Abstract Details

Title Pseudobulbar Affect (PBA) in an Incident ALS Cohort: Results from the Apulia Registry

Topic Anterior Horn

Presentation(s) P07 - (-)

Poster/Presentation
Number 073

Objective

Background BACKGROUND: PBA is characterized by involuntary and uncontrollable laughing and/or crying episodes, occurring in several neurological diseases. PBA may impair patients' quality of life, but is under-recognized and undertreated. No data are available from incident ALS cohorts.

Design/Methods DESIGN/METHODS: Incident ALS cases, diagnosed in 2011 and 2012, according to El Escorial criteria were enrolled from a prospective population based registry in Apulia, Southern Italy. Neurological status at entry was assessed using a standard neurological examination and the revised ALS Functional Rating Scale (ALSFRS-r). The Center for Neurologic Study-Lability Scale (CNS-LS), a 7-item self-administered questionnaire, was used to evaluate the presence and the severity of PBA. Total scores range from 7 to 35. A score ? 13 was used to identify PBA presence.

Results RESULTS: Eighty-one sporadic incident ALS cases were enrolled. Median disease duration was 17 months (range 2.4-143.5), median onset-diagnosis interval (ODI) 12 months (range 2-131), median ALSFRS-r at baseline 36/48 (range 3-47) and median ALSFRS-r bulbar sub-score 9/12 (range 0-12). Neurological examination revealed presence of PBA in 19/81 patients (23%). Pathological CNS-LS score was found in 32/81 patients (39.5%). Median total CNS-LS score was 9/35 (range 7-29). There was a negative correlation between total CNS-LS score and disease duration (p=0.009; rs:-0.29), ODI (p=0.002; rs:-0.34) and ALSFRS-r bulbar sub-score (p=0.022; rs:-0.28). Pathological CNS-LS was associated with signs of upper motor neuron (UMN) involvement and presence of PBA at the neurological examination (p<0.0001).

Conclusions CONCLUSIONS: This study shows that PBA may be present in a considerable percentage of incident ALS cases at the beginning of the disease and may be related to a more severe course and to UMN involvement.

Authors/Disclosures
PRESENTER	No disclosure on file
Helmut Butzkueven, MD, MBBS	Dr. Butzkueven has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Oxford Health Policy Forum. The institution of Dr. Butzkueven has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Biogen. The institution of Dr. Butzkueven has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Merck. The institution of Dr. Butzkueven has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Novartis. The institution of Dr. Butzkueven has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Roche. Dr. Butzkueven has received personal compensation in the range of $10,000-$49,999 for serving as an officer or member of the Board of Directors for MSBase . The institution of Dr. Butzkueven has received research support from NHMRC. The institution of Dr. Butzkueven has received research support from Biogen. The institution of Dr. Butzkueven has received research support from Roche. The institution of Dr. Butzkueven has received research support from Novartis.
	No disclosure on file
	No disclosure on file
	No disclosure on file
Rosa Capozzo	No disclosure on file
	No disclosure on file
Eustachio D'Errico	No disclosure on file
Isabella L. Simone (Insitute of Neurologist, Univerisity of Bari Italy)	No disclosure on file
Giancarlo Logroscino, MD, PhD, FAAN (University of Bari)	Dr. Logroscino has nothing to disclose.

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