Progressive degenerative movement conditions
Atypical parkinsonism is used for a group of progressive degenerative brain conditions that cause symptoms similar to Parkinson’s disease, but that behave differently over time.
These conditions usually:
- progress more quickly than Parkinson’s disease
- involve additional symptoms beyond movement
- respond poorly or only briefly to standard Parkinson’s medication.
It can arise from more than one underlying disease process.
What is atypical parkinsonism?
Atypical parkinsonism is not a single disease but a category describing several distinct conditions that share features with Parkinson’s disease while also having their own characteristic patterns and distinct pathological changes in the brain. Recognising early signs such as frequent falls, changes in eye movements, bladder disturbances, erectile dysfunction, fainting or cognitive changes can help individuals seek timely support and feel empowered to manage their health.
There are several recognised conditions grouped under this term, each with a different pattern of additional features:
- Progressive supranuclear palsy (PSP) – early falls (often backwards), stiffness and difficulty moving the eyes up and down.
- Multiple system atrophy (MSA) – a combination of movement difficulties, balance disturbance and autonomic problems, such as light-headedness on standing and bladder dysfunction.
- Corticobasal degeneration (CBD) – asymmetric stiffness and clumsiness, often affecting one side of the body more than the other, sometimes with jerky movements or a limb that feels ‘foreign’.
- Dementia with Lewy bodies-related parkinsonism – movement symptoms occurring alongside early cognitive fluctuations, visual hallucinations and sleep disturbance.
Over time, atypical parkinsonism typically affects:
- movement, balance and coordination
- speech and swallowing
- eye movements (in some subtypes)
- autonomic functions, such as blood pressure and bladder control (in some subtypes)
- thinking, behaviour and daily living skills, particularly later in the condition.
What happens in the brain?
Atypical parkinsonism is associated with progressive damage in brain regions involved in movement control, including the basal ganglia, brainstem and, in some subtypes, the cerebellum or frontal regions. The underlying protein change depends on the specific condition:
- PSP and corticobasal degeneration are usually associated with a build-up of abnormal tau protein.
- Multiple system atrophy is usually associated with a build-up of abnormal alpha-synuclein protein, the same protein involved in Parkinson’s disease and dementia with Lewy bodies.
This means atypical parkinsonism is not one disease, but a movement-led way that different underlying brain diseases can present.
What increases the risk of atypical parkinsonism?
Risk factors vary somewhat by subtype, since atypical parkinsonism covers several distinct underlying diseases, though some general patterns are recognised.
Factors that can’t be changed include:
- age – most atypical parkinsonian conditions become more common with increasing age, typically presenting in the 60s and 70s
- genetics – most cases occur sporadically, without a family history, though rare genetic factors have been identified in a small proportion of cases, particularly for corticobasal degeneration and some MSA cases.
Factors that may be modifiable are not well established for atypical parkinsonism specifically, since these conditions are less strongly linked to cardiovascular or lifestyle-related risk factors than, for example, vascular parkinsonism. General brain health measures are reasonable to encourage but haven’t been shown to reduce the risk of these specific conditions meaningfully.
These conditions are rare, and their causes are not fully understood, but ongoing research aims to improve understanding and management, offering hope for future advancements.
Common patient questions
It can be, depending on the specific condition. Some atypical parkinsonian syndromes involve early or prominent changes in thinking, planning or behaviour, while others affect movement and autonomic function more than cognition, at least initially.
Most cases occur sporadically, without a family history. Rare genetic factors may slightly increase risk in some conditions, but atypical parkinsonism is not typically considered an inherited group of conditions.
There is currently no treatment that stops or reverses these conditions. Still, a focus on managing symptoms and maintaining safety can help preserve quality of life through a multidisciplinary approach that includes physiotherapy, speech therapy and medication trials where appropriate.