Journal
NEUROLOGY
Volume 68, Issue 19, Pages 1571-1575Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1212/01.wnl.0000260965.20021.47
Keywords
-
Categories
Funding
- NINDS NIH HHS [R21 NS051738, NS051738] Funding Source: Medline
Ask authors/readers for more resources
Objective: To localize and analyze the anatomic distribution of upper motor neuron (UMN) and lower motor neuron (LMN) loss in patients with ALS early in their disease when motor manifestations were still relatively focal using clinical examination signs. Methods: We reviewed records of 100 patients with ALS who were evaluated when the diagnosis was first established or suspected. From the patient history, we ascertained the body region of first symptoms and the time course. From the physical examination, we separately graded severity of UMN and LMN signs in each body region, indexed these to the body region of first symptoms, and sorted and analyzed the data. Results: Motor manifestations began in one body region in 98% of patients. UMN and LMN signs were both maximal in these same body regions, but they were independent of each other in severity and their outward distribution, which conformed to neuronal anatomy. The outward distribution of both UMN and LMN signs seemed more directed to caudal body regions than to rostral ones. Conclusions: Motor neuron degeneration in ALS is a focal process at both upper and lower motor neuron levels of the motor system. At each level, it begins corresponding to the same peripheral body region and then advances contiguously and separately to summate over time.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available