Annals of Indian Academy of Neurology
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   2016| April-June  | Volume 19 | Issue 2  
    Online since May 12, 2016

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Approach to a case of myeloneuropathy
Ravindra Kumar Garg, Hardeep Singh Malhotra, Neeraj Kumar
April-June 2016, 19(2):183-187
DOI:10.4103/0972-2327.182303  PMID:27293327
Myeloneuropathy is a frequently encountered condition and often poses a diagnostic challenge. A variety of nutritional, toxic, metabolic, infective, inflammatory, and paraneoplastic disorders can present with myeloneuropathy. Deficiencies of vitamin B12, folic acid, copper, and vitamin E may lead to myeloneuropathy with a clinical picture of subacute combined degeneration of the spinal cord. Among infective causes, chikungunya virus has been shown to produce a syndrome similar to myeloneuropathy. Vacuolar myelopathy seen in human immunodeficiency virus (HIV) infection is clinically very similar to subacute combined degeneration. A paraneoplastic myeloneuropathy, an immune-mediated disorder associated with an underlying malignancy, may rarely be seen with breast cancer. Tropical myeloneuropathies are classified into two overlapping clinical entities — tropical ataxic neuropathy and tropical spastic paraparesis. Tropical spastic paraparesis, a chronic noncompressive myelopathy, has frequently been reported from South India. Establishing the correct diagnosis of myeloneuropathy is important because compressive myelopathies may pose diagnostic confusion. Magnetic resonance imaging (MRI) in subacute combined degeneration of the spinal cord typically reveals characteristic signal changes on T2-weighted images of the cervical spinal cord. Once the presence of myeloneuropathy is established, all these patients should be subjected to a battery of tests. Blood levels of vitamin B12, folic acid, vitamins A, D, E, and K, along with levels of iron, methylmalonic acid, homocysteine, and calcium should be assessed. The pattern of neurologic involvement and results obtained from a battery of biochemical tests often help in establishing the correct diagnosis.
  8,855 602 -
Understanding migraine: Potential role of neurogenic inflammation
Rakesh Malhotra
April-June 2016, 19(2):175-182
DOI:10.4103/0972-2327.182302  PMID:27293326
Neurogenic inflammation, a well-defined pathophysiologial process is characterized by the release of potent vasoactive neuropeptides, predominantly calcitonin gene-related peptide (CGRP), substance P (SP), and neurokinin A from activated peripheral nociceptive sensory nerve terminals (usually C and A delta-fibers). These peptides lead to a cascade of inflammatory tissue responses including arteriolar vasodilation, plasma protein extravasation, and degranulation of mast cells in their peripheral target tissue. Neurogenic inflammatory processes have long been implicated as a possible mechanism involved in the pathophysiology of various human diseases of the nervous system, respiratory system, gastrointestinal tract, urogenital tract, and skin. The recent development of several innovative experimental migraine models has provided evidence suggestive of the involvement of neuropeptides (SP, neurokinin A, and CGRP) in migraine headache. Antidromic stimulation of nociceptive fibers of the trigeminal nerve resulted in a neurogenic inflammatory response with marked increase in plasma protein extravasation from dural blood vessels by the release of various sensory neuropeptides. Several clinically effective abortive antimigraine medications, such as ergots and triptans, have been shown to attenuate the release of neuropeptide and neurogenic plasma protein extravasation. These findings provide support for the validity of using animal models to investigate mechanisms of neurogenic inflammation in migraine. These also further strengthen the notion of migraine being a neuroinflammatory disease. In the clinical context, there is a paucity of knowledge and awareness among physicians regarding the role of neurogenic inflammation in migraine. Improved understanding of the molecular biology, pharmacology, and pathophysiology of neurogenic inflammation may provide the practitioner the context-specific feedback to identify the novel and most effective therapeutic approach to treatment. With this objective, the present review summarizes the evidence supporting the involvement of neurogenic inflammation and neuropeptides in the pathophysiology and pharmacology of migraine headache as well as its potential significance in better tailoring therapeutic interventions in migraine or other neurological disorders. In addition, we have briefly highlighted the pathophysiological role of neurogenic inflammation in various other neurological disorders.
