Abstract
Neuropathology studies of amyotrophic lateral sclerosis (ALS) and animal
models of ALS reveal a strong association between aberrant protein
accumulation and motor neuron damage and activated microglia and
astrocytes, the resident CNS innate immune cells. While the role of
neuroinflammation in the pathology of ALS is unclear imaging studies
support the idea that innate immune activation occurs early disease in
both humans and rodent models of ALS. In addition to innate immunity,
emerging studies also reveal the presence of peripheral monocytes,
macrophages, and lymphocytes in the CNS as well as at the neuromuscular
junction. To better understand the association of neuroinflammation
(innate and adaptive) with disease progression paraclinical studies
including the use of biomarkers and imaging modalities allow monitoring
of immune parameters in the disease process. Such approaches are
important for patient stratification, selection, and inclusion in
clinical trials, as well as to provide readouts of response to therapy.
Here, we discuss the different imaging modalities e.g., MRI, MRS, PET as
well as other approaches including biomarkers of inflammation in ALS,
aid the understanding of the underlying immune mechanisms associated
with motor neuron degeneration in ALS.