Search

Many centres in the UK care for patients with motor neuron disease (MND) in a multidisciplinary clinic (MDC). It has been demonstrated that such care results in better prognosis for survival than care from a general neurology clinic (GNC). Whether this is due to higher use of disease-modifying interventions or an independent factor of attendance at a specialist clinic has not been established. Hence, we performed a retrospective review of hospital notes of patients with MND who were diagnosed and followed up in a GNC between 1998 and 2002 and in an MDC between 2006 and 2010. Overall, 162 patients attended a GNC, and 255 attended the MDC. The median survival from diagnosis was 19 months for patients who attended the MDC, compared to 11 months for those attending the GNC (hazard ratio 0.51, 95% CI 0.41-0.64). The Cox hazards model identified attendance at an MDC as an independently positive prognostic factor (HR 1.93, 95% CI 1.37-2.72, p < 0.001). We concluded that care at an MDC improves survival. While this effect is augmented by the increased use of riluzole, NIV and PEG, the data suggest that coordinated care independently improves the prognosis of MND patients. [ABSTRACT FROM AUTHOR]

Introduction: The contribution of cranial and thoracic region electromyography (EMG) to diagnostic criteria for amyotrophic lateral sclerosis (ALS) has not been evaluated.Methods: Clinical and EMG data from each craniospinal region were retrospectively assessed in 470 patients; 214 had ALS. Changes to diagnostic classification in Awaji-Shima and revised El Escorial criteria after withdrawal of cranial/thoracic EMG data were ascertained.Results: Sensitivity for lower motor neuron involvement in ALS was highest in the cervical/lumbar regions; specificity was highest in cranial/thoracic regions. Cranial EMG contributed to definite/probable Awaji-Shima categorization in 1.4% of patients. Thoracic EMG made no contribution. For revised El Escorial criteria, cranial and thoracic data reclassified 1% and 5% of patients, respectively.Conclusion: Cranial EMG data make small contributions to both criteria, whereas thoracic data contribute only to the revised El Escorial criteria. However, cranial and thoracic region abnormalities are specific in ALS. Consideration should be given to allowing greater diagnostic contribution from thoracic EMG. Muscle Nerve 54: 378-385, 2016. [ABSTRACT FROM AUTHOR]