{"title":"Clarification on \"Myotonic Dystrophy Type 1 - An Atypical Presentation\".","authors":"Joydeep Mukherjee, Sourav Nanda, Amar Kumar Mishra, Arindam Biswas","doi":"10.4103/aian.aian_341_24","DOIUrl":"10.4103/aian.aian_341_24","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141295415","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Aishwarya Ghosh, Palash Kumar Malo, Sadhana Singh, S Monisha, Thomas Gregor Issac
{"title":"Reduced Cerebellar White Matter Volume in Musicians: A Marker of Musical Expertise.","authors":"Aishwarya Ghosh, Palash Kumar Malo, Sadhana Singh, S Monisha, Thomas Gregor Issac","doi":"10.4103/aian.aian_261_24","DOIUrl":"10.4103/aian.aian_261_24","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141987267","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"An Instructional Module for Functional Dissociative Seizures (FDS) in Hindi: Enhancing Communication and Management in India.","authors":"Manjari Tripathi, Sandhya Sharma, Avinash Ganapule, Rekha Dwivedi, Kirandeep Kaur, Vasundhara Shukla, Saranya Gomathy, Prarthana Chandra, Rajesh Kumar Singh, Deepti Vibha, Rajesh Sagar, Mamta Sood, Ashima Nehra, Shashank Tripathi","doi":"10.4103/aian.aian_305_24","DOIUrl":"10.4103/aian.aian_305_24","url":null,"abstract":"<p><strong>Background and objectives: </strong>Functional dissociative seizures (FDS) are very common in neurology clinics, they straddle both neurology and psychiatry. In India, scarce resources and guidelines are available for effectively conveying their diagnosis and management, which leads to a substantial gap in care. We aimed to provide an instructional module for FDS, tailored to clinicians, patients, and caregivers in Hindi, to enhance communication of diagnosis and facilitate suitable management strategies.</p><p><strong>Methods: </strong>The instructional module development process was based on clinical settings and expert opinions. A standard translation process was performed to ensure the linguistic and cultural equivalence. Validation was carried out by a panel of 15 validators, comprising 13 neurologists and two psychiatrists, to identify key psychoeducation components and their significance. They were provided a questionnaire consisting of five questions that were related to completeness, understandability, legibility, clarification, and usefulness of educational material for clinicians, patients, and caregivers. Items were rated using a 5-point Likert scale.</p><p><strong>Results: </strong>There was agreement on four items (Q1, Q3, Q4, and Q5), whereas approximately 85% of the validators agreed on the remaining one item (Q2). The instructional module demonstrated high content validity as indicated by both item level-content validity index (0.98) and scale-level content validity index (0.97), alongside content validity ratio and modified kappa statistics.</p><p><strong>Conclusions: </strong>The developed Hindi instructional module for FDS serves as a valuable tool to enhance the communication of FDS diagnosis and dispel misconceptions. This offers a comprehensive resource for clinicians, patients, and caregivers in India, potentially bridging gap in FDS care.</p>","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142456559","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Optic Neuritis - The Evolving Spectrum.","authors":"Abdullah Al-Ani, Fiona Costello","doi":"10.4103/aian.aian_602_24","DOIUrl":"10.4103/aian.aian_602_24","url":null,"abstract":"<p><p>Optic neuritis (ON) is an inflammatory condition that affects the optic nerve and may be associated with various central nervous system demyelinating conditions, infectious diseases, and systemic autoimmune syndromes. This manuscript sheds light on the epidemiologic patterns and diverse clinical features of ON, emphasizing the importance of early detection and prompt treatment. Various studies have revealed geographic and ethnic variations across ON subtypes, which are likely related to the incidence and prevalence of co-associated disorders. Distinguishing ON subtypes may be challenging and requires use of paraclinical tools. Treatment strategies differ depending on the etiology, further highlighting the importance of accurately identifying specific ON subtypes in a timely manner.</p>","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142456576","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Cardiac MRI in Duchenne and Becker Muscular Dystrophy.","authors":"Manu Santhappan Girija, Deepak Menon, Kiran Polavarapu, Veeramani Preethish-Kumar, Seena Vengalil, Saraswati Nashi, Madassu Keertipriya, Mainak Bardhan, Priya Treesa Thomas, Valasani Ravi Kiran, Vikas Nishadham, Arun Sadasivan, Akshata Huddar, Gopi Krishnan Unnikrishnan, Ashita Barthur, Atchayaram Nalini","doi":"10.4103/aian.aian_988_23","DOIUrl":"10.4103/aian.aian_988_23","url":null,"abstract":"<p><strong>Background and objectives: </strong>Cardiovascular magnetic resonance imaging (CMRI) is the noninvasive technique of choice for early detection of cardiac involvement in Duchenne and Becker muscular dystrophy (DMD and BMD, respectively), but is seldom used in routine clinical practice in the Indian context. We sought to determine the prevalence of CMRI abnormalities in patients with DMD and BMD and to compare the CMRI parameters with the phenotypic and genotypic characteristics.