{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,10]],"date-time":"2026-07-10T15:02:24Z","timestamp":1783695744727,"version":"3.55.0"},"reference-count":52,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2021,2,26]],"date-time":"2021-02-26T00:00:00Z","timestamp":1614297600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003130","name":"Fonds Wetenschappelijk Onderzoek","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003130","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004040","name":"KU Leuven","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004040","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003246","name":"Nederlandse Organisatie voor Wetenschappelijk Onderzoek","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003246","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Neurol."],"abstract":"<jats:p>Hereditary spastic paraplegia (HSP) is a neurological, genetic disorder that predominantly presents with lower limb spasticity and muscle weakness. Pediatric pure HSP types with infancy or childhood symptom onset resemble in clinical presentation to children with bilateral spastic cerebral palsy (SCP). Hence, treatment approaches in these patient groups are analogous. Altered muscle characteristics, including reduced medial gastrocnemius (MG) muscle growth and hyperreflexia have been quantified in children with SCP, using 3D-freehand ultrasound (3DfUS) and instrumented assessments of hyperreflexia, respectively. However, these muscle data have not yet been studied in children with HSP. Therefore, we aimed to explore these MG muscle characteristics in HSP and to test the hypothesis that these data differ from those of children with SCP and typically developing (TD) children. A total of 41 children were retrospectively enrolled including (1) nine children with HSP (ages of 9\u201317 years with gross motor function levels I and II), (2) 17 age-and severity-matched SCP children, and (3) 15 age-matched typically developing children (TD). Clinically, children with HSP showed significantly increased presence and severity of ankle clonus compared with SCP (<jats:italic>p<\/jats:italic> = 0.009). Compared with TD, both HSP and SCP had significantly smaller MG muscle volume normalized to body mass (<jats:italic>p<\/jats:italic> \u2264 0.001). Hyperreflexia did not significantly differ between the HSP and SCP group. In addition to the observed pathological muscle activity for both the low-velocity and the change in high-velocity and low-velocity stretches in the two groups, children with HSP tended to present higher muscle activity in response to increased stretch velocity compared with those with SCP. This exploratory study is the first to reveal MG muscle volume deficits in children with HSP. Moreover, high-velocity-dependent hyperreflexia and ankle clonus is observed in children with HSP. Instrumented impairment assessments suggested similar altered MG muscle characteristics in pure HSP type with pediatric onset compared to bilateral SCP. This finding needs to be confirmed in larger sample sizes. Hence, the study results might indicate analogous treatment approaches in these two patient groups.<\/jats:p>","DOI":"10.3389\/fneur.2021.635032","type":"journal-article","created":{"date-parts":[[2021,2,26]],"date-time":"2021-02-26T07:07:46Z","timestamp":1614323266000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":11,"title":["Muscle Characteristics in Pediatric Hereditary Spastic Paraplegia vs. Bilateral Spastic Cerebral Palsy: An Exploratory Study"],"prefix":"10.3389","volume":"12","author":[{"given":"Nathalie","family":"De Beukelaer","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lynn","family":"Bar-On","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Britta","family":"Hanssen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicky","family":"Peeters","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandra","family":"Prinsen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Els","family":"Ortibus","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaat","family":"Desloovere","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anja","family":"Van Campenhout","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2021,2,26]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"1151","DOI":"10.1016\/S0140-6736(83)92879-9","article-title":"Classification of the hereditary ataxias and paraplegias","volume":"321","author":"Harding","year":"1983","journal-title":"Lancet."},{"key":"B2","doi-asserted-by":"publisher","first-page":"1127","DOI":"10.1016\/S1474-4422(08)70258-8","article-title":"Hereditary spastic paraplegia: clinical features and pathogenetic mechanisms","volume":"7","author":"Salinas","year":"2008","journal-title":"Lancet Neurol."},{"key":"B3","doi-asserted-by":"publisher","first-page":"571","DOI":"10.1007\/s00415-004-0366-7","article-title":"Gait analysis of sporadic and hereditary spastic paraplegia","volume":"251","author":"Klebe","year":"2004","journal-title":"J Neurol."},{"key":"B4","doi-asserted-by":"publisher","first-page":"293","DOI":"10.1055\/s-0034-1386767","article-title":"Hereditary spastic paraplegia: clinical principles and genetic advances","volume":"34","author":"Fink","year":"2014","journal-title":"Semin Neurol."},{"key":"B5","doi-asserted-by":"publisher","first-page":"15082","DOI":"10.1038\/nrdp.2015.82","article-title":"Cerebral palsy","volume":"2","author":"Graham","year":"2016","journal-title":"Nat Rev"},{"key":"B6","doi-asserted-by":"publisher","first-page":"3","DOI":"10.3389\/fneur.2019.00003","article-title":"Management of hereditary spastic paraplegia: a systematic review of the literature","volume":"10","author":"Bellofatto","year":"2019","journal-title":"Front Neurol."},{"key":"B7","doi-asserted-by":"publisher","first-page":"206","DOI":"10.1111\/j.1468-1331.2006.01617.x","article-title":"Botulinum toxin injection in patients with hereditary spastic paraparesis","volume":"14","author":"Rousseaux","year":"2007","journal-title":"Eur J Neurol."},{"key":"B8","doi-asserted-by":"publisher","first-page":"794","DOI":"10.1111\/j.1469-8749.2010.03686.x","article-title":"Gross muscle morphology and structure in spastic cerebral palsy: a systematic review","volume":"52","author":"Barrett","year":"2010","journal-title":"Dev Med Child Neurol."},{"key":"B9","doi-asserted-by":"publisher","first-page":"263","DOI":"10.1111\/dmcn.14714","article-title":"Measuring skeletal muscle morphology and architecture with imaging modalities in children with cerebral palsy: a scoping review","volume":"63","author":"Williams","year":"2020","journal-title":"Dev Med Child Neurol."},{"key":"B10","doi-asserted-by":"publisher","first-page":"360","DOI":"10.1002\/mus.23326","article-title":"Childhood muscle morphology and strength: alterations over six months of growth","volume":"46","author":"Pitcher","year":"2012","journal-title":"Muscle Nerve."},{"key":"B11","doi-asserted-by":"publisher","first-page":"579","DOI":"10.3109\/09638288.2014.935492","article-title":"Does muscle size matter? 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