Parkinson-Plus Syndromes: Multiple System Atrophy

by Roy Strowd, MD

My Notes
  • Required.
Save Cancel
    Learning Material 2
    • PDF
      Slides Parkinson-Plus Syndromes.pdf
    • PDF
      Download Lecture Overview
    Report mistake

    00:01 All right, well let's talk about and learn a little bit more about the four common Parkinson's-plus syndromes, and we're going to review each of them.

    00:09 First, let's start with multiple system atrophy.

    00:12 MSA is an adult onset sporadic, rapidly progressive multisystem, neurodegenerative fatal disease that's characterized by Parkinsonian features, so bradykinesia, rigidity, postural instability.

    00:25 And it can also include cerebellar dysfunction, autonomic dysfunction, or just primary Parkinsonian symptoms.

    00:33 We often see urogenital dysfunction and prominent autonomic features, as well as in some cases, corticospinal disorders.

    00:43 What's going on in MSA? Well, what we see here is spreading of aberrant alpha-synuclein.

    00:50 MSA is an alpha-synuclein apathy like idiopathic parkinson's disease.

    00:56 There spreading of alpha-synuclein deposits from neurons to glial cells.

    01:00 This results in chronic alterations in glial cell function.

    01:04 There's impairment of the trophic function between the oligodendrocytes and the axons, as well as secondary axonal damage and extensive demyelination that is occurring.

    01:14 The glial and the myelin are both involved in multiple system atrophy and the pathology is spread throughout the brain.

    01:23 There is an inflammatory cascade that is thought to be induced by this process, and then subsequently secondary neurodegeneration is thought to follow.

    01:33 What are the clinical manifestations what a patients present with? Well, the history is important.

    01:38 With MSA, we see early autonomic dysfunction.

    01:41 Orthostasis, erectile dysfunction and incontinence.

    01:44 We need to ask for those, we need to interrogate those.

    01:47 They're not always apparent when evaluating a patient and their key features to cue in on in a test question or clinical exam.

    01:57 On physical exam, we're looking for Parkinsonian symptoms as well as other associated symptoms.

    02:02 And there are three types of the MSA.

    02:04 MSA of the Parkinsonian type, MSA of the cerebellar type, and MSA that just involves early autonomic dysfunction.

    02:12 In Parkinsonian predominant cases, we see akinesia or bradykinesia, prominent axial rigidity, postural instability.

    02:20 We may or may not see tremor in the vast majority of these patients, we don't see tremor.

    02:25 In contrast, a Parkinson's disease and cognitive function is typically preserved though we may see some subcortical dementia early in the disease.

    02:34 In the cerebellar predominant cases, we see early ataxia.

    02:38 We can see problems with equilibrium and gait problems in those patients.

    02:43 Dysdiadochokinesis or difficulty with rapid alternating movements is present on exam and we really want to look for that when evaluating patients with a possible Parkinson's-plus syndrome.

    02:55 And then dysarthria, as well as ocular abnormalities can be observed.

    03:01 The diagnosis is made clinically we don't use imaging to diagnose multiple system atrophy.

    03:07 But we can see imaging abnormalities and I'm demonstrating some of the more severe and significant in stage changes that we can see on the brain.

    03:15 Here we're looking at MRI scans of the brain, the far left is a T2 and the others are sagittal T1 images.

    03:22 All the way on the right is normal.

    03:23 And we're looking at that midline, that parasagittal image, we see the brainstem, a nice healthy pons, midbrain on top of the pons, and a dominant corpus callosum with the cortex above it.

    03:38 In moderate to severe cases of MSA, we can see atrophy of those subcortical brainstem and cerebellar circuits.

    03:47 Here you can see on the axial section all the way to the left, atrophy of the middle cerebellar peduncles.

    03:54 There's prominent basal cisterns, you see more spinal fluid in those basal cisterns than you would normally.

    04:00 And this is indicative of atrophy.

    04:02 We can't see a lesion.

