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2022| July-August | Volume 25 | Issue 4
Online since
September 9, 2022
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ORIGINAL ARTICLES
Study Protocol: IMPETUS: Implementing a uniform stroke care pathway in medical colleges of India: IMPETUS
Stroke
Rohit Bhatia, Partha Haldar, Inder Puri, MV Padma Srivastava, Sanjeev Bhoi, Menka Jha, Anupam Dey, Suprava Naik, Satyabrata Guru, Mamta Bhushan Singh, VY Vishnu, Roopa Rajan, Anu Gupta, Deepti Vibha, Awadh Kishore Pandit, Ayush Agarwal, Manish Salunkhe, Gunjan Singh, Deepshikha Prasad, Samhita Panda, Sucharita Anand, Amit Kumar Rohila, Pushpinder S Khera, Sarbesh Tiwari, S Bhaskar, Mayank Garg, Niraj Kumar, Minakshi Dhar, Ashutosh Tiwari, Naman Agrawal, Garuda Buchi Raju, Jyoti Garg, Biman Kanti Ray, Amit Bhardwaj, Alok Verma, Nikhil Dongre, Gurpreet Chhina, Raminder Sibia, Rupinderjeet Kaur, Paresh Zanzmera, Amit Gamit, Thomas Iype, Ravinder Garg, Sulena Singh, Ashok Kumar, Abhay Ranjan, Vijay Sardana, Dilip Soni, Bharat Bhushan, Rajinder K Dhamija, Alvee Saluja, Kiran Bala, Surekha Dabla, Divya Goswami, Abhimanyu Agarwal, Sudhir Shah, Shalin Shah, Mayank Patel, Pranav Joshi, Sanjiv Awasthi, Smita Nath, Shishir Chandan, Rupali Malik, Neera Chowdhury
July-August 2022, 25(4):640-646
DOI
:10.4103/aian.aian_1033_21
Introduction:
In India, a national program for stroke (national programme for the control of cardiovascular diseases, diabetes, cancer, and stroke) and stroke management guidelines exist. Its successful implementation would need an organized system of stroke care in practice. However, many challenges exist including lack of awareness, prehospital notification systems, stroke ready hospitals, infrastructural weaknesses, and rehabilitation. We present here a protocol to investigate the feasibility and fidelity of implementing a uniform stroke care pathway in medical colleges of India.
Methods and Analysis:
This is a multicentric, prospective, multiphase, mixed-method, quasi-experimental implementation study intended to examine the changes in a select set of stroke care-related indicators over time within the sites exposed to the same implementation strategy. We shall conduct process evaluation of the implementation process as well as evaluate the effect of the implementation strategy using the interrupted time series design. During implementation phase, education and training about standard stroke care pathway will be provided to all stakeholders of implementing sites. Patient-level outcomes in the form of modified Rankin Scale score will be collected for all consecutive patients throughout the study. Process evaluation outcomes will be collected and reported in the form of various stroke care indicators. We will report level and trend changes in various indicators during the three study phases.
Discussion:
Acute stroke requires timely detection, management, and secondary prevention. Implementation of the uniform stroke care pathway is a unique opportunity to promote the requirements of homogenous stroke care in medical colleges of India.
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EDITORIAL
ALS treatment- We Still Await the “Magic Cocktail”
Jasmine Shimin Koh, Su Rong Fam, Peng Soon Ng, Thirugnanam Umapathi
July-August 2022, 25(4):577-578
DOI
:10.4103/aian.aian_397_22
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Ticagrelor in stroke prevention new wine in an old bottle?
MV Padma Srivastava, Venugopalan Y Vishnu
July-August 2022, 25(4):575-576
DOI
:10.4103/aian.aian_315_22
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AIAN REVIEWS
Mechanical thrombectomy: Review
Chintan Prajapati, Vikram Huded, Niranjan Mahajan, Anirudh Kulkarni
July-August 2022, 25(4):606-615
DOI
:10.4103/aian.aian_29_22
Large vessel occlusion stroke contributes to disability and mortality out of proportion to its incidence. Over time it was noted that intravenous thrombolysis alone was not sufficient for this stroke type. Slowly, endovascular approach and mechanical clot retrieval have come out to be the biggest advances in the field of neurology as well as modern medicine. Although the careful selection of patients is needed as standardized by landmark trials. At the same time, thrombectomy is now being studied in patients excluded by previous trials and is seemingly coming out be effective in the vast majority of patients with large vessel occlusions. Further, techniques and devices are getting refined day by day to achieve the maximum possible benefit.
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ORIGINAL ARTICLES
Serotonin receptor agonist and risk of paresthesia in migraine patients: A dose-response model-based (network) meta-analysis
Sayed Aliul Hasan Abdi, Shabihul Fatma Sayed, Jamuna Bhaskar
July-August 2022, 25(4):669-675
DOI
:10.4103/aian.aian_972_21
Background:
Migraine may be an important factor for paresthesia in the limbs, especially in the upper limbs. In several patients, paresthesia is responsible for a low quality of life. Treatment with the serotonin agonist may be a triggering factor for paresthesia in certain patients. Various serotonin receptor agonists are available for migraine treatment. We performed a meta-analysis of updated clinical trials of the serotonin agonist to figure out the risk of paresthesia.
Methods:
PubMed, Embase, and Cochrane Library databases were searched for clinical trials that evaluated the serotonin agonist for migraine treatment versus placebo. The main outcomes were to perform dose-response model-based network meta-analysis of different serotonin agonists and to compute the relative risk for paresthesia. In addition, probability of paresthesia among various treatments was estimated by the Surface Under the Cumulative Ranking (SUCRA) method. The R 4.30 and Rev Man 5.3 softwares were used to perform meta-analysis.
