{"doi":"10.1002/mds.70059","title":"Temporal Muscle Thickness Correlates with Functional Decline in Huntington's Disease","abstract":"Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by a cytosine-adenine-guanine (CAG) trinucleotide expansion on chromosome 4. Clinical features include chorea or parkinsonism, psychiatric symptoms, and progressive cognitive decline. As the disease progresses, patients develop functional impairment, weight loss, and dependence in daily life. Earlier symptom onset is associated with longer CAG repeat expansions. The CAG-age product (CAP) score provides an estimate of the predicted onset of motor symptoms and cumulative disease burden.1 Clinical staging commonly relies on the total functional capacity (TFC) of the Unified Huntington's Disease Rating Scale (UHDRS),2 capturing the patients’ ability to perform essential everyday activities. Beyond central nervous system pathology, HD affects peripheral tissues such as skeletal muscle, contributing to weight loss even in early disease stages.3 Reduced muscle mass is associated with morbidity, loss of independence, and poorer quality of life.4 Despite its clinical relevance, muscle status is rarely assessed in HD, largely because of the limitations of conventional measurement methods. Temporal muscle thickness (TMT), obtainable from standard cranial magnetic resonance imaging (MRI), has recently emerged as a reliable surrogate for skeletal muscle mass.4 Reduced skeletal muscle mass has been associated with longer hospital stays, recurrent hospitalizations, and increased risk of complications.5 This study evaluated TMT on routine cranial MRI in patients with HD and examined associations with clinical disease parameters. Data were derived from an HD patient cohort who were enrolled in a longitudinal observational study at the Medical University of Innsbruck.6 Ethical approval was obtained, and all participants gave informed consent. Clinical assessments included motor symptoms (total motor score of the UHDRS [UHDRS-TMS]), functionality (UHDRS-TFC), cognition (symbol digit modalities test [SDMT]), body weight, and body mass index (BMI). CAP scores were calculated.1 Inclusion required the availability of a 3-Tesla cranial MRI acquired after clinical testing. TMT was measured manually on axial noncontrast T1-weighted three-dimensional magnetization-prepared rapid acquisition with gradient echo sequences by an experienced neuroimaging rater, following established protocols.3 The mean of bilateral measurements was used for analysis. Statistical analyses were performed with SPSS 30. Fifty-five patients with HD in clinical stages 1 to 3 based on functional capacities and motor symptoms were included. Demographic characteristics were comparable, with no significant differences in age or CAG repeat length. Mean TMT was 8.7 mm (±2.7) in males and 7.8 mm (±2.2) in females (P = 0.179). TMT was associated with greater disease burden as indicated by higher clinical stages (Table 1). Reduced TMT correlated with TMS, TFC, CAP score, body weight, BMI, and SDMT. This study demonstrates progressive TMT decline across clinical stages of HD, reflecting both neurological deterioration and systemic musculoskeletal involvement. The findings support TMT as a potential disease progression marker and emphasize musculoskeletal decline as an early feature of HD. TMT measurement may complement clinical and nutritional assessments, enabling earlier detection and monitoring of physical and functional decline. Incorporating TMT assessment offers a noninvasive method to monitor systemic changes in HD, even in patients with relatively preserved neurological function. (1) Statistical analysis: A. Design, B. Execution, C. Analysis; (2) Manuscript: A. Writing, B. Editing of final version of the manuscript. G.H.: 1A, 1B, 1C, 2A, 2B C.C.: 1A, 1B, 2A, 2B M.P.: 2B K.S.: 2B F.C.: 2B B.W.: 2B F.K.: 2B P.M.: 2B A.D.: 2B A.G.: 2B S.L.: 2B K.S.: 2B B.H.: 1A, 1B, 1C, 2A, 2B We thank all participants who voluntarily took part in this study. B.H., A.D., and K.S. are members of the European Reference ","journal":"Movement Disorders","year":2025,"id":575544,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":0,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9557,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2025-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":1483802,"name":"Clancy Cerejo","orcid":null,"position":1,"is_corresponding":false},{"id":1361631,"name":"Marina Peball","orcid":"0000-0002-0791-7300","position":2,"is_corresponding":false},{"id":1483377,"name":"Katarína Schwarzová","orcid":"0009-0006-0350-7861","position":3,"is_corresponding":false},{"id":1483378,"name":"Federico Carbone","orcid":"0000-0002-6909-0150","position":4,"is_corresponding":false},{"id":1483803,"name":"Bernadette Wimmer","orcid":null,"position":5,"is_corresponding":false},{"id":348670,"name":"Florian Krismer","orcid":"0000-0002-4493-5073","position":6,"is_corresponding":false},{"id":1337024,"name":"Philipp Mahlknecht","orcid":"0000-0003-0671-0516","position":7,"is_corresponding":false},{"id":1483379,"name":"Atbin Djamshidian","orcid":"0000-0001-7174-6000","position":8,"is_corresponding":false},{"id":1483380,"name":"Astrid Grams","orcid":"0000-0003-2304-3946","position":9,"is_corresponding":false},{"id":1483804,"name":"Samuel Labrecque","orcid":null,"position":10,"is_corresponding":false},{"id":348662,"name":"Klaus Seppi","orcid":"0000-0001-6503-1455","position":11,"is_corresponding":false},{"id":1361632,"name":"Beatrice Heim","orcid":"0000-0002-6749-3044","position":12,"is_corresponding":false},{"id":1483801,"name":"Greta Hemicker","orcid":null,"position":0,"is_corresponding":true}],"reference_count":6,"raw_metadata":null,"created_at":"2026-07-19T02:57:48.486077Z","pmid":"40977449","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}