    04:03 We see what's leftover from neurodegeneration that's atrophy.

    04:07 Similarly, on the 2 sagittal images in the middle, we see atrophy of the olive and the pons and the olivopontocerebellar circuits.

    04:20 And you can see mild atrophy on the left image and then more severe atrophy on the right.

    04:25 And all of this gives rise to Parkinsonian features and likely prominent cerebellar findings in these patients.

    04:35 Now let's talk a little bit about some of the MSA variants.

    04:38 There's an MSAP or a Parkinsonian type.

    04:41 It was formerly called striatonigral degeneration, and this is MSA with prominent Parkinsonian features.

    04:48 We may see tremor, we see the typical Parkinsonian signs, and early autonomic failure and that's really what differentiates this from PD.

    04:57 In the cerebellar type, this was formerly called olivopontocerebellar atrophy.

    05:02 And like the MRI scans we looked at in the last slide, we see prominent olive, pons and cerebellar atrophy.

    05:09 Here, the patients present with prominent cerebellar dysfunction, gait ataxia, limb ataxia, ataxic, dysarthria, gaze-evoked nystagmus, axial rigidity problems and dysmetria.

    05:22 In the MSA-A type, this was formerly known as shy drager disease.

    05:27 This is MSA with prominent autonomic features and patients present with a predominance of orthostatic hypotension, as well as potentially incontinence and erectile dysfunction.

    05:38 And this orthostatic hypotension is very, very difficult to control.

    05:42 Patients stand up and become hypotensive and pass out.

    05:45 And many of them develop supine hypertension.

    05:48 So they lay down and they're hypertensive.

    05:50 They stand up and they're hypotensive.

    05:51 And it's very difficult to control their autonomic nervous system externally.

    05:59 How about treatment? How do we treat MSA? Well, there's no definitive disease modifying therapy.

    06:05 We do think about supportive care therapy.

    06:08 Botox injections can be used for focal dystonia is that can develop.

    06:12 Orthostatic hypotension is the most significant symptom we manage.

    06:16 We think about using agents that will increase blood pressure.

    06:19 Fludrocortisone is first line and midodrine is often second line and occasionally we'll need third or other fourth line agents.

    06:26 And urogenital symptoms are not uncommon and we consider agents to treat a neurogenic bladder.

    About the Lecture

    The lecture Parkinson-Plus Syndromes: Multiple System Atrophy by Roy Strowd, MD is from the course Parkinson-Plus Syndromes.

    Included Quiz Questions

    1. Multiple system atrophy
    2. Lewy body dementia
    3. Parkinson disease
    4. Alzheimer disease
    5. Corticobasal degeneration
    1. Alpha-synucleinopathy
    2. B-amyloidopathy
    3. Tauopathy
    4. Prion disease
    1. Neurons spread aberrant alpha-synuclein to glial cells, leading to an inflammatory cascade.
    2. Alpha-synuclein deposits in the brainstem and basal ganglia, leading to autolysis.
    3. Amyloid infiltrates the gray matter, creating a nidus for alpha-synuclein binding.
    4. Alpha-synuclein mediates degeneration of the frontal and temporal regions of the brain.
    1. Both erectile dysfunction and orthostatic hypotension
    2. Hallucinations
    3. Erectile dysfunction
    4. Orthostatic hypotension
    5. Both orthostatic hypotension and hallucinations
    1. Subcortical brainstem and cerebellar atrophy
    2. Frontal and temporal lobe atrophy
    3. Frontal and parietal lobe atrophy
    4. Biventricular enlargement
    1. Fludrocortisone
    2. Epinephrine
    3. Phenylephrine
    4. Propranolol
    5. Ginkgo biloba

    Author of lecture Parkinson-Plus Syndromes: Multiple System Atrophy

     Roy Strowd, MD

    Roy Strowd, MD

    Customer reviews

    5,0 of 5 stars
    5 Stars
    4 Stars
    3 Stars
    2 Stars
    1  Star