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2015| January-March | Volume 18 | Issue 1
Online since
February 10, 2015
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HISTORY OF NEUROLOGY
The neuropsychiatric ailment of Vincent Van Gogh
Kalyan B Bhattacharyya, Saurabh Rai
January-March 2015, 18(1):6-9
DOI
:10.4103/0972-2327.145286
PMID
:25745302
Vincent Van Gogh is one of the most celebrated creative artists of all time. All his life, he was afflicted by some kind of neurological or psychiatric disorder, which remains a mystery even today. Many historians and his personal physicians believe that he suffered from epilepsy while others felt that he was affected by Ménière's disease. Features such as hypergraphia, atypical sexuality, and viscosity of thinking suggest the possibility of Gastaut-Geschwind phenomenon, a known complication of complex partial seizure. On the contrary, some historians feel that he was forced to sever his right ear in order to get relief from troublesome tinnitus, a complication of Ménière's disease. He was addicted to the liquor absinthe, which is known to lead to xanthopsia, and many authorities argue that this was the reason for his penchant for the deep and bright yellow color in many of his paintings. Others have suggested the possibility of bipolar disorder, sunstroke, acute intermittent porphyria, and digitalis toxicity as well.
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ORIGINAL ARTICLES
Comparison of Levetiracetam and sodium Valproate in migraine prophylaxis: A randomized placebo-controlled study
Homa Sadeghian, Rouzbeh Motiei-Langroudi
January-March 2015, 18(1):45-48
DOI
:10.4103/0972-2327.144290
PMID
:25745310
Background:
Migraine is a chronic and disabling disorder. Treatment of migraine often comprises of symptomatic (abortive) and preventive (prophylactic) treatment. The current drugs used in migraine prophylaxis include antidepressant drugs (Serotonin Reuptake Inhibitors, Tricyclic antidepressants), and anti-epileptic drugs (valproate, gabapentin, etc).
Objective:
The objective of our study was to assess the efficacy and tolerability of levetiracetam in adult migraine prophylaxis, compared to valproate and placebo.
Materials and Methods:
We conducted a prospective, randomized, placebo-controlled study. A total of 85 patients were randomized to receive levetiracetam 500 mg/d (
n
= 27), valproate 500 mg/d (
n
= 32) or placebo (
n
= 26). The patients were evaluated for treatment efficacy after 6 months. Efficacy was assessed as a more than 50% decrease in headache frequency.
Results:
In levetiracetam group, 17 (63.0%) patients experienced a more than 50% decrease in headache frequency, while this efficacy number was 21 (65.6%) for valproate group and 4 (15.4%) for placebo group. The difference was not statistically significant between levetiracetam and valproate, while it was significant when comparing either levetiracetam or valproate to placebo.
Conclusion:
Compared to placebo, levetiracetam offers improvement in headache frequency in patients with migraine. The efficacy of levetiracetam in migraine prophylaxis is comparable to currently used drugs such as valproate.
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Impulse control disorders and related behaviours (ICD-RBs) in Parkinson's disease patients: Assessment using "Questionnaire for impulsive-compulsive disorders in Parkinson's disease" (QUIP)
Ashish Sharma, Vinay Goyal, Madhuri Behari, Achal Srivastva, Garima Shukla, Deepti Vibha
January-March 2015, 18(1):49-59
DOI
:10.4103/0972-2327.144311
PMID
:25745311
Background:
There is limited data on the prevalence of impulse control disorder and related behaviors (ICD-RBs) in Indian patients with Parkinson's Disease (PD). In the context of potential genetic and environmental factors affecting the expression of ICD-RBs, studying other multiethnic populations may bring in-sights into the mechanisms of these disorders.
Objectives:
To ascertain point prevalence estimate of ICD-RBs in Indian PD patients, using the validated "Questionnaire for Impulsive-Compulsive Disorders in Parkinson's disease (QUIP)" and to examine their association with Dopamine replacement therapy (DRT).
Materials and Methods:
This was a hospital based observational cross-sectional study. After taking informed consent, patients and their informants (spouse, or primary caregiver) were made to complete the QUIP, and were instructed to answer questions based on behaviors that occurred anytime during PD that lasted at least four consecutive weeks.
Results:
Total of 299 patients participated in the study. At least one ICD-RB was present in 128 (42.8%), at least one Impulse control disorder (ICD) was present in 74 (24.75%) and at least one Impulse control related compulsive behaviour (ICRB) was present in 93 (31.1%) patients. Punding was the most frequent (12.4%) followed by hyper sexuality (11.04%), compulsive hobbyism (9.4%), compulsive shopping (8.4%), compulsive medication use (7.7%), compulsive eating (5.35%), walkabout (4%) and pathological gambling (3.3%). ≥ 2 ICD-RBs were observed in 15.7% of patients. After multivariate analysis, younger age of onset, being unmarried were specifically associated with presence of ICD. Longer disease duration was specifically associated with presence of ICRB. Whereas smoking and higher dopamine levodopa equivalent daily doses (DA LEDD) were associated with both presence of ICD and ICRB. Higher LD LEDD was specifically associated with presence of ICD-RB.
