%0 Journal Article
%A Kessler, Christoph
%A Wilke, Carlo
%A Hengel, Holger
%A Rattay, Tim W
%A Maleska Maceski, Aleksandra
%A Kuhle, Jens
%A Schöls, Ludger
%A Schüle, Rebecca
%T Serum NfL, but not GFAP, differentiates primary lateral sclerosis from adrenomyeloneuropathy and hereditary spastic paraplegia type 4.
%J Amyotrophic lateral sclerosis & frontotemporal degeneration
%V 27
%N 1-2
%@ 2167-8421
%C Abingdon
%I Taylor Francis Group
%M DZNE-2026-00158
%P 110 - 117
%D 2026
%X Neurodegenerative upper motor neuron (UMN) syndromes ranging from primary lateral sclerosis (PLS) to pure and complicated types of hereditary spastic paraplegia (HSP) remain challenging to differentiate clinically, especially in the early stages of disease. As they share the hallmark of spastic paraparesis, easily accessible biomarkers are warranted to facilitate an early diagnosis.We examined serum neurofilament light chain (sNfL) and serum glial fibrillary acidic protein (sGFAP) as diagnostic biomarkers to differentiate PLS from HSP, represented by two paradigmatic subtypes: SPG4, the most common type of pure HSP, and adrenomyeloneuropathy (AMN), a common complicated form of HSP. In addition to sNfL and sGFAP raw levels, we used age-adjusted z-scores to account for age-related biomarker level increases.In our cohort of 18 PLS patients, 18 AMN patients, 25 SPG4 patients and 60 controls, sNfL z-scores were higher in PLS than in SPG4 (p < 0.001), AMN (p = 0.03), and controls (p < 0.001). Furthermore, sNfL z-scores allowed distinguishing PLS from SPG4 (AUC 0.82, 95
%K Humans
%K Male
%K Female
%K Middle Aged
%K Spastic Paraplegia, Hereditary: blood
%K Spastic Paraplegia, Hereditary: diagnosis
%K Neurofilament Proteins: blood
%K Glial Fibrillary Acidic Protein: blood
%K Adult
%K Adrenoleukodystrophy: blood
%K Adrenoleukodystrophy: diagnosis
%K Biomarkers: blood
%K Diagnosis, Differential
%K Aged
%K Young Adult
%K Cohort Studies
%K Biomarker (Other)
%K GFAP (Other)
%K HSP (Other)
%K NfL (Other)
%K PLS (Other)
%K Neurofilament Proteins (NLM Chemicals)
%K Glial Fibrillary Acidic Protein (NLM Chemicals)
%K neurofilament protein L (NLM Chemicals)
%K Biomarkers (NLM Chemicals)
%K GFAP protein, human (NLM Chemicals)
%F PUB:(DE-HGF)16
%9 Journal Article
%$ pmid:40961460
%R 10.1080/21678421.2025.2557936
%U https://pub.dzne.de/record/285033