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024 7 _ |a 10.1080/21678421.2025.2557936
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037 _ _ |a DZNE-2026-00158
041 _ _ |a English
082 _ _ |a 610
100 1 _ |a Kessler, Christoph
|b 0
245 _ _ |a Serum NfL, but not GFAP, differentiates primary lateral sclerosis from adrenomyeloneuropathy and hereditary spastic paraplegia type 4.
260 _ _ |a Abingdon
|c 2026
|b Taylor Francis Group
336 7 _ |a article
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336 7 _ |a ARTICLE
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336 7 _ |a Journal Article
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520 _ _ |a 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% CI 0.67-0.98) and-slightly less accurate-from AMN (AUC 0.77, 95% CI 0.60-0.95). sGFAP z-scores did not differ significantly between groups.Our study suggests that serum NfL, but not GFAP, is a potential diagnostic biomarker in degenerative UMN diseases and may help to differentiate PLS from pure and complicated forms of HSP. Our results indicate that axonal degeneration-the source of NfL release-is predominant over astrocytic pathology-the source of GFAP release-in PLS, AMN, and SPG4.
536 _ _ |a 353 - Clinical and Health Care Research (POF4-353)
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588 _ _ |a Dataset connected to CrossRef, PubMed, , Journals: pub.dzne.de
650 _ 7 |a Biomarker
|2 Other
650 _ 7 |a GFAP
|2 Other
650 _ 7 |a HSP
|2 Other
650 _ 7 |a NfL
|2 Other
650 _ 7 |a PLS
|2 Other
650 _ 7 |a Neurofilament Proteins
|2 NLM Chemicals
650 _ 7 |a Glial Fibrillary Acidic Protein
|2 NLM Chemicals
650 _ 7 |a neurofilament protein L
|2 NLM Chemicals
650 _ 7 |a Biomarkers
|2 NLM Chemicals
650 _ 7 |a GFAP protein, human
|2 NLM Chemicals
650 _ 2 |a Humans
|2 MeSH
650 _ 2 |a Male
|2 MeSH
650 _ 2 |a Female
|2 MeSH
650 _ 2 |a Middle Aged
|2 MeSH
650 _ 2 |a Spastic Paraplegia, Hereditary: blood
|2 MeSH
650 _ 2 |a Spastic Paraplegia, Hereditary: diagnosis
|2 MeSH
650 _ 2 |a Neurofilament Proteins: blood
|2 MeSH
650 _ 2 |a Glial Fibrillary Acidic Protein: blood
|2 MeSH
650 _ 2 |a Adult
|2 MeSH
650 _ 2 |a Adrenoleukodystrophy: blood
|2 MeSH
650 _ 2 |a Adrenoleukodystrophy: diagnosis
|2 MeSH
650 _ 2 |a Biomarkers: blood
|2 MeSH
650 _ 2 |a Diagnosis, Differential
|2 MeSH
650 _ 2 |a Aged
|2 MeSH
650 _ 2 |a Young Adult
|2 MeSH
650 _ 2 |a Cohort Studies
|2 MeSH
700 1 _ |a Wilke, Carlo
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700 1 _ |a Hengel, Holger
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700 1 _ |a Rattay, Tim W
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700 1 _ |a Maleska Maceski, Aleksandra
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700 1 _ |a Kuhle, Jens
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700 1 _ |a Schöls, Ludger
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700 1 _ |a Schüle, Rebecca
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773 _ _ |a 10.1080/21678421.2025.2557936
|g Vol. 27, no. 1-2, p. 110 - 117
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|t Amyotrophic lateral sclerosis & frontotemporal degeneration
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