  5,568 134 -
Infantile spasms: A prognostic evaluation
Mary Iype, Geetha Saradakutty, Puthuvathra Abdul Mohammed Kunju, Devi Mohan, Muttathu Krishnapanicker Chandrasekharan Nair, Babu George, Shahanaz M Ahamed
April-June 2016, 19(2):228-235
DOI:10.4103/0972-2327.173314  PMID:27293335
Background: Few papers address the comprehensive prognosis in infantile spasms and look into the seizure profile and psychomotor outcome. Objective: We aimed to follow up children with infantile spasms to study: a) the etiology, demographics, semiology, electroencephalogram (EEG), and radiological pattern; b) seizure control, psychomotor development, and EEG resolution with treatment; c) the effects of various factors on the control of spasms, resolution of EEG changes, and psychomotor development at 3-year follow-up. Materials and Methods: Fifty newly diagnosed cases with a 1-12 month age of onset and who had hypsarrhythmia in their EEG were recruited and 43 were followed up for 3 years. Results: Of the children followed up, 51% were seizure-free and 37% had a normal EEG at the 3-year follow-up. Autistic features were seen in 74% of the children. Only 22.7% among the seizure-free (11.6% of the total) children had normal vision and hearing, speech with narration, writing skills, gross and fine motor development, and no autism or hyperactivity. On multivariate analysis, two factors could predict bad seizure outcome — the occurrence of other seizures in addition to infantile spasms and no response to 28 days of adrenocorticotropic hormone (ACTH). No predictor could be identified for abnormal psychomotor development. Discussion and Conclusion: In our study, we could demonstrate two factors that predict seizure freedom. The cognitive outcome and seizure control in this group of children are comparable to the existing literature. However, the cognitive outcome revealed by our study and the survey of the literature are discouraging.
  4,094 113 -
Knowledge, attitude, and practice in relation to stroke: A community-based study from Kolkata, West Bengal, India
Sujata Das, Avijit Hazra, Biman Kanti Ray, Malay Ghosal, Arijit Chaudhury, Tapas Kumar Banerjee, Shyamal Kumar Das
April-June 2016, 19(2):221-227
DOI:10.4103/0972-2327.176857  PMID:27293334
Background: The rising incidence of stroke in India indicates the importance of evaluating the existing knowledge, attitude, and practice (KAP) in the community, which is essential for stroke control. Objective: To explore and compare stroke-related KAP among participants from stroke-affected families (SAFs) and nonstroke-affected families (NSFs). Design: Using stratified random sampling, a three-phase house-to-house survey was conducted in Kolkata, West Bengal, India. First, field investigators screened subjects of stroke; second, the neurologist confirmed positive cases; and third, under supervision of the neuropsychologist, a validated questionnaire on KAP was administered to participants from SAFs and age-matched NSAFs from the same neighborhood. Results: The KAP questionnaire was administered to 282 participants each from both groups. Knowledge about stroke prevailed in 97% participants and was significantly higher in the SAF group. Both SAF and NSAF groups had better knowledge about prominent symptoms of stroke (loss of consciousness and paralysis) and admitted it as emergency situation requiring hospitalization and that it was potentially preventable. Those persons belonging to the SAF group, however, had lesser knowledge of the risk factors such as diabetes (P < 0.001), smoking (P < 0.014), alcoholism (P < .0.0001), family history (P < .0.0001) and mild stroke symptoms such as headache, (P < 0.001), vomiting (P < 0.001), and fits (P 0.003) as compared to the NSAF group. Conclusions: Persons from both SAF and non-SAF groups are aware about stroke but possess lesser knowledge about the many symptoms of stroke and risk factors, indicating the necessity of enhancement of existence knowledge on symptoms for better diagnosis and of risk factors for better prevention.
  3,991 131 -
Prions, prion-like prionoids, and neurodegenerative disordersVacancy
Ashok Verma
April-June 2016, 19(2):169-174
DOI:10.4103/0972-2327.179979  PMID:27293325
Prion diseases or transmissible spongiform encephalopathies are fatal neurodegenerative diseases characterized by the aggregation and deposition of the misfolded prion protein in the brain. α-synuclein (α-syn)-associated multiple system atrophy has been recently shown to be caused by a bona fide α-syn prion strain. Several other misfolded native proteins such as β-amyloid, tau and TDP-43 share some aspects of prions although none of them is shown to be transmissible in nature or in experimental animals. However, these prion-like “prionoids” are causal to a variety of neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, and amyotrophic lateral sclerosis. The remarkable recent discovery of at least two new α-syn prion strains and their transmissibility in transgenic mice and in vitro cell models raises a distinct question as to whether some specific strain of other prionoids could have the capability of disease transmission in a manner similar to prions. In this overview, we briefly describe human and other mammalian prion diseases and comment on certain similarities between prion and prionoid and the possibility of prion-like transmissibility of some prionoid strains.