Conclusions:
Our study revealed a relatively higher frequency of ICD-RBs, probably because of the use of screening instrument and because we combined both ICDs and ICRBs. Also high proportion of DA use (81.6%) among our patients might be responsible. The role of genetic factors that might increase the risk of developing ICD-RBs in this population needs further exploration.
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Plasmapheresis in neurological disorders: Experience from a tertiary care hospital in South India
V Abdul Gafoor, James Jose, K Saifudheen, Mohamed Musthafa
January-March 2015, 18(1):15-19
DOI
:10.4103/0972-2327.144301
PMID
:25745304
Background:
Therapeutic plasma exchange (PE) or plasmapheresis is the treatment of choice in many neurological disorders. Even though it is safe in experienced hands, there is a major concern about its safety among physicians.
Objectives:
To analyze our experience with 230 patients who underwent PE for various neurological disorders.
Materials and Methods:
Retrospective review of PE procedures done during a period of 48 months, from July 2007 to June 2011 in a tertiary care teaching hospital in South India. Indications, clinical results and technical factors are discussed.
Results:
The main indication for PE was GBS (203 patients; 88.3%). Age of patients ranged from 14-65 (mean = 42.3 years). The most common complications were paraesthesias and/or cramps (36.1%) and hypotension (32.2%). Four pregnant patients who underwent PE had good recovery with one intrauterine death. There was no mortality.
Conclusion:
The analysis of 240 cases of PE done in our department shows that the procedure is safe, with only minimal procedure related complications and no mortality.
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Pattern and risk factors of stroke in the young among stroke patients admitted in medical college hospital, Thiruvananthapuram
Praveen Prasannakumar Subha, Sabarinadh Muraleedharan Pillai Geethakumari, Mohan Athira, Zinia Thajudeen Nujum
January-March 2015, 18(1):20-23
DOI
:10.4103/0972-2327.144293
PMID
:25745305
Background:
Stroke in the young is particularly tragic because of its potential to create a long-term burden on the victims, their families, and the community. There had been relatively few studies on young stroke in Kerala's socio-economic setup, that too encapsulating the mentioned apparently relevant dimensions of stroke in the young
. Objective:
To study the prevalence, patterns and risk factors of young stroke.
Settings and Design:
A cross-sectional study with case control comparison at Government Medical College Hospital, Thiruvananthapuram, Kerala, India.
Materials and and Methods:
Total 100 stroke patients were identified over a period of 2 months, and data were collected on the basis of questionnaire developed for the purpose.
Results:
Of 100 stroke patients, 15 had stroke in the young, among which 9 (60%) had ishaemic stroke. Hypertension was the most common risk factor. Smoking, alcohol, atrial fibrillation, and hyperlipidemia were found to be more common in cases (young stroke) when compared with controls. Alcohol use and atrial fibrillation were significantly higher among young stroke patients. Physical inactivity was significantly lesser in those with stroke in the young than elderly. Atrial fibrillation emerged as an independent risk factor of stroke in the young with adjusted odds ratio of 6.18 (1.31-29.21).
Conclusion:
In all, 15% of total stroke occurred in young adults <50 years. The proportion of hemorrhagic stroke in young adults is higher than in elderly. Atrial fibrillation is identified as an independent risk factor of stroke in the young. Compared with stroke in elderly alcohol use, smoking, hyperlipidemia, and cardiac diseases, which are known risk factors, are higher in young stroke.
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CASE REPORTS
Becker's nevus with neurofibromatosis type 1
Sumit Kar, Krishnan Preetha, Nidhi Yadav, Bhushan Madke, Nitin Gangane
January-March 2015, 18(1):90-92
DOI
:10.4103/0972-2327.144281
PMID
:25745321
Neurofibromatosis type 1 is an autosomal dominant disorder which primarily affects the growth and development of neural cell tissues. It presents as multiple tumor-like growths over the skin that arises from the nerves and is associated with other abnormalities like pigmentation over the skin and bone deformities. Becker's nevus or hairy pigmented epidermal nevus is a benign cutaneous hamartoma which is characterized by hyperpigmented macule with hypertrichosis. It is rarely associated with neurofibromatosis. We report a 22-years-old male with coexistent Becker's nevus and type 1 neurofibromatosis.
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ORIGINAL ARTICLES
Pediatric Guillain-Barré syndrome: Indicators for a severe course
Muhammet Ali Varkal, Tuğçe Aksu Uzunhan, Nur Aydınlı, Barış Ekici, Mine Çalışkan, Meral Özmen
January-March 2015, 18(1):24-28
DOI
:10.4103/0972-2327.144274
PMID
:25745306
Objectives:
This study aims to retrospectively evaluate pediatric Guillain-Barré syndrome cases in a tertiary center in Istanbul, Turkey.
Materials and Methods:
The data of 40 patients with Guillain-Barré syndrome who had been admitted to the Department of Pediatrics at the Istanbul University Medical Faculty between 2005 and 2011 were collected. Mann-Whitney U, Kruskal-Wallis, chi-square, and Fisher's exact tests were used for statistical analysis.
