Annals of Indian Academy of Neurology
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   2014| July-September  | Volume 17 | Issue 3  
    Online since August 12, 2014

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The ketogenic diet and other dietary treatments for refractory epilepsy in children
Suvasini Sharma, Puneet Jain
July-September 2014, 17(3):253-258
DOI:10.4103/0972-2327.138471  PMID:25221391
The ketogenic diet is a high-fat, low-carbohydrate, and restricted protein diet that is useful in patients with refractory epilepsy. The efficacy of the ketogenic diet is better than most of the new antiepileptic drugs. Other modifications of the diet are also beneficial, such as the modified Atkins diet and the low glycemic index treatment. There is a lack of awareness of the ketogenic diet as a treatment modality for epilepsy amongst pediatricians and neurologists. In this review, the use of the ketogenic diet and other dietary treatments in refractory epilepsy is discussed. The Indian experience with the use of these dietary treatments is also briefly reviewed.
  5,542 236 1
Myopathies of endocrine disorders: A prospective clinical and biochemical study
Vikas Sharma, Papori Borah, Lakshya J Basumatary, Marami Das, Munindra Goswami, Ashok K Kayal
July-September 2014, 17(3):298-302
DOI:10.4103/0972-2327.138505  PMID:25221399
Introduction: Major categories of endocrine myopathy include those associated with: Adrenal dysfunction (as in Cushing's disease or steroid myopathy); thyroid dysfunction (as in myxedema coma or thyrotoxic myopathy); vitamin D deficiency; parathyroid dysfunction; and pituitary dysfunction. Steroid myopathy is the most common endocrine myopathy. Objective: To study the etiology, varied presentations, and outcome after therapy of patients with endocrine myopathies. Materials and Methods: Myopathy was evaluated by the standard clinical procedures: Detailed clinical history, manual muscle strength testing, and creatine phosphokinase (CPK). Endocrine disorders were diagnosed as per clinical features and biochemical parameters. The treatment was given to patients as per underlying endocrine disease. Myopathy was assessed before and after treatment. Results: Out of the 37 patients who were diagnosed with endocrine myopathies, thyroid dysfunction was the most common cause (17 cases), followed by vitamin D deficiency in nine, adrenal dysfunction in six, parathyroid dysfunction in three, and pituitary dysfunction in two. Some patients had atypical presentation (repeated falls in one, tongue fasciculations in one, neck weakness in five, one with ptosis and facial weakness, asymmetrical onset in one, and calf hypertrophy in one. The serum creatine kinase (CK) concentration did not correlate with muscle weakness. Following the treatment regimen which was specific for a given myopathy, 26 patients recovered fully. Conclusion: We found varied clinical presentations of endocrine myopathies. All the patients with neuromuscular complaints should be investigated for endocrine causes because significant number of them recovers fully with specific treatment.
  4,457 134 1
Relapsing Anti-NMDAR Encephalitis after a gap of eight years in a girl from North-East India
Ashok Kumar Kayal, Marami Das, Suvorit Bhowmick, Baiakmenlang Synmon
July-September 2014, 17(3):349-351
DOI:10.4103/0972-2327.138526  PMID:25221411
It has been just 7 years since the discovery of anti-NMDAR encephalitis as distinct immune-mediated encephalitis and we have such cases being reported from our country. Herein, we describe a case of a 13-year-old girl who had relapsing encephalitis consisting of multiple types of difficult-to-control seizures, abnormal behavior, language disintegration, memory loss and abnormal movements eight years after the first clinical attack. In 2005, when she was 5 yearsold, anti-NMDAR encephalitis was not yet discovered and she was provisionally diagnosed as a case of viral encephalitis. During her second attack in 2013, antibodies against NMDAR were demonstrated by immunofluoresence in serum (1:10). This is the first report from our country of a case of relapsing anti-NMDAR encephalitis of such a long duration, successfully treated by immunotherapy.
  4,051 69 -
Relapsing Guillain-Barre syndrome in pregnancy and postpartum
S Meenakshi-Sundaram, K Swaminathan, SN Karthik, S Bharathi
July-September 2014, 17(3):352-354
DOI:10.4103/0972-2327.138527  PMID:25221412
Guillain-Barre syndrome (GBS) rarely complicates pregnancy, but can be associated with high maternal and perinatal morbidity if not properly identified and treated. A high index of suspicion, supportive measures, access to intensive care unit and intravenous immunoglobulin (IVIG) therapy are cornerstones of management in GBS complicating pregnancy. Neurologists and Obstetricians should be aware of the risks of relapsing GBS in the immediate postpartum period. Surgery and anesthesia may be triggers for relapse in association with an overall increase in pro-inflammatory cytokines in the postpartum period. We report a unique case of GBS complicating pregnancy in the third trimester followed by a relapse in the postpartum period. She made a good recovery with supportive measures and a repeat course of IVIG during the relapse.