  3,402 127 -
Clinical profile and treatment outcome of febrile infection-related epilepsy syndrome in South Indian children
Sandeep B Patil, Arun Grace Roy, Kollencheri Puthenveettil Vinayan
April-June 2016, 19(2):188-194
DOI:10.4103/0972-2327.173305  PMID:27293328
Purpose: To describe the clinical features and outcome of febrile infection-related epilepsy syndrome (FIRES), a catastrophic epileptic encephalopathy, in a cohort of South Indian children. Materials and Methods: We performed a retrospective chart review of a cohort of children with previously normal development who presented with status epilepticus or encephalopathy with recurrent seizures following a nonspecific febrile illness during the period between January 2007 and January 2012. They were divided into two groups super refractory status epilepticus (SRSE) and refractory status epilepticus (RSE) depending on the duration and severity of the seizures. Key Findings: Fifteen children who met the inclusion criteria were included for the final analysis. The age of the children at presentation ranged 3-15 years (median 6.3 years). All the children presented with prolonged or recurrent seizures occurring 1-12 days (median 4 days) after the onset of fever. Eight children had SRSE while seven children had refractory seizures with encephalopathy. Cerebrospinal fluid (CSF) analysis was done in all the children in the acute phase, and the cell count ranged 0-12 cells/μL (median 2 cells/μL) with normal sugar and protein levels. Initial neuroimaging done in all children (MRI in 10 and CT in 5), and it was normal in 13 children. Treatment modalities included multiple antiepileptic drugs (AEDs) (4-9 drugs) (median 5 drugs). Midazolam (MDZ) infusion was administered in seven patients. Eight patients required barbiturate coma to suppress the seizure activity. The duration of the barbiturate coma ranged 2-90 days (median 3 days). Steroids were used in 14 children and intravenous immunoglobulin (2 g/kg) in 7 children. Three children died in the acute phase. All children were maintained on multiple AEDs till the last follow-up, the number of AEDs ranged 1-6 (median 5 AEDs). The patients with super refractory status in the acute phase were found to be more severely disabled at the follow-up; the median score of these patients on the Glasgow Outcome Scale (GOS) was 2 compared to 5 in the RSE group. Significance: This study reports one of the largest single center cohorts from India, with an adverse long-term developmental and seizure outcome. The duration and severity of seizures in the acute period correlated directly with the short-term and long-term clinical outcomes. There is an urgent need for developing new effective therapeutic strategies to treat this acute catastrophic epileptic syndrome.
  3,135 149 -
Development of a dot blot assay with antibodies to recombinant “core” 14-3-3 protein: Evaluation of its usefulness in diagnosis of Creutzfeldt–Jakob disease
Sarada Subramanian, Anita Mahadevan, Parthasarathy Satishchandra, Susarla Krishna Shankar
April-June 2016, 19(2):205-210
DOI:10.4103/0972-2327.176867  PMID:27293331
Background and Purpose: Definitive diagnosis of Creutzfeldt–Jakob disease (CJD) requires demonstration of infective prion protein (PrPSc) in brain tissues by immunohistochemistry or immunoblot, making antemortem diagnosis of CJD difficult. The World Health Organization (WHO) recommends detection of 14-3-3 protein in cerebrospinal fluid (CSF) in cases of dementia, with clinical correlation, as a useful diagnostic marker for CJD, obviating the need for brain biopsy.This facility is currently available in only a few specialized centers in the West and no commercial kit is available for clinical diagnostic use in India. Hence the objective of this study was to develop an in-house sensitive assay for quantitation of 14-3-3 protein and to evaluate its diagnostic potential to detect 14-3-3 proteins in CSF as a biomarker in suspected cases of CJD. Materials and Methods: A minigene expressing the “core” 14-3-3 protein was synthesized by overlapping polymerase chain reaction (PCR) and the recombinant protein was produced by employing a bacterial expression system. Polyclonal antibodies raised in rabbit against the purified recombinant protein were used for developing a dot blot assay with avidin-biotin technology for signal amplification and quantitation of 14-3-3 protein in CSF. Results: The results in the present study suggest the diagnostic potential of the dot blot method with about 10-fold difference (P< 0.001) in the CSF levels of 14-3-3 protein between the CJD cases (N= 50) and disease controls (N= 70). The receiver operating characteristic (ROC) analysis of the results suggested an optimal cutoff value of 2 ng/mL. Conclusions: We have developed an indigenous, economical, and sensitive dot blot method for the quantitation of 14-3-3 protein in CSF.
  2,676 67 -
An unusual case of cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy with occipital lobe involvement
Bhavesh Trikamji, Mariam Thomas, Gasser Hathout, Shrikant Mishra
April-June 2016, 19(2):272-274
DOI:10.4103/0972-2327.173403  PMID:27293347
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an autosomal dominant angiopathy caused by a mutation in the notch 3 gene on chromosome 19. Clinically, patients may be asymptomatic or can present with recurrent ischemic episodes and strokes leading to dementia, depression, pseudobulbar palsy, and hemi- or quadraplegia. Additional manifestations that have been described include migraine (mostly with aura), psychiatric disturbances, and epileptic seizures. Neuroimaging is essential to the diagnosis of CADASIL. On imaging CADASIL is characterized by symmetric involvement by confluent lesions located subcortically in the frontal and temporal lobes as well as in the insula, periventricularly, in the centrum semiovale, in the internal and external capsule, basal ganglia, and brain stem; with relative sparing of the fronto-orbital and the occipital subcortical regions. We describe a 49 year old male with CADASIL with absence of temporal lobe findings on MRI but predominant lesions within the periventricular white matter, occipital lobes with extension into the subcortical frontal lobes, corpus callosum and cerebellar white matter. Although CADASIL characteristically presents with anterior temporal lobe involvement, these findings may be absent and our case addresses the atypical imaging findings in CADASIL.
