{"title":"The electroencephalogram in the diagnosis and classification of status epilepticus: a practical guide.","authors":"Hannah Ford, Udaya Seneviratne","doi":"10.1136/pn-2024-004336","DOIUrl":"10.1136/pn-2024-004336","url":null,"abstract":"<p><p>Status epilepticus is a serious neurological emergency requiring swift recognition and treatment. Presentations with prominent motor features are easily recognised but it can be challenging to diagnose those with subtle or no motor features. Electroencephalogram (EEG) remains indispensable in diagnosing, classifying, monitoring and prognosticating of status epilepticus. There are several separate classification systems for seizures, epilepsy and status epilepticus, incorporating clinical features, causes and EEG correlates. This review focuses on using EEG in status epilepticus and provides a practical approach to diagnosis and classification aligning with the current International League Against Epilepsy and American Clinical Neurophysiology Society definitions.</p>","PeriodicalId":39343,"journal":{"name":"PRACTICAL NEUROLOGY","volume":" ","pages":"396-410"},"PeriodicalIF":2.3,"publicationDate":"2025-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143075886","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Granulomatous polyangiitis with pachymeningitis: a case report.","authors":"Whei Chuern Yeoh, Cristina Estrach, Jagdish Nair","doi":"10.1136/pn-2024-004463","DOIUrl":"10.1136/pn-2024-004463","url":null,"abstract":"<p><p>A 33-year-old man had 1 month of right-sided headache with multiple right-sided cranial neuropathies. He had a history of recurrent sinusitis, elevated inflammatory markers and positive cytoplasmic anti-neutrophilic cytoplasmic antigens (c-ANCA) anti-proteinase 3 (anti-PR3). MR scans of the brain and cervical spine identified an inflammatory mass at the skull base with hypertrophic pachymeningitis and a sigmoid sinus thrombosis. Cerebrospinal fluid examination excluded infection and a meningeal biopsy confirmed vasculitis. CT scan of the chest showed multiple lung nodules with cavitation. We gave intravenous cyclophosphamide and corticosteroids as induction therapy for granulomatosis with polyangiitis. Unfortunately, during the induction phase, he developed diffuse alveolar haemorrhage and progressive mononeuritis multiplex. We subsequently gave intravenous rituximab and C5a receptor antagonist, avacopan. He recovered well and remains in remission.</p>","PeriodicalId":39343,"journal":{"name":"PRACTICAL NEUROLOGY","volume":" ","pages":"470-474"},"PeriodicalIF":2.3,"publicationDate":"2025-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143568260","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Gashirai K Mbizvo, Anna Visca, Saif Huda, Andrew J Larner
{"title":"Thinking beyond papilloedema: importance of cells in the vitreous.","authors":"Gashirai K Mbizvo, Anna Visca, Saif Huda, Andrew J Larner","doi":"10.1136/pn-2024-004453","DOIUrl":"10.1136/pn-2024-004453","url":null,"abstract":"","PeriodicalId":39343,"journal":{"name":"PRACTICAL NEUROLOGY","volume":" ","pages":"490-492"},"PeriodicalIF":2.3,"publicationDate":"2025-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143626404","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Saiju Jacob, Maria Elena Farrugia, Channa Hewamadduma, Fiona Norwood, Marguerite Hill, Maria Isabel Leite, John Paul McConville, Ashwin Arnold Pinto, Jennifer Spillane, Jon Sussman, Stuart Viegas
{"title":"Association of British Neurologists (ABN) autoimmune myasthenia gravis management guidelines (2025 update).","authors":"Saiju Jacob, Maria Elena Farrugia, Channa Hewamadduma, Fiona Norwood, Marguerite Hill, Maria Isabel Leite, John Paul McConville, Ashwin Arnold Pinto, Jennifer Spillane, Jon Sussman, Stuart Viegas","doi":"10.1136/pn-2025-004655","DOIUrl":"10.1136/pn-2025-004655","url":null,"abstract":"<p><p>The 2025 update of the Association of British Neurologists guidelines for the management of autoimmune myasthenia gravis (MG) emphasises several points that are distinct from the 2015 guidelines based on recent research and publications. The main differences from the previous guidelines are: (1) The recommendation to prescribe daily steroids rather than the alternate day regimen is now standard practice. (2) There is a clear emphasis on the beneficial effects of early thymectomy. (3) Randomised controlled trial evidence now supports early use of rituximab (within 1 year of generalised disease onset), although the evidence is less robust, but still likely to be useful, in established treatment-refractory MG. (4) Finally, several clinical trials have been published for newer targeted therapies in MG, predominantly those that inhibit the complement and neonatal Fc gamma receptor pathways, the roles of which are being slowly established especially in patients unresponsive to conventional therapy.</p>","PeriodicalId":39343,"journal":{"name":"PRACTICAL NEUROLOGY","volume":"25 5","pages":"422-437"},"PeriodicalIF":2.3,"publicationDate":"2025-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145056028","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Azza Mahmoud, Divyanshu Dubey, Ajith Goonetilleke, Gina Lee, Suzi Elhabet, Iftikhar Ahmad, Mohammad Dogar, Anu Jacob