  4,031 71 -
The national institute of health toolbox
Madhuri Khilari, Sunil K Narayan
July-September 2014, 17(3):247-252
DOI:10.4103/0972-2327.138464  PMID:25221390
  3,905 115 -
Prevalence of fatigue in Guillain-Barre syndrome in neurological rehabilitation setting
Prajna Ranjani, Meeka Khanna, Anupam Gupta, Madhu Nagappa, Arun B Taly, Partha Haldar
July-September 2014, 17(3):331-335
DOI:10.4103/0972-2327.138521  PMID:25221406
Background: Fatigue contributes significantly to the morbidity and affects the quality of life adversely in Guillain-Barre Syndrome (GBS). Objective: To determine the prevalence of fatigue in GBS in neurological rehabilitation setting and to study its clinical correlates. Materials and Methods: We performed secondary analysis of data of patients with GBS admitted in neurological rehabilitation ward of a tertiary care centre, recorded at both admission and discharge. Assessment of fatigue was done by Fatigue Severity Scale (FSS), disability-status by Hughe's Disability Scale (HDS), functional-status by Barthel Index, anxiety/depression by Hospital Anxiety Depression Scale, sleep disturbances by Pittsburgh Sleep Quality Index and muscle weakness by Medical Research Council sum scores. Results: A total of 90 patients (62 men) with mean age 34 years (95% CI 32.2, 37.7) were included. Median duration of, stay at neurological rehabilitation ward was 30 days, while that of symptoms was 18.5 days. Presence of fatigue at admission (FSS ≥ 4 in 39% patients) was associated with ventilator requirement (P = 0.021) and neuropathic pain (P = 0.03). Presence of fatigue at discharge (FSS ≥ 4 in 12% patients) was associated with disability- HDS (≥3) (P = 0.008), presence of anxiety (P = 0.042) and duration of stay at rehabilitation ward (P = 0.02). Fatigue did not correlate with age, gender, antecedent illness, muscle weakness, depression and sleep disturbances. Conclusion: Fatigue is prevalent in GBS during early recovery phase of illness. Despite motor recovery fatigue may persist. Knowledge about fatigue as burden of disease in these patients will improve patient care.
  3,568 79 -
Duchenne/Becker muscular dystrophy: A report on clinical, biochemical, and genetic study in Gujarat population, India
Mandava V Rao, Gaurang M Sindhav, Jitendra J Mehta
July-September 2014, 17(3):303-307
DOI:10.4103/0972-2327.138508  PMID:25221400
Objective: In India, various groups have studied different regions to find out deletion pattern of dystrophin gene. We have investigated its deletion pattern among Duchenne/Becker muscular dystrophy (D/BMD) patients across Gujarat. Moreover, in this study we also correlate the same with reading frame rule. However, we too consider various clinicopathological features to establish as adjunct indices when deletion detection fails. Materials and Methods: In this pilot study, a total of 88 D/BMD patients consulting at our centers in Gujarat, India were included. All patients were reviewed on basis of their clinical characteristics, tested by three primer sets of 10-plex, 9-plex, and 7-plex polymerase chain reaction (PCR) for genetic analysis; whereas, biochemical indices were measured using automated biochemical analyzers. Results: The diagnosis of D/BMD was confirmed by multiplex-PCR (M-PCR) in D/BMD patients. A number of 65 (73.86%) out of 88 patients showed deletion in dystrophin gene. The exon 50 (58.46%) was the most frequent deletion found in our study. The mean age of onset of DMD and BMD was 4.09 ΁ 0.15 and 7.14 ΁ 0.55 years, respectively. In patients, mean creatine phosphokinase (CPK), lactate dehydrogenase (LDH), and myoglobin levels were elevated significantly (P < 0.05) in comparison to controls. Addition to CPK, LDH and myoglobin are good adjunct when deletion detection failed. These data are further in accordance with world literature when correlated with frame rule. Conclusion: The analysis has been carried out for the first time for a total of 88 D/BMD patients particularly from Gujarat, India. More research is essential to elucidate specific mutation pattern in association with management and therapies of proband.