Results:
A total of 30 placebo-controlled clinical trials (29,154 subjects) were included in the study to perform dose-response model-based network meta-analysis to explore the risk of paresthesia with different serotonin agonists versus placebo. The drugs Topiramate 200 mg, Lasmiditan 400 mg, and Zolmitriptan 10 mg showed higher relative risks for paraesthesia as 2.71, 2.2, and 2.42, respectively. However, the SUCRA probabilities of paresthesia for each treatment in the network were higher for Lasmiditan.
Conclusions:
This meta-analysis of reported placebo-controlled clinical trials suggests that the SUCRA probabilities for the manifestation of paresthesia are higher with Lasmiditan. The relative risk of paresthesia is higher with the use of Topiramate 200 mg, Lasmiditan 400 mg, and Zolmitriptan 10 mg. In addition, Lasmiditan exhibited a gradual dose-response of relative risk for the manifestation of paresthesia.
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AIAN REVIEWS
The genetic landscape of polymicrogyria
Jesmy James, Mary Iype, Mithran Omana Surendran, Ayyappan Anitha, Sanjeev V Thomas
July-August 2022, 25(4):616-626
DOI
:10.4103/aian.aian_97_22
Polymicrogyria (PMG) is a relatively common complex malformation with cortical development, characterized by an exorbitant number of abnormally tiny gyri separated by shallow sulci. It is a neuronal migration disorder. Familial cases of PMG and the manifestation of PMG in patients with chromosomal aberrations and mutations indicate their important role of genetics in this disorder. The highly stereotyped and well-conserved nature of the cortical folding pattern in humans is suggestive of the genetic regulation of the process. The chromosomal abnormalities observed in PMG include deletions, duplications, chromosomal rearrangements, and aneuploidies. Two of the most common deletions in PMG are 22q11.2 deletion and 1p36 deletion. Further, mutations in several genes such as
GPR56
,
TUBB2B
,
SRPX2
,
PAX6
,
EOMES
,
WDR62
,
TUBA8
,
KIAA1279
, and
COL18A1
are known to be associated with PMG. Intriguingly, these genes are responsible only for a small number of cases of PMG. The protein products of these genes are implicated in diverse molecular and cellular functions. Taken together, PMG could be the result of the disruption of several biological pathways. Different modes of Mendelian inheritance and non-Mendelian inheritance are seen in PMG. We have suggested a gene panel that can be used for the detection of malformations of cortical development.
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ORIGINAL ARTICLES
Predictors of early neurological deterioration following intravenous thrombolysis: Difference between risk factors for ischemic and hemorrhagic worsening
Srikumar B Nair, Deepthi Somarajan, Rammohan K Pillai, Keerthi Balachandran, Sona Sathian
July-August 2022, 25(4):627-633
DOI
:10.4103/aian.aian_893_21
Background:
Prevention of early neurological deterioration (END) is becoming an important therapeutic target in acute ischemic stroke management. The aim of the study is to ascertain the causes and predictors of early neurological deterioration following thrombolysis and determine the predictive value of IScore.
Methods:
In this single center prospective study, we analyzed clinical, imaging and outcome data in 168 patients thrombolyzed intravenously ≤4.5 hours from onset of stroke. Early neurological deterioration was defined as worsening ≥2 points in the NIHSS score at 24 hours.
Results:
END occurred in 34 patients (20%) and caused significantly worse short term outcome. Ischemic END (ENDi) (n = 23) was twice as common as symptomatic hemorrhage (ENDh) (n = 11). Ischemia progression (n = 15) was the most common cause. Early malignant edema was another major cause. On multivariate analysis, significant predictors (p <.05) were proximal artery occlusion [all END (p <.001), ENDi and ENDh], previous ischemic insults (all END) and raised diastolic blood pressure (ENDh). ENDi was more common in those with carotid artery occlusion, large vessel disease and previous ischemic insults. ENDh was more common in those with raised diastolic blood pressure and NIHSS-ASPECTS mismatch. For patients with NIHSS <14, IScore >105 and for NIHSS ≥14, IScore >175 was associated with higher risk of END.
Conclusion:
END occurs in one fifth of patients after intravenous thrombolysis; ENDi outnumbers ENDh. Proximal artery occlusion is a major predictor for END. Potentially modifiable risk factors include admission hyperglycemia and elevated blood pressures. Distinct factors characterize ENDh and ENDi and can guide prevention and management strategies. IScore identifies patients at risk for END.
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AIAN REVIEWS
Fever, seizures and encephalopathy: From bush fires to firestorms
Prabhjot Kaur, Suvasini Sharma, Ramesh Konanki, Asuri N Prasad
July-August 2022, 25(4):587-600
DOI
:10.4103/aian.aian_12_22
Fever-associated seizures and febrile encephalopathy are common neurological problems in children. Infections of the nervous system are responsible for the majority of cases. However, there is a spectrum of infection-associated and inflammatory conditions associated with the triad of fever, seizures, and encephalopathy. Apart from complex febrile seizures and febrile status epilepticus, fever infection-related epilepsy syndrome of childhood (FIRES), infantile hemiconvulsion hemiplegia epilepsy syndrome (IHHE), acute encephalopathy with delayed diffusion restriction (AESD), acute necrotizing encephalopathy of childhood (ANE), and reversible splenial lesion syndrome (RESLES) are age-related clinical phenotypes of fever-related epilepsy and encephalopathy. Awareness of these entities is important for appropriate diagnosis and the prompt use of immunomodulatory/immunosuppressive therapies. In this review, we discuss the pathophysiology, clinical phenotypes, and management approaches of these fever-related seizure and encephalopathy states.