  2,651 60 -
Persistent post-stroke dysphagia treated with cricopharyngeal myotomy
Sruthi S Nair, Arathy Jalaja Surendaran, Jayakumar R Menon, Sapna Erat Sreedharan, Padmavathy N Sylaja
April-June 2016, 19(2):249-251
DOI:10.4103/0972-2327.160055  PMID:27293339
Post-stroke dysphagia is a common problem after stroke. About 8-13% patients have persistent dysphagia and are unable to return to pre-stroke diet even after 6 months of stroke. Use of percutaneous endoscopic gastrostomy (PEG) may be required in these patients, which may be psychologically unacceptable and impair the quality of life. In those with cricopharyngeal dysfunction leading on to refractory post-stroke dysphagia, cricopharyngeal myotomy and injection of botulinum toxin are the treatment options. We present a case of vertebrobasilar stroke who had persistent dysphagia due to cricopharyngeal dysfunction with good recovery of swallowing function following cricopharyngeal myotomy 1.5 years after the stroke.
  2,598 57 -
Clinical and polysomnographic predictors of severe obstructive sleep apnea in the South Indian population
Sapna Erat Sreedharan, Pragati Agrawal, Ramachandrapillai S Rajith, Shana Nair, Sankara P Sarma, Ashalatha Radhakrishnan
April-June 2016, 19(2):216-220
DOI:10.4103/0972-2327.173315  PMID:27293333
Background: With the emergence of lifestyle diseases in epidemic proportions, obstructive sleep apnea (OSA) is being increasingly recognized in less developed countries as well. Aim: We sought to study the demographic, clinical, and polysomnographic (PSG) predictors of OSA severity in a cohort of South Indian patients. Materials and Methods: Consecutive patients with PSG proven OSA [apnea hypopnea index (AHI) ≥5/h] were prospectively recruited. The study period was from January 2012 to December 2012. Demographic data, history of vascular risk factors, substance abuse, sleep quality, snoring, and witnessed apneas were collected using a structured pro forma. In addition, PSG variables such as AHI, sleep latency and efficiency, duration of slow wave and rapid eye movement (REM) sleep, and other parameters were collected. Correlations between AHI severity and clinical and PSG parameters were done. Results: There were 152 (119 males and 33 females) subjects with a mean age of 53.8 years and body mass index (BMI) of 29.31. Mean AHI was 36.2/h (range: 5.1-110) and 66 subjects had severe OSA. Around 12% had the presenting complaint as insomnia, mainly of sleep maintenance. Of the subjects, 35% had witnessed apneas and 67% had excessive daytime sleepiness (EDS); 40% of patients had ≥2 risk factors. PSG parameters showed short sleep onset latency with a high arousal index. Mean apnea duration was 24.92 s. We found that age >55 years, BMI >25 kg/m2, witnessed apneas, EDS, hypertension, dyslipidemia, reduced slow wave sleep duration, mean apnea duration >20 s, and desaturation index >10/h correlated well with OSA severity while the arousal index, sleep latency and efficiency, and exposure to smoking and alcohol showed no association. Conclusions: Older subjects with witnessed apneas are likely to have more severe OSA. Even though overall sleep architecture was similar between the groups, severe OSA had shorter slow wave sleep, longer apneas, and higher nocturnal hypoxemia.
  2,504 102 -
Cardiac cephalalgia: First case from India
Sanjay Prakash, Nirav Panchani, Chaturbhuj Rathore, Prayag Makwana, Mitali Rathod
April-June 2016, 19(2):252-254
DOI:10.4103/0972-2327.165467  PMID:27293340
A 67-year-old male smoker had exertional headaches for 2 years. The headaches were holocephalic, very severe, excruciating, and occasionally accompanied by nausea. Physical examinations and neuroimaging were normal. Electrocardiogram (ECG) showed old infarct in inferior leads. Sublingual nitrate provided relief in headaches. Stress test was positive with recurrence of similar headaches with ECG changes suggestive of myocardial ischemia. Coronary angiogram revealed three-vessel disease. Coronary artery bypass surgery provided complete resolution of headaches.
  2,394 106 -
Periodic electroencephalogram discharges in a case of Lafora body disease: An unusual finding
Rajendra Singh Jain, Arti Gupta, Pankaj Kumar Gupta, Rakesh Agrawal
April-June 2016, 19(2):269-271
DOI:10.4103/0972-2327.176862  PMID:27293346
Lafora body disease (LBD) is a form of progressive myoclonic epilepsy, characterized by seizures, myoclonic jerks, cognitive decline, ataxia, and intracellular polyglucosan inclusion bodies (Lafora bodies) in the neurons, heart, skeletal muscle, liver, and sweat gland duct cells. Electroencephalogram (EEG) findings in LBD may include multiple spikes and wave discharges, photosensitivity, multifocal epileptiform discharges, and progressive slowing in background activity. Periodicity in epileptiform discharges has not been frequently depicted in LBD. We herein report an unusual case of LBD who showed generalized periodic epileptiform discharges in EEG.