  3,027 136 -
Varied presentations of moyamoya disease in a tertiary care hospital of north-east India
Papori Borah, Vikas Sharma, Lakshya Jyoti Basumatary, Marami Das, Munindra Goswami, Ashok K Kayal
July-September 2014, 17(3):317-320
DOI:10.4103/0972-2327.138518  PMID:25221403
Introduction: Moyamoya disease is a chronic progressive cerebrovascular disorder, characterized by stenosis or occlusion of bilateral internal carotid arteries (ICAs), anterior cerebral arteries (ACAs) and middle cerebral arteries (MCAs), accompanied by a collateral network of vessels formed at the base of the brain. Ischemia and intracranial hemorrhage are the common typical manifestations. However moyamoya disease has been associated with atypical presentations like headache, seizures and involuntary movements. Although frequently reported from Asian countries like Japan, China and Korea, only few studies reported on clinical manifestations of moyamoya disease from India. Objectives: To study the varied presentations of moyamoya disease in a tertiary care hospital of north-east India. Material and Methods: Relevant investigations were done to rule out other causes of moyamoya syndrome. Results: We report 6 cases of moyamoya disease with varied presentations from a tertiary care referral government hospital. Case 1, 2 and 6 presented with alternating hemiparesis. Case 3 had amaurosis fugax. Case 4 had history suggestive of ischemic stroke and presented with hemichorea. Case 4 had focal seizure as the only manifestation. Cases 4 and 5 notably had stenosis of posterior cerebral artery (PCA) in addition to stenosis of bilateral ICAs, ACAs and MCAs. Conclusion: Owing to its low incidence in India, moyamoya disease is easily overlooked as a possible diagnosis. However, because of its progressive nature, it is imperative to diagnose this disease early and offer surgical treatment to the patients.
  2,805 97 -
Hippocampal volumetry: Normative data in the Indian population
Aravind Narayan Mohandas, Rose Dawn Bharath, Parthipulli Vasuki Prathyusha, Arun K Gupta
July-September 2014, 17(3):267-271
DOI:10.4103/0972-2327.138482  PMID:25221393
Background: Mesial temporal sclerosis (MTS) is the most common cause of temporal lobe epilepsy. Quantitative analysis of the hippocampus using volumetry is commonly being used in the diagnosis of MTS and is being used as a marker in prognostication of seizure control. Although normative data for hippocampal volume (HV) is available for the western population, no such data is available for the Indian population. Aim: The aim of the study was to establish normative data for HV for the Indian population, which can aid in the accurate diagnosis of MTS. Materials and Methods: Magnetic resonance imaging (MRI) scans of 200 healthy volunteers were acquired using a 3 Tesla (3T) MRI scanner. Manual segmentation and volumetry was done using Siemens Syngo software. The data was analyzed using two tailed t-test to detect associations between HV and age, gender, and education. The data so obtained was also correlated with the data available from the rest of the world. Results: A mean HV of 2.411 cm 3 (standard deviation -0.299) was found in the study, which was significantly smaller when compared to the data from the western population. The right hippocampus was larger than the left, with a mean volume of 2.424 cm 3 and 2.398 cm 3 , respectively. HV was detected to be significantly higher in males. No association was found between HV and age and education. Conclusion: The values obtained in this study may be adopted as a standard in the evaluation of patients with intractable epilepsy.
  2,758 109 -
Lower motor neuron paralysis with extensive cord atrophy in parainfectious acute transverse myelitis
Sunil Pradhan, Ajit Kumar
July-September 2014, 17(3):347-348
DOI:10.4103/0972-2327.138525  PMID:25221410
We describe a young patient of acute transverse myelitis (ATM) who developed true lower motor neuron (LMN) type flaccid paraplegia as a result of anterior horn cell damage in the region of cord inflammation that extended from conus upwards up to the D4 transverse level. We infer that flaccidity in acute phase of ATM is not always due to spinal shock and may represent true LMN paralysis particularly if the long segment myelits is severe and extending up to last spinal segment.
  2,770 49 1
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
July-September 2014, 17(3):325-330
DOI:10.4103/0972-2327.138520  PMID:25221405
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.
