Quality of life of ALS and LIS patients with and without invasive mechanical ventilation.

There are very few studies where quality of life (QOL) is assessed in patients with complete physical and functional disability and dependence to invasive mechanical ventilation (IV). We compared QOL of amyotrophic lateral sclerosis (ALS) and locked-in-syndrome (LIS) patients with invasive mechanical ventilation to ALS and LIS patients without mechanical invasive ventilation. Thirty-four patients, 27 with ALS and seven with LIS (vascular or tumoral aetiology) were included in the study. Twelve had invasive ventilation, 22 had non-invasive ventilation, and in the non-invasive ventilation group, five of them had ventilation via mask. The following scales were used for patients: ALS Functional Rating Scale (ALSFRS), McGILL, Short-Form 36 (SF36), Beck Depression Inventory-II, the Toronto Alexithymia Scale and the anxiety inventory of Spielberger. Mean ALSFRS scores were significantly lower in the invasive ventilation group (IV) than in the non-invasive ventilation group. McGILL and SF36 were not significantly different between the IV group and the non-invasive ventilation group; there were no significant differences between the two groups for others scales either. Comparison between IV group and LIS without invasive mechanical ventilation revealed no significant difference for SF36 and McGILL QOL scores. QOL was not significantly different between the IV and not invasively ventilated patients, but ALSFRS was significantly lower in the IV group, and comparison of QOL scores between non-ventilated LIS patients who had the same score of dependence that invasively ventilated patients did not show any difference. Invasive mechanical ventilation for patients who accept tracheotomy allows life prolongation and their QOL is not affected; medical teams should be aware of that.

Valproic acid-induced encephalopathy in very long course treated patients.

PRIMARY OBJECTIVE: In patients receiving valproate (VPA) treatment, valproate induced-encephalopathy (VIE) is among the most serious adverse side-effects and hyperammonaemia is a frequent and well-recognized event. This report evaluates adult patients receiving VPA for several years. RESEARCH DESIGN: This study was conducted in adult patients receiving VPA for several years. Many studies have evaluated blood ammonia levels and VPA-related encephalopathy in patients whose treatment was initiated for a few months. Most studies were conducted in children. METHODS AND PROCEDURES: This study looked at retrospectively occurrences of VIE in 63 patients who were receiving VPA for a minimum duration of at least 2 years. At the beginning of the study basal ammonaemia was measured for all patients. MAIN OUTCOMES AND RESULTS: This study reports a rather frequent onset of VIE. Long duration of VPA treatment was not correlated with encephalopathy onset. In seven cases temporary administration of lactulose alone was effective and VPA was continued. CONCLUSIONS: This study confirms that, under VPA treatment, hyperammonaemia is a current event; 25.4% patients presented isolated elevated ammonaemia when receiving VPA for very long durations. VPA therapy should be monitored closely and clinicians must be warned of VPA encephalopathy signs.