  2,420 66 -
Mechanical thrombectomy for acute ischemic stroke in pregnancy using the penumbra system
Sanjith Aaron, NK Shyamkumar, Sunithi Alexander, P Suresh Babu, AT Prabhakar, Vinu Moses, TV Murthy, Mathew Alexander
April-June 2016, 19(2):261-263
DOI:10.4103/0972-2327.173302  PMID:27293343
Even though intravenous thrombolysis with tissue plasminogen activator (IV tPA) is the standard of care in acute ischemic stroke, its use in pregnancy is not clearly defined. Mechanical thrombectomy devices can be an option; however, literature on the use of such mechanical devices in stroke in pregnancy is lacking. Here we describe two cases that developed acute embolic stroke during pregnancy who were successfully treated by mechanical clot retrieval using the Penumbra system 28 (Penumbra Inc., Alameda, California, USA). To the best of our knowledge, these are the only case reports on the use of the Penumbra device in pregnant patients with acute ischemic stroke.
  2,368 71 -
Clinical and MRI findings of cerebellar agenesis in two living adult patients
Fazil Mustafa Gelal, Tugce Ozlem Kalayci, Mehmet Celebisoy, Levent Karakas, Hulya Erdogan Akkurt, Feray Koc
April-June 2016, 19(2):255-257
DOI:10.4103/0972-2327.160054  PMID:27293341
Cerebellar agenesis (CA) is an extremely rare entity. We present two adult patients with CA. The 61-year-old man had ataxia, dysarthria, abnormalities in cerebellar tests, severe cognitive impairment, and moderate mental retardation. The 26-year-old woman had dysmetria, dysdiadochokinesia, and dysarthria as well as mild cognitive impairment and mild mental retardation. Magnetic resonance imaging (MRI) showed complete absence of the cerebellum with small residual vermis. Brainstem was hypoplastic and structures above tentorium were normal. Supratentorial white matter bundles were unaffected in diffusion tensor tractography. Only few adult patients with CA have so far been published. These cases show that patients with CA present with a variety of developmental, clinical, and mental abnormalities; and emphasize the role of the cerebellum in normal motor, language, and mental development.
  2,341 52 -
Malignant transformation in a case of megalencephalic leukoencephalopathy with subcortical cysts: An extreme rarity in a rare disorder
Rajendra Singh Jain, Pankaj Kumar Gupta, Sunil Kumar, Rakesh Agrawal
April-June 2016, 19(2):242-244
DOI:10.4103/0972-2327.173303  PMID:27293337
Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is an autosomal recessive inherited disorder characterized by macrocephaly, progressive motor disability, seizures, mild cognitive decline, slow progression, and typical magnetic resonance imaging (MRI) findings. Age of onset of symptoms is described from birth to 25 years. Late onset presentation is very rare, only few cases have been reported worldwide. Most important clue for diagnosis is the characteristic MRI changes that include diffuse involvement of subcortical white matter mainly in frontoparietal region with relative sparing of central white matter along with subcortical cysts mostly in anterior temporal region. Cysts are usually benign and slowly progressive. Malignant transformation of cysts has not been reported as yet. We herein report a very unusual and probably the first case of MLC who presented to us in a unique manner with late onset and malignant transformation of cyst in left temporal region leading to rapid neurological decline. This case report highlights a possible life-threatening complication of a previously known slowly progressive disease warranting urgent neurosurgical intervention.
  2,222 65 -
Hippocampal volumes among older Indian adults: Comparison with Alzheimer's disease and mild cognitive impairment
Vikas Dhikav, Sharmila Duraisamy, Kuljeet Singh Anand, Umesh Chandra Garga
April-June 2016, 19(2):195-200
DOI:10.4103/0972-2327.176863  PMID:27293329
Background: Hippocampal volume data from India have recently been reported in younger adults. Data in older adults are unknown. The present paper describes hippocampal volume from India among older adults and compares the same with patients having Alzheimer's disease (AD) and mild cognitive impairment (MCI). Materials and Methods: A total of 32 cognitively normal subjects, 20 patients with AD, and 13 patients with MCI were enrolled. Patients were evaluated for the diagnosis of AD/MCI using the National Institute of Neurological and Communicative Disorders and Stroke and the Related Disorders Association criteria and the Clinical Dementia Rating (CDR) Scale (score = 0.5), respectively. Hippocampal volume was measured using magnetic resonance imaging (MRI) machine by manual segmentation (Megnatom Symphony 1.5T scanner) three-dimensional (3D) sequences. Results: Age and duration of illness in the MCI group were 70.6 ± 8.6 years and 1.9 ± 0.9 years, respectively. In the AD group, age and duration of illness were 72 ± 8.1 years and 3.1 ± 2.2 years, respectively. In cognitively normal subjects, the age range was 45-88 years (66.9 ± 10.32) years. Mean mini–mental status examination (MMSE) score of healthy subjects was 28.28 ± 1.33. In the MCI group, MMSE was 27.05 ± 1.79. In the AD group, MMSE was 13.32 ± 5.6. In the healthy group, the hippocampal volume was 2.73 ± 0.53 cm3 on the left side and 2.77 ± 0.6 cm3 on the right side. Likewise, in MCI, the volume on the left side was 2.35 ± 0.42 cm3 and the volume on the right side was 2.36 ± 0.38 cm3. Similarly, in the AD group, the volume on the right side was 1.64 ± 0.55 cm3 and on the left side it was 1.59 ± 0.55 cm3. Post hoc analysis using Tukey's honestly significant difference (HSD) showed, using analysis of variance (ANOVA) that there was a statistically significant difference between healthy and AD (P ≤ 0.01), and between healthy and MCI (P ≤ 0.01) subjects. There was a correlation between MMSE score and hippocampal volume in the AD group. Conclusion: The volume of the hippocampus in older Indian adults was 2.77 ± 0. 6 cm3 on the right side and 2.73 ± 0.52 cm3 on the left side. There was a significant hippocampal volume loss in MCI/AD compared to cognitively normal subjects.