  2,671 92 2
Major outcomes of patients with tuberculous meningitis on directly observed thrice a week regime
Thomas Iype, Ayyappan Kunjukrishna Pillai, Ajith Cherian, Zinia T Nujum, Chithra Pushpa, Dalus Dae, Vijayakumar Krishnapillai
July-September 2014, 17(3):281-286
DOI:10.4103/0972-2327.138496  PMID:25221396
Background: Revised National Tuberculosis Control Programme (RNTCP) of Government of India provides intermittent thrice-a-week directly observed treatment short course (RNTCP regimen). Objective: Assessments of all-cause mortality and nine-month morbidity outcomes of patients with tuberculous meningitis (TBM) on RNTCP regimen. Materials and Methods: We prospectively followed up patients registered with RNTCP center, with a diagnosis of TBM from January 1 st , 2010 to December 31 st , 2011. Morbidity was assessed using modified Rankin Scale (mRS). Results: We had 43 patients with median duration for follow-up of 396 days and that of survivors of 425 days. Two patients defaulted. Fourteen patients (32.5%) had mRS score of 4 to 6 and 29 had mRS of 0 to 3 after 9-month treatment. Severe disability was not related to any factor on logistic regression. Severe disability was seen in one patient (6.66%) among the 15 patients with stage 1, nine (37.5%) out of 24 patients with stage 2 and three (75%) out of 4 patients with stage 3 disease. Eight patients died (18.6%) of whom 4 died during the intensive phase and 4 during the continuation phase of RNTCP regimen. Mortality was independently related to treatment failure with adjusted Hazard ratio of 8.29 (CI: 1.38-49.78) (P = 0.02). One patient (6.66%) died out of the 15 patients with stage 1 disease, 5 (20.8%) out of 24 patients with stage 2 disease and 2 (50%) out of the 4 with stage 3 disease. Discussion and Conclusion: RNTCP regimen was associated with good compliance, comparable mortality and morbidity.
  2,300 130 -
Response to abdominal hysterectomy with bilateral salpingo-oophorectomy in postmenopausal woman with anti-yo antibody mediated paraneoplastic cerebellar degeneration
Amita Bhargava, Bharat Bhushan, Gaurav M Kasundra, Khichar Shubhakaran, Guruprasad S Pujar, Basavaraj Banakar
July-September 2014, 17(3):355-357
DOI:10.4103/0972-2327.138528  PMID:25221413
Paraneoplastic cerebellar degeneration (PCD) is a rare neurological disorder characterized by a widespread loss of Purkinje cells associated with a progressive pancerebellar dysfunction. PCD often precedes the cancer diagnosis by months to years. Here, we report a case of 44-year old postmenopausal woman who presented with PCD symptoms and high levels of anti-Yo antibodies titer since 8 months. We failed to conclude any neoplastic focus after thorough laboratory and imaging study. She minimally responded to methylprednisolone and immunoglobulin therapies. Despite therapy she was severely disabled. Planned abdominal hysterectomy with bilateral salpingo-oophorectomy (AHBSO) was done, histology revealed grade IIA borderline serous papillary carcinoma of ovary. Her neurological deficit responded dramatically to AHBSO. It is first case report who emphasize the response of AHBSO with presentation of anti-Yo antibody-mediated PCD and hidden nidus in post menopausal women.
  2,342 64 1
Pharmacogenetic evaluation of ABCB1, Cyp2C9, Cyp2C19 and methylene tetrahydrofolate reductase polymorphisms in teratogenicity of anti-epileptic drugs in women with epilepsy
Manna Jose, Moinak Banerjee, Anila Mathew, Tashi Bharadwaj, Neetha Vijayan, Sanjeev V Thomas
July-September 2014, 17(3):259-266
DOI:10.4103/0972-2327.138475  PMID:25221392
Aim: Pregnancy in women with epilepsy (WWE) who are on anti-epileptic drugs (AEDs) has two- to three-fold increased risk of fetal malformations. AEDs are mostly metabolized by Cyp2C9, Cyp2C19 and Cyp3A4 and transported by ABCB1. Patients on AED therapy can have folate deficiency. We hypothesize that the polymorphisms in ABCB1, Cyp2C9, Cyp2C19 and methylene tetrahydrofolate reductase (MTHFR) might result in differential expression resulting in differential drug transport, drug metabolism and folate metabolism, which in turn may contribute to the teratogenic impact of AEDs. Materials and Methods: The ABCB1, Cyp2C9, Cyp2C19 and MTHFR polymorphisms were genotyped for their role in teratogenic potential and the nature of teratogenecity in response to AED treatment in WWE. The allelic, genotypic associations were tested in 266 WWE comprising of 143 WWE who had given birth to babies with WWE-malformation (WWE-M) and 123 WWE who had normal offsprings (WWE-N). Results: In WWE-M, CC genotype of Ex07 + 139C/T was overrepresented (P = 0.0032) whereas the poor metabolizer allele *2 and *2 *2 genotype of CYP2C219 was significantly higher in comparison to WWE-N group (P = 0.007 and P = 0.005, respectively). All these observations were independent of the nature of malformation (cardiac vs. non cardiac malformations). Conclusion: Our study indicates the possibility that ABCB1 and Cyp2C19 may play a pivotal role in the AED induced teratogenesis, which is independent of nature of malformation. This is one of the first reports indicating the pharmacogenetic role of Cyp2C19 and ABCB1 in teratogenesis of AED in pregnant WWE.
