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Neurology, ophthalmology and psychiatry
Published in Shibley Rahman, Avinash Sharma, MRCP Part 2 Best of Five Practice Questions, 2018
Shibley Rahman, Avinash Sharma
On examination, he appears fit and well with a blood pressure of 122/78 mmHg. Cardiovascular, respiratory and abdominal examination are all normal. Neurological examination reveals normal tone and bulk but sporadic fasciculations are seen in the quadriceps, brachioradialis, biceps, triceps, calves and pectoralis muscles. No fasciculations are seen in the tongue. He is generally strong in all muscle groups tested, with 5/5 strength. Reflexes are all preserved with flexor plantar responses. Sensation is intact. Investigations are normal. What is the likely diagnosis? alcohol-related myopathyamyotrophic lateral sclerosisbenign fasciculation syndromechronic poliomyelitishypokalaemic periodic paralysis
ALSUntangled #65: glucocorticoid corticosteroids
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2023
Jill Ann Goslinga, Mark Terrelonge, Richard Bedlack, Paul Barkhaus, Benjamin Barnes, Tulio Bertorini, Mark Bromberg, Gregory Carter, Amy Chen, Jesse Crayle, Mazen Dimachkie, Leanne Jiang, Gleb Levitsky, Isaac Lund, Sarah Martin, Christopher Mcdermott, Gary Pattee, Kaitlyn Pierce, Dylan Ratner, Lenka Slachtova, Yuyao Sun, Paul Wicks
In January 2013, a former Physiology professor at Wake Forest University School of Medicine (10) published an account of his purported ALS remission with the aid of anti-inflammatory medications. At age 32, he developed fasciculations in his left index finger, with rapid spread to the rest of his body. His fasciculations increased over the subsequent year, and he developed dysarthria and sialorrhea, followed by weakness and loss of coordination. He was never formally diagnosed with ALS, but he had consulted once with a neurologist who mentioned the disease. He self-administered the fluorinated corticosteroid oral triamcinolone (“Aristocort”), with which he was performing laboratory research. Over the first week, his fasciculations reduced by 50%. He continued varied steroid treatments over the subsequent decade and noted gradual improvement in his weakness and dysarthria despite persistent fasciculations. After stopping steroid use, he lived until age 91 when he died of respiratory disease. Given he was never formally diagnosed with ALS and may have had a form of a benign fasciculation syndrome, his case is unlikely to represent true recovery from ALS due to steroid treatment.
Chitotriosidase as biomarker for early stage amyotrophic lateral sclerosis: a multicenter study
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2021
Petra Steinacker, Emily Feneberg, Steffen Halbgebauer, Simon Witzel, Federico Verde, Patrick Oeckl, Philip Van Damme, Nayana Gaur, Elizabeth Gray, Julian Grosskreutz, Claude G. Jardel, Mykyta Kachanov, Jens Kuhle, Foudil Lamari, Aleksandra Maceski, Maria Del Mar Amador, Benjamin Mayer, Claudia Morelli, Susanne Petri, Koen Poesen, Joost Raaphorst, François Salachas, Vincenzo Silani, Martin R. Turner, Marcel M. Verbeek, Alexander E. Volk, Jochen H. Weishaupt, Patrick Weydt, Albert C. Ludolph, Markus Otto
Early and late symptomatic ALS patients had similar CHIT1 levels (p = 0.0667) and both could be discriminated from Con, DCon, and oMND with slightly better results for early diagnosed ALS (Figure 2(A)). In the DCon group there were some patients with relatively high CHIT1 levels, especially a patient with radiculopathy (17660 pg/mL) and a patient with benign fasciculation syndrome (14130 pg/mL). Looking at the oMND patients we found lower CHIT1 levels in SMA/SBMA (2757 ± 1455 pg/mL) compared to PLS (5736 ± 4476 pg/mL).