Results:
Mean patient age was 5.4 ± 3.0 years; 20 out of 40 patients (50%) were female and 20 (50%) were male. Preceding infection was detected in 32 cases (80%). Six patients had speech impairment. Out of eight patients with respiratory distress (20%), five required respiratory support (12.5%) of which three of them had speech impairment as well. According to nerve conduction studies, 21 patients (52.5%) had acute inflammatory demyelinating polyradiculoneuropathy, 14 (35%) had acute motor axonal neuropathy, and five (12.5%) had acute motor-sensory axonal neuropathy. Thirty-three patients (82.5%) received intravenous immunglobulin, 3 (7.5%) underwent plasmapheresis and 4 (10%) received both. Time until recovery (
P
= 0.022) and time until aided (
P
= 0.036) and unaided (
P
= 0.027) walking were longer in patients with acute gastrointestinal infection than in those with upper respiratory tract infection (
P
< 0.05). Time until response to treatment (
P
= 0.001), time until aided (
P
= 0.001) and unaided (
P
= 0.002) walking, and time until complete recovery (
P
= 0.002) were longer in acute motor axonal neuropathy cases as compared to acute inflammatory demyelinating polyradiculoneuropathy cases.
Conclusion:
Recovery was longer with acute gastrointestinal infection and acute motor axonal neuropathy. Speech impairment could be a clinical clue for the need of mechanical ventilation.
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Seizures in patients with cerebral hemiatrophy: A prognostic evaluation
Anupam Jaiswal, Ravindra Kumar Garg, Hardeep Singh Malhotra, Rajesh Verma, Maneesh Kumar Singh
January-March 2015, 18(1):39-44
DOI
:10.4103/0972-2327.144296
PMID
:25745309
Purpose:
Cerebral hemiatrophy is a common childhood disease. It clinically manifests with seizures, hemiparesis and mental retardation.
Materials and Methods:
In this prospective study, previously untreated patients with seizures and cerebral hemiatrophy were recruited. Cerebral hemiatrophy was diagnosed on the basis of hemispheric ratio. Patients with acquired hemiconvulsion, hemiplegia, and epilepsy (HHE) syndrome were included in group A. Group B included patients with congenital HHE syndrome. Patients were followed up for 6 months for seizure recurrence.
Results:
Out of 42 patients 26 were in group A and 16 were in group B. After 6 months, there was significant reduction in seizure frequency (
P
< 0.0001) in both the groups. At least 50% reduction in seizure frequency was noted in all the patients. Complete seizure freedom was observed in 15 (35.7%) patients. Seizure recurrences were significantly higher (
P
= 0.008) in group A. On univariate analysis, predictors of seizure recurrences were history of febrile seizures (
P
= 0.013), hippocampal sclerosis (
P
= 0.001), thalamic atrophy (
P
= 0.001), basal ganglia atrophy (
P
= 0.001), cerebellar atrophy (
P
= 0.01), ventricular dilatation (
P
= 0.001), epileptiform discharges at presentation (
P
= 0.023), complex partial seizures (
P
= 0.006) and status epilepticus (
P
= 0.02). On multivariate analysis, hemispheric ratio was the only significant factor for seizure recurrence.
Conclusion:
Patients with congenital hemiatrophy had better seizure control than that in patients with HHE syndrome.
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CASE REPORTS
Generalized chorea due to delayed encephalopathy after acute carbon monoxide intoxication
Yueh-Feng Sung, Ming-Hua Chen, Giia-Sheun Peng, Jiunn-Tay Lee
January-March 2015, 18(1):108-110
DOI
:10.4103/0972-2327.144288
PMID
:25745326
Movement disorder due to delayed encephalopathy after carbon monoxide (CO) intoxication is uncommon. Generalized chorea, presenting as an initial symptom of delayed encephalopathy, is extremely rare. We describe a 60-year-old woman, who had completely recovered from acute CO poisoning, developed mental and behavioral changes, urinary incontinence and generalized chorea 2 weeks thereafter. T2-weighted brain magnetic resonance imaging showed extensive hyperintensity of the bilateral periventricular and subcortical white matter and the globus pallidus. Brain single-photon emission computed tomography (SPECT) with technetium-99 ethylene cysteine dimer showed inhomogeneous perfusion in the cerebral cortex, with decreased uptake in bilateral frontal regions. Delayed encephalopathy after acute CO intoxication was diagnosed, and the symptoms gradually improved after hyperbaric oxygen therapy (HBOT). This case report demonstrates that generalized chorea may be one of the initial presenting symptoms of delayed encephalopathy after acute CO intoxication. We hypothesize that the generalized chorea in our patient may have been caused by the subcortical white matter lesions, which most likely interrupted the basal ganglia-thalamocortical circuits and that HBOT may be the treatment of choice for such patients.
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ORIGINAL ARTICLES
Neuropsychiatric co-morbidities in non-demented Parkinson's disease
Nirendra Kumar Rai, Vinay Goyal, Nand Kumar, Garima Shukla, Achal Kumar Srivastava, Sumit Singh, Madhuri Behari
January-March 2015, 18(1):33-38
DOI
:10.4103/0972-2327.144287
PMID
:25745308
Objective:
To evaluate neuropsychiatric co-morbidities (depression, psychosis and anxiety) in non-demented patients with Parkinson's disease (PD).
