Fast Progression in Amyotrophic Lateral Sclerosis Is Associated With Greater TDP-43 Burden in Spinal Cord

Abstract Upper and lower motor neuron pathologies are critical to the autopsy diagnosis of amyotrophic lateral sclerosis (ALS). Further investigation is needed to determine how the relative burden of these pathologies affects the disease course. We performed a blinded, retrospective study of 38 ALS...

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Bibliographic Details
Published in:Journal of neuropathology and experimental neurology 2021-08, Vol.80 (8), p.754-763
Main Authors: Cathcart, Sahara J, Appel, Stanley H, Peterson, Leif E, Greene, Ericka P, Powell, Suzanne Z, Arumanayagam, Anithachristy S, Rivera, Andreana L, Cykowski, Matthew D
Format: Article
Language:English
Subjects:
Adult
Aged
Amyotrophic lateral sclerosis
Amyotrophic Lateral Sclerosis - metabolism
Amyotrophic Lateral Sclerosis - pathology
Autopsies
Binding proteins
Brain research
C9orf72 Protein - metabolism
Development and progression
Disease Progression
DNA binding proteins
DNA-Binding Proteins - metabolism
Female
Health aspects
Hospitals
Humans
Male
Medical research
Medicine, Experimental
Middle Aged
Motor Cortex - metabolism
Motor Cortex - pathology
Neurons
Original
Pathology
Patients
Protein binding
Proteins
Spinal cord
Spinal Cord - metabolism
Spinal Cord - pathology
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Summary:Abstract Upper and lower motor neuron pathologies are critical to the autopsy diagnosis of amyotrophic lateral sclerosis (ALS). Further investigation is needed to determine how the relative burden of these pathologies affects the disease course. We performed a blinded, retrospective study of 38 ALS patients, examining the association between pathologic measures in motor cortex, hypoglossal nucleus, and lumbar cord with clinical data, including progression rate and disease duration, site of symptom onset, and upper and lower motor neuron signs. The most critical finding in our study was that TAR DNA-binding protein 43 kDa (TDP-43) pathologic burden in lumbar cord and hypoglossal nucleus was significantly associated with a faster progression rate with reduced survival (p 
ISSN:0022-3069
1554-6578
DOI:10.1093/jnen/nlab061