Neurobehavioral dysfunction in ALS has a negative effect on outcome and use of PEG and NIV.

OBJECTIVE: To assess the effect of neurobehavioral dysfunction on amyotrophic lateral sclerosis (ALS) survival and on the use of life-prolonging therapies in a population-based setting. METHODS: Of the 132 patients diagnosed with ALS in the province of Torino, Italy, between January 1, 2007, and June 30, 2008, 128 participated in the study. Neurobehavioral dysfunction was assessed with the Frontal Systems Behavior Scale (FrSBe), using the Family Rating forms, administered within 4 months from diagnosis. RESULTS: The 128 patients included 71 men and 57 women, with a mean age at onset of 64.7 (SD 11) years. Forty-one patients (32.0%) had a neurobehavioral dysfunction and 9 (7.0%) an isolated dysexecutive behavior. Enteral nutrition (EN) and noninvasive ventilation (NIV) were performed with similar frequencies in patients with and without neurobehavioral dysfunction. Patients with neurobehavioral dysfunction had a significantly shorter survival than those with a normal FrSBe score (median survival, 3.3 vs 4.3 years; p = 0.02). Patients with isolated dysexecutive behavior had a shorter survival than those without neurobehavioral dysfunction (median survival, 2.5 vs 4.5 years; p = 0.03). Patients with neurobehavioral dysfunction had a shorter survival after EN and NIV, while patients with isolated dysexecutive behavior had a shorter survival after NIV but not after EN. The negative effect of comorbid neurobehavioral dysfunction and of isolated dysexecutive behavior on survival persisted under the Cox multivariate model. CONCLUSIONS: The presence of neurobehavioral dysfunction or of isolate dysexecutive behavior in ALS at diagnosis is a strong predictor of a poor outcome, partially related to a reduced efficacy of life-prolonging therapies.

Neurobehavioral symptoms in ALS are negatively related to caregivers' burden and quality of life.

OBJECTIVE: To evaluate the frequency of neurobehavioral symptoms related to FTLD in a consecutive series of amyotrophic lateral sclerosis (ALS) patients and to assess their influence on patients' and caregivers' mood, burden, and quality of life. METHODS: A total of 70 couples of ALS patients and their caregivers consecutively seen in our ALS clinic were separately interviewed using a battery of tests assessing frontotemporal-related neurobehavioral symptoms, emotional status, and quality of life. Patients' behavioral abnormalities were assessed with the Frontal Systems Behavior Scale (FrSBe). Caregiver burden was assessed with the Caregiver Burden Inventory (CBI). RESULTS: According to caregivers' evaluations, 34 (48.6%) patients had FrSBe pathological scores at the time of the interview. According to patients' evaluation, 9 (12.9%) patients had pathological scores at the time of the interview. In caregivers' assessment, at the time of the interview the most commonly impaired neurobehavioral domain was apathy (39 patients, 55.7%), followed by executive dysfunction (32, 45.7%) and disinhibition (18, 25.7%). Neurobehavioral symptoms were related to the presence of bulbar symptoms at the time of the interview, but not to patients' age, gender, or physical status (ALS-FRS score). Patients' neurobehavioral symptoms were significantly related to lower caregivers' quality of life, highest depression, and highest burden, both in univariate and in multivariable analyses. CONCLUSIONS: Neurobehavioral symptoms were present in 50% of our ALS patients and were related to bulbar symptoms. They have a profound negative impact on caregivers' psychological status and were highly related with caregivers' burden.

ALS patients and caregivers communication preferences and information seeking behaviour.

To evaluate information preferences and information seeking behaviour in ALS patients and caregivers. Sixty ALS patients and caregivers couples were interviewed using a structured questionnaire about the content of diagnosis communication and their information seeking behaviour. The patients (35 men and 25 women) had a mean age of 63.4 years (SD 9.5). The caregivers (21 men and 39 women) had a mean age of 53.3 years (SD 14.9). The overall satisfaction with bad news communication and the impression that the physician had understood their feelings were higher amongst patients. Both parties indicated that the most important aspects to be informed were current researches, disease-modifying therapies and ALS outcome. Approximately 55% of patients and 83.3% of caregivers searched for information from sources outside the healthcare system. The most frequently checked source was internet, although its reliability was rated low. The caring neurologist should better attune the content of communication to patients' and caregivers' preferences, trying to understand what they want to know and encouraging them to make precise questions. Health professionals should be aware that ALS patients and caregivers often use internet to obtain information and should help them to better sort-out and interpret the news they found.

A cross sectional study on determinants of quality of life in ALS.

BACKGROUND: Understanding the determinants of quality of life (QoL) in amyotrophic lateral sclerosis (ALS) has become increasingly important with the recent emphasis on the comprehensive management of patients. OBJECTIVE: To evaluate the determinants of QoL in ALS using two scales with different theoretical constructs: the Schedule for the Evaluation of QoL-Direct Weighting (SEIQoL-DW), which evaluates subjective aspects of QoL, and the McGill QoL Questionnaire (MQOL), which evaluates both health related and non-health related factors of QoL. METHODS: Eighty consecutive patients with ALS underwent a battery of tests evaluating QoL and a series of physical, emotional, psychological, and socioeconomic predictor variables. A stepwise linear regression model was used to compare QoL scores and explicatory variables. RESULTS: SEIQoL-DW score was related to social support, depression, religiosity, and socioeconomic status. Total MQOL score was related to social support, socioeconomic status, and clinical status. MQOL single item score (MQOL-SIS) was related to social support, depression, social withdrawal, and socioeconomic status. SEIQoL-DW score was not related to total MQOL score. Conversely, a significant correlation was found between SEIQoL-DW and MQOL-SIS. CONCLUSIONS: With both QoL scales, the most important explicatory variable of QoL was the self perceived quality of social support. Physical status was not relevant in determining QoL. This study indicates that health related QoL measures are not adequate to assess QoL in patients with ALS, because their appreciation of QoL mainly relies on psychological, supportive, and spiritual factors. Therapeutic interventions should consider the psychological needs of patients and pay greater attention to caregivers' issues.