{"title":"Oligoclonal bands.","authors":"Mark D Willis, Karim L Kreft, Bethan Dancey","doi":"10.1136/pn-2023-003814","DOIUrl":"10.1136/pn-2023-003814","url":null,"abstract":"<p><p>Oligoclonal bands (OCBs) represent the presence of intrathecal immunoglobulin G (IgG) as detected by isoelectric focusing and immunofixation. Cerebrospinal fluid (CSF) analysed alongside a paired serum sample gives five different immunofixation patterns. These are: type 1-the normal physiological state with no intrathecal IgG synthesis; type 2-evidence for intrathecal IgG synthesis, with CSF-restricted OCBs; type 3-evidence for intrathecal IgG synthesis, with CSF-restricted OCBs, but with additional, identical bands in the CSF and serum; type 4-absence of intrathecal IgG synthesis, but with identical OCBs in CSF and serum; and type 5-absence of intrathecal IgG synthesis, with a monoclonal band in CSF and serum. Analysis of these patterns can help to diagnose a range of neurological conditions, including multiple sclerosis. However, it is important to interpret OCB results alongside other CSF tests and their clinical context.</p>","PeriodicalId":39343,"journal":{"name":"PRACTICAL NEUROLOGY","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141471336","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}
Dylan Selbst, Sai Sachin Divakaruni, Carly Weber-Levine, Elias S Sotirchos
{"title":"Seizures and skin crawling: neurocysticercosis with subcutaneous cysticercosis.","authors":"Dylan Selbst, Sai Sachin Divakaruni, Carly Weber-Levine, Elias S Sotirchos","doi":"10.1136/pn-2024-004188","DOIUrl":"10.1136/pn-2024-004188","url":null,"abstract":"","PeriodicalId":39343,"journal":{"name":"PRACTICAL NEUROLOGY","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141321770","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":"'A Ray of Darkness' by Margiad Evans.","authors":"Yasmin Zid, Phil E M Smith","doi":"10.1136/pn-2024-004132","DOIUrl":"10.1136/pn-2024-004132","url":null,"abstract":"","PeriodicalId":39343,"journal":{"name":"PRACTICAL NEUROLOGY","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140132823","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}
Kaminie Moodley, Anandan A Moodley, Stephanie Efthymiou, Henry Houlden, Pierre L A Bill, Vinod B Patel, Simon Rinaldi
{"title":"Combined central and peripheral demyelination in two siblings, immune mediated or genetic?","authors":"Kaminie Moodley, Anandan A Moodley, Stephanie Efthymiou, Henry Houlden, Pierre L A Bill, Vinod B Patel, Simon Rinaldi","doi":"10.1136/pn-2024-004114","DOIUrl":"10.1136/pn-2024-004114","url":null,"abstract":"<p><p>We report unusual cases of combined central and peripheral demyelination in two siblings related to pregnancy, each presenting with progressive tetraparesis and cranial nerve palsies. The elder sister had a relapsing-remitting course with optic nerve dysfunction and died during a relapse from respiratory insufficiency. The younger sister presented with disorientation and acute-onset limb and facial weakness. She responded well to corticosteroid therapy. Their clinical presentation, response to immunomodulatory therapy, nerve conduction studies, cerebrospinal fluid and histology supported an acquired demyelinating cause. Whole-exome sequencing identified variants in two genes not previously linked to this clinical phenotype. Serological tests for antibody-mediated demyelination were negative. Despite the undefined pathogenesis, these cases provide a platform to explore the confluence of genetic, immune and environmental factors in the context of acquired demyelination. We discuss the differential diagnosis and a diagnostic approach to such cases from the perspectives of neuroimmunology and neurogenetics.</p>","PeriodicalId":39343,"journal":{"name":"PRACTICAL NEUROLOGY","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11420716/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141499191","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Hawraman Ramadan, Ibrahim Hassan, Reham Elgafy, Souhel Najjar
{"title":"Influenza A-associated acute necrotising encephalopathy","authors":"Hawraman Ramadan, Ibrahim Hassan, Reham Elgafy, Souhel Najjar","doi":"10.1136/pn-2024-004249","DOIUrl":"https://doi.org/10.1136/pn-2024-004249","url":null,"abstract":"A previously healthy woman in her 20s was brought to the emergency department with a convulsive seizure. Over the preceding 24 hours, she had developed flu-like symptoms, including cough, headache and fever (39°C). She was started on lorazepam with a loading dose of levetiracetam and given empirical intravenous acyclovir and ceftriaxone. A CT scan of the head and blood tests for inflammatory markers, full blood count and renal and liver profile were normal. She was intubated and admitted to the medical intensive care unit due to a persistently low Glasgow coma scale score (7–8) and recurrent seizures. Cerebrospinal fluid (CSF) examination showed two white cells/µL (≤5), glucose 4.06 