From the Guidelines
Shy-Drager syndrome, a subtype of multiple system atrophy (MSA), is characterized by predominant autonomic dysfunction, and its management focuses on controlling symptoms such as orthostatic hypotension, urinary incontinence, and movement disorders. The symptoms of Shy-Drager syndrome include orthostatic hypotension, urinary incontinence or retention, erectile dysfunction in men, constipation, reduced sweating, and movement disorders such as parkinsonism or cerebellar ataxia 1.
Key Symptoms:
- Orthostatic hypotension, causing dizziness and fainting
- Urinary incontinence or retention
- Erectile dysfunction in men
- Constipation
- Reduced sweating
- Movement disorders such as parkinsonism (slowness, stiffness, tremor) or cerebellar ataxia (poor coordination, unsteady gait)
Management:
- For orthostatic hypotension, measures include increasing salt and fluid intake, wearing compression stockings, elevating the head of the bed, and medications like midodrine or fludrocortisone 1
- Parkinsonism symptoms may partially respond to levodopa, though response is typically less robust than in Parkinson's disease 1
- Urinary problems can be managed with anticholinergics like oxybutynin for incontinence or intermittent catheterization for retention
- Physical therapy helps maintain mobility, while speech therapy addresses communication difficulties As there is no treatment to stop disease progression, the goal of management is to improve the quality of life by controlling symptoms 1.
From the Research
Symptoms of Shy-Drager Syndrome
- Orthostatic hypotension, which is a sudden drop in blood pressure upon standing 2, 3, 4
- Urinary symptoms, such as incontinence or retention 2, 3, 4
- Erectile impotence in males 2, 3
- Nocturnal stridor, which is a high-pitched sound while breathing in 2
- Rigidity and akinesia (stiffness and lack of movement) without tremors 2, 5
- Cerebellar signs, such as ataxia (loss of coordination) 2, 4
- Autonomic nervous system dysfunction, leading to symptoms like syncope, constipation, fecal incontinence, and cardiac arrhythmias 5
Management of Shy-Drager Syndrome
- Treatment of hypertonic-akinetic syndrome with levodopa/carbidopa and agonists of dopamine receptors, although this may increase the frequency of hypotonic episodes 3
- Use of muscarinic receptor agonists, such as bethanechol chloride, to improve cholinergic dysfunction and autonomic functions 6
- A coordinate, multidisciplinary team approach is suggested for nursing care and family coping with Shy-Drager Syndrome 5