The access of drugs into the central nervous system (CNS) is regulated
by the blood-brain barrier (BBB) and blood-spinal cord barrier (BSCB). A
large body of evidence supports perturbation of these barriers in
neurodegenerative diseases, including Alzheimer’s disease and
Parkinson’s disease. Modifications to the BBB and BSCB are also reported
in amyotrophic lateral sclerosis (ALS), albeit these modifications have
received less attention relative to those in other neurodegenerative
diseases. Such alterations to the BBB and BSCB have the potential to
impact on CNS exposure of drugs in ALS, modulating the effectiveness of
drugs intended to reach the brain and the toxicity of drugs that are not
intended to reach the brain. Given the clinical importance of these
phenomena, this review will summarise reported modifications to the BBB
and BSCB in ALS, discuss their impact on CNS drug exposure and suggest
further research directions so as to optimise medicine use in people
with ALS.