Journal of the Neurological Sciences最新文献

{"title":"Neurosarcoidosis of the hypothalamus, infundibulum, and pituitary gland: A retrospective cohort study","authors":"Max Herman,&nbsp;Katherine Russell,&nbsp;Hang Shi,&nbsp;James G. Greene,&nbsp;Spencer K. Hutto","doi":"10.1016/j.jns.2026.125807","DOIUrl":"10.1016/j.jns.2026.125807","url":null,"abstract":"<div><h3>Background</h3><div>Hypothalamic-pituitary involvement is common in neurosarcoidosis, but dedicated studies describing its impact are limited.</div></div><div><h3>Objective</h3><div>To determine the clinicoradiographic characteristics, treatment responsiveness, and long-term outcomes of hypothalamic-pituitary neurosarcoidosis.</div></div><div><h3>Methods</h3><div>Patients with definite or probable neurosarcoidosis were included if they exhibited radiographic involvement of the hypothalamus, infundibulum, and/or pituitary and were divided into symptomatic and asymptomatic groups. When available, radiographic features were directly reviewed by the authors.</div></div><div><h3>Results</h3><div>Thirty-one patients (31/247 total cohort, 12.6%) were included, 19 (61.3%) being symptomatic. Chronic presentations were typical (13/19, 68.4%), frequently with polydipsia, polyuria, memory impairment, and gonadal dysfunction. On MRI, lesions were centered in the infundibulum (28/29, 96.6%) and inferior hypothalamus (28/29, 96.6%), typically expansile (23/28, 82.1%) and homogeneously enhancing (25/28, 89.3%). Other inflammatory foci in the neuraxis (27/29, 93.1%; especially cranial nerves and meninges) and body (30/31, 96.8%) were routinely seen. Nearly all responded to corticosteroids acutely (17/18, 94.4%). Most (13/19, 68.4%) required two or more lines of maintenance treatments, with TNF inhibitor regimens most often successful (8/12, 66.7%). Symptomatic patients experienced worse functional outcomes (mRS 3 vs 1, <em>p</em> 0.027) and a higher relapse risk (12/19 vs 3/12, <em>p</em> 0.048) than asymptomatic patients. Despite improved or resolved imaging (18/19, 94.7%), almost all symptomatic patients (18/19, 94.7%) were on corticosteroids and/or hormonal treatments at last follow-up (median 55 months).</div></div><div><h3>Conclusion</h3><div>Symptomatic hypothalamic-pituitary neurosarcoidosis is a severe phenotype with high rates of disability, relapse, treatment refractoriness, and long-term hormonal and steroidal treatments despite improving radiographic abnormalities.</div></div>","PeriodicalId":17417,"journal":{"name":"Journal of the Neurological Sciences","volume":"482 ","pages":"Article 125807"},"PeriodicalIF":3.2,"publicationDate":"2026-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146190423","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Opisthotonus: Revisiting a classic movement disorder","authors":"José Fidel Baizabal-Carvallo ,&nbsp;Joseph Jankovic","doi":"10.1016/j.jns.2026.125782","DOIUrl":"10.1016/j.jns.2026.125782","url":null,"abstract":"<div><div>Opisthotonus is characterized by backward arching of the trunk, associated with retrocollis, caused by involuntary contractions of extensor paraspinal muscles. Classical etiologies include tetanus, strychnine intoxication, dystonia (particularly tardive dystonia and neurodegeneration with brain iron accumulation), seizures, and functional movement disorders (MDs). Inborn errors of metabolism, epileptic encephalopathies and other gene mutations should be considered in newborns and infants presenting with opisthotonus. We surveyed 1216 consecutive patients presenting for evaluation in a tertiary care center for MDs and identified 17 (1.4%) with opisthotonus. Functional opisthotonus represented the most common cause, present in 9 (53%) patients. Other etiologies included tardive dystonia (<em>n</em> = 2), dystonia secondary to cavernous hemangioma in the basal ganglia (<em>n</em> = 1), dystonia due to hypoxic encephalopathy (n = 1), and 4 with idiopathic dystonia. Identifying the underlying etiology of opisthotonus is important to guide therapeutic strategies. Benzodiazepines, baclofen, anticholinergics, and botulinum toxin are common pharmacological options, whereas deep brain stimulation and surgical correction of the underlying cause should be considered in selected cases.