  2,208 77 -
Unusual idiopathic normal pressure hydrocephalus patient with marked asymmetric and upper body parkinsonism
Kyunghun Kang, Dongho Choi, Ho-Won Lee
April-June 2016, 19(2):245-248
DOI:10.4103/0972-2327.160057  PMID:27293338
Asymmetry of parkinsonian symptoms is strong evidence toward the diagnosis of Parkinson's disease (PD). Lower body parkinsonism is characteristic in idiopathic normal pressure hydrocephalus (INPH). We report an unusual INPH patient with marked asymmetric and upper body parkinsonism. An 83-year-old man presented with gait impairment and asymmetric clumsiness of movement. According to the Unified Parkinson's Disease Rating Scale (UPDRS), the motor subscore was 12 in the left limb and 8 in the right. The score was 14 for both the upper and lower body. After the cerebrospinal fluid tap test (CSFTT), he showed marked improvement in the upper body score. A loss of asymmetry of parkinsonian signs, with greater improvement in the left limb, was presented. Fluorinated N-3-fluoropropyl-2β-carbomethoxy-3β-(4-iodophenyl)-nortropane (F-18 FP-CIT) positron emission tomography (PET) imaging was normal. In the differential diagnosis of elderly patients presenting with parkinsonism compatible with PD, we might need to consider a diagnosis of INPH.
  2,159 60 -
Clinical and radiologic features and their relationships with neurofunctional scores in patients with acute cerebellar infarct
Hasan Huseyin Kozak, Ali Ulvi Uca, Necdet Poyraz, Suleyman Omer Anliacik, Osman Serhat Tokgoz
April-June 2016, 19(2):211-215
DOI:10.4103/0972-2327.177351  PMID:27293332
Background: Cerebellar infarct is a rare condition with very nonspecific clinical features. The aim of this study was to assess the full spectrum of the clinical characteristics, neuroimaging findings and neurofunctional analyses of cerebellar infarction, and the relationship between them. Materials and Methods: Data were collected from 59 patients admitted to our department during an 8-year period. We retrospectively analyzed the relationship between demographic characteristics, clinical symptomatology, etiological factors, functional condition, vascular distribution, frequency of subcortical white matter lesions (WMLs), and concomitant lesion outside the cerebellum in patients with acute cerebellar infarct (ACI) at time of admission. Results: The mean age in our series was 65.2 years, with most being male (57.6%). The posterior inferior cerebellar (PICA) artery was the most commonly affected territory at 62.7%. There was concomitant lesion outside the cerebellum in 45.7%. The main etiology in PICA was cardioembolism. While mean National Institutes of Health Stroke Scale on admission was 2.08 ± 1.67 in study group, modified Rankin Scale (mRS) on admission was detected to be mRS1 (n: 44, 74.5%) and mRS2 (n: 12, 20.3%) most frequently. Fourteen (35%) patients were detected to be in Fazekas stage 0; 11 (27.5%) patients in Fazekas stage 1; 6 (15%) patients in Fazekas stage 2; and 9 (22.5%) patients in Fazekas stage 3. Conclusion: Cerebellar infarct is very heterogeneous. The other cerebral area infarcts which accompany ACI negatively affect neurologic functional scores. Although it is difficult to detect the relationship between WMLs and neurologic functional severity, timely detection of risk factors and their modulation may be associated with prevention and treatability of WMLs, and this may be one of the important points for prevention of stroke-related disability.
  2,132 73 -
Bilateral familial Hirayama disease in a father and daughter
Sanjay Pandey, Shruti Jain
April-June 2016, 19(2):264-266
DOI:10.4103/0972-2327.173410  PMID:27293344
We are reporting a case of bilateral familial Hirayama disease where a father and daughter are the affected members of the family with the similar distribution of their weakness and wasting. To the best of our knowledge, bilateral familial Hirayama disease has not been described in father and daughter.
