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Motor neuron disease (MND) is a devastating neurodegenerative condition associated with considerable disability and a poor prognosis. Despite improvements in symptomatic management in recent years, few therapies are available which modify survival. However, the challenge to find successful treatments would be greatly assisted by clearer elucidation of the underlying pathoaetiology. Many potential exogenous risk factors have been proposed as part of a gene-environment interaction in the aetiology of MND. A growing interest in the role of vigorous physical activity in the development of MND has followed reports of a higher than expected incidence of the disease in professional sports people. Such an association is also supported by current hypotheses concerning the cellular and genetic mechanisms of MND. However, evidence from epidemiological research remains conflicting and inconclusive. This article reviews the existing literature regarding physical activity as a risk factor for MND and the potential biological and genetic plausibility for this association. [ABSTRACT FROM AUTHOR]

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative condition, respiratory failure being the commonest cause of death. Quality of life and survival can be improved by supporting respiratory function with non-invasive ventilation. Transcutaneous carbon dioxide monitoring is a non-invasive method of measuring arterial carbon dioxide levels enabling simple and efficient screening for respiratory failure. The aim of this study was to validate the accuracy of carbon dioxide level recorded transcutaneously with a TOSCA 500 monitor. It is a prospective, observational study of 40 consecutive patients with ALS, recruited from a specialist ALS clinic. The partial pressure of carbon dioxide (PCO2) in each patient was determined by both transcutaneous monitoring and by an arterialized ear lobe capillary blood sample. The carbon dioxide (CO2) levels obtained with these two methods were compared by Bland-Altman analysis. The results showed that the mean difference between arterialized and transcutaneous readings was − 0.083 kPa (SD 0.318). The Bland-Altman limits of agreement ranged from 0.553 to − 0.719 kPa. The difference was < 0.5 kPa in 90% of the recordings. Four of the 40 measurements had a difference of > 0.5 kPa, with a maximum recorded difference of 0.95 kPa. In conclusion, non-invasive carbon dioxide monitoring using a TOSCA monitor is a useful clinical tool in neurology practice. Users should be aware of the possibility of occasional inaccurate readings. A clinically unexpected or incompatible reading should be verified with a blood gas analysis, especially when a decision to provide ventilatory support is required. [ABSTRACT FROM AUTHOR]

Placement of a gastrostomy tube remains the gold standard procedure to maintain nutrition in patients with motor neuron disease (MND) and bulbar muscle weakness. Percutaneous endoscopic gastrostomy (PEG) is the most commonly used procedure in this context. Per-oral image guided gastrostomy (PIG) is a new hybrid technique used successfully in non-MND patients. We have modified the PIG technique to improve patient tolerability and have undertaken a pilot evaluation of PIG compared to PEG in MND patients. Nineteen PIG and 16 PEG procedures performed over a period of four years were evaluated. Pre-procedural forced vital capacity (FVC), procedural oxygen saturation, post-procedural complications and survival duration were recorded. Results showed that a gastrostomy tube was successfully placed in 95% of the PIG group and 80% of the PEG group. Rates of minor complications were comparable in both groups (21% in PIG, 23% in PEG). No life-threatening complications occurred in either group. Procedural mean oxygen saturations were higher in the PIG group compared to the PEG group ( p < 0.001). No significant survival differences were observed. This study provides evidence for the use of the PIG procedure as a safe and well tolerated alternative to PEG in MND patients. [ABSTRACT FROM AUTHOR]