Cure MSA

Country specific patient information

Multiple system atrophy

 

The term multiple system atrophy (MSA) denotes a sporadic progressive neurodegenerative disorder of adult onset and of unknown aetiology which is clinically characterized by autonomic failure, parkinsonism, cerebellar ataxia, and pyramidal signs in any combination. One recent epidemiological survey has found a prevalence rate of 4.4/100.000, and another an incidence rate of 3/100.000/year.
Two major motor presentations can be distinguished clinically. Parkinsonian features predominate in 80% of patients (MSA-P subtype), cerebellar ataxia is the major motor feature in 20% of patients (MSA-C subtype).
The disease affects both men and women, it usually starts in the 6th decade and relentlessly progresses with death occuring after on average 9 years.

 

Clinical features in 203 pathologically verified MSA cases

I. Autonomic symptoms

Frequency

CIs (95%)

Urinary incontinence

55%

(48-62)

Postural faintness (incl. syncope)

51%

(44-58)

Impotence

47%

(37-56)

Recurrent syncope (>2)

18%

(12-23)

Urinary retention

18%

(13-24)

Faecal incontinence

12%

(8-17)

II. Parkinsonism

 

 

Akinesia

83%

(77-88)

*Tremor

67%

(60-73)

present at rest

39%

(32-46)

pill-rolling

8%

(5-12)

jerky

3%

(1-6)

not specified

25%

(18-32)

Rigidity

63%

(56-70)

L-Dopa response

 

 

best - poor

72%

(62-83)

-good

28%

(17-38)

Last -poor

95%

(75-92)

good

5%

(1-12)

Dyskinesias

27%

(17-38)

orofacial

15%

(8-25)

limbs

10%

(4-19)

Fluctuations

24%

(15-35)

III. Cerebellar signs

 

 

Gait ataxia

49%

(42-56)

Limb ataxia

47%

(40-54)

*Intention tremor

24%

(18-30)

Nystagmus

23%

(18-30)

IV. Pyramidal signs

 

 

Hyperreflexia

46%

(39-53)

Extensor plantar response

41%

(34-48)

Spasticity

10%

(6-15)

V. Other features

 

 

Intellectual deterioration

 

 

mild

22%

(17-29)

moderate

2%

(1-5)

severe

0.5%

(0-3)

Stridor

13%

(9-18)

Dystonia

12%

(8-17)

Anisocoria

8%

(5-12)

Contractures

7%

(4-11)

 

*Some patients had > 1 type of tremor.

 

Bradykinesia, rigidity, postural and rest tremor as well as dysequilibrium and gait unsteadiness characterize parkinsonism associated with MSA. Up to 90% of patients with MSA-P treated fail to show a sustained, long-term response to L-dopa. The cerebellar disorder comprises gait ataxia, limb kinetic ataxia and scanning dysarthria as well as cerebellar occulomotor disturbances. Autonomic failure in MSA manifests predominantly as symptomatic orthostatic hypotension, frequently associated with impaired or absent reflex tachycardia upon standing. Erectile dysturbances represent the most common and often the earliest feature of male MSA patients. In addition, patients may note increased constipation and hypo- or anhydrosis. Urological features are common and include urinary urgency, frequency, nocturia and urge incontinence. The clinical diagnosis of MSA rests largely on history and physical examination. Additional investigations are particularly helpful in excluding differential diagnoses, however, they may also support a presumptive clinical diagnosis