1 Ahuja A, Gothi D, Joshi J. A 52-year-old man with daytime sleepiness, sialorrhea, and facial fasciculations. Chest 2006; 130:287–290 2 Servera E, Sancho J. Appropriate management of respiratory problems is of utmost importance in the treatment of patients with amyotrophic lateral sclerosis [editorial]. Chest 2005; 127:1879–1882 3 Aboussouan LS, Khan SU, Meeker DP, et al. Effect of noninvasive...

OBJECTIVE To study the expiratory muscle force and the ability to cough estimated by the peak expiratory flow and peak cough flow in patients with Duchenne muscular dystrophy and amyotrophic lateral sclerosis. DESIGN A total of 27 patients with amyotrophic lateral sclerosis and 52 patients with Duchenne muscular dystrophy were studied. From the group of 144 normal subjects of this laboratory,...

Evidence from human post mortem, in vivo and animal model studies implicates the neuroimmune system and activated microglia in the pathology of amyotrophic lateral sclerosis. The study aim was to further evaluate in vivo neuroinflammation in individuals with amyotrophic lateral sclerosis using [(11)C]-PBR28 positron emission tomography. Ten patients with amyotrophic lateral sclerosis (seven mal...

BACKGROUND Use of statins has been associated with an amyotrophic lateral sclerosis-like syndrome in 2 analyses of overlapping surveillance databases that record adverse events potentially related to prescription drug use. We assessed whether statin use is associated with the occurrence of amyotrophic lateral sclerosis and other motor neuron disorders. METHODS AND RESULTS We conducted a popul...

Heterozygous missense mutations in the N-terminal motor or coiled-coil domains of the kinesin family member 5A (KIF5A) gene cause monogenic spastic paraplegia (HSP10) and Charcot-Marie-Tooth disease type 2 (CMT2). Moreover, heterozygous de novo frame-shift mutations in the C-terminal domain of KIF5A are associated with neonatal intractable myoclonus, a neurodevelopmental syndrome. These finding...

Secretion of prolactin in nine patients with amyotrophic lateral sclerosis and in seven healthy men was investigated with the use of metoclopramide stimulation and bromocriptine inhibition tests. Blood serum prolactin concentration was determined in the basal state and 30, 60 and 120 minutes after oral administration of 10 mg metoclopramide or 2.5 mg bromocriptine. A period of 3 days intervened...

OBJECTIVE To describe the prevalence and severity of periodic limb movements during sleep in amyotrophic lateral sclerosis patients and to explore this fact as a predictor of severity of the condition with respect to mortality. METHODS In this case-control study, questionnaire and polysomnographic data were analyzed from 35 amyotrophic lateral sclerosis patients. Controls were matched by age,...

Riluzole, a benzothiazole derivative, has been shown to be effective in prolonging survival in amyotrophic lateral sclerosis. The mechanisms by which riluzole exerts neuroprotective effects in amyotrophic lateral sclerosis remains to be fully elucidated, although inhibition of glutamatergic transmission and modulation of Na + channel function have been proposed. In an attempt to determine the m...

The aim of this study was to determine first, if benign fasciculations and those in amyotrophic lateral sclerosis can de distinguished on the basis of their waveforms or firing characteristics, and second to determine how fasciculation parameters evolved with progression of amyotrophic lateral sclerosis. Fasciculation potentials recorded from 63 muscles of 28 patients with definite amyotrophic ...

I propose in this paper shortly to consider some cases of paraplegia in which the main symptoms were those that are taken to indicate sclerosis of the crossed pyra-midal tracts in the spinal cord, and in which the arms were not affected. The symptoms of this lateral sclerosis are paraplegia, with spasm and rigidity of muscles, and marked increase of myotatic irritability. The lateral sclerosis ...