Brain : a journal of neurology
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Although ventilatory failure is the most common cause of death in amyotrophic lateral sclerosis (ALS) and measurement of respiratory muscle strength (RMS) has been shown to have prognostic value, no single test of strength can predict the presence of hypercapnia reliably. RMS was measured in 81 ALS patients to evaluate the relationship between tests of RMS and the presence of ventilatory failure, defined as a carbon dioxide tension > or = 6 kPa. We studied the predictive value of vital capacity (VC), static inspiratory and expiratory mouth pressures (MIP, MEP), maximal sniff oesophageal (sniff P(oes)), transdiaphragmatic (sniff P(di)) and nasal (SNP) pressure, cough gastric (cough P(gas)) pressure and transdiaphragmatic pressure after bilateral cervical magnetic phrenic nerve stimulation (CMS P(di)) to identify the risk of ventilatory failure in the whole group and in subgroups of patients with and without significant bulbar involvement. ⋯ In particular, the non-invasive SNP is more sensitive than VC and MIP, suggesting that it could usefully be included in tests of respiratory muscle strength in ALS and will be helpful in assessing the risk of ventilatory failure. In patients with significant bulbar involvement, tests of respiratory muscle strength do not predict hypercapnia. Sleep-disordered breathing is correlated with RMS and the novel tests of RMS having the strongest relationship with the degree of sleep disturbance.
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Some accounts of body representations postulate a real-time representation of the body in space generated by proprioceptive, somatosensory, vestibular and other sensory inputs; this representation has often been termed the 'body schema'. To examine whether the body schema is influenced by peripheral factors such as pain, we asked patients with chronic unilateral arm pain to determine the laterality of pictured hands presented at different orientations. ⋯ Importantly, a significant interaction between the magnitude of mental rotation and limb was observed: RTs were longer for the painful arm than for the unaffected arm for large-amplitude imagined movements; controls exhibited symmetrical RTs. These findings suggest that the body schema is influenced by pain and that this task may provide an objective measure of pain.