Annals of Indian Academy of Neurology最新文献

{"title":"Unmasking Osmotic Demyelination Syndrome/Extrapontine Myelinolysis in Acute Intermittent Porphyria: Preventable Complications-Challenges in Diagnosis and Management.","authors":"Vykuntaraju K Gowda, Priyanka A Nayak, Uddhava V Kinhal, Amaresh Roy, Varunvenkat M Srinivasan","doi":"10.4103/aian.aian_920_24","DOIUrl":"https://doi.org/10.4103/aian.aian_920_24","url":null,"abstract":"<p><strong>Abstract: </strong>Acute intermittent porphyria (AIP) is a dominant mendelian disorder caused due to deficiency of the enzyme porphobilinogen deaminase. It classically presents with pain abdomen, hypertensive crisis, electrolyte imbalance, mostly hyponatremia, and neuropsychiatric involvement. We report a case of a 12-year-old boy with AIP who experienced an acute crisis and later developed altered sensorium and seizures. Upon evaluation, he was found to have severe hyponatremia, which was secondary to the syndrome of inappropriate antidiuretic hormone secretion. His condition was corrected with intravenous hypertonic saline, and his sodium levels normalized over 2-3 days. Despite the successful correction of sodium levels, he developed extrapyramidal symptoms a week later. Magnetic resonance imaging of the brain revealed extrapontine myelinolysis. He was treated with intravenous steroids, which led to significant improvement. At 1-month follow-up, there were no neurological deficits.</p>","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143676697","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":"A Comparison of Transnasal Versus Ultrasound-Guided Suprazygomatic Approaches for Sphenopalatine Ganglion Blocks in Persistent Idiopathic Facial Pain.","authors":"Ezgi Can, Ömer T Akkaya","doi":"10.4103/aian.aian_713_24","DOIUrl":"https://doi.org/10.4103/aian.aian_713_24","url":null,"abstract":"<p><strong>Background and objectives: </strong>The majority of patients with persistent idiopathic facial pain (PIFP) do not respond to conservative treatment and thus require alternative therapeutic modalities. This study compared the effects of transnasal and ultrasound (US)-guided suprazygomatic approaches for sphenopalatine ganglion (SPG) block on pain management in refractory PIFP.</p><p><strong>Methods: </strong>This study included 38 patients with PIFP who underwent transnasal (Group T, n = 18) and US-guided suprazygomatic (Group U, n = 20) SPG block. Numeric Rating Scale (NRS) and Head Impact Test-6 (HIT-6) were used to assess therapeutic efficacy. Clinical evaluations were performed according to the scores recorded at baseline, 1 h, 1 week, and 4 weeks.</p><p><strong>Results: </strong>Transnasal and US-guided suprazygomatic SPG blocks in patients with PIFP provided effective and successful treatment results between groups at 1 h, 1 week, and 4 weeks compared to baseline (P < 0.001). However, when NRS scores were compared at 1 h and 4 weeks, the US-guided suprazygomatic block provided significantly better pain relief (P = 0.041 and P = 0.030, respectively). No adverse effects were observed, except transient facial paresthesia described in two patients in Group U.</p><p><strong>Conclusions: </strong>This retrospective study concluded that US-guided suprazygomatic SPG block can provide effective pain relief in PIFP patients at 4-week follow-up. This method can be regarded as an alternative treatment option, given that it is both safe and simple to apply.</p>","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-03-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143633190","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":"Muscle MRI Pattern in Dysferlinopathy and its Correlation with Dysferlin Gait.","authors":"Tanushree Chawla, Dipti Baskar, Kiran Polavarapu, Veeramani Preethish-Kumar, Saraswati Nashi, Seena Vengalil, Karthik Kulanthaivelu, Jitender Saini, Akshata Huddar, Gopikrishnan Unnikrishnan, Bevinahalli Nanjegowda Nandeesh, Atchayaram Nalini","doi":"10.4103/aian.aian_987_24","DOIUrl":"https://doi.org/10.4103/aian.aian_987_24","url":null,"abstract":"<p><strong>Background and objectives: </strong>Magnetic resonance imaging (MRI) in dysferlinopathy has consistently demonstrated a particular pattern of affliction. We aimed to study muscle MRI characteristics of lower limbs in limb girdle muscular dystrophy (LGMD)-R2 phenotypes and correlate them with the gait pattern.</p><p><strong>Methods: </strong>Forty genetically and/or biopsy-proven cases of dysferlinopathy underwent muscle MRI of the lower limbs. The pattern and extent of fatty infiltration and edema were recorded. Spearman's correlation analysis was used to find the correlation between muscle involvement and demographics, duration of illness, Muscular Dystrophy Functional Rating Scale (MDFRS), genotype, and gait pattern.</p><p><strong>Results: </strong>Mean age at onset and duration of illness at evaluation were 21.5 ± 6.3 years and 7.15 ± 4.95 years, respectively. Male: Female of patients was 2:1. Long head of biceps femoris was most severely involved with relative sparing of short head. Specific MRI pattern was noted based on phenotype, though no genotypic correlation was observed. Adductor magnus and semimembranosus were more severely involved in LGMD and proximodistal (PD) forms compared to Miyoshi muscular dystrophy type 1 phenotype. In addition, tibialis posterior and extensor hallucis longus were more severely involved in PD compared to MM and LGMD phenotypes. MDFRS mobility domain and duration of illness correlated with MRI findings. Gait pattern analysis revealed more severe involvement of flexor hallucis longus compared to extensor hallucis longus.