MSA Overview in Continuum, August 2016

An overview of diagnosing atypical parkinsonian disorders, including multiple system atrophy (MSA), was published in the August 2016 issue of Continuum, a journal for neurologists.

The article describes MSA as follows:

MSA is characterized by variable presentations of parkinsonism, cerebellar and pyramidal signs, and autonomic dysfunction. … Two clinical phenotypes are generally distinguished by predominant parkinsonism (MSA–parkinsonian type [MSA-P]) or predominant cerebellar ataxia (MSA–cerebellar type [MSA-C]). Median age of onset for MSA is 58 years of age, which is younger than that of PSP and CBD. No MSA cases have been identified younger than age 30, whereas for PSP the cutoff age is 50 years. Disease progression is faster than in PD and mean survival is approximately 6 to 9 years, consistent with more widespread neurodegeneration.

There’s nothing new in the article (except the very brief mentions of dementia in MSA – a bit more on that below) but it provides a good summary of current knowledge about MSA.  The article discusses:

* MSA-P symptoms, including parkinsonism, tremor (distal, myoclonic), rigidity, dystonia, anterocollis, early falls, dysarthria, dysphonia, autonomic failure (69%), dyskinesia (orofacial common).  A case study of someone with MSA-P is provided.

* MSA-C symptoms, including limb ataxia, gait ataxia, early falls, dysarthria (ataxic), dysphagia, gaze impairment, emotionality

* MSA pathology

* MSA diagnostics

* MSA treatments

Some excerpts are copied below.

The author lists “progressive dementia” as a possible symptom of MSA-C.  And, the author points to an article from 2010 that estimates “dementia in 14% to 16% of patients with MSA.”  I’ll have to read that 2010 article to see if there’s autopsy confirmation and if they really mean “dementia” or “cognitive impairment.”ed below.

The abstract is available at no charge. Amazingly, the full article seems to be available at no charge; grab it while you can! See:

Diagnostic Approach to Atypical Parkinsonian Syndromes.
Nikolaus R. McFarland
Continuum. 2016 Aug;22(4 Movement Disorders):1117-42.
PubMed ID#: 27495201

Link to Abstract

Link to Full article

Happy reading!


Excerpts from
Diagnostic Approach to Atypical Parkinsonian Syndromes.
Nikolaus R. McFarland
Continuum. 2016 Aug;22(4 Movement Disorders):1117-42.


* Multiple system atrophy–parkinsonian type may be differentiated from Parkinson disease by its more symmetrical appearance, atypical tremor, dystonia (antecollis), early dysarthria/dysphonia, gait and postural instability, dysautonomia, and rapid progression.

* Multiple system atrophy–cerebellar type is one of the most common causes of sporadic, adult-onset ataxia and is distinguished by parkinsonism, dysautonomia, and rapid progression.

* Pharmacologic treatment of orthostatic hypotension may include enhancing blood volume with fludrocortisone or desmopressin or adding drugs that increase vascular resistance such as midodrine, droxidopa, or pyridostigmine.