</div></div>","PeriodicalId":17417,"journal":{"name":"Journal of the Neurological Sciences","volume":"482 ","pages":"Article 125782"},"PeriodicalIF":3.2,"publicationDate":"2026-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146105862","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The impact of adding optical coherence tomography in MS diagnostic criteria on the classification of tumefactive demyelination","authors":"Fiorella S. Guido ,&nbsp;John J. Chen ,&nbsp;Paul A. Decker ,&nbsp;Mahboubeh Fereidan-Esfahani ,&nbsp;Kevin D. Chodnicki ,&nbsp;Deena A. Tajfirouz ,&nbsp;Eoin P. Flanagan ,&nbsp;Orhun H. Kantarci ,&nbsp;B. Mark Keegan ,&nbsp;Sean J. Pittock ,&nbsp;Jan-Mendelt Tillema ,&nbsp;Claudia F. Lucchinetti ,&nbsp;Jeanette E. Eckel-Passow ,&nbsp;W. Oliver Tobin","doi":"10.1016/j.jns.2026.125739","DOIUrl":"10.1016/j.jns.2026.125739","url":null,"abstract":"<div><h3>Background</h3><div>De novo tumefactive demyelination (TD) poses a diagnostic challenge, with lower rates of cerebrospinal fluid (CSF) oligoclonal bands and lower female-to-male ratio than typical onset multiple sclerosis (MS). Only about half fulfill the 2017 McDonald diagnostic criteria for MS at presentation. Therefore, our aim was to assess optic nerve involvement by optical coherence tomography (OCT) in patients with TD. Further, whether the presence of abnormalities on OCT would allow additional TD patients to be diagnosed with MS when the optic nerve is added as a fifth topography to the Barkhof criteria or 2017 McDonald criteria.</div></div><div><h3>Methods</h3><div>Observational retrospective chart review of patients seen at a tertiary referral center from 1/1/1990 to 2/21/2024 with TD. Inclusion criteria were: 1) brain MRI showing an active TD lesion; 2) medical records with at least one available clinical assessment by a neurologist; 3) available Cirrus OCT data to review. Exclusion criteria were: 1) patients with final diagnosis of vasculitis, abscess, CNS malignancies, or MOGAD; 2) presence of confounding ocular disease.</div></div><div><h3>Results</h3><div>OCT was available in 68 patients with TD. The tumefactive attack was the first demyelinating event in 45 (67 %) patients. At presentation, 29/68 (43 %) fulfilled 3 of 4 Barkhof criteria, and 34/68 (50 %) fulfilled the 2017 McDonald criteria for the diagnosis of MS. A clinical history of optic neuritis was present in 9/68 (13 %) patients and OCT was abnormal in 9/9 (100 %) of those patients. Regardless of clinical history, OCT was abnormal in 22/68 (32 %) patients. Therefore, OCT identified abnormalities suggestive of prior optic neuritis in 13/59 (22 %) TD patients without a clinical history of optic neuritis. If the optic nerve, as a firth topography, was assessed by OCT 39/68 (57 %) would fulfill McDonald criteria.</div></div><div><h3>Conclusions</h3><div>OCT in patients presenting with TD lesions can frequently identify both clinical and subclinical optic neuritis. The addition of the optic nerve as a fifth topography assessed by OCT to fulfill dissemination in space criteria would allow additional TD patients to be diagnosed with MS.</div></div>","PeriodicalId":17417,"journal":{"name":"Journal of the Neurological Sciences","volume":"481 ","pages":"Article 125739"},"PeriodicalIF":3.2,"publicationDate":"2026-02-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145966568","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Methodological considerations on benzodiazepine exposure and dementia risk: Commentary on Legrand et al.","authors":"Tian Ruan ,&nbsp;Tao Yu ,&nbsp;Fang Zhao ,&nbsp;Yaohua Tang","doi":"10.1016/j.jns.2026.125736","DOIUrl":"10.1016/j.jns.2026.125736","url":null,"abstract":"","PeriodicalId":17417,"journal":{"name":"Journal of the Neurological Sciences","volume":"481 ","pages":"Article 125736"},"PeriodicalIF":3.2,"publicationDate":"2026-02-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146018976","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diabetic lumbosacral radiculoplexus neuropathy after glucagon-like peptide 1 receptor agonist use: A case series","authors":"Swathy Chandrashekhar ,&nbsp;Long Davalos ,&nbsp;Richeek Pradhan ,&nbsp;Pritikanta Paul","doi":"10.1016/j.jns.2026.125755","DOIUrl":"10.1016/j.jns.2026.125755","url":null,"abstract":"<div><h3>Background</h3><div>Diabetic Lumbosacral Radiculoplexus Neuropathy (DLRPN) is a rare form of debilitating neuropathy, usually preceded by rapid glycemic control.</div></div><div><h3>Methods</h3><div>We describe a case series of patients with DLRPN who had been exposed to GLP-1 Receptor Agonists (GLP-1 RA) prior to symptom onset.</div></div><div><h3>Results</h3><div>Six patients (3 men; aged 53–73) with type 2 diabetes developed sudden-onset, asymmetric lower limb pain followed by weakness-bilateral in 5/6. Most had substantial weight loss (35–52 lbs) and rapid HbA1c decline (&gt;5) in months preceding symptoms. Four patients had electrophysiologic evidence of lumbosacral plexopathy; imaging was supportive in 2. One patient received intravenous steroids with improvement; others were managed supportively, with 3 showing stabilization or mild recovery.</div></div><div><h3>Discussion</h3><div>This series highlights a potential association between rapid glycemic and weight changes from GLP-1 RA use and DLRPN. Clinicians should be alert to subacute neuropathy with muscle weakness in patients undergoing aggressive glycemic control.