  2,105 69 -
Osteoporosis in patients with stroke: A cross-sectional study
Snezana Tomasevic-Todorovic, Dusica Simic-Panic, Aleksandar Knezevic, Cila Demesi-Drljan, Dusan Maric, Fahad Hanna
April-June 2016, 19(2):286-288
DOI:10.4103/0972-2327.173409  PMID:27293356
  2,091 45 -
Necrotizing autoimmune myopathy
Sachin Suresh Babu, Laxmi Khanna, Ravindra Kumar Saran, Gaurav Mittal, Sudhir Peter, Ivy Sebastian
April-June 2016, 19(2):288-290
DOI:10.4103/0972-2327.176864  PMID:27293357
  2,061 71 -
Sporadic and familial myoclonic dystonia: Report of three cases and review of literature
Kalyan B Bhattacharyya, Arijit Roy, Atanu Biswas, Ashutosh Pal
April-June 2016, 19(2):258-260
DOI:10.4103/0972-2327.168625  PMID:27293342
Myoclonic dystonia refers to a clinical syndrome characterized by rapid jerky movements along with dystonic posturing of the limbs. Clinically, it is characterized by sudden, brief, electric shock-like movements, mostly involving the upper extremities, shoulders, neck and trunk. Characteristically, the movements wane with consumption of small dose of alcohol in about 50% of cases. Additionally, dystonic contractions are observed in most of the patients in the affected body parts and some patients may exhibit cervical dystonia or graphospasm as well. It may manifest as an autosomal dominant condition or sometimes, as a sporadic entity, though there are doubts whether these represent cases with reduced penetrance. The condition is usually treated with a combination of an anticholinergic agent like, benztropine, pimozide and tetrabenazine. We report one sporadic case and one familial case where the father and the son are affected. The cases were collected from the Movement Disorders Clinic of Bangur Institute of Neurosciences, Kolkata, West Bengal in a period of ten months. Myoclonic dystonia is a rare condition and to the best of our knowledge, this series is the first one reported from our country. Videos of the patients are also provided with the article.
  2,049 64 -
Late-onset myoclonic epilepsy in Down syndrome (LOMEDS): A spectrum of progressive myoclonic epilepsy — Case report
Chandra Mohan Sharma, Rajendra Kumar Pandey, Banshi Lal Kumawat, Dinesh Khandelwal
April-June 2016, 19(2):267-268
DOI:10.4103/0972-2327.173411  PMID:27293345
Cognitive decline and epilepsy are well recognized complication of Down syndrome (DS). Here, we intend to present a case of 28 year old male who presented with progressive mental regression, gait ataxia and myoclonic jerking especially on awakening in morning. His EEG was normal and karyotyping revealed trisomy of chromosome 21. Very few cases had been described in literature of late-onset myoclonic epilepsy in DS. This is first case report from India and our aim is to propose the inclusion of this entity in the spectrum of progressive myoclonic epilepsies but still more cases are yet to be found.
  1,979 58 -
Atypical magnetic resonance imaging features in subacute sclerosing panencephalitis
Biplab Das, Manoj Kumar Goyal, Manish Modi, Sahil Mehta, Sudheer Chakravarthi, Vivek Lal, Sameer Vyas
April-June 2016, 19(2):275-276
DOI:10.4103/0972-2327.173304  PMID:27293348
Objectives: Subacute sclerosing panencephalitis (SSPE) is rare chronic, progressive encephalitis that affects primarily children and young adults, caused by a persistent infection with measles virus. No cure for SSPE exists, but the condition can be managed by medication if treatment is started at an early stage. Methods and Results: Heterogeneity of imaging findings in SSPE is not very uncommon. But pial and gyral enhancements are very rarely noticed. Significant asymmetric onset as well as pial-gyral enhancements is not reported. Herein we present a case of 16 years adolescent of SSPE having remarkable asymmetric pial-gyral enhancements, which were misinterpreted as tubercular infection. Conclusion: Early diagnosis and treatment is encouraging in SSPE, although it is not curable with current therapy. Clinico-radiological and electrophysiological correlation is very important in diagnosis of SSPE, more gravely in patients having atypical image findings as in our index case.
  1,855 83 -
”Missing clot” during mechanical thrombectomy in acute stroke using Solitaire stent retrieval system
Vikram Huded, Vivek Nambiar, Romnesh De Souza, Vikram Bohra, Ritesh Ramankutty
April-June 2016, 19(2):279-280
DOI:10.4103/0972-2327.176866  PMID:27293350
Stent retrieval system is an established treatment modality in acute ischemic stroke with large vessel occlusion. Here, we describe a complication which occurred during mechanical thrombectomy in three cases where the clot dislodged during retrieval. There was a possibility of the clot getting reinjected into the artery with possible dire consequences.