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LETTERS TO THE EDITORS
Progressive fulminant necrotizing myelitis in COVID-19 infection; case report of a rare complication
Hossein Azin, Alireza Vakilian, Mohammad Reza Najafi, Rostam Seifaddini, Mahdieh Azin
July-August 2022, 25(4):781-785
DOI
:10.4103/aian.aian_442_21
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ORIGINAL ARTICLES
Spinocerebellar ataxia 12 patients have better quality of life than spinocerebellar ataxia 1 and 2
Surekha Dabla, Divyani Garg, Rajeev Aggarwal, Nand Kumar, Mohammad Faruq, Roopa Rajan, Garima Shukla, Vinay Goyal, Ravindra Mohan Pandey, Achal Kumar Srivastava
July-August 2022, 25(4):647-653
DOI
:10.4103/aian.aian_611_21
Background:
Spinocerebellar ataxia is a neurodegenerative disease. Information on comparative assessment of quality of life (QoL) among SCAs, particularly SCA 12, is scarce. We aimed to compare health-related QoL in SCA 1, 2 and 12.
Methods:
We conducted a cross-sectional study among individuals with genetically-confirmed SCAs. Ataxia severity was assessed using Brief Ataxia Rating Scale (BARS), independence in activities of daily living (ADL) using Katz index (Katz ADL) and depression using Beck's Depression Inventory-II (BDI-II). QoL was assessed via Short Form Health Survey version 2.0 (SF-36).
Results:
We enrolled 89 individuals (SCA1 = 17, SCA2 = 43, SCA12 = 29; 56% males). Mean age at onset (41.0 ± 11.6 for SCA12 versus 24.9 + 7.0 for SCA1 and 28.8 ± 9.8 years for SCA2) was significantly higher among SCA12. SCA12 had lower BARS (mean score 4.1 ± 4.5 versus 10.6 ± 4.6 for SCA1 and 12.5 ± 4.5 for SCA2). SCA12 scored better on all SF-36 subdomains including Physical (PCS) and Mental Component Summary (MCS) scores. PCS score amongst SCA12 was 44.4 ± 9.0 versus 30.4 ± 9.1 for SCA1 and 33.3 ± 8.9 for SCA2. MCS score for SCA12 was 51.4 ± 11.4 versus 41.8 ± 11.5 for SCA1 and 41.8 ± 11.2 for SCA2. SCA12 had lower mean BDI scores (5.0 ± 6.0) versus SCA1 (9.5 ± 11.6) and SCA2 (10.9 ± 10.3). BARS and BDI emerged as significant predictors of most SF-36 subdomains.
Conclusions:
Our study suggests that despite older age and comparable disease duration, SCA12 patients experience better QoL, less severe depression and ataxia versus SCA1 and SCA2. Severity of ataxia and depression are significant predictors of QoL among the three SCA types.
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Adaptation and validation of a sinhala version of the radbound oral motor inventory (ROMP) for parkinson's disease
P D. M. H . Gamage, MS Mohideen, Prasanna Galhena, N Weerasinghe, MP Kumbukage, Tharuka Herath, Sunethra Senanayake, D Sirisena, SJ Senanayake, Inuka Kishara Gooneratne
July-August 2022, 25(4):688-691
DOI
:10.4103/aian.aian_953_21
Background:
The Radboud Oral Motor Inventory for Parkinson's disease (ROMP) is a patient-rated assessment measuring patients' perceptions of speech, swallowing, and saliva control among patients with idiopathic Parkinson's disease (IPD).
Objective:
The present study was carried out to adapt and validate the Sinhala version of the ROMP questionnaire in a Sinhala-speaking patient cohort diagnosed with IPD.
Materials and Methods:
The study population consisted of patients diagnosed with IPD attending a tertiary care neurology clinic at the National Hospital of Sri Lanka. ROMP was translated from English to Sinhala, and an expert committee verified its content. Construct validity was assessed by correlating the Sinhala ROMP scores with the subscales in speech, salivation, and swallowing of the Unified Parkinson's Disease Rating Scale and with five-point Likert-type scale to assess dysarthria, dysphagia, and drooling by a speech and language therapist. Test–retest reproducibility was assessed by repeating the questionnaire in 2 weeks.
Results:
A cohort of 21 patients was evaluated (male to female ratio = 2.5:1, mean age was 58.8 [±8.3] years). The Spearman's correlations between ROMP and the Likert-type scale assessment, that is, speech
r
= 0.85 (
P
< 0.01), swallowing
r
= 0.86 (
P
< 0.01), and drooling
r
= 0.88 (
P
< 0.01), and subscales of the UPDRS were statistically significant, that is, speech
r
= 0.75 (
P
< 0.01), swallowing
r
= 0.96 (
P
< 0.01), and salivation
r
= 0.94 (
P
< 0.01). Reproducibility of the three domains and total intraclass correlation coefficients indicated a high level of agreement in test–retest reproducibility (range: 0.98–0.99). The three subdomains of the instrument also had excellent internal consistency (total Cronbach's α = 0.99).
Conclusion:
The Sinhala version of ROMP has proved to be a good assessment tool for dysphagia, dysarthria, and drooling in the early stage of IPD patients.
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Respiratory dysfunction in parkinson's disease: Relation with dysautonomia
Meghashree Sampath, Geetanjali Bade, Vinay Goyal, Achal K Srivastava, Ashok K Jaryal, Kishore K Deepak, Anjana Talwar
July-August 2022, 25(4):683-687
DOI
:10.4103/aian.aian_940_21
Background:
Parkinson's disease (PD) is a neurodegenerative disease perceived as a motor disorder. It is most commonly associated with autonomic dysfunction, affecting multiple systems. This altered autonomic control might be reflected by a parallel change in the airway caliber of these patients.
Aim:
To correlate the pulmonary impairment in patients with Parkinson's disease with the underlying dysautonomia.
Materials and Methods:
A total of 30 patients with Parkinson's disease participated in the study. Heart rate (HR) variability was recorded for 5 min to assess the autonomic dysfunction, followed by impulse oscillometry (IOS) and spirometry. IOS being an effort independent technique uses sound waves at different frequencies (5–25 Hz) to measure the airway impedance.