</p><p><strong>Conclusions: </strong>Muscle involvement differed based on the phenotype. Characteristic great toe extension in PD phenotype showed an imaging correlation with more severe involvement of flexor hallucis longus compared to extensor hallucis longus. Thus, imaging can be a potential biomarker to study the evolution and severity of disease in dysferlinopathy.</p>","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-03-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143623275","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":"Novel Case of Bilateral Posterior Canal Benign Paroxysmal Positional Vertigo (BPPV) with Double Canal Switch and Canal Jam Following Treatment with Epley Maneuver.","authors":"Ajay K Vats, Andrea Castellucci, Sudhir Kothari, Shreya Vats, Priyanka Minocha","doi":"10.4103/aian.aian_944_24","DOIUrl":"https://doi.org/10.4103/aian.aian_944_24","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-03-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143623277","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":"Catch Me If You Can! - The \"Whack-a-Mole\" Sign for Functional Tremor.","authors":"Sayooja Sachithanandan, Asish Vijayaraghavan, Kiren George Koshy, Divya Kalikavil Puthanveedu, Syam Krishnan","doi":"10.4103/aian.aian_760_24","DOIUrl":"https://doi.org/10.4103/aian.aian_760_24","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143555684","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":"Frequency, Clinical Features, and Imaging Comparisons of Seropositive versus Seronegative Autoimmune Encephalitis in a Tertiary Care Setting.","authors":"Bhavani Madduluri, Shaik A Jabeen, Reshma S Shaik, Sireesha Yareeda, Suryaprabha Turaga, Madhur K Srivastava, Charan Neeradi","doi":"10.4103/aian.aian_718_24","DOIUrl":"https://doi.org/10.4103/aian.aian_718_24","url":null,"abstract":"<p><strong>Background and objectives: </strong>Seronegative autoimmune encephalitis (SNAIE) is an enigmatic entity which lacks detectable autoantibodies, leading to a diagnostic delay. Our objective was to study the frequency of SNAIE and compare the clinical, electrophysiological, and imaging characteristics of patients with SNAIE to those in patients with seropositive autoimmune encephalitis (SPAIE).</p><p><strong>Methods: </strong>This ambispective observational study was undertaken at a tertiary care center in South India for 7 years. The demographic data, clinical features, cerebrospinal fluid (CSF) analysis findings, electroencephalogram (EEG), and 3 T magnetic resonance imaging (MRI), and 18F fluoro-2-deoxy-d-glucose positron emission tomography computerized tomography (18F FDG PET-CT) findings were compared.</p><p><strong>Results: </strong>Of the 64 subjects enrolled, SNAIE frequency was 62.5%, with a notable 3:1 male-to-female ratio. Seizure was the predominant clinical presentation in SNAIE compared to SPAIE (50% vs. 20%, P value 0.02). Refractory seizures (45% vs. 4.5%) and status epilepticus (37.5% vs. 0%) were more common in SNAIE ( P value 0.001), whereas involuntary movements and faciobrachial dystonic seizures were common in SPAIE (66% vs. 16%, P value 0.04). CSF pleocytosis was frequently seen in SPAIE (50% vs. 17%, P value 0.006). EEG findings in SNAIE included increased epileptiform discharges and built-up rhythms. FDG PET abnormalities were observed in both groups (91% SPAIE, 97% SNAIE), but the seropositive group exhibited more temporal lobe involvement (58% vs. 30%, P = 0.036) and SNAIE had more diffuse involvement.</p><p><strong>Conclusions: </strong>SNAIE exhibited male predominance, primarily presenting with isolated seizure as the initial presentation. EEG and FDG PET findings distinguished the two groups, emphasizing the importance of these modalities as biomarkers in the early detection and management of SNAIE.</p>","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143536367","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":"Prolonged Survival in Subacute Sclerosing Panencephalitis: Case Series with a Review of Literature on \"Long Survivors\".","authors":"Archita Makharia, Ayush Agarwal, Ajay Garg, Achal K Srivastava, Divyani Garg","doi":"10.4103/aian.aian_871_24","DOIUrl":"https://doi.org/10.4103/aian.aian_871_24","url":null,"abstract":"<p><strong>Abstract: </strong>Subacute sclerosing panencephalitis (SSPE) is a progressive and usually fatal disorder, arising from persistent measles virus infection in the brain. Exceptionally, prolonged survival has been noted. We report four such patients and also review current literature on long survival in SSPE.</p>","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143536389","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":"Jaw-Opening Myoclonus: A Rare and Distinctive Sign in Subacute Sclerosing Panencephalitis.","authors":"Bhavani Madduluri, Suryaprabha Turaga, Yenni Harika, Sabeeha Naaz, S Arun","doi":"10.4103/aian.aian_1075_24","DOIUrl":"https://doi.org/10.4103/aian.aian_1075_24","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143536385","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":"When Tuberculosis Lurks: The Silent Risk of Central Nervous System Infection Following In vitro Fertilization.","authors":"Suparna Kumar, Govind Madhaw","doi":"10.4103/aian.aian_1018_24","DOIUrl":"https://doi.org/10.4103/aian.aian_1018_24","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143522349","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":"Isolated Central and Peripheral Nervous System Relapse in Chronic Myeloid Leukemia: A Report of Two Cases with Review of Literature.","authors":"Farsana Mustafa, Divyani Garg, Ajay Garg, Ayush Agarwal, Lavleen Singh, Mounika Pallati, Akash Jha, Achal Kumar Srivastava","doi":"10.4103/aian.aian_841_24","DOIUrl":"https://doi.org/10.4103/aian.aian_841_24","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143522346","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}