  1,857 61 -
Mutations in GABRG2 receptor gene are not a major factor in the pathogenesis of mesial temporal lobe epilepsy in Indian population
Aparna Banerjee Dixit, Jyotirmoy Banerjee, Abuzar Ansari, Manjari Tripathi, Sarat P Chandra
April-June 2016, 19(2):236-241
DOI:10.4103/0972-2327.182304  PMID:27293336
Aim: This study is focused on GABRG2 gene sequence variations in patients with mesial temporal lobe epilepsy (mTLE). The GABAAreceptor is a heteropentameric receptor and alpha-1 beta-2 gamma-2 subunits combination is most abundant and present in almost all regions of the brain. The gamma-2 subunit (GABRG2) gene mutations have been reported in different epilepsy pathologies. In the present study we have looked for GABRG2 gene sequence variations in patients with mTLE. Materials and Methods: Twenty patients (12 females and eight males, age 4.6-38 years) with MTLE were recruited for this investigation. Patients were recommended for epilepsy surgery after all clinical investigations as per the epilepsy protocol. Ethnically matched glioma or meningioma patients were considered as nonepileptic controls. During temporal lobectomy of amygdalohippocampectomy, hippocampal brain tissue samples were resected guided by intraoperative electrocorticography (ECoG) activity. All 11 exons of GABRG2 gene with their flanking intronic regions were amplified by polymerase chain reaction (PCR) and screened by DNA sequencing analysis for sequence variations. Statistical Analysis Used: Comparison of allele frequencies between patient and control groups was determined using a c2 test. Results and Conclusions: Total five DNA sequence variations were identified, three in exonic regions (c.643A > G, rs211035), (c.T > A, rs424740), and (c.C > T, rs418210) and two in intronic regions (c.751 + 41A > G, rs211034) and (c.751 + 52G > A, rs 34281163). Allele frequencies of variants identified in this study did not differ between patients and normal controls. Thus, we conclude that GABRG2 gene may not be playing significant role in the development of epilepsy or as a susceptibility gene in patients with MTLE in Indian population.
  1,857 52 -
Topiramate precipitating mania in bipolar disorder
NA Uvais, VS Sreeraj
April-June 2016, 19(2):283-284
DOI:10.4103/0972-2327.176858  PMID:27293353
  1,808 75 -
In memoriam for Prof. Noshir H. Wadia
Bhim Sen Singhal, Sarosh M Katrak
April-June 2016, 19(2):292-293
  1,736 88 -
Coexistence of central nucleus, cores, and rods: Diagnostic relevance
Sathiyabama Dhinakaran, Rashmi Santhosh Kumar, Ravindra Thakkar, Gayathri Narayanappa
April-June 2016, 19(2):201-204
DOI:10.4103/0972-2327.176861  PMID:27293330
Background: Congenital myopathies (CMs) though considered distinct disorders, simultaneous occurrence of central nucleus, nemaline rods, and cores in the same biopsy are scarcely reported. Objective: A retrospective reassessment of cases diagnosed as CMs to look for multiple pathologies missed, if any, during the initial diagnosis. Materials and Methods: Enzyme histochemical, and immunohistochemical-stained slides from 125 cases diagnosed as congenital myopathy were reassessed. Results: The study revealed 15 cases (12%) of congenital myopathy with more than one morphological feature. Central nucleus with cores (n = 11), central nucleus, nemaline rods and cores (n = 3), and nemaline rods with cores (n = 1). 4/11 cases were diagnosed as centronuclear myopathy (CNM) in the first instance; in addition, cores were revealed on reassessment. Discussion: The prevalence of CMs of all neuromuscular disorders is approximately 6 in 100,000 live births, with regional variations. Three main defined CMs include centro nuclear myopathy (CNM), nemaline rod myopathy (NRM), and central core disease (CCD). However, they are more diverse with overlapping clinical and histopathological features, thus broadening the spectra within each category of congenital myopathy. Conclusion: Identification of cases with overlap of pathological features has diagnostic relevance.
  1,725 58 -
Hashimoto's encephalopathy as a treatable cause of corticobasal disease
Sasi Kumar Sheetal, Robert Mathew, Byju Peethambaran
April-June 2016, 19(2):285-286
DOI:10.4103/0972-2327.176859  PMID:27293355
  1,709 58 -
Autoimmune encephalitis
Beuy Joob, Viroj Wiwanitkit
April-June 2016, 19(2):284-284
DOI:10.4103/0972-2327.176868  PMID:27293354
  1,681 68 -
Frontal “wasting”: Cortical arteriovenous malformation causing hand wasting
Aastha Takkar, Sahil Mehta, Chirag K Ahuja, Manoj Kumar Goyal, Manish Modi, Kanchan K Mukherjee, Ashish Pathak
April-June 2016, 19(2):277-278
DOI:10.4103/0972-2327.176865  PMID:27293349
  1,539 72 -
Did Mozart suffer from Tourette's syndrome?
PA Bhaskar
April-June 2016, 19(2):282-282
DOI:10.4103/0972-2327.173309  PMID:27293352
  1,567 43 -
Defining role of vitamin D in cognitive decline
Sunil Kumar Raina
April-June 2016, 19(2):290-291
DOI:10.4103/0972-2327.176860  PMID:27293358
  1,503 51 -
Utility of cerebrospinal fluid cortisol level in acute bacterial meningitis
Anish Mehta, Rohan R Mahale, Uchil Sudhir, Mahendra Javali, Rangasetty Srinivasa
April-June 2016, 19(2):281-282
DOI:10.4103/0972-2327.173412  PMID:27293351
  1,439 48 -
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