Results:
There was a significant decrease in SDSD (6.60 (10.18–6.01) vs. 12.22 (13.95–11.30);
P
= 0.04), RMSSD (6.59 (10.17–5.50) vs. 12.20 (13.93–11.28);
P
= 0.04), and total power (315.8 (506.3–120.7) vs. 771.3 (799.0–643.6);
P
= 0.04) in stage II as compared to stage I. Resistance at 20 Hz (R20) was found to be positively correlated with SDSD (r = 0.40,
P
= 0.04), RMSSD (r = 0.40,
P
= 0.04), and HF (r = 0.41,
P
= 0.03).
Conclusion:
Amongst the PD population, any changes in the parasympathetic component (responsible for bronchoconstriction) due to the underlying dysautonomia might be reflected as increased airway resistance in the pulmonary system.
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Quality of life and concerns of parkinson's disease patients and their caregivers during COVID-19 pandemic: An indian study
Sourav Banerjee, Adreesh Mukherjee, Bidisha Bhattacharyya, Kochupurackal P Mohanakumar, Atanu Biswas
July-August 2022, 25(4):676-682
DOI
:10.4103/aian.aian_905_21
Objectives:
Parkinson's disease (PD) patients have suffered during the coronavirus disease 2019 pandemic, with worsening of both motor and nonmotor symptoms. We conducted this study to evaluate the quality of life (QoL) and concerns of PD patients and their caregivers.
Methods:
The study was conducted in mixed method, where the baseline data was taken by face-to-face interview during the unlock phase of December 2020 to March 2021, when there was no lockdown. This included demography, Hoehn and Yahr (HY) stage, Parkinson's Disease Questionnaire-8 (PDQ-8), and Parkinson's Disease Questionnaire for Carer (PDQ-Carer). During the second wave of COVID-19 (April–June 2021), telephonic interview was conducted using Depression, Anxiety Stress Scale- 21 Items (DASS-21), PDQ-8, PDQ-Carer, and open-ended questions regarding their concerns.
Results:
Compared with the baseline data, PDQ-8 and PDQ-Carer scores showed significant worsening during the second wave. DASS-21 scores had significant correlation with PDQ-Carer and PDQ-8 scores. Female patients reported poorer QoL. Caregivers of non-vaccinated patients had worse PDQ-Carer Strain scores. There was no significant association between worsening of motor symptoms and PDQ-8 and PDQ-Carer scores. More than 80% patients and 70% caregivers reported anxiety and depression. Their concerns were regarding difficulties due to social isolation, restriction of activity, and financial constraints. Additionally, there were worries about patient care, vaccination, and recurrence of the wave.
Conclusions:
The QoL of both patients and their caregivers were affected by the pandemic. A significant proportion had anxiety and depression, and this correlated with QoL. There were some important concerns on various aspects of the pandemic.
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AIAN REVIEWS
Cognition in trinucleotide repeat spinocerebellar ataxias: A review
Ayush Agarwal, Pankaj , Mohd Faruq, Ajay Garg, Achal K Srivastava
July-August 2022, 25(4):601-605
DOI
:10.4103/aian.aian_63_22
Spinocerebellar ataxias (SCAs) comprise a group of complex and heterogeneous hereditary neurodegenerative disorders characterized by cerebellar ataxia, with ophthalmoplegia, pyramidal and extrapyramidal features, peripheral neuropathy, motor neuron disease, pigmentary retinopathy, epilepsy, and dementia in varying proportions. Cognitive impairment is not frequent in SCAs but is rarely noticed since it gets camouflaged behind the exorbitant ataxic manifestations of the disease. The exact incidence and extent of cognitive impairment in these rare disorders are not known due to the heterogeneity between different SCA types and different modalities of testing employed in different studies. Through our review, we have summarized the cognitive aspects of SCA and can safely conclude that cognitive dysfunction is common in some SCA types when compared to others. Not only is it important to appreciate its presence as a symptom complex in SCA but also is the need to actively search and treat it to improve the patients' quality of life.
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NOTICE OF RETRACTION
Retraction: Seronegative autoimmune encephalitis: A challenge for the neurologist
July-August 2022, 25(4):786-786
DOI
:10.4103/0972-2327.355739
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ORIGINAL ARTICLES
Propensity of stroke in standard versus various aortic arch variants: A 200 patients study
Swapnil Samadhiya, Vijay Sardana, Bharat Bhushan, Dilip Maheshwari, Seeta Ram Yadav, Ravi Goyal
July-August 2022, 25(4):634-639
DOI
:10.4103/aian.aian_710_21
Background:
Abnormal origin of arteries from the aortic arch could alter the hemodynamics. Therefore, aortic arch variations might predispose patients to atherosclerosis, which would increase the stroke risk by impending thrombus formation.
Objectives:
To investigate the prevalence of various types of the anatomy of the aortic arch in ischemic stroke patients and determine if aortic morphology has any effect on early-onset strokes.
Materials and Methods:
Observational study including 200 imaging-confirmed (Non Contrast Computed Tomography(NCCT)/magnetic resonance imaging [MRI] of the brain) acute ischemic stroke patients. This was followed by computed tomography [CT]/MRI angiography of the arch of the aorta, neck vessels, and intracranial arteries. The occurrence of various types of standard and other aortic arch variants was studied. The prevalence of stroke and its characteristics were analyzed for demographics, types, location, and the predominant side of involvement among standard arch variants and standard versus various aortic arch variants. A
P
value < 0.05 was considered significant.
Results:
Standard arch Type I was the most common (
P
< 0.0001). Age at stroke onset in Type 1 was 61.83 years ± 2.78 years, in Type 2 was 59.8 years ± 3.55 years, and in Type 3 was 60.96 years ± 3.56 years (
P
= 0.0012). Among the bovine aortic arch, age at stroke presentation in Type A was 53.33 years ± 8.35 years, in Type B was 53.36 years ± 7.4 years, and in Type C was 63.25 years ± 9.25 years (
P
< 0.0001).