Background:
Non-motor symptoms like neuropsychiatric co-morbidities are common in Parkinson's disease and may predate motor symptoms. Currently there is scarcity of data regarding neuropsychiatry manifestations in Indian patients with PD.
Methods:
In this cross-sectional study consecutive 126 non-demented patients with PD (MMSE ≥25) were enrolled. They were assessed using Unified Parkinson's disease rating scale (UPDRS), Hoehn & Yahr (H&Y) stage, Schwab and England (S&E) scale of activity of daily life. Mini-international neuropsychiatric interview (MINI) was used for diagnosis of depression, psychosis and anxiety. Beck's depression inventory (BDI), Brief psychiatric rating scale (BSRS) and Hamilton rating scale for anxiety (HAM-A) scales were used for assessment of severity of depression, psychosis and anxiety respectively.
Results:
Mean age and duration of disease was 57.9 ± 10.9 years and 7.3 ± 3.6 years respectively. At least one of the neuropsychiatric co-morbidity was present in 64% patients. Depression, suicidal risk, psychosis and anxiety were present in 43.7%, 31%, 23.8% and 35.7% respectively. Visual hallucinations (20.6%) were most frequent, followed by tactile (13.5%), auditory (7.2%) and olfactory hallucinations (1.6%). Patients with depression had higher motor disability (UPDRS-motor score 33.1 ± 14.0 vs 27.3 ± 13.3; and UPDRS-total 50.7 ± 21.8 vs 41.0 ± 20.3, all p values <0.05). Patients with psychosis were older (63.6 ± 8.0 years vs 56.1 ± 11.1 years,
p
< 0.05) and had longer duration of illness (8.6 ± 3.4 years vs 6.9 ± 3.5,
p
< 0.05).
Conclusions:
About two third patients with Parkinson's disease have associated neuropsychiatric co-morbidities. Depression was more frequent in patients with higher disability and psychosis with longer duration of disease and older age. These co-morbidities need to be addressed during management of patients with PD.
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Adaptation and validation of stroke-aphasia quality of life (SAQOL-39) scale to Hindi
Ishita H Mitra, Gopee Krishnan
January-March 2015, 18(1):29-32
DOI
:10.4103/0972-2327.144276
PMID
:25745307
Background:
Stroke is a major detriment to the quality of life (QOL) in its victims. Several functional limitations following stroke contribute to the denigrated QOL in this population. Aphasia, a disturbance in the comprehension, processing, and/or expression of language, is a common consequence of stroke. Yet, in most Indian languages, including the national language (Hindi), there are no published tools to measure the QOL of persons with stroke-aphasia.
Objective:
The current study was carried out to adapt and validate a well-known tool to measure the QOL (i.e., Stroke-Aphasia Quality of Life-39; SAQOL-39) to Hindi.
Materials and Methods:
We presented the original (English) version of the SAQOL-39 to a group of six Hindi-speaking Speech Language Pathologists hailing from the central and northern regions of India to examine the sociocultural suitability of items and indicate modifications, if any. The linguistic adaptation was performed through a forward-backward translation scheme. The socioculturally and linguistically adapted (to Hindi) version was then administered on a group of 84 Hindi-speaking persons with aphasia to examine the acceptability, test-retest reliability as well as the internal consistency of the instrument.
Results:
The SAQOL-39 in Hindi exhibited high test-retest reliability (ICC = 0.9) as well as acceptability with minimal missing data. This instrument exhibited high internal consistency (Chronbach's ∝ = 0.98) as well as the both item-to-total and inter-domain correlations.
Conclusions:
The socioculturally and linguistically adapted Hindi version of SAQOL-39 is a robust tool to measure the QOL of persons with stroke-aphasia. It may serve as an essential tool to measure the QOL in this population for both clinical and research purposes.
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CASE REPORTS
Fazio Londe syndrome: A treatable disorder
Poovazhagi Varadarajan, Vimal Thayanathi, Leema C Pauline
January-March 2015, 18(1):87-89
DOI
:10.4103/0972-2327.144283
PMID
:25745320
Fazio Londe Syndrome is a rare neurological disorder presenting with progressive bulbar palsy with respiratory failure. Initially considered to have an unrelenting course, is now found to be due to mutations in the
SLC52A3
gene which encodes the intestinal (hRFT2) riboflavin transporter in some children. We report an 11-year-old child with features of Fazio Londe syndrome who presented to our Institute with respiratory failure.
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REVIEW ARTICLE
Management of REM sleep behavior disorder: An evidence based review
Preeti Devnani, Racheal Fernandes
January-March 2015, 18(1):1-5
DOI
:10.4103/0972-2327.150927
PMID
:25745301
Rapid eye movement (REM) sleep behavior disorder (RBD) is characterized by dream enactment behavior resulting from a loss of REM skeletal muscle atonia. The neurobiology of REM sleep and the characteristic features of REM atonia have an important basis for understanding the aggravating etiologies the proposed pharmacological interventions in its management. This review outlines the evidence for behavioral and therapeutic measures along with evidence-based guidelines for their implementation, impact on falls, and effect on polysomnography (PSG) while highlighting the non-motor, autonomic, and cognitive impact of this entity. PubMed databases were reviewed upto May 2013 in peer-reviewed scientific literature regarding the pathophysiology and management of RBD in adults. The literature was graded according to the Oxford centre of evidence-based Medicine Levels. An early intervention that helps prevent consequences such as falls and provides a base for intervention with neuroprotective mechanisms and allocates a unique platform that RBD portrays with its high risk of disease conversion with a sufficiently long latency. RBD provides a unique platform with its high risk of disease conversion with a sufficiently long latency, providing an opportunity for early intervention both to prevent consequences such as falls and provide a base for intervention with neuroprotective mechanisms.
