Abstract Details

Title Correlation of cognitive impairment and KING’s and MiTos staging in ALS: a cross-sectional population-based study.

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) S54 - Motor Neuron Disease (4:36 PM-4:47 PM)

Poster/Presentation
Number 007

Objective

To assess the relationship between cognitive impairment and clinical staging in ALS using a population-based cohort.

Background

It remains unclear whether the cognitive status of ALS patients worsens with disease progression. Previous longitudinal studies suggested that cognitive function declined in patients cognitively impaired at baseline while unimpaired patients tended to remain cognitively intact. However, in a recent cross-sectional study the frequency of ALS-specific cognitive deficits and behavioral impairment increased with the increasing in King’s Clinical Staging System.

Design/Methods A total of 785 ALS patients incident between 2007 and 2015 in Piedmont region and detected through the Piemonte and Valle d’Aosta register for ALS (PARALS) have been included in the study. Cognitive status was assessed in blind by two ALS-expert neuropsychologists and classified according to the novel diagnostic criteria (ALS with frontotemporal dementia, ALS-FTD, ALS with combined cognitive and behavioural impairment, ALScbi, ALS with cognitive impairment, ALSci, ALS with behavioural impairment, ALSbi, and cognitively normal ALS, ALS-CN). Clinical staging was assessed using both the King’s and the MiTos stagings.

Results According to Kings’s staging, the frequency of ALS-FTD progressively increased from 16.9% in stage 2 to 44.4% in stage 4. Similarly, according to MiToS staging the frequency of ALS-FTD significantly increased from 20.7% in stage 0 to 47.6% in stage 2. The frequency of ALSci, ALSbi and ALScbi increased from King’s stage 0 to King’s stage 3 and decreased thereafter. Overall, in more severe stages of both staging systems about 60% of patients showed some degrees of cognitive impairment (King’s stage 4, 61.1%, MiToS stage 2, 61.9%).

Conclusions Although limited by the cross-sectional design, this large population-based study showed that the frequency of ALS-FTD increases with increasing in clinical stage, suggesting that at least in a part of ALS patients cognitive impairment worsens during the course of the disease.

Authors/Disclosures
Rosario Vasta, MD (University of Turin, Department of Neurosciences) PRESENTER	Dr. Vasta has nothing to disclose.
	No disclosure on file
	No disclosure on file
Andrea Calvo, MD, PhD, FAAN (Dept. of Neuroscience, University of Turin)	Dr. Calvo has nothing to disclose.
Cristina Moglia (University of Torino)	Cristina Moglia has nothing to disclose.
Antonio Canosa	Antonio Canosa has nothing to disclose.
Umberto Manera, MD (Department of Neuroscience "Rita Levi Montalcini" - University of Torino)	Dr. Manera has nothing to disclose.
	No disclosure on file
Gabriele Mora, MD	Dr. Mora has nothing to disclose.
	No disclosure on file
	No disclosure on file
	No disclosure on file
Letizia Mazzini	No disclosure on file
Barbara Iazzolino	Barbara Iazzolino has nothing to disclose.
Adriano Chio, MD, FAAN (Dept. of Neuroscience, University of Turin)	Dr. Chio has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Cytokinetics. Dr. Chio has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Mitsubishi. Dr. Chio has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Chio has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Corcept.

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