Conclusions:
Standard aortic arch Type 2, bovine aortic arch Type A, and Type B are associated with an early age at stroke presentation. During routine carotid evaluation by CT or MR angiography in stroke patients, it would be better to evaluate the aortic arch as well, especially in young patients.
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GRAND ROUND
Carotid artery stenting for a nonagenarian presenting as stuttering stroke
Saman Fatima, Ajay Garg, Leve Joseph, MV Padma Srivastava, Deepti Vibha, Raghav Bansal, Ashish Bindra, Manjari Tripathi, Rajesh Kumar Singh, Arunmozhimaran Elavarasi
July-August 2022, 25(4):715-717
DOI
:10.4103/aian.aian_14_22
Carotid artery stenting (CAS) is performed in patients with minor strokes and transient ischemic attacks (TIAs) to prevent further strokes. However, most operators do not intervene in older adults. We had a 92-year patient with recurrent minor strokes with two possible proximate causes – cardioembolism and significant symptomatic left carotid stenosis. This patient continued to have recurrent ischemic events in the left carotid territory despite optimum management of the cardioembolic source with dual antiplatelets and anticoagulation and was successfully treated with left CAS. The role of carotid revascularization in older patients with high-grade symptomatic carotid stenosis and cardiac comorbidities is discussed.
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EDITORIAL COMMENTARY
Management of wilson disease: The quest continues
Gautam Kamila, Biswaroop Chakrabarty
July-August 2022, 25(4):585-586
DOI
:10.4103/aian.aian_961_21
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EDITORIAL
Prioritizing neuro-rehabilitation services in low-and middle-income countries: Needs, challenges and possible solutions
Alvee Saluja, Rajinder K Dhamija
July-August 2022, 25(4):579-582
DOI
:10.4103/aian.aian_499_22
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LETTERS TO THE EDITOR
Age and gender differences in the cognitive reserve index
Danijel Slavic, Velimir Tomic, Željka Nikolaševic, Nevena Djurdjevic, Nada Naumovic
July-August 2022, 25(4):767-770
DOI
:10.4103/aian.aian_1106_21
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ORIGINAL ARTICLES
Assessment of therapeutic response of edaravone and riluzole combination therapy in amyotrophic lateral sclerosis patients
Swapnil Samadhiya, Vijay Sardana, Bharat Bhushan, Dilip Maheshwari, Ravi Goyal, Pankaj
July-August 2022, 25(4):692-697
DOI
:10.4103/aian.aian_1083_21
Background:
Amyotrophic lateral sclerosis (ALS) is a progressive disease characterized by chronic degeneration of upper and lower motor neurons and finally death within 3–5 years usually because of respiratory failure. Riluzole and edaravone are presently available treatments. It may be better to try combination therapy rather than taking individual medications.
Objectives:
To compare the effectiveness of (edaravone + riluzole) combination therapy versus riluzole therapy alone in slowing down the progression of ALS and to evaluate the role of serum creatinine as a marker of disease progression.
Materials and Methods:
Observational, randomized, parallel assignment, open label study. Thirty patients with definite and probable ALS were randomly assigned to two treatment groups. The case group received (riluzole + edaravone) for the initial 6 months, followed by riluzole for the next 6 months. The control group received riluzole for 12 months. After 6 and 12 months, changes in ALS functional rating scale (ALSFRS-R), mRS, and Japanese ALS scores were determined.
P
value <.05 was considered significant.
Results:
An increase in mRS at 6 months in the case group versus control group was 0.07 versus 0.20, respectively (p =0.02). At 12 months, it was 0.47 versus 0.53, respectively (p =0.17). A decrease in serum creatinine at 6 months in case group versus control group was 0.08 versus 0.09, respectively (p =.82). There was no change in ALS FRS for bulbar symptoms (salivation), 3.46 versus 3.46 in the case group (p =.018) for the first 6 months.
Conclusions:
Combined with riluzole, edaravone slows disease progression and is safe, but the effect is short-term. Bulbar symptoms respond better to combination therapy. The serum creatinine is helpful in monitoring disease progression.
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IMAGES IN NEUROLOGY
Giant stellate “Cord Sign” in cerebral venous sinus thromboses
Praveen Kesav, Seby John, Khalid Al-Sharif, Syed Irteza Hussain
July-August 2022, 25(4):708-709
DOI
:10.4103/aian.aian_16_22
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ORIGINAL ARTICLES
Annual cost of care of Parkinson's Disease and its determinants in North India – A cost of illness study with patient perspective
Vipin Ola, Inder Puri, Divya Goswami, Deepti Vibha, Garima Shukla, Vinay Goyal, Achal Srivastava, Madhuri Behari
July-August 2022, 25(4):660-663
DOI
:10.4103/aian.aian_779_21
Background:
Parkinson's disease (PD) is a slowly progressive and disabling disorder, so the cost of illness may change with time. We aimed this study to know the annual cost of care of PD in India.
Methods:
After ethics approval, a prospective cohort study was conducted at the movement disorder clinic of tertiary care hospital for 2 years (2014–2016). The outcomes were a description of the total annual direct cost of Parkinson's disease including health care as well as non-health care cost. We also did correlation analysis to know the determinants of the total cost.
Results:
A total of 200 consecutive patients of PD with 141 (70.50%) males and 59 (29.50%) females with a mean age of 56.84 ± 10.51 years were enrolled. The annual Median Direct cost of care was INR 27,315.0 (IQR 13636.6–44908.4), whereas the Indirect cost was INR 21,400 (IQR 9800 – 96800). Cost on drugs (Direct health care) formed 68.50% (Median) of the total Direct cost. Total direct cost formed 11.38% of the Median total yearly income of our patients. Of the direct cost, the Median expenditure on drugs was INR 18,712.8 (8064.0 -30696.0). Only 5% of patients had health care insurance. The total direct cost was determined by the stage of Parkinson's disease and duration of disease (
P
= < 0.01) but not predicted by age, gender, age at onset, and the yearly income of patients.