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CASE REPORTS
Bright splenium of a psychotic mind
Shankar C Udaya, Bhumir N Chauhan, Vivek J Philip
January-March 2015, 18(1):80-83
DOI
:10.4103/0972-2327.145287
PMID
:25745318
A twenty-one-year old primigravida presented two weeks postpartum with new onset of behavioral alteration and irrelevant talking without fever, headache, or seizures. Magnetic resonance imaging (MRI) of brain showed a lesion with diffusion restriction in splenium of corpus callosum. A possibility of postpartum psychosis was considered after ruling out various etiologies for splenium hyperintense lesion. Near complete resolution of symptoms was noted after initiation of antipsychotic medications. Literature has described multiple etiologies for lesions involving splenium of corpus callosum on brain imaging. We present this case as a unique clinical presentation of splenial pathology in the form of postpartum psychosis.
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2
IMAGES IN NEUROLOGY
"Dot sign" in dengue encephalitis
Thomas Mathew, Sagar Badachi, Gosala Raja Kukkuta Sarma, Raghunandan Nadig
January-March 2015, 18(1):77-79
DOI
:10.4103/0972-2327.144306
PMID
:25745317
Neuro radiological findings in Dengue encephalitis are non specific .Here we report a case of Dengue encephalitis with transient splenial hyperintensity appearing as dot sign on magnetic resonance imaging of brain.
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4,367
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CASE REPORTS
Spinocerebellar ataxia-10 with paranoid schizophrenia
Bhavesh Trikamji, Parampreet Singh, Shrikant Mishra
January-March 2015, 18(1):93-95
DOI
:10.4103/0972-2327.144285
PMID
:25745322
Spino-cerebellar ataxia type 10 (SCA10) is an autosomal dominant disorder that is characterized by cerebellar ataxia, seizures and nystagmus with a fragmented pursuit. Schizophrenia has been reported with SCAs 1 and 2 yet in SCA 10, psychiatric manifestations are uncommon. We report a Hispanic family involving a father and his four children with SCA10 genetic mutation. Two of his children, a 20-year-old female and a 23-year-old male, presented with gradually progressive spino-cerebellar ataxia and paranoid schizophrenia. Neurological examination revealed ocular dysmetria, dysdiadokinesia, impaired finger-to-nose exam, gait ataxia and hyperreflexia in both the cases. Additionally, they had a history of psychosis with destructive behavior, depression and paranoid delusions with auditory hallucinations. Serology and CSF studies were unremarkable and MRI brain revealed cerebellar volume loss. Ultimately, a test for ATAXIN-10 mutation was positive thus confirming the diagnosis of SCA10 in father and his four children. We now endeavor to investigate the association between schizophrenia and SCA10.
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Central pontine myelinolysis presenting as isolated sixth nerve palsy in third trimester of pregnancy
Tushar Divakar Gosavi, Siew Ju See
January-March 2015, 18(1):84-86
DOI
:10.4103/0972-2327.144291
PMID
:25745319
A 30-year-old primigravida presented with isolated left sixth nerve palsy at 38 weeks gestation. Her MRI showed a lesion consistent with central pontine myelinolysis (CPM). Extensive investigations did not reveal any secondary cause for the CPM. She recovered spontaneously in 2 weeks with complete resolution of her MRI changes. To our knowledge, this is the first report of CPM occurring in third trimester in the absence of identifiable secondary causes and of CPM presenting as an isolated sixth nerve palsy. We discuss the reported causes of CPM in pregnancy, possible pathophysiologic mechanisms involved and the anatomic basis of the unique clinical presentation of sixth nerve palsy in our case.
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4,353
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Episodic neurological dysfunction in hereditary peripheral neuropathy
Girish Baburao Kulkarni, Pooja Mailankody, Pawanraj Palu Isnwara, Chandrajit Prasad, Veerendrakumar Mustare
January-March 2015, 18(1):111-114
DOI
:10.4103/0972-2327.144314
PMID
:25745327
Episodic transient neurological symptoms are an important set of problems presenting to a neurologist in his routine practice. Occasionally, detailed clinical history including past and family history supplemented with focused examination can bring out a rare cause for such symptoms. We describe in this report in a young male presenting with episodic focal neurological dysfunction, with family history of similar episodes in mother and brother. Examination showed features of pes cavus and peripheral neuropathy for which patient was asymptomatic. Mother and brother were established cases of hereditary neuropathy. Imaging on multiple occasions showed reversible white matter abnormalities. Clinical suspicion of X-linked Charcot-Marie-Tooth disease type 1 (CMT1X) was confirmed with detection of mutation in Gap Junction B1 (GJB1) gene, which codes for connexin 32 protein (c.425G>A; p.R142Q hemizygous mutation). Though this mutation has been already reported in CMTX patients, it has not been associated with transient neurological dysfunctions. This is probably the first reported case of CMTX patient with transient neurological dysfunction from India, whose family members had similar episodes.
