Synopsis
Recognise the combination of progressive autonomic failure with poorly responsive parkinsonism or cerebellar ataxia, identify airway and syncope hazards and provide anticipatory multidisciplinary care.
- Multiple system atrophy is a progressive synucleinopathy combining autonomic failure with parkinsonism, cerebellar dysfunction or both; MSA-P and MSA-C describe the predominant motor pattern.
- Early urinary urgency, incontinence or retention, erectile dysfunction and neurogenic orthostatic hypotension are major clues when they are disproportionate to motor disability.
- Parkinsonism is commonly symmetrical or axial and responds poorly or only transiently to levodopa, although a supervised adequate trial remains worthwhile in many patients.
Key red flags
Standing produces light-headedness, dim vision, weakness, neck or shoulder ache and sometimes syncope, with a substantial pressure fall and an insufficient compensatory pulse rise after confounders are addressed.
Investigation priorities
Document orthostatic physiology, symptom correlation and competing medicine or volume effects.
Management branches
Progressive parkinsonism or ataxia occurs with prominent urinary, sexual or blood-pressure dysfunction.
- Build a dated timeline of erectile, bladder, bowel, sweating, blood-pressure, sleep, speech, swallowing and motor symptoms and reconcile medicines that could reproduce them.
- Examine standing physiology, eye movements, parkinsonism, cerebellar and pyramidal signs and gait; measure post-void residual and obtain MRI to exclude alternatives and seek supportive features.
Key medicines
Levodopa combination therapyUndertake an adequately dosed specialist trial, titrated to recorded functional targets and individual tolerance.
MidodrineA common start is 2.5 mg three times daily while upright, titrated under specialist guidance.