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Hands
Published in Tor Wo Chiu, Stone’s Plastic Surgery Facts, 2018
The AION is a branch of the median nerve that arises 4–6 cm below the elbow and supplies FPL, FDP (index and middle) and PQ. There may be multiple sites of compression in the distal forearm: Gantzer’s muscle (accessory head FPL).Muscles and fascial bands at their origins, e.g. PT, FDS, FCR.Aberrant radial artery.Thrombosis of the ulnar collateral vessel, aberrant radial artery in the forearm.Trauma – fractures/penetrating injury.HNPP (hereditary neuropathy with liability to pressure palsies) should be considered in those with multiple nerve compressions (spontaneous recovery is common); AD inheritance, with deletion/mutation in PMP22 on chromosome 17 (genetic testing is available).
Issues and controversies involving the peripheral nervous system evaluation
Published in James W. Albers, Stanley Berent, Neurobehavioral Toxicology: Neurological and Neuropsychological Perspectives, 2005
James W. Albers, Stanley Berent
The experimental evidence indicating that neurotoxins predispose peripheral nerves to local damage at common entrapment sites derives from studies of diphtheric neuropathy (Hopkins & Morgan-Hughes, 1969). Additional examples exist. Hereditary neuropathy with liability to pressure palsies (HNLPP) is a disorder associated with recurrent, transient pressure palsies at common sites of nerve compression. After many years of repeated episodes of pressure-related mononeuropathies, patients who have HNLPP sometimes develop fixed impairments. On occasion, patients with HNLPP develop a generalized neuropathy (Felice, Poole, Blaivas, & Albers, 1994). This dominantly inherited disorder is associated with nerve biopsy evidence of tomacula or sausage-like swellings of myelinated nerve fibers. The role of the tomaculous swellings in the pathogenesis of conduction slowing at common sites of entrapment is uncertain. Nevertheless, these individuals have a familial disorder that predisposes them to developing isolated mononeuropathies. Therefore, the patient with unsuspected HNLPP, who is seen for the first time with a focal median mononeuropathy, presumably has a generalized abnormality that renders him or her susceptible to future progression and recurrence. Another example of a predisposition to mononeuropathy that presumably represents an underlying peripheral abnormality is the syndrome of familial recurrent Bell’s palsy and ocular motor nerve palsies (Aldrich, Beck, & Albers, 1987).
ATTRv amyloidosis Italian Registry: clinical and epidemiological data
Published in Amyloid, 2020
Massimo Russo, Laura Obici, Ilaria Bartolomei, Francesco Cappelli, Marco Luigetti, Silvia Fenu, Tiziana Cavallaro, Maria Grazia Chiappini, Chiara Gemelli, Luca Guglielmo Pradotto, Fiore Manganelli, Luca Leonardi, Filomena My, Simone Sampaolo, Chiara Briani, Luca Gentile, Claudia Stancanelli, Eleonora Di Buduo, Paolo Pacciolla, Fabrizio Salvi, Silvia Casagrande, Giulia Bisogni, Daniela Calabrese, Fiammetta Vanoli, Giuseppe Di Iorio, Giovanni Antonini, Lucio Santoro, Alessandro Mauro, Marina Grandis, Marco Di Girolamo, Gian Maria Fabrizi, Davide Pareyson, Mario Sabatelli, Federico Perfetto, Claudio Rapezzi, Giampaolo Merlini, Anna Mazzeo, Giuseppe Vita
Forty-eight patients (18.5%) received a misdiagnosis. All of them were probands, i.e., 48/163 (29.4%). The most frequent misdiagnosis reported was chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in 25 patients. Other diagnoses were hypertrophic cardiomyopathy in 6, AL amyloidosis in 5, fibromyalgia in 2, motor neuron disease in 2, coronary artery disease in 2, hypertensive cardiomyopathy in 1, aortic stenosis in 1, vascular encephalopathy in 1, combined adrenal cortical insufficiency and gonadal insufficiency in 1, diverticulitis in 1, hereditary neuropathy with liability to pressure palsies in 1. Diagnosis of lumbar spinal stenosis (LSS) was a cause of diagnostic delay in sixteen additional patients.