Conclusion:
Annual cost of care of Parkinson's disease is high and increases with the duration of the disease as well as the progression of the disease.
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LETTERS TO THE EDITORS
Adult-Onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) in an Indian man
Tejaswi Rao, Boby Varkey Maramattom
July-August 2022, 25(4):720-722
DOI
:10.4103/aian.aian_919_21
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713
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LIGHTER MOMENTS (ARTWORK OR PHOTOGRAPHY)
Lullabies: Behold there is a Bird…
Syed Seerath Mukhtar, Aasim Ur Rehman Ganie
July-August 2022, 25(4):714-714
DOI
:10.4103/aian.aian_495_22
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696
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LETTERS TO THE EDITORS
Dramatic recovery of freezing of gait with subthalamic nucleus deep brain stimulation
Halil Onder, Bilge Kocer, Selcuk Comoglu
July-August 2022, 25(4):743-744
DOI
:10.4103/aian.aian_980_21
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682
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Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) masquerading primary progressive aphasia
Sheetal Goyal, KJ Darshini, Dinesh Sharma, Saraswathi Nashi, Faheem Arshad, VP Vandana, Suvarna Alladi
July-August 2022, 25(4):777-779
DOI
:10.4103/aian.aian_1077_21
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698
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EDITORIAL
Quantifying quality of life after stroke
MV Padma Srivastava, Venugopalan Y Vishnu
July-August 2022, 25(4):583-584
DOI
:10.4103/aian.aian_116_22
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678
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LETTERS TO THE EDITORS
Brivaracetam induced encephalopathy: Why mend something which isn't broken
Ayush Agarwal, Jerry George, Mohit Anand, Abu Tahir, Venugopalan Y Vishnu, Mamta Bhushan Singh, MV Padma Srivastava
July-August 2022, 25(4):740-742
DOI
:10.4103/aian.aian_1115_21
[FULL TEXT]
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660
51
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Muscle cramps profile among spinocerebellar ataxias
Gustavo Leite Franklin, Carlos Henrique Ferreira Camargo, Daniel Balaban, João Matheus Marcon, Salmo Raskin, Alex Tiburtino Meira, Hélio Afonso Ghizoni Teive
July-August 2022, 25(4):718-719
DOI
:10.4103/aian.aian_942_21
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659
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The detailed İnvestigation of eyelid-opening apraxia after subthalamic nucleus deep brain stimulation
Selcuk Comoglu, Bilge Kocer, Hayri Kertmen, Halil Onder
July-August 2022, 25(4):775-776
DOI
:10.4103/aian.aian_1_22
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642
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ORIGINAL ARTICLES
Cranial autonomic symptoms in migraine: An observational study
Ashutosh Tiwari, Pradeep K Maurya, Abdul Qavi, Dinkar Kulshreshtha, Anup K Thacker, Ajai K Singh
July-August 2022, 25(4):654-659
DOI
:10.4103/aian.aian_948_21
Objective:
Our aim was to observe frequency of cranial autonomic symptoms (CAS) in migraineurs (primary) and its relation with laterality of headache or other factors, if any.
Background:
Migraine episodes have headaches with or without aura, and sometimes associated with systemic autonomic nervous system symptoms. Primarily presence of cranial autonomic symptoms suggests diagnosis of TACs. But many studies reported cranial autonomic symptoms (CAS) ranging from 26% to 80% in migraine patients.
Material and Methods:
Consecutive patients of migraine attending our headache clinic were included in our study. Presence of CAS was recorded with respect to ocular, nasal, facial and aural symptoms along with headache characteristics and laterality information. Detailed clinical examination was performed. We used ICHD 3 (beta version) criteria.
Results:
Our study cohort comprised of 200 patients having mean (± SD) age 31.12 (± 10.67) years. There were 157 (78.5%), females. Out of 200 patients, 148 (74%) were having at least one CAS, of which 70% were having 2 or more CAS. Frequency of CAS was lacrimation (45.5%), conjunctival injection (34.5%), eyelid edema (34%), aural fullness (27.5%), facial sweating (25%), facial flushing (17.5%), nasal congestion (9%), rhinorrhea (5%) and ptosis (4%). Bilateral CAS was present in 129 (87%) and unilateral CAS in 19 (13%) (OR 35.31; 95% CI 9.19 to 135.7), (P < 0.0001). Sunlight as a trigger was present in all 148 (100%) patients.
Conclusion:
Our study showed that CASs in migraine is common and bilateral. Sunlight triggers headache in almost all CAS positive patients.
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IMAGES IN NEUROLOGY
Subclavian steal syndrome due to Takayasu arteritis
Jerry George, Ayush Agarwal, Divya M Radhakrishnan, Achal K Srivastava
July-August 2022, 25(4):707-707
DOI
:10.4103/aian.AIAN_25_22
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LETTERS TO THE EDITORS
A curious case of eosinophilic meningitis in a family in Southern India
Poornima N Nambiar, TP Javed Ahamed, Sarika Sasidharan, Lovely Catherine Mathew, KA Salam
July-August 2022, 25(4):757-759
DOI
:10.4103/aian.aian_95_22
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CASE REPORT
Perry syndrome with a novel mutation and a rare presentation: First report from India
Pramod Krishnan, Gosala R K. Sarma, Uday Murgod, Murali Srinivas, Ajit K Roy
July-August 2022, 25(4):703-706
DOI
:10.4103/aian.aian_890_21
Objective:
To characterize the first patient of Perry syndrome reported from India.
Methods:
A 62-year-old gentleman presented with acute encephalopathy, hypercapnia, central hypoventilation, and seizures. He required ventilatory support for persistent respiratory failure even after the resolution of the encephalopathy. History revealed symptoms of orthostatic hypotension, episodes of shallow breathing, unsteadiness of gait, anxiety and depression, and significant weight loss for the previous two years. His mother and elder brother had succumbed to a similar illness. Investigations for neuromuscular diseases, including myasthenia and Pompes disease, were negative. Genetic tests for muscular dystrophies and myopathies, investigations for infectious, autoimmune, and para-neoplastic diseases were negative. Neuroimaging and electrophysiological studies were unremarkable. During his hospital stay, he developed rigidity and bradykinesia.
