Abstract

AbstractBackgroundBlood brain barrier (BBB) dysfunction is suggested to have a causal role in neurodegenerative disorders. Cerebrospinal fluid (CSF) total protein (TP) is a proxy for CSF albumin quotient, which is an indicator of BBB breakdown. Normative values for CSF TP are based on young and healthy individuals, and there is a lack of understanding of how this biomarker changes with aging and neurodegeneration. The aim of this study is to examine the CSF profile in a cohort of patients with ALS‐FTD spectrum disorders and to determine relationship between the CSF profile and cognitive and motor measures in these patients.MethodCSF from participants with ALS‐FTD spectrum disorders enrolled in the NIH protocol “Investigating Complex Neurodegenerative Disorders related to Amyotrophic Lateral Sclerosis and Frontotemporal Dementia” were included (n = 63). We described the distribution of the CSF profile in these participants and compared the findings with existing literature. We then examined the relationship between CSF measures and functional rating scales and cognitive and motor measures.ResultThe average total protein was 47.77 mg/dL (SD = 38.29). The average CSF glucose was 66.00 mg/dL (SD = 12.80). There was a negative correlation (p = 0.0102) between CSF TP and the ALS Functional Rating Score. We will further investigate the relationship between the CSF profile and measures of cognitive and motor performance.ConclusionOur findings show that CSF TP is elevated in patients with ALS‐FTD spectrum disorders and higher CSF TP level correlated with greater disease severity in ALS. We will further investigate the relationship between CSF TP and other measures of cognitive and motor function.

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