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AVP-923 palliates pseudobulbar affect in ALS. Overall benefits of treatment are reflected in fewer episodes of crying and laughing and improvements in overall quality of life and quality of relationships.

(AAN) is charged with developing practice parameters for physicians. This evidence-based review addresses some of the major management issues in patients with ALS, and highlights the many areas in which more research is needed.Justification. ALS is a progressive, degenerative motor neuron disease of unknown cause. Muscle atrophy and spasticity in limb and bulbar muscles result in weakness and loss of ambulation, oropharyngeal dysfunction, weight loss, and ultimately respiratory failure. Although advances in understanding the pathophysiology of ALS have stimulated the development of new drug therapies, 1 the mainstay of treatment for ALS patients remains symptomatic management.The practice parameters presented here comprise the first recommendations for the management of ALS based on a prescribed review and analysis of the peer-reviewed literature. These practice parameters were developed to improve the care and the quality of life of people with ALS by providing a rational basis for managing the disease.Description of the process. A multidisciplinary task force, all with extensive ALS experience, included 19 physicians, 3 patients with ALS, 1 gastroenterologist, 1 pulmonologist, 1 occupational therapist whose mother has ALS, and 1 nurse. In addition, consultants with expertise on ethics, practice parameter development, and medical library research participated in the process. The task force agreed to investigate five areas: 1) informing the patient and the family about the diagnosis and prognosis (also called "breaking the news") of ALS; 2) symptomatic treatment; 3) nutrition, and decisions about percutaneous endoscopic gastroscopy (PEG); 4) respiratory insufficiency and mechanical ventilation; and 5) advance directives and palliative care. To help achieve this goal, they developed several guiding principles or attributes of care: Principles of ALS management1. High priority should be placed on patient self-determination or autonomy as an underlying assumption in the therapeutic relationship. Delivery of both information and care must take into consideration the cultural and psychosocial context of the patient and the family. 2. Patients and families need information that is timed appropriately for decision making, and delivered well in advance of major management crossroads, especially for respiratory care. Moreover, decision making is a dynamic process that may be subject to change as the disease becomes more severe.The investigators and institutions of the ALS Practice Parameters Task Force are listed in the Appendix on page 1320.

DMq markedly reduced PBA frequency and severity, decreasing the condition's detrimental impact on a patient's life, with satisfactory safety and high tolerability. The findings expand the clinical evidence that DMq may be an important treatment for patients suffering from the socially debilitating symptoms of PBA.

Inevitably there are some omissions m such a document and many of the symptoms of patients with ALS are not addressed m this first document but will be taken up m future iterations. Of particular importance m the document is the evidence-based recommendation about the value of percutaneous endoscopic gastrostomy and the importance of timmg m presentmg this option to patients. Similarly, strong evidence-based recommendations are made about the utility of noninvasive mechanical ventilation and the beneficial impact that this has on symptoms of hypoventilation and on survival. Finally, management of patients m the termmal phase of the disease usmg the highest standard of palliative care is discussed m some detail. Perhaps one of the most important contributions of this document is the highlighting of areas where further research is needed. This has been done m each of the major sections of the paper. More rigorous clinical investigations are still needed to help answer some of the most pressmg management issues for patients with ALS. Mission Statement The Quality Standards Subcommittee of the American Academy of Neurology (AAN) is charged with developmg practice parameters for physicians. This evidence-based review addresses some of the major management issues m patients with amyotrophic lateral sclerosis (ALS), and highlights the many areas where more research is needed.

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