</p><p><strong>Methods: </strong>A prospective, observational study was conducted on patients genetically diagnosed with DMD and BMD who could complete CMRI between March 2020 and March 2022. Abnormal CMRI was the presence of any late gadolinium enhancement (LGE) that signifies myocardial fibrosis (LGE positivity), regional wall motion abnormality, or reduced left ventricular ejection fraction (LVEF <55%).</p><p><strong>Results: </strong>A total of 46 patients were included: 38 patients with DMD and eight with BMD. Cardiac abnormality was seen in 23 (50%) patients. LGE was more common than impaired LVEF in DMD (16, 42.1%), while impaired LVEF was more common in BMD (5, 62.5%). LGE was most frequently found in lateral wall (18/19) followed by inferior (6/19), septal (5/19), anterior (2/19), and apex (1/19). Among the various clinicodemographic parameters, only age ( r = 0.495, P = 0.002) and disease duration ( r = 0.407, P = 0.011) were found to significantly correlate with LGE in patients with DMD. No association was found between the various CMRI parameters and the genotype.</p><p><strong>Conclusions: </strong>The current study highlights the differences in myocardial fibrosis and LV dysfunction between DMD and BMD, along with other CMRI parameters. Notably, a genotype-CMRI correlation was not found in the current cohort, which needs to be further explored.</p>","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142339789","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Exploring Uncommon Neurologic Complication in Young Diabetics with Osmotic Demyelination Syndrome: A Case Series and Literature Review.","authors":"Manisha Gupta, Ramesh Gurjar, Arpit Agrawal, Abhijeet Kumar Kohat, Abhishek Kumar","doi":"10.4103/aian.aian_390_24","DOIUrl":"10.4103/aian.aian_390_24","url":null,"abstract":"<p><p>Osmotic demyelination syndrome (ODS) is well known to be associated with the rapid correction of hyponatremia. However, there is limited literature on its link to persistent or corrected hyperglycemic states in diabetic patients. We report two young patients with ODS and hyperglycemia. Neuroimaging and blood tests indicated central pontine and extrapontine myelinolysis with a hyperglycemic hyperosmolar state (HHS). These unusual cases provide insight into the neurologic complications of hyperglycemia.</p>","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141900748","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Effectiveness of Home-Based Self-monitoring of Blood Pressure in a Primary Care set-up of India (EASE-BP): Protocol for an Open-Label Randomized Controlled Trial.","authors":"Rohit Bhatia, Partha Haldar, Imnameren Longkumer, Madakasira Vasantha Padma Srivastava, Puneet Misra, Gautam Sharma, Mamta Bhushan Singh, Rakesh Kumar, Harshal Ramesh Salve","doi":"10.4103/aian.aian_471_24","DOIUrl":"10.4103/aian.aian_471_24","url":null,"abstract":"<p><strong>Background and objectives: </strong>Rising hypertension from inadequate detection, noncompliance with treatment, and poor control presents a major public health challenge. Previously, adopting the strategy of self-monitoring of blood pressure (SMBP) showed better hypertension detection, control, and medication adherence. However, evidence from India is scarce. Here, we present the trial protocol for the effectiveness of SMBP intervention in hypertension detection, blood pressure (BP) control, and medication adherence in a primary care set-up.</p><p><strong>Methods: </strong>EASE-BP is an open-label randomized trial in two distinct adult populations - part-A self-reported non-hypertensive population (total sample size: 400) and part-B known hypertensive population (total sample size: 332). The two populations will be sampled from two separate villages under the Ballabgarh Health and Demographic Surveillance System. In both the populations, participants randomized in the intervention arm will receive a validated electronic BP device and will be advised to self-monitor BP for at least twice a month (or more). Those in the control arm will be advised to monitor BP for at least twice a month (or more) at the nearby health center. Participants in both arms will receive education on hypertension and atherosclerotic disease. In addition, adult family members in the households of randomized