Relationship between cerebrospinal fluid/serum albumin quotient and phenotype in amyotrophic lateral sclerosis: a retrospective study on 328 patients.

Autor: Verde F; IRCCS Istituto Auxologico Italiano, Department of Neurology and Laboratory of Neuroscience, Piazzale Brescia, 20, 20149, Milan, Italy. f.verde@auxologico.it.; Department of Pathophysiology and Transplantation, 'Dino Ferrari' Center, Università degli Studi di Milano, Milan, Italy. f.verde@auxologico.it., Ferrari I; IRCCS Istituto Auxologico Italiano, Department of Neurology and Laboratory of Neuroscience, Piazzale Brescia, 20, 20149, Milan, Italy.; School of Medicine, Università degli Studi di Milano, Milan, Italy., Maranzano A; IRCCS Istituto Auxologico Italiano, Department of Neurology and Laboratory of Neuroscience, Piazzale Brescia, 20, 20149, Milan, Italy.; Neurology Residency Program, Università degli Studi di Milano, Milan, Italy., Ciusani E; Laboratory of Neurological Biochemistry and Neuropharmacology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy., Torre S; IRCCS Istituto Auxologico Italiano, Department of Neurology and Laboratory of Neuroscience, Piazzale Brescia, 20, 20149, Milan, Italy., Milone I; IRCCS Istituto Auxologico Italiano, Department of Neurology and Laboratory of Neuroscience, Piazzale Brescia, 20, 20149, Milan, Italy., Colombo E; IRCCS Istituto Auxologico Italiano, Department of Neurology and Laboratory of Neuroscience, Piazzale Brescia, 20, 20149, Milan, Italy., Doretti A; IRCCS Istituto Auxologico Italiano, Department of Neurology and Laboratory of Neuroscience, Piazzale Brescia, 20, 20149, Milan, Italy., Peverelli S; IRCCS Istituto Auxologico Italiano, Department of Neurology and Laboratory of Neuroscience, Piazzale Brescia, 20, 20149, Milan, Italy., Ratti A; IRCCS Istituto Auxologico Italiano, Department of Neurology and Laboratory of Neuroscience, Piazzale Brescia, 20, 20149, Milan, Italy.; Department of Medical Biotechnologies and Translational Medicine, Università degli Studi di Milano, Milan, Italy., Maderna L; IRCCS Istituto Auxologico Italiano, Department of Neurology and Laboratory of Neuroscience, Piazzale Brescia, 20, 20149, Milan, Italy., Poletti B; IRCCS Istituto Auxologico Italiano, Department of Neurology and Laboratory of Neuroscience, Piazzale Brescia, 20, 20149, Milan, Italy., Messina S; IRCCS Istituto Auxologico Italiano, Department of Neurology and Laboratory of Neuroscience, Piazzale Brescia, 20, 20149, Milan, Italy., Morelli C; IRCCS Istituto Auxologico Italiano, Department of Neurology and Laboratory of Neuroscience, Piazzale Brescia, 20, 20149, Milan, Italy., Silani V; IRCCS Istituto Auxologico Italiano, Department of Neurology and Laboratory of Neuroscience, Piazzale Brescia, 20, 20149, Milan, Italy.; Department of Pathophysiology and Transplantation, 'Dino Ferrari' Center, Università degli Studi di Milano, Milan, Italy., Ticozzi N; IRCCS Istituto Auxologico Italiano, Department of Neurology and Laboratory of Neuroscience, Piazzale Brescia, 20, 20149, Milan, Italy.; Department of Pathophysiology and Transplantation, 'Dino Ferrari' Center, Università degli Studi di Milano, Milan, Italy.
Jazyk: angličtina
Zdroj: Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology [Neurol Sci] 2023 May; Vol. 44 (5), pp. 1679-1685. Date of Electronic Publication: 2023 Jan 17.
DOI: 10.1007/s10072-023-06604-3
Abstrakt: Background: We analysed the relationship between cerebrospinal fluid (CSF)/serum albumin quotient (Q-Alb) and phenotype in a large cohort of patients with amyotrophic lateral sclerosis (ALS).
Methods: Three hundred twenty-eight single-centre consecutive patients with ALS were evaluated for Q-Alb, basic epidemiological and clinical data, motor phenotype, cognitive/behavioural impairment, clinical staging, clinical and neurophysiological indexes of upper (UMN) and lower motor neuron (LMN) dysfunction, and presence of ALS gene mutations.
Results: Q-Alb did not correlate with age but was independently associated with sex, with male patients having higher levels than female ones; the site of onset was not independently associated with Q-Alb. Q-Alb was not associated with motor phenotype, cognitive/behavioural impairment, disease stage, progression rate, survival, or genetic mutations. Among measures of UMN and LMN dysfunction, Q-Alb only had a weak positive correlation with an electromyography-based index of active limb denervation.
Conclusion: Previous work has documented increased Q-Alb in ALS compared to unaffected individuals. This, together with the absence of associations with nearly all ALS phenotypic features in our cohort, suggests dysfunction of the blood-CSF barrier as a shared, phenotype-independent element in ALS pathophysiology. However, correlation with the active denervation index could point to barrier dysfunction as a local driver of LMN degeneration.
(© 2023. Fondazione Società Italiana di Neurologia.)
Databáze: MEDLINE
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