Nutritional perils, the immunoglobulin paradox, and a flickering candle - Editors' Highlights June 2025
May 16, 2025
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Delve into the complexities of newly identified diseases like IgG4-related disorders and the nuances of genetics with repeat expansion disorders. Explore the nutritional dangers linked to bariatric surgeries and the risks of restrictive vegan diets. Learn about Hoover's sign and its role in functional neurological disorders, as well as the critical review of immunoglobulin therapies in neurology. Finally, engage with the ethical challenges in caring for patients with prolonged disorders of consciousness, highlighting the need for empathy and clear communication.
IgG4-related diseases require careful histopathological examination for accurate diagnosis since serum IgG4 tests lack specificity.
Repeat expansion disorders necessitate alternative diagnostic approaches due to their elusiveness in standard whole genome sequencing.
Nutritional neuropathies post-bariatric surgery highlight critical dietary monitoring needs to prevent acute neuropathies caused by deficiencies.
Deep dives
IgG4-Related Diseases: A Complex Challenge
IgG4-related diseases are chronic, multi-system disorders that often mimic other conditions, making diagnosis challenging for neurologists. The discussion emphasizes the need for a solid tissue diagnosis, as serum IgG4 tests are not sensitive or specific enough. Histopathological examination is critical, focusing on cellular infiltration, fibrosis, and vascular obliteration. The episode highlights the importance of recognizing and diagnosing this condition, as many neurologists may look back at their patients and retrospectively identify cases that fit the profile.
Understanding Repeat Expansion Disorders
Repeat expansion disorders encompass a range of genetic conditions characterized by the abnormal repetition of certain DNA sequences. The podcast discusses how these disorders often elude detection by standard whole genome sequencing, necessitating a different diagnostic approach. Clinicians need to be aware of the effects of repeat size on clinical symptoms, as the severity of the condition often correlates with the size of the expansion. Additionally, the phenomenon of anticipation, where the disorder worsens with each generation, is a crucial consideration in genetic counseling.
Nutritional Neuropathies on the Rise
Nutritional neuropathies, particularly following bariatric surgery, have shown a resurgence as significant health concerns. The discussion revolves around specific cases where patients developed acute neuropathies due to thiamine deficiency post-surgery, emphasizing the critical need for timely diagnosis and treatment. It highlights the inadequacies in measuring thiamine levels and the resultant risks posed to patients, particularly those undergoing restrictive diets. Awareness of dietary deficiencies, especially among those with modern dietary practices, is crucial for preventing these conditions.
Hoover's Sign in Functional Neurological Disorders
Hoover's sign serves as an important diagnostic tool for assessing functional neurological disorders (FND), but its interpretation must be approached with caution. The podcast elaborates on how Hoover's sign can also indicate issues of agency, making it relevant in a broader context beyond FND, such as in conditions like Parkinson's disease. Clinicians are encouraged to utilize Hoover's sign in conjunction with a comprehensive assessment that includes patient history and interaction with family. This approach enhances understanding and management of functional symptoms in a sensitive manner.
Using Immunoglobulins in Neurology: A Practical Overview
The practical approach to using immunoglobulins in neurology emphasizes their role as immunomodulatory rather than immunosuppressive treatments. The episode discusses various conditions such as Guillain-Barré syndrome and Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) where immunoglobulins are indicated and the necessity of robust diagnostic processes. It also raises considerations about the appropriate use of immunoglobulins, highlighting the importance of treatment duration and side effects management. Cost considerations are addressed, reflecting the need for judicious and evidence-based use of this valuable resource in clinical practice.
This episode’s theme is new diseases, whether they are newly described, or old diseases back in a new form. We start off with IgG4-related disease, a chronic multisystem disorder that is still being understood. Then there is a genetics paper on repeat expansion disorders, a group of conditions often thought as disparate but with quite a few commonalities. Next along is a fascinating case following gastric sleeve bariatric surgery, highlighting the dangers of travelling for unsupervised surgeries, and raising the question, “Are you getting enough soil in your diet?” There’s also a discussion of what else Hoover’s sign might represent, and the evolution of immunoglobulin use. Closing the episode is a paper offering a masterclass on the assessment of best interests in prolonged disorder of consciousness.
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Production and editing by Brian O'Toole. Thank you for listening.
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