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4,211
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Unilateral rubral tremors in Wilson's disease treated with dimercaprol
Rahul T Chakor, Haresh Bharote, Nishikant Eklare, Khansabegum Tamboli
January-March 2015, 18(1):115-116
DOI
:10.4103/0972-2327.144286
PMID
:25745328
Tremors are reported as the most frequent neurological manifestation of Wilson's disease (WD) in some series. Postural tremors, rest tremors, action tremors and wing-beating (rubral) tremors are the different types of tremors seen in WD. We report a patient of WD with unilateral rubral tremors refractory to 1-year therapy with Penicillamine and anti-tremor medications. The tremors decreased considerably after adding chelation therapy with dimercaprol. Combination of Penicillamine and dimercaprol is an effective decoppering measure in rubral tremors of WD.
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ORIGINAL ARTICLES
Quantitative EEG and medial temporal lobe atrophy in
Alzheimer's dementia
: Preliminary study
Soo-Ji Lee, Min-Ho Park, Sang-Soon Park, Jin-Young Ahn, Jae-Hyeok Heo
January-March 2015, 18(1):10-14
DOI
:10.4103/0972-2327.145284
PMID
:25745303
Backgrounds:
The electroencephalogram (EEG) abnormalities in Alzheimer's disease (AD) have been widely reported, and medial temporal lobe atrophy (MTLA) is one of the hallmarks in early stage of AD. We aimed to assess the relationship between EEG abnormalities and MTLA and its clinical validity.
Materials and Methods:
A total of 18 patients with AD were recruited (the mean age: 77.83 years). Baseline EEGs were analyzed with quantitative spectral analysis. MTLA was assessed by a T1-axial visual rating scale (VRS).
Results:
In relative power spectrum analysis according to the right MTLA severity, the power of theta waves in C4, T4, F4, F8, and T5 increased significantly and the power of beta waves in T6, C4, T4, F8, T5, P3, T3, and F7 decreased significantly in severe atrophy group. In relative power spectrum analysis according to the left MTLA severity, the power of theta waves in T3 increased significantly and that of beta waves in P4, T6, C4, F4, F8, T5, P3, C3, T3, F3, and F7 decreased significantly in severe atrophy group.
Conclusion:
The severe MTLA group, regardless of laterality, showed more severe quantitative EEG alterations. These results suggest that quantitative EEG abnormalities are correlated with the MTLA, which may play an important role in AD process.
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RESIDENTS CORNER
Atypical lytic lesions of skull: Clinical and radiological correlation
Sureka Binit, Mahesh Kumar Mittal, Aliza Mittal, Brij Bhushan Thukral
January-March 2015, 18(1):117-119
DOI
:10.4103/0972-2327.144309
PMID
:25745329
Imaging alone cannot differentiate various isolated atypical lytic lesions involving the skull. Clinical and radiological correlation is mandatory in reaching to a diagnosis. Histopathology remains the gold standard. We describe few atypical cases presenting as isolated lytic lesions of skull with characteristic imaging findings and a brief clinical approach to reach towards the diagnosis.
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4,029
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1
CASE REPORTS
Alien hand and leg as the presenting feature of probable sporadic Creutzfeldt-Jakob disease: A rare presentation of a rare disease
Banshi Lal Kumawat, Chandra Mohan Sharma, Kunal Nath, Mihir Acharya, Dinesh Khandelwal, Deepak Jain
January-March 2015, 18(1):99-102
DOI
:10.4103/0972-2327.144278
PMID
:25745324
Sporadic Creutzfeldt-Jakob disease (sCJD) can have varied clinical presentation depending upon the genotype at codon 129. The common presenting clinical features of sCJD are rapid onset cognitive impairment, ataxia, psychosis and visual signs (field defects, distortion, cortical blindness). Alien limb sign was first described in patients with corpus callosal tumors and later with other neurodegenerative conditions like corticobasal degeneration. Alien hand complaints as the presenting feature of sCJD has been described in literature, but simultaneous alien hand and leg has been rarely described as presenting feature of sCJD. We describe here a case of a 55-year-old man who presented with progressive left alien hand and leg as the sole clinical manifestation of probable sCJD.
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3,897
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LETTERS TO THE EDITOR
Tacrolimus associated thrombotic thrombocytopenic purpura (TTP) and stroke in the young
Boby Varkey Maramattom, Rahul Patil, Punnoose Thomas, Binu Upendran
January-March 2015, 18(1):126-127
DOI
:10.4103/0972-2327.144282
PMID
:25745335
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3,790
82
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ORIGINAL ARTICLES
Test-retest reliability of transcarpal sensory NCV method for diagnosis of carpal tunnel syndrome
Seyed Mostafa Jazayeri, Alireza Ashraf, Hajar Karimian, Ali Moghari, Asma Azadeh
January-March 2015, 18(1):60-62
DOI
:10.4103/0972-2327.145285
PMID
:25745312
Background:
Carpal Tunnel Syndrome (CTS) is the most frequent entrapment neuropathy affecting the upper extremity. There are a variety of electrodiagnostic methods available for documenting median neuropathy in CTS. In some studies, determining the sensory NCV across the palm-wrist segment has been introduced as the most sensitive diagnostic procedure for CTS. The aim of this study was to investigate the test-retest reliability of transcarpal median sensory NCV method for the diagnosis of CTS.
