Extremely Painful Multifocal Acquired Predominant Axonal Sensorimotor Neuropathy of the Upper Limb |
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Authors: | Doris Lieba‐Samal MD Marieke H J van Rosmalen MD Irene M F van Balken MD Aad Verrips MD PhD Jop Mostert MD PhD Sigrid Pillen MD PhD Nens van Alfen MD PhD |
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Institution: | 1. Department of Neurology, Medical University of Vienna, Vienna, AustriaDrs Lieba‐Samal and van Eijk contributed equally to this work.;2. Department of Neurology and Clinical Neurophysiology, Radboud University Medical Center, Donders Center for Neuroscience, Nijmegen, the Netherlands;3. Department of Neurology, Canisius Wilhelmina Hospital, Nijmegen, the Netherlands;4. Department of Neurology, Rijnstate Hospital, Arnhem, the Netherlands;5. Kempenhaeghe, Sleep Medicine Center, Heeze, the Netherlands |
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Abstract: | The differential diagnosis of upper extremity mononeuritis multiplex includes neuralgic amyotrophy, vasculitic neuropathy, and Lewis‐Sumner syndrome. We describe 3 patients initially suspected of neuralgic amyotrophy, who had an extremely painful, protracted, progressive disease course, not fitting one of these established diagnoses. Nerve ultrasonography showed focal caliber changes of the roots, plexus, and limb nerves. Electromyography showed predominant multifocal axonopathy. Ongoing autoimmune neuropathy was suspected. Steroid treatment provided temporary relief, and intravenous immunoglobulin A sustained pain decrease and functional improvement. These patients appear to have extremely painful axonal inflammatory neuropathy, with a good response to immune‐modulating treatment. |
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Keywords: | brachial plexus high‐frequency imaging inflammatory neuropathy Lewis‐Sumner syndrome musculoskeletal neuralgic amyotrophy neuromuscular ultrasound neurosonology (adult) peripheral nerve treatment |
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