Publications by authors named "Ryan Keh"
A young woman with Rogers syndrome (thiamine-responsive megaloblastic anaemia, diabetes mellitus and sensorineural deafness) presented with headache, recurrent supraventricular tachycardia and features of an upper gastrointestinal bleed, 1 month after radiofrequency cardiac ablation for supraventricular tachycardia. She deteriorated rapidly after endoscopy and subsequently died. Brain imaging during the acute deterioration showed diffuse intracranial air embolism and hypoxic-ischaemic injury.
View Article and Find Full Text PDFValid, responsive blood biomarkers specific to peripheral nerve damage would improve management of peripheral nervous system (PNS) diseases. Neurofilament light chain (NfL) is sensitive for detecting axonal pathology but is not specific to PNS damage, as it is expressed throughout the PNS and CNS. Peripherin, another intermediate filament protein, is almost exclusively expressed in peripheral nerve axons.
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- POEMS syndrome is a rare and complex condition often misdiagnosed, leading to a significant delay in treatment and potential disability for patients.
- There are no consensus guidelines for managing POEMS, but this text offers a comprehensive plan for diagnosis and treatment, emphasizing the need for a multidisciplinary team of specialists.
- Treatment primarily focuses on addressing the underlying plasma cell issues, alongside supportive care for various complications, with a strong call for more clinical trials due to the current lack of robust evidence.
Rasch-built overall disability scale for POEMS syndrome (POEMS-RODS).
J Peripher Nerv Syst
December 2022
Article Synopsis
- Patient-reported outcome measures can effectively gauge disease severity and are meaningful for patients' lives, particularly in complex disorders like POEMS, which affects multiple body systems and causes disabling neuropathy.
- The research introduces a new scale, the Rasch-built Overall Disability Scale (POEMS-RODS), specifically designed for POEMS, created from a preliminary questionnaire which underwent thorough testing and refinement.
- The final version of the POEMS-RODS includes 23 relevant items that show reliable results, but further international studies are needed to verify its effectiveness and adaptability in different populations.
POEMS syndrome is a rareparaneoplastic disorder driven by an underlying low level plasma cell dyscrasiaand associated with elevated serum vascular endothelial growth factor (VEGF). Dueto its rarity, there are no internationally agreed standards of care, with verylimited data to guide management in the relapse setting. Agents used in myelomaare rational choices and have been employed.
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- In clinical neurology, effective serological biomarkers for assessing the peripheral nervous system are limited, leading to reliance on alternative diagnostic methods.
- Accurate understanding and application of these biomarkers are crucial to prevent misdiagnosis and ensure timely treatment adjustments.
- The text explores the benefits and drawbacks of existing biomarkers and suggests potential advancements for future use.
Article Synopsis
- - The study aimed to investigate the potential link between COVID-19 vaccination and Guillain-Barré syndrome (GBS) using data from the NHS in England and UK hospitals.
- - A total of 996 GBS cases were reported, with a notable increase in March-April 2021; 198 of these cases occurred within six weeks of receiving the first dose of the ChAdOx1 nCoV-19 vaccine (AstraZeneca).
- - The study found a slight increase in GBS cases associated with the first dose of the AstraZeneca vaccine, but no excess risk was observed for second doses or for the Pfizer and Moderna vaccines.
Article Synopsis
- This study analyzes the effectiveness of immunoglobulin (Ig) treatment for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy with conduction block (MMNCB) over a period from 2009 to 2020, focusing on monitoring patient outcomes through grip strength and disability scales.
- Results indicate minimal group changes in outcome measures over time, but significant intra-individual variation is noted, with a considerable percentage of patients showing changes exceeding the minimal clinically important differences (MCID).
- The study identifies trends of improvement and deterioration among patients, particularly linking older age with deterioration in CIDP, and highlights the complexity of interpreting outcome measures to guide
IgG pan-neurofascin antibodies identify a severe yet treatable neuropathy with a high mortality.
J Neurol Neurosurg Psychiatry
October 2021
Objectives: We aimed to define the clinical and serological characteristics of pan-neurofascin antibody-positive patients.
Methods: We tested serum from patients with suspected immune-mediated neuropathies for antibodies directed against nodal/paranodal protein antigens using a live cell-based assay and solid-phase platform. The clinical and serological characteristics of antibody-positive and seronegative patients were then compared.
Article Synopsis
- The study focuses on switching patients with inflammatory neuropathies from intravenous immunoglobulin (IVIg) to subcutaneous immunoglobulin (SCIg), assessing the effectiveness of dosing strategies.* -
- A total of 44 out of 56 patients maintained their SCIg treatment for an average of 3.3 years with stable clinical outcomes; however, some needed dose adjustments over time for better management.* -
- The findings suggest that adjusting doses based on patient response is a viable method for maintaining effective SCIg therapy, although some patients reverted back to IVIg due to relapses or personal choice.*
Article Synopsis
- This study investigated the potential link between COVID-19 and Guillain-Barré syndrome (GBS) by analyzing GBS cases in the UK before and during the COVID-19 pandemic.
- The researchers found that GBS incidence decreased during the pandemic and did not show a correlation with COVID-19 infection rates across different regions.
- Although some GBS cases were associated with COVID-19, there were no significant differences in clinical features between COVID-19 positive and negative cases, suggesting no strong evidence linking the virus as a cause of GBS.
Therapeutic plasma exchange (TPE) involves the extracorporeal separation of plasma from the cellular components of blood with replacement fluid, such as human albumin or fresh frozen plasma. A number of studies across the world revealed that more than one third of TPE procedures were performed for neurological disorders. Myasthenia gravis (MG), Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) were the most frequently cited indications for TPE, followed by multiple sclerosis (MS).
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