What are the signs and symptoms of multiple system atrophy (MSA)?

Updated: Sep 24, 2018
  • Author: Stephen M Bloomfield, MD; Chief Editor: Selim R Benbadis, MD  more...
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Answer

Answer

In a study of 100 MSA patients, initial symptoms were orthostatic hypotension (68%), parkinsonism (46%), autonomic symptoms (41%), and cerebellar signs and symptoms (5%). Nearly all patients eventually develop parkinsonism and autonomic symptoms. Orthostatic hypotension develops in 66% of patients. Cerebellar symptoms develop in 50% of patients during the course of the disease.

Autonomic failure manifests as urinary dysfunction, orthostatic hypotension, erectile dysfunction, or impotence, which are observed early in nearly all men with MSA. Other common manifestations are urinary frequency, urgency, incontinence, or incomplete bladder emptying. On electromyelography (EMG), abnormal sphincteric results have been noted in MSA and can be useful ancillary findings. The early display of autonomic dysfunction is believed to anticipate a worse prognosis. [6, 7]

Patients with parkinsonism typically have asymmetric tremor, bradykinesia, rigidity, and postural instability. The tremor tends to be postural, irregular, and jerky, unlike the typical pill-rolling tremor of idiopathic parkinsonism. The dysarthria observed in patients with MSA tends to be hypokinetic. Those with cerebellar features present with gait and limb ataxia, ataxic dysarthria, and sustained gaze-evoked nystagmus. They also tend to develop saccadic pursuit movements.

Extensor plantar responses and hyperreflexia are present in patients with corticospinal dysfunction. Respiratory stridor is observed in 33% of patients; however, they rarely require a tracheostomy. Cognitive dysfunction is less common than in other Parkinson-plus syndromes, such as PSP or corticobasal degeneration (CBD).


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