  2,235 97 1
Movement disorders of probable infectious origin
Ketan Jhunjhunwala, M Netravathi, Pramod Kumar Pal
July-September 2014, 17(3):292-297
DOI:10.4103/0972-2327.138503  PMID:25221398
Background: Movement disorders (MDs) associated with infections remains an important debilitating disorder in the Asian countries. Objectives: The objective of the following study is to report the clinical and imaging profile of a large cohort of patients with MDs probably associated with infection. Materials and Methods: This was a chart review of 35 patients (F:M-15:20) presenting with MD in the Neurology services of National Institute of Mental Health and Neurosciences, India. The demographic profile, type of infection, time from infection to MD, phenomenology of MD and magnetic resonance imaging (MRI) findings were reviewed. Results: The mean age at presentation was 22.6 ± 13.3 years, (5-60), age of onset of MD was 15.7 ± 15 years, and duration of symptoms was 6.9 ± 8.1 years (42 days to 32 years). The mean latency of onset of MD after the infection was 5.9 ± 4.2 weeks. The phenomenology of MD were: (1) Pure dystonia-28.6%, (2) dystonia with choreoathetosis-22.9%, (3) Parkinsonism-14.6%, (4) pure tremor, hemiballismus, myoclonus and chorea-2.9% each, and (5) mixed MD-22.9%. Most often the MD was generalized (60%), followed by right upper limb (31.4%) and left upper limb (8.6%). A viral encephalitic type of neuroinfection was the most common infection (85.7%), which was associated with MD. Abnormalities of brain MRI, seen in 79.2%, included signal changes in (1) thalamus-52.0%, (2) putamen and subcortical white matter-16% each, (3) pons-12%, (4) striatopallidum, striatum and grey matter-8% each, and (5) caudate, cerebellum, lentiform nucleus, midbrain and subthalamic nucleus-4.0% each. Conclusions: MDs associated with infection were the most often post-encephalitic. Dystonia was the most common MD, and thalamus was the most common anatomical site involved.
  2,170 140 1
Hirayama disease with juvenile myoclonic epilepsy: A case report
Jin-Sung Park, Sung-Pa Park, Jong-Geun Seo
July-September 2014, 17(3):358-360
DOI:10.4103/0972-2327.138529  PMID:25221414
Hirayama disease (HD) is rare, but benign anterior horn cell disease, predominantly affecting young men. One of the symptoms, besides weakness, is abnormal movement in the hand. Juvenile myoclonic epilepsy (JME) is one of the most common types of generalized epilepsies and can be recognized by a myoclonic jerk and electroencephalography (EEG) features. We report the case of a 19-year-old male who had HD, with unilateral abnormal movement in the hand, which was diagnosed as JME. We should consider performing an EEG in patients with HD, who present with atypical hand movements, in order to differentiate it from seizure.
  2,211 62 -
Alterations in Polysomnographic (PSG) profile in drug-naïve Parkinson's disease
Sanju P Joy, Sanjib Sinha, Pramod Kumar Pal, Samhita Panda, Mariamma Philip, Arun B Taly
July-September 2014, 17(3):287-291
DOI:10.4103/0972-2327.138501  PMID:25221397
Objective: We studied the changes in Polysomnographic (PSG) profile in drug-naïve patients of Parkinson's disease (PD) who underwent evaluation with sleep overnight PSG. Materials and Methods: This prospective study included 30 with newly diagnosed levodopa-naïve patients with PD, fulfilling the UK-PD society brain bank clinical diagnostic criteria (M:F = 25:5; age: 57.2 ± 10.7 years). The disease severity scales and sleep related questionnaires were administered, and then patients were subjected to overnight PSG. Results: The mean duration of illness was 9.7 ± 9.5 months. The mean Hoehn and Yahr stage was 1.8 ± 0.4. The mean Unified Parkinson's Disease Rating Scale (UPDRS) motor score improved from 27.7 ± 9.2 to 17.5 ± 8.9 with sustained usage of levodopa. Nocturnal sleep as assessed by Pittsburgh Sleep Quality Index (PSQI) was impaired in 10 (33.3%) patients (mean PSQI score: 5.1 ± 3.1). Excessive day time somnolence was recorded in three patients with Epworth Sleepiness Scale (ESS) score ≥ 10 (mean ESS score: 4.0 ± 3.4). PSG analysis revealed that poor sleep efficiency of <85% was present in 86.7% of patients (mean: 68.3 ± 21.3%). The latencies to sleep onset (mean: 49.8 ± 67.0 minutes) and stage 2 sleep (36.5 ± 13.1%) were prolonged while slow wave sleep was shortened. Respiration during sleep was significantly impaired in which 43.3% had impaired apnoea hyperpnoea index (AHI) ≥5, mean AHI: 8.3 ± 12.1). Apnoeic episodes were predominantly obstructive (obstructive sleep apnea, OSA index = 2.2 ± 5.1). These patients had periodic leg movement (PLM) disorder (56.7% had PLM index of 5 or more, mean PLMI: 27.53 ± 4 9.05) that resulted in excessive daytime somnolence. Conclusions: To conclude, sleep macro-architecture is altered in frequently and variably in levodopa-naοve patients of PD and the alterations are possibly due to disease process per se.