{"title":"Paraneoplastic KLHL11 encephalitis presenting with progressive tinnitus, hearing loss and ataxia.","authors":"Azza Mahmoud, Divyanshu Dubey, Ajith Goonetilleke, Gina Lee, Suzi Elhabet, Iftikhar Ahmad, Mohammad Dogar, Anu Jacob","doi":"10.1136/pn-2024-004498","DOIUrl":"10.1136/pn-2024-004498","url":null,"abstract":"<p><p>Limbic encephalitis is associated with various tumours and antibodies. We describe a 33-year-old man with progressive cochleovestibular symptoms, cerebellar ataxia, myoclonus and antibodies against Kelch-like protein 11 (KLHL11). MR scan of the brain showed hippocampal T2 hyperintensities. FDG PET scan was normal. Biopsy of his enlarged left testes showed fibrosis without neoplasia. Immunotherapy so far has not helped significantly.</p>","PeriodicalId":39343,"journal":{"name":"PRACTICAL NEUROLOGY","volume":" ","pages":"443-447"},"PeriodicalIF":2.3,"publicationDate":"2025-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143469531","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Late-onset Pompe disease mimicking oculopharyngeal muscular dystrophy.","authors":"Moiz Mikail, Matthew Gladman","doi":"10.1136/pn-2024-004506","DOIUrl":"10.1136/pn-2024-004506","url":null,"abstract":"<p><p>Pompe disease is a genetic condition that results in glycogen accumulation in tissues, presenting from early infancy to late adulthood with various neurological and non-neurological features. We describe a woman in her late 70s with a slowly progressive onset (over years) of ptosis, oropharyngeal dysphagia and dysarthria, who was found to have late-onset Pompe disease. This case illustrates an atypical phenotype of late-onset Pompe disease that closely mimicked oculopharyngeal muscular dystrophy. Pompe disease is relatively easily identified using dried blood spot testing as a screening test. Enzyme replacement therapy for Pompe disease has increased the importance of its timely diagnosis, and recognising the variability in its presentation.</p>","PeriodicalId":39343,"journal":{"name":"PRACTICAL NEUROLOGY","volume":" ","pages":"466-469"},"PeriodicalIF":2.3,"publicationDate":"2025-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143543835","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Clarithromycin-induced myoclonic status epilepticus.","authors":"Mahmut Sami Biçimveren","doi":"10.1136/pn-2024-004464","DOIUrl":"10.1136/pn-2024-004464","url":null,"abstract":"<p><p>Myoclonus is a sudden brief involuntary activity and is either epileptic or non-epileptic. Myoclonic seizures are common in juvenile myoclonic epilepsy and in the much rarer childhood epilepsies, such as Dravet syndrome and Doose syndrome. However, they also occur at any age in adults. Myoclonic seizures may occur in cortical stroke, Alzheimer's disease, Lance-Adams syndrome, autoimmune limbic encephalitis and toxic-metabolic disorders. Clarithromycin may also cause myoclonic status epilepticus. We report a patient with myoclonic status epilepticus induced by clarithromycin.</p>","PeriodicalId":39343,"journal":{"name":"PRACTICAL NEUROLOGY","volume":" ","pages":"455-457"},"PeriodicalIF":2.3,"publicationDate":"2025-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143469461","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Catastrophic antiphospholipid antibody syndrome provoked by eltrombopag.","authors":"Bhargav Chandan Sumanth T, Aditya Vijayakrishnan Nair, Angel Miraclin T, George Abraham Ninan, Brunda K, Leena Rv, Harshad Arvind Vanjare, Joshi Nutan Damodar, Yamuna Naik, Appaswamy Thirumal Prabhakar, Ajith Sivadasan, Vivek Mathew, Sanjith Aaron","doi":"10.1136/pn-2025-004512","DOIUrl":"10.1136/pn-2025-004512","url":null,"abstract":"<p><p>Antiphospholipid antibody syndrome (APS) may rarely present with isolated thrombocytopenia that may be diagnosed and treated as immune thrombocytopenic purpura. A 32-year-old woman was given eltrombopag (a thrombopoietin receptor agonist) to treat presumed immune thrombocytopenic purpura. She developed catastrophic APS, with multiple ischaemic infarcts, microvascular coronary thrombosis and digital gangrene. She improved after stopping eltrombopag and starting immunotherapy (corticosteroids, intravenous immunoglobulin and rituximab) but was left with significant neurological morbidity.</p>","PeriodicalId":39343,"journal":{"name":"PRACTICAL NEUROLOGY","volume":" ","pages":"448-451"},"PeriodicalIF":2.3,"publicationDate":"2025-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143693776","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}