Materials and Methods:
Twenty-three patients with clinical symptoms of CTS were tested two times by two different practitioners in one session and again by the first practitioner after one week. Stimulation of the median nerve was performed in the wrist and palm, with a conduction distance maximum of 7 cm, reliabilities of median nerves sensory nerve action potential latencies with stimulation at wrist and palm (W-SNAP, P-SNAP) and its transcarpal NCV were assessed with intraclass correlation coefficient (ICC).
Results:
Comparison of the obtained values, which were done by two practitioners in one session showed ICC of W-SNAP latency, P-SNAP latency and transcarpal NCV of 0.93, 0.88 and 0.87, respectively and values that were done by one practitioner in two sessions with one-week interval showed ICC of 0.60, 0.50 and 0.47, respectively.
Conclusion:
Our findings suggest excellent interpractitioner test-retest reliability of transcarpal median sensory NCV method for diagnosing CTS.
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CASE REPORTS
Subacute sclerosing panencephalitis in a child with human immunodeficiency virus (HIV) infection on antiretroviral therapy
Karthik Muthusamy, Sangeetha Yoganathan, Maya Mary Thomas, Mathew Alexander, Valsan Philip Verghese
January-March 2015, 18(1):96-98
DOI
:10.4103/0972-2327.144299
PMID
:25745323
Subacute Sclerosing Panencephalitis (SSPE) in HIV-infected children is a scarcely reported entity with previous reports describing fulminant course. The impact of highly active antiretroviral therapy (HAART) in altering its course remains unknown. We describe a child with HIV infection, who developed measles at 5 months of age and later developed SSPE at 14 years of age, remaining stable at 7 month follow-up, while on HAART for WHO (World Health Organisation) stage IV disease. The dynamics of HIV-related immunosuppression has an impact on the clinical course of SSPE. Contrary to reported cases of fulminant progression, a classic presentation with slow progression can be expected in children on HAART. We reemphasize the recommendation of "early measles vaccination" to prevent measles infection and subsequent SSPE in these children with an increasingly good life expectancy in the era of HAART.
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3,699
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IMAGES IN NEUROLOGY
Hypertrophic multiple cranial neuropathies: An unusual presentation of primary CNS lymphoma
Satish V Khadilkar, Ashish G Bhutada, Chetan R Chaudhari, Vernon Velho, Shilpa Domkundwar, Girish Muzumdar
January-March 2015, 18(1):74-76
DOI
:10.4103/0972-2327.144305
PMID
:25745316
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3,642
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CASE REPORTS
Ictal Generalized EEG Attenuation (IGEA) and hypopnea in a child with occipital type 1 cortical dysplasia - Is it a biomarker for SUDEP?
Ganne Chaitanya, Santosh N Subbareddy, Jayabal Velmurugan, Arima Arivazhagan, Bharath D Rose, Anita Mahadevan, Madhu Nagappa, Parayil S Bindu, Malla Bhaskara Rao, Arun B Taly, Parthasarathy Satishchandra, Sanjib Sinha
January-March 2015, 18(1):103-107
DOI
:10.4103/0972-2327.144279
PMID
:25745325
An interesting association of ictal hypopnea and ictal generalized EEG attenuation (IGEA) as possible marker of sudden unexpected death in epilepsy (SUDEP) is reported. We describe a 5-years-old girl with left focal seizures with secondary generalization due to right occipital cortical dysplasia presenting with ictal hypopnea and IGEA. She had repeated episodes of the ictal apnoea in the past requiring ventilator support and intensive care unit (ICU) admission during episodes of status epilepticus. The IGEA lasted for 0.26-4.68 seconds coinciding with the ictal hypopnea during which both clinical seizure and electrical epileptic activity stopped. Review of literature showed correlation between post-ictal apnoea and post ictal generalized EEG suppression and increased risk for SUDEP. The report adds to the growing body of literature on peri-ictal apnea, about its association with IGEA might be considered as a marker for SUDEP. She is seizure free for 4 months following surgery.
