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Regional Spreading Pattern Is Associated with Clinical Phenotype in Amyotrophic Lateral Sclerosis by Alessio Maranzano; Federico Verde; Eleonora Colombo; Barbara Poletti; Alberto Doretti; Ruggero Bonetti; Delia Gagliardi; Megi Meneri; Luca Maderna; Stefano Messina; Stefania Corti; Claudia Morelli; Vincenzo Silani; Nicola Ticozzi is a Neuroscience article available to read on EtoBox.

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## Abstract Increasing evidence shows that disease spreading in amyotrophic lateral sclerosis (ALS) follows a preferential pattern with more frequent involvement of contiguous regions from the site of symptom onset. The aim of our study was to assess if: (i) the burden of upper (UMN) and lower motor neuron (LMN) involvement influences directionality of disease spreading; (ii) specific patterns of disease progression are associated with motor and neuropsychological features of different ALS subtypes (classic, bulbar, primary lateral sclerosis, UMN-predominant, progressive muscular atrophy, flail arm, flail leg); and (iii) specific clinical features may help identify ALS subtypes, which remain localized to the site of onset for a prolonged time (regionally entrenching ALS). A single-centre, retrospective cohort of 913 Italian ALS patients was evaluated to assess correlations between directionality of the disease process after symptom onset and motor/neuropsychological phenotype. All patients underwent an extensive evaluation including the following clinical scales: Penn Upper Motor Neuron Score (PUMNS), MRC Scale for Muscle Strength and the Edinburgh Cognitive and Behavioural ALS Scr

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Author

Alessio Maranzano; Federico Verde; Eleonora Colombo; Barbara Poletti; Alberto Doretti; Ruggero Bonetti; Delia Gagliardi; Megi Meneri; Luca Maderna; Stefano Messina; Stefania Corti; Claudia Morelli; Vincenzo Silani; Nicola Ticozzi

Publisher

Oxford University Press (OUP)

Published

2023

Language

EN

Field

Neuroscience (Life Sciences)