participants will be encouraged to monitor their BP. All participants and family members will be instructed to maintain a log of BP recordings in a diary that will be provided to them. Data will be collected at baseline and then every month for three-months. The primary outcome in part-A will be the incidence of new cases of hypertension during 3 months follow-up and in part-B will be the difference in SBP and rate of medication adherence at 3 months follow-up. Secondary outcomes will include (1) uptake of the intervention, (2) any stroke, myocardial infarction, or death, (3) additional yield of new cases of hypertension among the family members, and (4) integrating the intervention into the national programs for long-term noncommunicable disease goals.</p><p><strong>Conclusion: </strong>EASE-BP will help understand if SMBP improves hypertension detection, BP control, and medication adherence. Consequently, this will appraise policymakers on whether provision of BP instruments may be added to the national program for hypertension prevention and control.</p><p><strong>Trial registry number: </strong>This trial was registered under Clinical Trial Registry - India with reference number CTRI/2023/02/049949.</p>","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142456561","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ajay K Vats, Shreya Vats, Sudhir Kothari, Nishant Aswani
{"title":"Atypical Benign Paroxysmal Positional Vertigo: Concomitant Posterior Semicircular Cupulolithiasis with Ipsicanal Nonampullary Arm Posterior Semicircular Canalolithiasis.","authors":"Ajay K Vats, Shreya Vats, Sudhir Kothari, Nishant Aswani","doi":"10.4103/aian.aian_357_24","DOIUrl":"10.4103/aian.aian_357_24","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142387438","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Saranya B Gomathy, Yamini Priyanka, Ajay Garg, William L Macken, Ayush Agarwal, Tanveer Ahmed, Rohit Bhatia, Vinay Goel, Kanwaljeet Garg, Robert Ds Pitceathly, Mary M Reilly, Michael G Hanna, Mv Padma Srivastava, Venugopalan Y Vishnu
{"title":"Phenotypic, Electrophysiologic, and Imaging Spectrum of Hirayama Disease from Northern India.","authors":"Saranya B Gomathy, Yamini Priyanka, Ajay Garg, William L Macken, Ayush Agarwal, Tanveer Ahmed, Rohit Bhatia, Vinay Goel, Kanwaljeet Garg, Robert Ds Pitceathly, Mary M Reilly, Michael G Hanna, Mv Padma Srivastava, Venugopalan Y Vishnu","doi":"10.4103/aian.aian_348_24","DOIUrl":"10.4103/aian.aian_348_24","url":null,"abstract":"<p><strong>Background and objectives: </strong>Cervical flexion-induced myelopathy, also known as Hirayama disease (HD), is a lower motor neuron disorder seen mainly in adolescents and young adults, affecting the C7-T1 myotomes, presenting as asymmetric weakness with wasting of one or both the distal upper limbs. We aimed to describe the clinical features, electrophysiology, and radiologic features of HD in a tertiary care institute in northern India.</p><p><strong>Methods: </strong>One hundred and forty patients between 2017 and 2022 with clinical and imaging features consistent with HD were retrospectively reviewed from the All India Institute of Medical Sciences-Comprehensive Neuromuscular Diseases center database.</p><p><strong>Results: </strong>Majority were males with the mean age of onset of illness being 17.8 years. The median duration of the symptoms was 3 (1.5-4) years. Sixty-nine (49%) patients had unilateral involvement, and the disease was actively progressing in 88 (63%) patients at presentation. Two families had history of HD in two (1.4%) siblings. Electromyography showed abnormal findings in the clinically involved limb in all the patients and in the clinically uninvolved limb in 17/50 (34%) patients. Flexion magnetic resonance imaging (MRI) demonstrated forward dural displacement in 134 (96%) patients and asymmetric cord flattening in 124 (88.5%) patients. Disability was graded as mild, moderate, and severe; 12 (13%) had severe disability. The majority were managed conservatively, and four underwent surgery for HD.</p><p><strong>Conclusion: </strong>A high index of suspicion of HD needs to be kept in a young male presenting with distal upper limb weakness and atrophy. Dynamic MRI of the cervical spine in young adults presenting with hand wasting is inevitable. This disease needs to be managed aggressively and early to prevent serious dysfunction and loss of productivity.</p>","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142456577","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"My Birdsongs in Clinical Neurology.","authors":"Satish V Khadilkar","doi":"10.4103/aian.aian_840_24","DOIUrl":"https://doi.org/10.4103/aian.aian_840_24","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142493428","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}