What are the clinical features of multiple system atrophy (MSA)?

Updated: Oct 17, 2018
  • Author: André Diedrich, MD, PhD; Chief Editor: Selim R Benbadis, MD  more...
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Answer

Features indicating the presence of MSA (tables 2a and 2b) or of another disorder (Table 3) are described below. (Corticospinal tract dysfunction with extensor plantar response with hyperreflexia [pyramidal sign] is not used to categorize MSA.) (See DDx.)

Table 2a. Main Features for the Diagnosis of MSA (Open Table in a new window)

Clinical Domain

Feature

Comment

Autonomic

dysfunction

Severe orthostatic hypotension (OH)

  • Asymptomatic

  • Symptomatic

OH is defined as blood pressure fall by at least 30mm Hg systolic and 15mm Hg diastolic within 3 minutes of standing from a previous 3-minute interval in the recumbent position.**

Urogenital dysfunction

Urinary incontinence (UI) or incomplete bladder emptying

UI is defined as persistent, involuntary, partial or total bladder emptying.

ED usually occurs before symptomatic OH.***

Erectile dysfunction (ED) in men

Parkinsonian features

(87% incidence *)

Bradykinesia (BK)

BK is slowness of voluntary movement with progressive reduction in speed and amplitude during repetitive actions.

PI not caused by primary visual, vestibular, cerebellar, or proprioceptive dysfunction.

Rigidity

Postural instability (PI)

Tremor - Postural, resting, or both

Cerebellar dysfunction

(54% incidence *)

Gait ataxia (GA)

GA is a wide-based stance with steps of irregular length and direction.

Sustained gaze-evoked nystagmus

Ataxic dysarthria

Limb ataxia

Oculomotor dysfunction

Coritcospinal tract dysfunction

Extensor plantar response with hyperreflexia

Babinsky sign, Pyramidal sign

*Incidence of clinical features recorded during the lifetimes of 203 patients (Gilman et al [2] ).

**OH caused by drugs, food, temperature, deconditioning, or diabetes are excluded.

***ED does not count in the definition of onset of disease, because it is a general feature in older people.

Table 2b. Additional Features for the Diagnosis of Possible MSA* (Open Table in a new window)

Category

Additional Features

 

Possible

MSA-P

Possible

MSA-C

  • Babinski sign with hyperreflexia

  • Stridor

 

Possible

MSA-P

  • Rapidly progressive parkinsonism

  • Poor response to levodopa

  • Postural instability within 3 years of motor onset

  • Gait ataxia, cerebellar dysarthria, limb ataxia, or cerebellar oculomotor dysfunction

  • Dysphagia within 5 years of motor onset

  • Atrophy on magnetic resonance imaging (MRI) of putamen, middle cerebellar peduncle, pons, or cerebellum

  • Hypometabolism on 2-[fluorine-18]fluoro-2-deoxy-D-glucose (FDG) positron emission tomography (PET) scanning in putamen, brainstem, or cerebellum

 

Possible

MSA-C

  • Parkinsonism (bradykinesia and rigidity)

  • Atrophy on MRI of the putamen, middle cerebellar peduncle, or pons

  • Hypometabolism on FDG-PET in the putamen

  • Presynaptic striatonigral dopaminergic denervation on single-photon emission computed tomography (SPECT) or PET scanning

*Modified from second consensus [6]

 

Table 3. Characteristics That Do Not Support the Diagnosis of MSA (Open Table in a new window)

Procedure

Nonsupporting Features

History taking

  • Symptomatic onset at < 30 years

  • Onset after age 75 years

  • Family history of ataxia or parkinsonism

  • Systemic diseases or other identifiable causes for features listed in Table 2a

  • Hallucinations unrelated to medication

  • Dementia

Physical examination

  • Classic parkinsonian pill-rolling rest tremor

  • Clinically significant neuropathy

  • Prominent slowing of vertical saccades or vertical supranuclear gaze palsy

  • Evidence of focal cortical dysfunction, such as aphasia, alien limb syndrome, and parietal dysfunction

Laboratory study

  • Metabolic, molecular genetic, and imaging evidence of alternative cause of features listed in Table 2a

  • White matter lesions suggesting multiple sclerosis


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