  2,192 58 1
Adrenomyeloneuropathy with bulbar palsy: A rare association
Vishal Annaji Chafale, Satish Arunkumar Lahoti, Atanu Biswas, Arijit Roy, Asit Kumar Senapati
July-September 2014, 17(3):361-363
DOI:10.4103/0972-2327.138530  PMID:25221415
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.
  2,161 52 -
Intergenotypic variation of Vitamin B12 and Folate in AD: In north Indian population
Neelam Chhillar, Neeraj Kumar Singh, Basu Dev Banerjee, Kiran Bala, Mitra Basu, Deepika Sharma
July-September 2014, 17(3):308-312
DOI:10.4103/0972-2327.138510  PMID:25221401
Objectives: Changes in lifestyle habits such as diet modification or supplementation have been indicated as probable protective factors for a number of chronic conditions including Alzheimer's disease (AD). With this background, we aim to hypothesize that whether C677T polymorphism of methylenetetrahydrofolate reductase (MTHFR) gene contributes towards the risk of developing AD and its association with vitamin B12 and folate levels. Materials and Methods: A case-control study comprising of total 200 subjects, within the age group of 50-85 years. Their blood samples were analyzed for serum folate, vitamin B12 levels, and MTHFR C677T polymorphism by restriction fragment length polymorphism (RFLP). Results: The mean plasma levels of vitamin B12 and folate were significantly lower in study group when compared to the control group (P < 0.001). Genotypic and allelic frequency of MTHFR gene in both groups was found to be significant (P < 0.05). The intergenotypic variations of vitamin B12 and folate were found to be significant (P < 0.001). Conclusion: We concluded that the subjects with homozygous mutated alleles are more prone to AD and also pointed out the influence of presence/absence of MTHFR T allelic variants on serum folate and vitamin B12 levels.
  2,122 48 1
Recurrence rate of seizure following discontinuation of anti-epileptic drugs in patients with normal long term electroencephalography
V Abdul Gafoor, Kondanath Saifudheen, James Jose
July-September 2014, 17(3):277-280
DOI:10.4103/0972-2327.138492  PMID:25221395
Background: The usefulness of electroencephalography (EEG) in predicting seizure recurrence after antiepileptic drugs (AED) tapering is a controversial subject. There have been no studies which tested the additional yield of long-term over routine EEG recordings in predicting seizure recurrence after AED withdrawal. Objective: The primary objective of our study is to determine the recurrence rate of seizure following AED withdrawal in patients with focal epilepsy, unknown cause who had normal long-term electroencephalography (LTEEG) and secondary objective is to analyze the variables that would predict seizure recurrence. Materials and Methods: This was a prospective observational study. A total of 91 patients were included. 62 patients who had normal routine and LTEEG entered the final phase of the study were followed-up regularly for 1 year or until seizure recurrence whichever was earlier. Results: A total number of 91 patients were enrolled for the first phase of the study. Of these, 13 (14.29%) patients had an abnormal routine EEG. Of the remaining patients, another 16 (17.58%) had abnormal LTEEG. The remaining 62 patients with normal routine and long-term EEG entered the final phase of the study. Of these, 17 patients (27.41%) had seizure recurrence during the follow-up while 45 (72.58%) remained seizure free until the end of the 1 year follow-up. The significant variables associated with a higher risk of seizure relapse were a positive past history of seizure recurrence on prior drug withdrawal (relative risk: 2.19, confidence interval: 1.01-4.74, P < 0.05) and the duration of epilepsy until seizure control was achieved (P < 0.009). Conclusions: The recurrence rate of seizure in patients with a normal LTEEG is 27.41%. A positive past history of seizure recurrence and a longer time to achieve seizure freedom with AED increased the risk of seizure recurrence.