</div></div>","PeriodicalId":17417,"journal":{"name":"Journal of the Neurological Sciences","volume":"481 ","pages":"Article 125755"},"PeriodicalIF":3.2,"publicationDate":"2026-02-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145979300","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Authors' reply to comment letter on ZILU25 study","authors":"Vincenzo Di Stefano ,&nbsp;Claudia Vinciguerra ,&nbsp;Michelangelo Maestri Tassoni ,&nbsp;Rita Frangiamore","doi":"10.1016/j.jns.2026.125758","DOIUrl":"10.1016/j.jns.2026.125758","url":null,"abstract":"","PeriodicalId":17417,"journal":{"name":"Journal of the Neurological Sciences","volume":"481 ","pages":"Article 125758"},"PeriodicalIF":3.2,"publicationDate":"2026-02-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146035239","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Author response: Impact of dysphagia on early psychosocial consequences after acute ischemic stroke","authors":"Anel Karisik ,&nbsp;Stefan Kiechl ,&nbsp;Michael Knoflach ,&nbsp;Lukas Mayer-Suess","doi":"10.1016/j.jns.2026.125763","DOIUrl":"10.1016/j.jns.2026.125763","url":null,"abstract":"","PeriodicalId":17417,"journal":{"name":"Journal of the Neurological Sciences","volume":"481 ","pages":"Article 125763"},"PeriodicalIF":3.2,"publicationDate":"2026-02-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146018918","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Response to the letter to the editor “Myotonic dystrophy and cancer risk: Insights, limitations, and the need for genetic certainty”","authors":"Bilal Hameed ,&nbsp;Shahinaz M. Gadalla","doi":"10.1016/j.jns.2025.125718","DOIUrl":"10.1016/j.jns.2025.125718","url":null,"abstract":"","PeriodicalId":17417,"journal":{"name":"Journal of the Neurological Sciences","volume":"481 ","pages":"Article 125718"},"PeriodicalIF":3.2,"publicationDate":"2026-02-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145891183","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Digital biomarkers in early Alzheimer's disease from wearable or portable technology: A scoping review","authors":"Mathias Holsey Gramkow ,&nbsp;Helena Sophia Coley Gleerup ,&nbsp;Anja Hviid Simonsen ,&nbsp;Gunhild Waldemar ,&nbsp;Kristian Steen Frederiksen","doi":"10.1016/j.jns.2026.125734","DOIUrl":"10.1016/j.jns.2026.125734","url":null,"abstract":"<div><h3>Background</h3><div>The pursuit of accurate biomarkers for early detection and disease monitoring of Alzheimer's disease (AD) has driven a growing interest in digital biomarkers. We aimed to map the research landscape of digital biomarkers in early AD obtained with wearable or portable digital health technologies (DHTs).</div></div><div><h3>Methods</h3><div>In our scoping review, we included original research on portable or wearable DHTs where digital biomarkers were measured in populations of early AD (mild cognitive impairment (MCI) or mild dementia). We searched MEDLINE, Web of Science and EMBASE with a wide search strategy with independent review and data extraction by two review team members. We charted data in tabular/graphical form.</div></div><div><h3>Results</h3><div>After deduplication and screening of 8893 records, we included 109 studies describing a wide array of wearable or portable DHTs that obtained digital biomarkers. The study population consisted of 3019 individuals with MCI due to AD (54 % female, weighted mean age 73 years), and 1942 individuals with mild AD (55 % female, weighted mean age 73 years). The most studied biomarkers were rest/activity (39 %), speech (17 %), and gait (14 %), with most studies focusing on one domain. Few studies reported outcomes associated with diagnosis (16 %) and prognosis (3 %).</div></div><div><h3>Conclusion</h3><div>We identified a growing evidence base investigating digital biomarkers in early AD. There is a paucity of studies examining diagnostic and prognostic properties, representing a knowledge gap. This overview may help to guide future research efforts to bridge the gap between the development and clinical implementation of digital biomarkers in early Alzheimer's disease.</div></div>","PeriodicalId":17417,"journal":{"name":"Journal of the Neurological Sciences","volume":"481 ","pages":"Article 125734"},"PeriodicalIF":3.2,"publicationDate":"2026-02-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145940311","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Letter to the editor: Association between benzodiazepines and dementia: A case-control study from Canadian health surveys and medico-administrative databases","authors":"Zhiyi Chen ,&nbsp;Longyao Zhang ,&nbsp;Xuezhu Zhang","doi":"10.1016/j.jns.2026.125738","DOIUrl":"10.1016/j.jns.2026.125738","url":null,"abstract":"","PeriodicalId":17417,"journal":{"name":"Journal of the Neurological Sciences","volume":"481 ","pages":"Article 125738"},"PeriodicalIF":3.2,"publicationDate":"2026-02-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145940313","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}