Results:
In view of the prominent respiratory failure, Parkinsonism, unexplained weight loss, and family history, he was tested for Perry syndrome. A heterozygous missense variation in Exon 2 of the DCTN1 gene that results in the substitution of Proline for Alanine at codon 45 (pA45P) was detected. This variant was not detected in his clinically unaffected brother. The clinical presentation and genetic test indicate Perry syndrome, a rare autosomal dominant fatal disease, which has never been reported from India. The patient improved with Levodopa and neurorehabilitation but eventually succumbed to his illness three years later.
Conclusion:
Perry syndrome, though rare, should be considered in the differential diagnosis of patients with a family history of Parkinsonism and central hypoventilation.
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Leukodystrophy-like presentation in a child: A case of hereditary spastic paraparesis-35
Kiruthiga Sugumar, Aakash Chandran Chidambaram, Jaikumar Govindaswamy Ramamoorthy, Tamil Selvan
July-August 2022, 25(4):745-746
DOI
:10.4103/aian.aian_120_22
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612
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The double whammy—Opsoclonus–Myoclonus–Ataxia syndrome and COVID-19: A case report
Revathy Vijayaraghavan, Vijayashankar Paramanandam, Muthukani Sankaranarayanan, Madhuri Kankanala, Pendem Hima Bindu, Safinaaz , Neetu Mariam Alex
July-August 2022, 25(4):731-733
DOI
:10.4103/aian.aian_1110_21
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606
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‘Cold and Locked in’: A frozen body and frozen eyes in end-stage ALS
Boby V Maramattom
July-August 2022, 25(4):725-727
DOI
:10.4103/aian.aian_1031_21
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A rare cause of acute febrile encephalopathy in a four year old boy–A case report with review of literature
Kiruthiga Sugumar, Vaishnavi Srinivasan, Aakash Chandran Chidambaram, Dhandapany Gunasekaran, Venkatesh Chandrasekaran, Narayanan Parameswaran, Bheemanathi H Srinivas
July-August 2022, 25(4):763-767
DOI
:10.4103/aian.aian_36_22
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ORIGINAL ARTICLES
Evaluation of asymptomatic carotid artery stenosis prior to coronary artery bypass grafting - A prospective observational study in North Indian population
Arghadip Bose, Krishna Prasad, Uma Debi, Harkant Singh, Prashant Panda, Saurabh Mehrotra
July-August 2022, 25(4):664-668
DOI
:10.4103/aian.aian_913_21
Background:
Carotid artery stenosis (CAS) is an established risk factor for peri-operative neurological complications in patients following coronary artery bypass grafting (CABG). However, routine pre-surgical screening for CAS is still a matter of debate. This study was conducted to study the prevalence of asymptomatic carotid artery disease in patients undergoing CABG and to determine the predictors of significant carotid stenosis in them.
Methods:
In this prospective observational study, 112 patients, who were planned for CABG, were enrolled, and their demographic details, risk factor profile, and coronary angiogram parameters were analyzed.
Results:
Carotid stenosis was observed in 75.0% of the study population with 11.6% having unilateral and 63.4% having bilateral carotid stenosis. A total of 56.2% of the patients had mild, 14.3% had moderate, and 4.5% had severe carotid stenosis. The presence of significant carotid stenosis showed a correlation with chronic stable angina (
P
= 0.009), significant left main (LM) disease (
P
= 0.001), chronic total occlusion (
P
= 0.043), and coronary vessel calcification (
P
= 0.004). Multivariate analysis of all the predictor variables in a regression model showed that significant LM disease (Odds ratio (OR):6.5,
P
= 0.002) and coronary artery calcification (OR: 4.3,
P
= 0.024) were the only independent predictors of significant CAS in the study population.
Conclusion:
The presence of significant carotid vessel stenosis in patients undergoing CABG in the Indian population has a stronger association with the chronicity of the coronary artery disease rather than the coronary atheroma load (as determined by the modified Gensini score). The presence of significant LM disease and coronary artery calcification may be useful in detecting high-risk patients for significant CAS during the pre-surgical workup.
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Management of children and adolescents with Wilson Disease and neurological worsening following D–Penicillamine therapy: A single centre experience
Madhan Kumar, TP Murugan, Arul P Lionel, Maya M Thomas, Pavithra Mannam, Sangeetha Yoganathan
July-August 2022, 25(4):698-702
DOI
:10.4103/aian.aian_519_21
Background:
Most centers in developing countries prefer chelation therapy with D-penicillamine for the management of Wilson's disease (WD) because of its easy availability and affordability. Neurological worsening following treatment with D-penicillamine is not uncommon. However, there is a paucity of Indian data on the incidence of neurological worsening in children and adolescents with WD following chelation therapy. Our study objectives were to identify the prevalence of neurological worsening in children and adolescents with WD following chelation with D–penicillamine therapy and to describe the management options and outcomes in these patients.
Materials and Methods:
In this retrospective chart review, children and adolescents with an established diagnosis of WD from 2010 to 2020 were identified from the hospital electronic database. Among these patients, data of children and adolescents with neurological worsening following D–penicillamine therapy were extracted and analyzed.
Results:
Neurological worsening was observed in 27/122 (22.1%) children and adolescents with WD on chelation therapy with D-penicillamine. Fifteen patients with neurological worsening following D-penicillamine therapy were managed with zinc monotherapy. Four patients were managed with a combination therapy of zinc and trientine. Five patients were treated with trientine monotherapy. Re-challenging with D-penicillamine at a lower dose followed by a slow dose escalation was attempted in three patients. Gradual clinical and functional status improvement was observed in 24 cases while one patient succumbed to pneumonia.
