The Canadian Movement Disorder Group
Multiple System Atrophy

MSA Coalition

http://www.multiple-system-atrophy.org

NIH information Page

https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Multiple-System-Atrophy

UK site

http://www.msatrust.org.uk

Multiple system Atrophy, abbreviated MSA defines a specific syndrome within the larger less well defined category of multiple system degenerations. The features of the MSA include: parkinsonism, cerebellar or corticospinal signs, orthostatic hypotension, impotence, and urinary incontinence or retention, usually preceding or within two years after the onset of the motor symptoms. The previous division into Shy Drager Syndrome (SDS), Striato Nigral Degeneration (SND), and Olivopontocerebellar Atrophy (OPCA) has been dropped. Immunohistochemistry demonstrates that these three disorders share a common pathology.

The latest diagnostic criteria are shown in the table below.

Wenning et. al. determined that the combination of autonomic insufficiency, speech or bulbar dysfunction, absence of dementia, postural instability with falls, poor response to levodopa, and absence of levodopa-induced confusion gave a diagnostic sensitivity and specificity greater than 90%.

Median age of onset of MSA is about age 55 years (range of 33 to 76). It affects men slightly more than women. Nearly half of patients are disabled or wheelchair bound within 5 years of the onset of motor symptoms. Mean survival is 6 to 7 years. 80% of MSA patients develop predominant parkinsonism (MSA-P) and 20% develop predominant cerebellar signs (MSA-C). The latter statistics are likely skewed by referral patterns to movement disorder centers. There is considerable overlap. Cerebellar features are present in over 40% of patients with SND type, and parkinsonism is detectable in 50% of OPCA type patients.

30% and 65% of MSA patients had a good levodopa response at some stage. Between 13% and 30% maintained some response through the course of the illness. 25% to 50% of those treated with levodopa had dyskinesias (particularly orofacial) and dystonia, even if they did not experience improvement in motor state.

Autonomic symptoms were the initial feature in 41% of patients, but ultimately 97% of patients developed some degree of autonomic dysfunction. The most frequent autonomic symptom in men was impotence and in women urinary incontinence. Orthostatic hypotension occurred in 68%.

A mild restriction of downgaze may develop in about 10% of MSA cases.. Anterior horn cell loss may occur but is uncommon. Anal sphincter EMG (90% have an abnormality) is a sensitive and specific diagnostic test for MSA. Even less frequently seen is a mild sensory neuropathy. Classic rest tremor is uncommon (29%). Cerebellar signs occurred in 54% of patients and upper motor neuron signs in 49% of the cases. MSA-P type generally demonstrates more tremor, pyramidal signs, and myoclonus than MSA-C type. Severe dementia is uncommon

Respiratory stridor (which ultimately occurs in 1/3 of cases) in combination with parkinsonism is highly suggests MSA until proven otherwise; although stridor can also occur in PD, it is exceptionally rare.

Multiple System Atrophy: Clinical Diagnostic Criteria

STRIATONIGRAL DEGENERATION Type

(predominant parkinsonism)

Sporadic adult-onset (age 30 years or above)

Possible

Non/poorly levodopa responsive parkinsonism*

Probable

Above**, plus severe symptomatic autonomic failure*** or cerebellar signs or pyramidal signs or pathological sphincter EMG 

 

 

Definite

Post mortem confirmed

OLIVOPONTOCEREBELLAR Type

(predominantly cerebellar)

Sporadic adult-onset (age 30 years or above)

Possible

Cerebellar syndrome with parkinsonism

 

Probable

Sporadic adult-onset cerebellar syndrome*

(with or without parkinsonism or pyramidal signs) plus severe symptomatic autonomic failure*** or pathological sphincter EMG

Definite

Post mortem confirmed

* Without DSM III dementia, generalized tendon areflexia, down gaze PSNP or other identifiable cause.

** Moderate or good, but often waning, response to levodopa may occur, in which case multiple atypical features need to be present.

*** Postural syncope or presyncope and/or urinary incontinence or retention not due to other causes.

Sporadic: one other case of typical clinical IPD among 1st or 2nd degree relatives allowable.

Frequency of individual clinical features in 203 cases of MSA

From Wenning, et al, MSA: A Review of 203 Pathologically proven Cases Movement Disorders Vol 12 No 2 1997 133-147

Autonomic symptoms

     Urinary incontinence      55%    

   Postural faintness           51%

   Impotence                    47%

   Recurrent syncope        18%

     Fecal incontince            12%

Parkinsonism

   Akinesia                     83%

   Tremor                       67%

     Rigidity                      63%

     Best L-Dopa response     

               Poor              72% 

               Good              28%

     Last  L-Dopa response

               Poor             95% 

               Good             5%

 Dyskinesias

               Orofacial        15%

               Limbs            10%

Fluctuations               24%

Cerebellar Signs

   Gait ataxia                      49%

   Limb ataxia                    47%

   Intention Tremor           24%

   Nystagmus                    23%

 

Pyramidal signs

   Hyperreflexia                  46%

   Babinski                         41%

   Spasticity                       10%

 

Other features

   Intellectual deterioration

       Mild                           22%

       Moderate                   2%

       Severe                        0.5%

       Stridor                      13%

         Dystonia                  12%

         Anisocoria                  8%

         Contractures              7%

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