Medicines for Corticobasal degeneration

What it is

Corticobasal degeneration (CBD) is a rare, progressive neurological condition in which certain areas of the brain gradually deteriorate over time, leading to increasing problems with movement and, often, thinking and other functions. It is one of a group of conditions related to a build-up of an abnormal protein in the brain (it is sometimes grouped with conditions such as progressive supranuclear palsy). CBD usually begins in later adult life, and progresses gradually over years. A characteristic feature of CBD is that it often affects one side of the body more than the other, at least initially. The symptoms can include: movement problems — such as stiffness and slowness of movement (which can resemble Parkinson’s disease), difficulty controlling a limb, jerky movements, and problems with coordination and balance; a particular difficulty in which a limb (often an arm) becomes difficult to use and control, sometimes with a feeling that it does not belong to the person or moves on its own (sometimes called an "alien limb"); problems with carrying out purposeful movements and tasks despite normal strength; and, over time, problems with thinking, memory, language, and other cognitive functions, and sometimes changes in behaviour or mood. As it progresses, CBD increasingly affects mobility, the ability to carry out daily activities, communication, and swallowing, and people need increasing support. CBD can be difficult to diagnose, particularly early on, as its features overlap with other conditions (such as Parkinson’s disease and other related conditions), and diagnosis is made by specialists based on the pattern of symptoms, examination, and tests to exclude other causes. There is currently no cure for CBD, and no treatment that stops its progression, but care focuses on managing the symptoms, maintaining function and quality of life for as long as possible, and supporting the person and family — through a multidisciplinary team including specialists and therapists, and, as the condition advances, supportive and palliative care. The key messages are that CBD is a rare, progressive brain condition causing movement and cognitive problems, often affecting one side of the body more, that there is no cure, and that symptoms, function, and support are managed by a specialist multidisciplinary team.

How it is treated

Corticobasal degeneration is managed by a specialist multidisciplinary team, focusing on managing the symptoms, 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 CBD is rare and its features overlap with other conditions, diagnosis is made by specialists (neurologists) based on the pattern of symptoms, examination, and tests (such as brain scans and other investigations) to build the diagnosis and exclude other causes; it can be difficult to diagnose, particularly early. Once diagnosed, care focuses on managing the range of symptoms and supporting the person over time. Management is multidisciplinary and tailored to the individual’s symptoms, and may include: physiotherapy to help with movement, stiffness, balance, and mobility, and to maintain function and prevent complications for as long as possible; occupational therapy to help with daily activities, aids, and adaptations to maintain independence and safety; speech and language therapy to help with any speech and communication difficulties and, importantly, with swallowing (which can be affected as the condition progresses); and management of specific symptoms — for example, medicines may be tried for some symptoms (such as stiffness or movement problems, though they are often less effective than in Parkinson’s disease), and treatments for other symptoms (such as mood, or others) as needed. Managing the cognitive and behavioural changes, and supporting communication and daily life, are part of care, with strategies and support. As CBD progresses and needs increase, care includes increasing support with mobility, daily living, communication, eating and swallowing (with appropriate support and, where needed, decisions about nutrition), and other needs, and supportive and palliative care focused on comfort and quality of life becomes an important part of care in the later stages. Support for the person and, importantly, for family and carers — practical, emotional, and social — is central, given the progressive and challenging nature of the condition, and support organisations (including those for CBD and related conditions) 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 a specialist multidisciplinary team manages the symptoms, helps maintain function and quality of life for as long as possible, and supports the person and family throughout, and that support and, in the later stages, palliative care focus on comfort and wellbeing; so specialist multidisciplinary care, symptom management, and support for the person and family are central to managing CBD.