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LETTERS TO THE EDITOR
Cerebral venous sinus thrombosis: Role of extra cranial collateral circulation
Chandramohan B Sharma, Jigar R Parekh, Dinesh Khandelwal, Bansi L Kumawat
January-March 2015, 18(1):121-123
DOI
:10.4103/0972-2327.144280
PMID
:25745332
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3,308
76
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Pseudo-dementia: A neuropsychological review
Ravikesh Tripathi, Seema Mehrotra
January-March 2015, 18(1):123-124
DOI
:10.4103/0972-2327.144302
PMID
:25745333
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3,203
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Neuropsychiatric profiles in patients with Alzheimer's disease and vascular dementia
Sunil Kumar Raina
January-March 2015, 18(1):120-121
DOI
:10.4103/0972-2327.144315
PMID
:25745331
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3,076
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SHORT COMMUNICATIONS
Challenges in understanding the epidemiology of acquired brain injury in India
Suresh Kumar Kamalakannan, Aashrai S. V. Gudlavalleti, Venkata S Murthy Gudlavalleti, Shifalika Goenka, Hannah Kuper
January-March 2015, 18(1):66-70
DOI
:10.4103/0972-2327.151047
PMID
:25745314
An acquired brain injury (ABI) is an injury to the brain, which is not hereditary, congenital, degenerative, or induced by birth trauma. In India, rapid urbanization, economic growth and changes in lifestyle have led to a tremendous increase in the incidence of ABI, so much so that it is being referred to as a 'silent epidemic'. Unlike developed countries, there is no well-established system for collecting and managing information on various diseases in India. Thus it is a daunting task to obtain reliable information about acquired brain injury. In the course of conducting a systematic review on the epidemiology of ABI in India, we recognized several challenges which hampered our effort. Inadequate case definition, lack of centralized reporting mechanisms, lack of population based studies, absence of standardized survey protocols and inadequate mortality statistics are some of the major obstacles. Following a standard case definition, linking multiple hospital-based registries, initiating a state or nationwide population-based registry, conducting population-based studies that are methodologically robust and introducing centralized, standard reporting mechanisms for ABI, are some of the strategies that could help facilitate a thorough investigation into the epidemiology and understanding of ABI. This may help improve policies on prevention and management of acquired brain injury in India.
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LETTERS TO THE EDITOR
Safe and successful intravenous thrombolysis for acute stroke in a menstruating woman
CJ Suresh Chandran
January-March 2015, 18(1):124-125
DOI
:10.4103/0972-2327.144294
PMID
:25745334
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2,922
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Cerebrospinal fluid cytokines, matrix metalloproteinases, HIV, and tuberculous meningitis
Sim Sai Tin, Viroj Wiwanitkit
January-March 2015, 18(1):120-120
DOI
:10.4103/0972-2327.144312
PMID
:25745330
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2,834
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SHORT COMMUNICATIONS
Group B streptococcal meningitis in children beyond the neonatal period in sub-Himalayan India
Divya Chauhan, Kiran Mokta, Anil Kanga, Neelam Grover, Digvijay Singh, Suruchi Bhagra
January-March 2015, 18(1):71-73
DOI
:10.4103/0972-2327.151049
PMID
:25745315
Objectives:
To evaluate clinicolaboratory profile and the outcomes in children (1 to 59 months) diagnosed with Group B streptococcus (GBS) meningitis over a period of 1 year.
Materials and Methods:
Cerebrospinal fluid (CSF) samples of 250 pediatric patients (1 to 59 months) admitted with suspected acute bacterial meningitis(ABM)were subjected to cell count, biochemical profile, culture, latex particle agglutination (LPA) and polymerase chain reaction (PCR).They were also evaluated for complications and were followed-up till 6 months after discharge.
Results:
Forty patients (25 boys and 15 girls), 16% of total suspected cases of ABM were diagnosed with GBS by LPA method and 30 (75%) out of these were above 3 months of age. The median duration of hospital stay was 7 days (range 1 to 72 days). State of coma was observed in two (5%) and one (2.5%) died, while 20 (50%) patients recovered completely.
Conclusion:
GBS should be considered as an important cause of ABM in Indian children beyond the neonatal period and further studies are warranted to determine the actual problem of the disease in our country.
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2,797
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LETTERS TO THE EDITOR
Rare case of congenital absence of left internal carotid artery
Syed Amer
January-March 2015, 18(1):128-129
DOI
:10.4103/0972-2327.144310
PMID
:25745336
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ORIGINAL ARTICLES
Hypertension and diabetes as risk factors for dementia: A secondary post-hoc analysis from north-west India
Sunil Kumar Raina, Vishav Chander, Sujeet Raina, Dinesh Kumar, Ashoo Grover, Ashok Bhardwaj
January-March 2015, 18(1):63-65
DOI
:10.4103/0972-2327.151045
PMID
:25745313
Introduction:
A relation between midlife risk factors (hypertension and diabetes) and dementia has been studied in past and an association has been documented, in spite of some studies pointing to the contrary.
Materials and Methods:
The study was based on post-hoc analysis of data obtained from a study conducted on an elderly population (60 years and above) from selected geographical areas (migrant, urban, rural and tribal) of the Himachal Pradesh state in North-West India.
Results:
Analysis of variance revealed an effect of risk factors on cognitive scores; however, post hoc Tukey's honest significant difference (HSD) test revealed that only hypertensives' demonstrated higher chances of scoring lower on cognitive measures.
Discussion:
The possibility that hypertension and diabetes affect dementia needs further evaluation, more so in Indian context.
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LETTERS TO THE EDITOR
Tuberculous meningitis on directly observed thrice a week regime
Sora Yasri, Viroj Wiwanitkit
January-March 2015, 18(1):129-129
DOI
:10.4103/0972-2327.144316
PMID
:25745337
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2,543
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© 2006 - Annals of Indian Academy of Neurology | Published by Wolters Kluwer -
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Online since 1
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March, 2006