  1,909 142 -
Multipoint incremental motor unit number estimation versus amyotrophic lateral sclerosis functional rating scale and the medical research council sum score as an outcome measure in amyotrophic lateral sclerosis
Sujit Abajirao Jagtap, Abraham Kuruvilla, Preetha Govind, Muralidharan D Nair, C Sarada, Ravi Prasad Varma
July-September 2014, 17(3):336-339
DOI:10.4103/0972-2327.138522  PMID:25221407
Introduction: Monitoring the disease progression in amyotrophic lateral sclerosis (ALS) is a challenge due to different rates of progression between patients. Besides clinical methods to monitor disease progression, such as the ALS functional rating scale (ALSFRS) and the medical research council (MRC) sum score, quantitative methods like motor unit number estimation (MUNE) are of interest. Objective: The objective of the present study is to evaluate the rate of progression in ALS using multipoint incremental MUNE and to compare MUNE, ALSFRS and MRC sum score at baseline and at 6 months for progression of the disease. Materials and Methods: Multipoint incremental MUNE using median nerve, ALS-FRS and MRC sum score was carried out in 29 ALS patients at baseline and then at 6 months. Results: Of the 29 ALS patients studied, the mean MUNE at baseline was 21.80 (standard deviation [SD]: 19.46, range 4-73), 15.9 in the spinal onset group (SD: 14.60) and 30.16 (SD: 22.89) in the bulbar onset group. Spinal onset patients had 74.02% of baseline MUNE value while bulbar onset patients had only 24.74% baseline value MUNE at 6 months follow-up (Unpaired t-test, P = 0.001). ALSFRS and MRC sum score showed statistically significant decline (P < 0.001) at 6 months follow-up. MUNE had the highest sensitivity for progression of the disease when compared to the ALS FRS and MRC sum score. Conclusion: Multipoint incremental MUNE is a valuable tool for outcome measure in ALS and other diseases characterized by motor unit loss. The rate of decline of multipoint incremental MUNE is more sensitive than that of MRC sum score and ALSFRS-R, when expressed as the percentage change from baseline.
  1,912 59 3
Derangements in bone mineral parameters and bone mineral density in south Indian subjects on antiepileptic medications
George Koshy, Ron Thomas Varghese, Dukhabandhu Naik, Hesargatta Shyamsunder Asha, Nihal Thomas, Mandalam Subramaniam Seshadri, Mathew Alexander, Maya Thomas, Sanjith Aaron, Thomas Vizhalil Paul
July-September 2014, 17(3):272-276
DOI:10.4103/0972-2327.138489  PMID:25221394
Background: Although there are reports describing the association of alternations of bone and mineral metabolism in epileptic patients with long-term anticonvulsant therapy, there are only limited Indian studies which have looked at this aspect. Objectives: This study was done to compare the prevalence of changes in bone mineral parameters and bone mineral density (BMD) in ambulant individuals on long-term anticonvulsant therapy with age- and body mass index (BMI)-matched healthy controls. Materials and Methods: There were 55 men (on medications for more than 6 months) and age- and BMI-matched 53 controls. Drug history, dietary calcium intake (DCI), and duration of sunlight exposure were recorded. Bone mineral parameters and BMD were measured. Results: The control group had a significantly higher daily DCI with mean ± SD of 396 ± 91 mg versus 326 ± 101 mg (P = 0.007) and more sunlight exposure of 234 ± 81 vs 167 ± 69 min (P = 0.05). BMD at the femoral neck was significantly lower in cases (0.783 ± 0.105 g/cm 2 ) when compared to controls (0.819 ± 0.114 g/cm 2 ). Majority of the patients (61%) had low femoral neck BMD (P = 0.04). There was no significant difference in the proportion of subjects with vitamin D deficiency (<20 ng/mL) between cases (n = 32) and controls (n = 37) (P = 0.234). Conclusions: Vitamin D deficiency was seen in both the groups in equal proportions, highlighting the existence of a high prevalence of this problem in India. Low femoral neck BMD found in cases may stress the need for supplementing calcium and treating vitamin D deficiency in this specific group. However, the benefit of such intervention has to be studied in a larger proportion of epileptic patients.
