Medicines for Multiple system atrophy

What it is

Multiple system atrophy (MSA) is a rare, progressive neurological condition in which several parts of the nervous system gradually deteriorate, affecting movement and the body’s automatic (autonomic) functions — those that are controlled without conscious effort, such as blood pressure, bladder and bowel function, and others. It is related to a build-up of an abnormal protein in the brain (it is grouped with conditions such as Parkinson’s disease in this respect, but is a distinct condition). MSA usually begins in later adult life and progresses over years. Because MSA affects "multiple systems", it causes a combination of features, which vary between individuals but can include: movement problems — such as stiffness and slowness of movement (parkinsonism, resembling Parkinson’s disease), and/or problems with balance and coordination (ataxia); and problems with the autonomic (automatic) functions — such as a drop in blood pressure on standing (causing dizziness or fainting), bladder problems (such as urgency, difficulty, or incontinence), bowel problems, sexual difficulties (such as erectile dysfunction), and problems with sweating and temperature control; other features can include speech and swallowing difficulties, breathing problems (such as during sleep), and, later, more widespread effects. The particular combination and prominence of features vary, and MSA is broadly divided into types depending on whether the movement or the balance features predominate. MSA can be difficult to diagnose, particularly early, as its features overlap with Parkinson’s disease and other conditions; the prominent autonomic symptoms (such as the blood pressure and bladder problems), and the way the condition progresses and responds to treatment, help distinguish it, and diagnosis is made by specialists based on the pattern of symptoms, examination, and tests. There is currently no cure for MSA, and no treatment that stops its progression, but care focuses on managing the symptoms (particularly the movement and autonomic symptoms, many of which can be helped), maintaining function and quality of life, and supporting the person and family, through a specialist multidisciplinary team and, as the condition advances, supportive and palliative care. The key messages are that MSA is a rare, progressive brain condition affecting movement and the body’s automatic functions, that there is no cure, and that a specialist team manages the symptoms (many of which can be helped) and supports the person and family.

How it is treated

Multiple system atrophy is managed by a specialist multidisciplinary team, focusing on managing the movement and autonomic symptoms (many of which can be helped), maintaining function and quality of life, and supporting the person and family; there is no cure or treatment to stop its progression, so care is supportive and individualised. Because MSA is rare and its features overlap with other conditions early on, diagnosis is made by specialists (neurologists) based on the combination of symptoms — particularly the movement problems together with prominent autonomic symptoms (such as blood pressure drops on standing and bladder problems) — along with examination and tests (such as scans and tests of autonomic function) to support the diagnosis and exclude other causes; diagnosis can be difficult, especially early. Once diagnosed, care focuses on managing the range of symptoms, many of which can be helped, and supporting the person over time. Management is multidisciplinary and tailored, and may include: managing the autonomic symptoms, which is an important part of care — for example, measures and medicines to manage the drop in blood pressure on standing (such as advice on rising slowly, increasing fluid and salt intake where appropriate, compression garments, and medicines); managing bladder problems (with measures, medicines, and specialist input); managing bowel, sexual, sweating, and other autonomic symptoms; and addressing these can significantly improve comfort and function; managing the movement symptoms — for example, medicines may be tried for the parkinsonism (though the response is often limited compared with Parkinson’s disease), and physiotherapy helps with movement, balance, and mobility; physiotherapy and occupational therapy to maintain movement, function, and daily living and provide aids and adaptations; speech and language therapy to help with speech and communication and, importantly, with swallowing (which is affected as the condition progresses); and management of breathing problems (including during sleep) and other symptoms as needed. As MSA progresses and needs increase, care includes increasing support with mobility, daily living, communication, swallowing and nutrition, and other needs, and supportive and palliative care focused on comfort and quality of life becomes important in the later stages. Support for the person and, importantly, for family and carers — practical, emotional, and social — is central, and support organisations (such as MSA charities) provide information, support, and community. Advance care planning, made with the person while they are able, helps ensure care reflects their wishes. The reassuring messages, within the seriousness of this progressive condition, are that many of the symptoms of MSA — particularly the autonomic symptoms — can be helped and managed, that a specialist multidisciplinary team manages the symptoms and supports function and quality of life for as long as possible, that support for the person and family is central, and that, in the later stages, palliative care focuses on comfort and wellbeing; so specialist multidisciplinary care, symptom management, and support for the person and family are central to managing MSA.