Key result
Muscle MRI disclosed asymmetric neurogenic muscle 'islands' alternating areas with and without fatty replacement, suggestive of Post-Poliomyelitis Syndrome.
Case Report (n=1)
Muscle MRI can be a useful imaging modality to distinguish post-poliomyelitis syndrome from other motor neuron diseases by revealing asymmetric neurogenic muscle islands.
A one-year-old male disclosed motor developmental delay, appendicular hypotonia, hyporeflexia, and weakness in the left lower limb. He started walking only at three years old. After this time, his symptoms were stable. At 31 years old, the patient started slight asymmetric and slowly progressive paraparesis and myalgia. He was referred with a suspicion of spinal muscular atrophy. Electromyography disclosed bilateral chronic lumbosacral denervation and mild acute denervation involving the right lower limb. Neuroimaging studies (brain and spinal MRI) and genetic testing (MLPA test for quantification of SMN1 and SMN2 copy numbers and hereditary neuropathy, non-5q, and other motor neuron disease panels) were normal. Muscle MRI disclosed asymmetric neurogenic muscle “islands” alternating areas with and without fatty replacement ([ Figure 1 ]),[ 1 ] suggestive of Post-Poliomyelitis Syndrome (PPS).[ 2 ] [ 3 ] Muscle MRI is useful to distinguish PPS from other motor neuron diseases.[ 1 ] [ 2 ] [ 3 ] Figure 1 Muscle MRI studies. Axial ( A-E ) and coronal ( H ) T1-weighted MRI shows asymmetric amyotrophy and marked fatty replacement involving bilateral vastus lateralis, right vastus intermedius, left biceps femoris, right vastus medialis, left tibialis anterior, and bilateral heads of gastrocnemius. Axial ( F,G ) and coronal ( I,J ) STIR MRI shows hyperintensity involving right vastus lateralis and tibialis anterior. Authors' Contributions VLB: conceptualization, data curation, formal analysis, writing – original draft preparation and supervision; HLCM, PHAF, FPS: conceptualization, formal analysis, writing – original draft preparation; IBF: conceptualization, data curation and writing – original draft preparation; PLS: BMLB, MATC, WBVRP: Conceptualization, data curation, formal analysis, writing – review & editing and supervision; PVSS, ASBO: Conceptualization, data curation and writing – review & editing. Publication History Received: 15 October 2023 Accepted: 03 December 2023 Article published online: 23 February 2024 © 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/) Thieme Revinter Publicações Ltda. Rua do Matoso 170, Rio de Janeiro, RJ, CEP 20270-135, Brazil Bibliographical Record Vinícius Lopes Braga, Heloísa Lopes Cohim Moreira, Pedro Henrique Almeida Fraiman, Filipe Pereira Sarmento, Igor Braga Farias, Paulo de Lima Serrano, Bruno de Mattos Lombardi Badia, Marco Antônio Troccoli Chieia, Wladimir Bocca Vieira de Rezende Pinto, Paulo Victor Sgobbi de Souza, Acary Souza Bulle Oliveira. Segmental areas of denervation in post-polio syndrome. Arq Neuropsiquiatr 2024; 82: s00441779507. DOI: 10.1055/s-0044-1779507
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Braga et al. (2024) conducted a case report in Post-polio syndrome (n=1). Muscle MRI was evaluated on Muscle MRI findings. Muscle MRI disclosed asymmetric neurogenic muscle 'islands' alternating areas with and without fatty replacement, suggestive of Post-Poliomyelitis Syndrome.
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