with intravenous rt-PA at Thammasat University Hospital from January 2010 to June 2012 was calculated. Patients were divided into three groups including symptomatic intracranial hemorrhage (sICH), asymptomatic intracranial hemorrhage (AsICH) and no intracerebral hemorrhage (NoICH). The primary outcome of analyses was sICH. Each parameter of the SEDAN score and correlation between score and sICH were analyzed with univariate and multivariate model. Results: 295 patients (18.6% of stroke admission) were treated with i.v. rt-PA. 13 patients (4.4%) had sICH and 31 patients (10.4%) had AsICH. Baseline blood sugar >12 mmol/l, early infarction, hyperdense cerebral artery, age >75 years-old and NIHSS ≥10(SEDAN) were associated with sICH by univariate analysis (P value = 0.018, <0.001, <0.001, 0.002 and 0.027 respectively). The rate of sICH occurrence was increased in accordance with the increasing of the SEDAN score. By multivariate analysis, odds ratio of baseline blood sugar >12 mmol/l, early infarction, hyperdense cerebral artery, age >75 years-old and NIHSS ≥10 were 1.248, 2.503, 1.107, 1.532 and 1.263 respectively. Conclusions: The SEDAN score was practical to use and predictive in Thai population. Each parameter of the SEDAN score was an independent risk factor for sICH after treatment with i.v. rt-PA.
  1,546 71 -
IMAGES IN NEUROLOGY
Gentle giant
Eswaradass Prasanna Venkatesan, Ramadoss Kalidoss, Balakrishnan Ramasamy, Gnanagurusamy Gnanashanmugham
April-June 2015, 18(2):231-232
DOI:10.4103/0972-2327.150610  PMID:26019425
  1,449 78 -
LETTERS TO THE EDITOR
Issues with normative data of hippocampal volumetry in Indian population
Kuljeet Singh Anand, Vikas Dhikav, Sharmila Doraswamy, Umesh Chandra Garga
April-June 2015, 18(2):259-259
DOI:10.4103/0972-2327.150607  PMID:26019437
  1,410 47 -
Author's Reply: Regarding issues with article on hippocampal volumetry
Aravind N Mohandas, Rose Dawn Bharath, Parthipulli Vasuki Prathyusha, Arun K Gupta
April-June 2015, 18(2):260-261
DOI:10.4103/0972-2327.152088  PMID:26019438
  1,108 37 -
Author's response on "Neuropsychiatric profiles in patients with Alzheimer's disease and vascular dementia"
Tushar Kanti Bandyopadhyay, Atanu Biswas, Arijit Roy, Deb Sankar Guin, Goutam Gangopadhyay, Sujit Sarkhel, Malay Kumar Ghoshal, Asit Kumar Senapati
April-June 2015, 18(2):256-256
DOI:10.4103/0972-2327.156565  PMID:26019435
  1,000 43 -
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