In the natural progression of amyotrophic lateral sclerosis (ALS), a state of malnutrition often develops, associated with reduced oral intake, caused by difficulties with swallowing, adequate salivation, and/or anorexia. The placement of a feeding tube should be considered at the point where patients demonstrate swallowing difficulty and/or alteration in nutritional status. The goal of this prospective work was to compare, in 50 patients having definite or probable ALS, the complications after endoscopic (PEG), or radiologically-guided gastrostomy (RIG) and to evaluate their effects on survival. RIG was proposed as first-line therapy when patients had a slow vital capacity (SVC) less than 50% of the theoretical value, or in a case where PEG was refused by the patient. Thirty patients underwent PEG procedure, and 20 had RIG. Among the decisional criteria for placing a gastrostomy tube, risk of aspiration demonstrated by using videofluoroscopy was more frequently observed in the RIG group (65%) than in the PEG group (19.2%) (p=0.002). The two patient populations at time of gastrostomy placement were comparable with respect to age, gender ratio, disease duration before gastrostomy, the form and the gravity of neurological involvement as well as for nutritional criteria. The only significant difference observed between the two groups was the level of the SVC, much lower for patients undergoing RIG: 51.6+/-25.0% versus 67.4+/-26.7% than for the PEG group (p=0.03). The frequency of all complications observed at the moment of placement of gastrostomy tube and during the first month of follow-up was not significantly different between the two groups. Kaplan-Meier survival curves from the date of gastrostomy placement were not different in univariate analysis (p=0.85). In multivariate analysis, survival was not different between one group and the other (p=0.28). The major interest of the RIG technique rests on its greater feasibility and on the possibility of utilizing it in ALS patients who have significant ventilatory compromise.

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The Dutch protocol for rehabilitative management was developed to provide an optimal and adequate care plan for patients with ALS. It is widely used in the Netherlands.

A 55-year-old woman with a progressive dementia and frontal syndrome was hospitalized because she was agitated every night after falling asleep (spoke, laughed, cried, tapped, kicked, walked, and fell down). She slept 5.5 hours during video polysomnography, but the theta rhythm electroencephalograph recording typical of sleep stages 1 to 2 and the spindles and K-complexes typical of sleep stage 2 contrasted with continuous muscular twitching, prominent rapid eye movements, vocalizations, and continuous, complex, purposeful movements typical of rapid eye movement (REM) sleep behavior disorder. This newly described stages 1--2 non-REM sleep behavior disorder suggests that central motor pattern generators were disinhibited during non-REM sleep.

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