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Background: The ELQ was developed to provide detailed information for experimental investigation of nature and extent of emotional lability in the ALS population.

 

Construct measured: This questionnaire assesses frequency, duration of episodes, relation to external events, degree of voluntary control, congruence with mood state and subsequent distress of patients with pathological laughter and crying and emotional lability.

 

Generic vs. disease specific: Disease specific

 

Means of administration: Administered as a structured interview

 

Intended respondent: Self-report by patient, proxy completion by caregiver in parallel version.

 

# of items: 33 in total, (includes 3 screening questions)

 

# of subscales and names of sub-scales: Laughing, Crying and Smiling

 

# of items per sub-scale: 11 items

 

Strengths: Since episodes of pathological laughing and crying may occur with varying frequency this measure asks about the previous four weeks. Includes a scale for abnormal smiling. A carer/proxy version is available to allow for the fact that patients and carers may disagree about the frequency with which such episodes occur. Has also been validated in an Italian version.

 

Weaknesses: No validation against clinical diagnosis.

Feasibility: Includes 3 screening questions which if answered negatively terminates the interview

 

Reliability: Cronbach's alpha for the Laughter scale= 0.8, Crying Scale =0.6. Test-retest reliability (4-6 week interval): intraclass correlation coefficients of 0.75 and 0.72 for Laughter and Crying Scales (Newsom-Davis 2004, Ph.D. Thesis)

 

Validity: Significant correlations between Crying and Total, Laughter and Total, Smiling and Total and Laughter and Smiling scores was significant at least p<0.05 (Newsom-Davis et al., 1999). Statistically significant between self and independent ratings for Total scores, Crying scores and Laughter scores at least p<0.05 and also for Crying (self-rated) and Total (Independent-rater) (Newson-Davis et al., 1999). Correlations between self- and independent rater versions for Total and Laughter scores significant at p<0.001(Palmieri et al., 2009). Some evidence of ability to differentiate between ALS subtypes in comparison to controls (Wicks et al., 2009).

 

Sensitivity to Change: No evidence of a significant change in scores over a six month period in ALS patients compared with controls (Abrahams et al., 2005).

 

Relationships to other variables: Self-rated Crying, Laughter and Total scores all correlated with bulbar impairment (p<0.01) (Newson-Davis et al., 1999), Total scores correlated with Total ALSFRS-R scores (p<.01); ALSFRS-Language (p<0.001); and ALSFRS-Swallowing (p<0.0001) scores. Laughing scores correlated with time since disease onset (p<0.001) (Palmieri et al, 2009); in addition, Total scores correlated with State anxiety (STAI Y1) scores (p<0.01); Crying correlated with Emotional Fragility (p<0.001) and Beck Depression inventory scores (p<0.001) STAI Y (p<0.01) and Trait anxiety (STAI Y2) scores ( p<0.01) (Palmieri et al, 2009). Total ELQ scores predictive of global measure of psychological distress in caregivers (Goldstein et al., 2006). No correlation with cognitive performance (Palmieri et al., 2009).