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

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Title: Relationship between cerebrospinal fluid/serum albumin quotient and phenotype in amyotrophic lateral sclerosis: a retrospective study on 328 patients.
Authors: Verde, Federico (AUTHOR), Ferrari, Ivan (AUTHOR), Maranzano, Alessio (AUTHOR), Ciusani, Emilio (AUTHOR), Torre, Silvia (AUTHOR), Milone, Ilaria (AUTHOR), Colombo, Eleonora (AUTHOR), Doretti, Alberto (AUTHOR), Peverelli, Silvia (AUTHOR), Ratti, Antonia (AUTHOR), Maderna, Luca (AUTHOR), Poletti, Barbara (AUTHOR), Messina, Stefano (AUTHOR), Morelli, Claudia (AUTHOR), Silani, Vincenzo (AUTHOR), Ticozzi, Nicola (AUTHOR)
Source: Neurological Sciences. May2023, Vol. 44 Issue 5, p1679-1685. 7p. 1 Chart.
Subjects: Amyotrophic lateral sclerosis, Cerebrospinal fluid, Serum albumin, Motor neurons, Phenotypes
Abstract: 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. [ABSTRACT FROM AUTHOR]
Copyright of Neurological Sciences is the property of Springer Nature and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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  Data: Relationship between cerebrospinal fluid/serum albumin quotient and phenotype in amyotrophic lateral sclerosis: a retrospective study on 328 patients.
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  Data: <searchLink fieldCode="JN" term="%22Neurological+Sciences%22">Neurological Sciences</searchLink>. May2023, Vol. 44 Issue 5, p1679-1685. 7p. 1 Chart.
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  Data: <searchLink fieldCode="DE" term="%22Amyotrophic+lateral+sclerosis%22">Amyotrophic lateral sclerosis</searchLink><br /><searchLink fieldCode="DE" term="%22Cerebrospinal+fluid%22">Cerebrospinal fluid</searchLink><br /><searchLink fieldCode="DE" term="%22Serum+albumin%22">Serum albumin</searchLink><br /><searchLink fieldCode="DE" term="%22Motor+neurons%22">Motor neurons</searchLink><br /><searchLink fieldCode="DE" term="%22Phenotypes%22">Phenotypes</searchLink>
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  Data: 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. [ABSTRACT FROM AUTHOR]
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  Data: <i>Copyright of Neurological Sciences is the property of Springer Nature and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.)
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        Value: 10.1007/s10072-023-06604-3
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      – Code: eng
        Text: English
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        PageCount: 7
        StartPage: 1679
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      – SubjectFull: Amyotrophic lateral sclerosis
        Type: general
      – SubjectFull: Cerebrospinal fluid
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      – SubjectFull: Serum albumin
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      – SubjectFull: Motor neurons
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      – TitleFull: Relationship between cerebrospinal fluid/serum albumin quotient and phenotype in amyotrophic lateral sclerosis: a retrospective study on 328 patients.
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              Text: May2023
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