Conclusion:
Children and adolescents with WD who had neurological worsening on D-penicillamine therapy may be managed with trientine. Zinc monotherapy with copper restricted diet was also found to be effective in non-affordable patients.
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LETTERS TO THE EDITORS
Encephalitis and ocular flutter due to an undifferentiated connective tissue disorder
Boby Varkey Maramattom, Joe Thomas
July-August 2022, 25(4):733-734
DOI
:10.4103/aian.aian_1079_21
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564
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Severe microcephaly and rapid deterioration due to cortical atrophy in early infancy: Consider TRAPPC4 trappopathy
Akgun Olmez, Selcan Zeybek
July-August 2022, 25(4):735-738
DOI
:10.4103/aian.aian_1108_21
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564
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LETTERS TO THE EDITOR
Co-existent Trigeminal Neuralgia and SUNCT: A Clinician's Dilemma
Anand Kumar, Shivani Rath, Ram Bhupal Reddy, Deepika Joshi
July-August 2022, 25(4):761-762
DOI
:10.4103/aian.aian_153_22
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544
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LETTERS TO THE EDITORS
Paroxysmal dyskinesia in a case of hypocalcemia with hypothyroidism and pseudohypoparathyroidism
A Sai Chandra Niveditha, Rebecca John, Sujith Ovallath
July-August 2022, 25(4):753-755
DOI
:10.4103/aian.aian_896_21
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553
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Posterior reversible encephalopathy syndrome: An atypical presentation of takayasu arteritis
Abhay Ranjan, Sanaullah Mudassir, Neetu Sinha, Nitin Pandey
July-August 2022, 25(4):727-729
DOI
:10.4103/aian.aian_1007_21
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528
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Prominent jaw closure dystonia, awake bruxism, and stereotypies in a patient with hypoglycemic encephalopathy
Asish Vijayaraghavan, Anjali Chouksey
July-August 2022, 25(4):759-760
DOI
:10.4103/aian.aian_39_22
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524
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LIGHTER MOMENTS (ARTWORK OR PHOTOGRAPHY)
Of loss and Tau
Divyani Garg
July-August 2022, 25(4):713-713
DOI
:10.4103/aian.aian_342_22
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500
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LETTERS TO THE EDITORS
Fusiform intracranial aneurysms in a CADASIL patient: A possibly missed association
Ayush Agarwal, Pritam Mazumdar, Pranjal Gupta, Ajay Garg, Divya M Radhakrishnan, Animesh Das, Awadh K Pandit, Achal K Srivastava
July-August 2022, 25(4):747-749
DOI
:10.4103/aian.aian_903_21
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508
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A Rare Case of Neuromuscular Oculoauditory Syndrome
Arumugom Archana, Tamil Selvan, Venkatraman R Kumar, K Abinaya, Nirupama Kasturi
July-August 2022, 25(4):780-781
DOI
:10.4103/aian.aian_67_22
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486
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IMAGES IN NEUROLOGY
The differential arterial flow signal sign: An early brain MRI sign of circulatory arrest
Vikas Bhatia, Saifullah Khalid, Raghav Seth, Atul Saroch
July-August 2022, 25(4):710-710
DOI
:10.4103/aian.aian_57_22
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472
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LETTERS TO THE EDITORS
Fumaric aciduria: A rare cause of refractory epilepsy
Sangeetha Yoganathan, Rangan Srinivasaraghavan, Madhan Kumar, Lisa Kratz, Shraddha Mular, Sniya V Sudhakar, Prateek Malik, Mahalakshmi Chandran, Maya Thomas, Sumita Danda
July-August 2022, 25(4):738-740
DOI
:10.4103/aian.aian_47_22
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472
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Acute fulminant encephalopathy in an adult due to ornithine transcarbamylase deficiency
Vivek K Nambiar, Aakash Shridharani, Sudheeran Kannoth, Siby Gopinath, Anand Kumar
July-August 2022, 25(4):722-724
DOI
:10.4103/aian.aian_1028_21
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454
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IMAGES IN NEUROLOGY
Spontaneous resolution of a lenticulostriate artery aneurysm
Subhadeep Gupta, Arpan Dutta, Uddalak Chakraborty, Biman K Ray, Deep Das, Rahul Kumar
July-August 2022, 25(4):711-712
DOI
:10.4103/aian.aian_141_22
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452
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LETTERS TO THE EDITORS
Posterior Circulation Approach for Anterior Circulation Thrombectomy in a Patient with Dysgenetic Internal Carotid Artery
Somenath Chatterjee, Karthikeyan Vanchilingam, Siddhartha Wuppalapati, Hemant Sonwalkar, Abhijit Das
July-August 2022, 25(4):749-752
DOI
:10.4103/aian.aian_22_22
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Core Disease in an adult
Ashmeet Kaur, Kusum Mathur, Anita Harsh
July-August 2022, 25(4):729-731
DOI
:10.4103/aian.aian_792_21
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454
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Nasu–Hakola disease – A rare type of presenile dementia
NC Krishnadas, Mansoor C Abdulla, Divya Pachat
July-August 2022, 25(4):771-772
DOI
:10.4103/aian.aian_1059_21
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438
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Early recurrence of holmes tremor after radiofrequency thalamotomy
Halil Onder, Bilge Kocer, Selcuk Comoglu
July-August 2022, 25(4):755-756
DOI
:10.4103/aian.aian_1107_21
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426
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Rapidly Progressive Cognitive Impairment with Faciobrachial Dystonic Seizures (FBDS) in Anti-LGI1 Encephalitis
Weifang Yin, Hui Ren, Xinglong Yang
July-August 2022, 25(4):773-774
DOI
:10.4103/aian.aian_70_22
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© 2006 - Annals of Indian Academy of Neurology | Published by Wolters Kluwer -
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Online since 1
st
March, 2006