mmol/L (2.5–4.4) and protein 0.96 g/L (0.15–0.45); Gram stain, culture, viral PCR panel and cytology were negative. Electroencephalogram showed severe diffuse theta-delta slowing consistent with encephalopathy. A nasopharyngeal swab tested positive for influenza A, H3N2 subtype. Blood and …","PeriodicalId":39343,"journal":{"name":"PRACTICAL NEUROLOGY","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142263027","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":"Diagnostic reasoning in challenging cases.","authors":"Aaron L Berkowitz","doi":"10.1136/pn-2023-003991","DOIUrl":"10.1136/pn-2023-003991","url":null,"abstract":"<p><p>Diagnostic reasoning relies on cognitive heuristics to recognise patterns of symptoms and signs in order to arrive at a diagnosis. These rules of thumb allow us to rapidly diagnose common conditions that present in typical ways. However, they may lead us astray when common conditions present atypically or when a patient has a rare condition or multiple conditions causing their constellation of symptoms, signs, and test results, rather than having a single diagnosis to explain them all. This article describes strategies to help counteract diagnostic pitfalls, to expand diagnostic possibilities and to make diagnostic progress with complex, multielement cases.</p>","PeriodicalId":39343,"journal":{"name":"PRACTICAL NEUROLOGY","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141180945","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":"Worked up but not worked out: inconclusive cases.","authors":"Neil J Scolding","doi":"10.1136/pn-2024-004207","DOIUrl":"10.1136/pn-2024-004207","url":null,"abstract":"","PeriodicalId":39343,"journal":{"name":"PRACTICAL NEUROLOGY","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141499195","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":"When to suspect inherited metabolic diseases.","authors":"Samuel Shribman, Robin H Lachmann","doi":"10.1136/pn-2024-004113","DOIUrl":"10.1136/pn-2024-004113","url":null,"abstract":"","PeriodicalId":39343,"journal":{"name":"PRACTICAL NEUROLOGY","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141499194","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}
John S Duncan, Virginia Beech, Fahmida Amin Chowdhury, Anna Miserocchi, Andrew McEvoy
{"title":"Complicated epilepsy surgery: importance of balancing benefit and deficit.","authors":"John S Duncan, Virginia Beech, Fahmida Amin Chowdhury, Anna Miserocchi, Andrew McEvoy","doi":"10.1136/pn-2023-003901","DOIUrl":"10.1136/pn-2023-003901","url":null,"abstract":"<p><p>The risk-benefit ratio of epilepsy surgery needs careful consideration, is different for each individual and requires a careful, informed dialogue between the person concerned and their medical advisers. We illustrate this process with Virginia, who has had refractory focal epilepsy from age 1 year and a left hemiparesis. At the age of 45 years, we discussed the possibility of epilepsy surgery and went through non-invasive investigations with structural and functional MRI, tractography, scalp video-EEG telemetry, neuropsychological and neuropsychiatric evaluations. This was followed by a decision to carry out intracranial EEG to define the area of seizure onset and its relation to an area of focal cortical dysplasia, eloquent cortex and tracts. We agreed to carry out a focal resection in the knowledge that this would result in a loss of left-hand function. One year later, Virginia is seizure-free on reduced medication. We describe the steps in the process with Virginia's views.</p>","PeriodicalId":39343,"journal":{"name":"PRACTICAL NEUROLOGY","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141184385","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}
Lu Yang, R J Paul Smith, Neil J Scolding, Claire M Rice
{"title":"IgG4-related disease in the nervous system","authors":"Lu Yang, R J Paul Smith, Neil J Scolding, Claire M Rice","doi":"10.1136/pn-2020-002863","DOIUrl":"https://doi.org/10.1136/pn-2020-002863","url":null,"abstract":"IgG4-related disease (IgG4-RD) is a recently described multisystemic disorder with a spectrum of manifestations that continue to be described. Nonetheless, there are recognised distinct patterns of disease. Neurological involvement is rare, particularly in isolation, but IgG4-RD may present with orbital disease, hypophysitis or pachymeningitis. Typically, it is highly responsive to treatment. This review highlights neurological manifestations of IgG4-RD and emphasises the importance of a high index of clinical suspicion to facilitate investigation and appropriate management, avoiding irreversible tissue damage and neurological dysfunction. We present a treatment algorithm for suggested management of IgG4-RD affecting the nervous system. No data are available.","PeriodicalId":39343,"journal":{"name":"PRACTICAL NEUROLOGY","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142204936","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}