EFNS task force on management of amyotrophic lateral sclerosis: guidelines for diagnosing and clinical care of patients and relatives

Abstract

Despite being one of the most devastating diseases known, there is little evidence for diagnosing and managing patients with amyotrophic lateral sclerosis (ALS). Although specific therapy is lacking, correct early diagnosis and introduction of symptomatic and specific therapy can have a profound influence on the care and quality of life of the patient and may increase survival time. This document addresses the optimal clinical approach to ALS. The final literature search was performed in the spring of 2005. Consensus recommendations are given graded according to the EFNS guidance regulations. Where there was lack of evidence but consensus was clear we have stated our opinion as good practice points. People affected with possible ALS should be examined as soon as possible by an experienced neurologist. Early diagnosis should be pursued and a number of investigations should be performed with high priority. The patient should be informed of the diagnosis by a consultant with a good knowledge of the patient and the disease. Following diagnosis, the patient and relatives should receive regular support from a multidisciplinary care team. Medication with riluzole should be initiated as early as possible. PEG is associated with improved nutrition and should be inserted early. The operation is hazardous in patients with vital capacity <50%. Non‐invasive positive pressure ventilation improves survival and quality of life but is underused. Maintaining the patients ability to communicate is essential. During the entire course of the disease, every effort should be made to maintain patient autonomy. Advance directives for palliative end of life care are important and should be fully discussed early with the patient and relatives respecting the patients social and cultural background.

Accepting or declining non-invasive ventilation or gastrostomy in amyotrophic lateral sclerosis: patients’ perspectives

“…While rates of acceptance of gastrostomy are not available for the UK, rates in the USA range from 0-63% [9]. Evidence-based guidelines based largely on expert opinion recommend the use of NIV in ALS [11][12][13][14].…”

The Present and the Future of Neuroimaging in Amyotrophic Lateral Sclerosis

“…Paraclinical and laboratory tests are used only to exclude "ALS-mimic" syndromes. 4,5 In ALS, the absence of a disease marker for UMN and LMN involvement has 2 main negative consequences. First, the delay from onset of the disease to diagnosis of ALS can vary between 13 and 18 months 6,7 ; and the diagnostic delay may even be greater in patients who present with isolated LMN signs.…”

Mental disorders in patients with lateral amyotrophic sclerosis and their family members

“…Это может способ-ствовать усилению действия препарата и минимизации его побочных эффектов [1,9]. Заслуживает внимания так-же факт частичного регресса когнитивных и психических нарушений при отмене холинолитиков, который нужда-ется в дальнейшем изучении.…”

“…Как известно, к методам, предусмотренным Между-народным стандартом лечения БАС, относят лечение пре-паратом рилузол, неинвазивной и искусственной венти-ляцией легких (ИВЛ), препаратами для коррекции слю-нотечения и психотропными, а также мероприятия, на-правленные на облегчение адаптации больного в быту [9]. Следует указать, что единственным методом лечения, ко-торый в настоящее время не входит в Международный стандарт лечения БАС, но может быть применен в случае показаний, -ранняя трахеостомия.…”