  1,806 108 -
Effects of oxysophoridine on amino acids after cerebral ischemic injury in mice
Tengfei Wang, Yuxiang Li, Peng Zhao, Jie Wang, Xiaomin Zhang, Yinju Hao, Juan Du, Chengjun Zhao, Tao Sun, Jianqiang Yu, Ru Zhou, Shaoju Jin
July-September 2014, 17(3):313-316
DOI:10.4103/0972-2327.138513  PMID:25221402
Background: Our previous studies demonstrated that oxysophoridine (OSR) had neuroprotective effects on mice through antioxidant and anti-apoptotic mechanisms. In this study, we investigated whether OSR could influence the release of amino acids in ischemic mice brains. Materials and Methods: Male ICR mice were scheduled to undergo 2 h middle cerebral artery occlusion (MCAO) and 24 h reperfusion. Before MCAO, mice in corresponding groups were intraperitoneally injected with OSR (62.5, 125 and 250 mg/kg) for seven successive days. After reperfusion, neurological scores were estimated, infarct volume and the brain water content were assessed. The levels of glutamate (Glu), aspartate (Asp), γ-aminobutyric acid (GABA) and Glycine (Gly) were measured by amino acid analyzer. Results: OSR significantly decreased neurological scores, reduced infarct volume and the brain water content. After treatment with OSR of 250 mg/kg, the contents of Glu, Asp, GABA and Gly in mice brains could maintain at a normal level compared with MCAO group mice. The Glu/GABA ratio was significantly decreased in OSR group mice. Conclusion: These findings indicate that OSR has a protective effect on cerebral ischemic injury and helps to maintain the amino acids homeostasis after reperfusion for a long time.
  1,595 40 -
Tumefactive Virchow-Robin spaces: A rare cause of obstructive hydrocephalus
Naseer A Choh, Feroze Shaheen, Irfan Robbani, Manjeet Singh, Tariq Gojwari
July-September 2014, 17(3):345-346
DOI:10.4103/0972-2327.138524  PMID:25221409
  1,512 54 -
Mild cognitive impairment: Profile of a cohort from a private sector memory clinic
Srikanth Srinivasan
July-September 2014, 17(3):340-344
DOI:10.4103/0972-2327.138523  PMID:25221408
Background: Private hospital memory clinics might see a different clientele than university or academic institutes due to referral biases. Objective: To characterize the profile of patients with mild cognitive impairment (MCI) from a private sector memory clinic. Materials and Methods: MCI was diagnosed according to revised clinical criteria of Petersen et al. For a subset of patients with MCI medial temporal atrophy and cerebral small vessel disease (white matter lesions and lacunes) were rated on magnetic resonance imaging (MRI) scans and analyzed for their contribution towards cognitive impairment. Results: Subjects with MCI formed one-third (113/371) of this memory clinic sample from a private hospital. MCI could be effectively diagnosed and subtyped using a brief cognitive scale (Concise Cognitive Test (CONCOG)). The amnestic MCI (single and multiple domains) subtype comprised the majority of cases with MCI. In a subsample of 33 patients, lacunar infarcts were more common than white matter lesions and hippocampal atrophy and were inversely associated with verbal fluency. Conclusions: MCI may be more commonly encountered in private hospital settings probably due to early referrals. It is possible to diagnose and subtype MCI using a brief cognitive instrument such as the CONCOG. In this sample, lacunar infarcts were more commonly encountered than medial temporal atrophy in such patients.
  1,477 53 -
Multimodal evoked potentials in spinocerebellar ataxia types 1, 2, and 3
Vijay Chandran, Ketan Jhunjhunwala, Meera Purushottam, Sanjeev Jain, Pramod Kumar Pal
July-September 2014, 17(3):321-324
DOI:10.4103/0972-2327.138519  PMID:25221404
Aims: Spinocerebellar ataxias (SCA) are a clinically heterogeneous group of disorders that are characterized by ataxia and an autosomal dominant pattern of inheritance. The aim of our study was to describe the findings of evoked potentials (EPs) among genetically proven SCA types 1, 2, and 3 and to additionally evaluate if EPs can be used to differentiate between them. Materials and Methods: Forty-three cases of genetically proven SCA (SCA1 = 19, SCA2 = 13, and SCA3 = 11) were evaluated with median somatosensory-EP (mSSEP), visual-EP (VEP), and brainstem auditory-evoked response (BAER) by standard procedures and compared with normative laboratory data. An EP was considered abnormal if latency was prolonged (>mean + 3 standard deviation (SD) of laboratory control data) or the waveform was absent or poorly defined. The waves studied were as follows: mSSEP - N20, VEP - P100 and BAER - interpeak latency 1-3 and 3-5. Results: EPs were abnormal in at least one modality in 90.9% of patients. The most common abnormality was of BAER (86.1%) followed by VEP (34.9%) and mSSEP (30.2%). The degree of abnormality in VEP, mSSEP, and BAER among patients with SCA1 was 42.1, 41.2, and 73.3%, respectively; among patients with SCA2 was 38.5, 27.3, and 100%, respectively; and among patients with SCA3 was 18.2, 37.5, and 88.9%, respectively. The differences between the subgroups of SCAs were not statistically significant. Conclusions: BAER was the most frequent abnormality in SCA types 1, 2, and 3; abnormalities of mSSEP were comparable in the three SCAs; whereas, abnormality of VEP was less often noted in SCA3.
  1,469 57 -
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