The natural history of motor neuron disease: Assessing the impact of specialist care

Abstract

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.

Challenges in the Understanding and Treatment of Amyotrophic Lateral Sclerosis/Motor Neuron Disease

“…Multiple interventions to address these significant determinants of health and longevity have implicated a multidisciplinary approach involving 1) dieticians (include altering food consistency, high-calorie supplements, and adequate hydration); 2) occupational therapy (hand braces, altered utensils, mobile arm supports, home modifications); 3) physical therapy (range of motion, bracing, seating, maintaining strength); 4) social work (home care assistance, financial support, emotional health); and 5) respiratory therapy (adequate ventilation, secretion management). Although large, controlled outcomes studies have not been completed, the benefit of such multidisciplinary care, on both survival and quality of life, has been widely accepted [111][112][113].…”

Multidisciplinary Care Improves Survival of Patients with Amyotrophic Lateral Sclerosis in the Unique Health System (SUS) in Brazil

“…Thus, studies with multidisciplinary units in the UK [24], Ireland [15] and Spain [18] have concluded that compared to ALS patients who were treated in neurology services, patients treated in multidisciplinary units have better survival, and patients whose symptoms began at the bulbar level especially benefited from multidisciplinary care. Nevertheless, the average one-year mortality rate is approximately 50% overall and 57% in bulbar cases [15].…”

“…Survival may be augmented by the increased use of riluzole, NIV and gastrostomy tube placement, but evidence shows that coordinated care independently improves the prognosis of ALS patients [24]. In the SUS, evidence also shows that multidisciplinary care reduces costs and the return visit frequency for patients with ALS [7].…”

DiPALS: Diaphragm Pacing in patients with Amyotrophic Lateral Sclerosis – a randomised controlled trial

“…prior NIV prescription 2. pre-existing implanted electrical device such as pacemaker or cardiac defibrillator 3. underlying cardiac or pulmonary diseases, or other disorders that would affect pulmonary tests independently of ALS (increased risk of general anaesthesia or adverse effect on survival over the course of the study) 4. women who were pregnant or breastfeeding at the time of screening SCIENTIFIC SUMMARY NIHR Journals Library www.journalslibrary.nihr.ac.uk xxiv 5. significant decision-making incapacity (patient suffered from major depression, schizophrenia, dementia or similar disorder) preventing informed consent by the patient 6. marked obesity affecting surgical access to diaphragm or significant scoliosis/chest wall deformity 7. the involvement in any respiratory trial that could have influenced the safety or outcome measures of the study within 3 months of the planned implantation of the device or during the year of follow-up 8. pre-existing diaphragm abnormality such as a hiatus hernia or paraoesophageal hernia of abdominal contents ascending into the thoracic cavity 9.…”