Parkinson-Plus Syndromes: Introduction

by Roy Strowd, MD

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    00:01 In this talk, let's review some of the Parkinson's Plus Syndromes.

    00:06 These are Parkinsonian syndromes, patients present with a Parkinsonism, but they're not idiopathic PD.

    00:15 First, let's talk a little bit about Parkinsonisms.

    00:17 There are two basic Parkinsonisms.

    00:20 One is a levodopa-responsive Parkinsonism, and that's idiopathic PD.

    00:25 Those patients have bradykinesia, rigidity, postural instability, often with tremor, and they're readily responsive to levodopa challenge.

    00:34 The second is levodopa-nonresponsive Parkinsonisms and these are our PD-plus syndromes that we'll review in this talk.

    00:46 Let's start with an overview of some of the common Parkinson's plus syndromes and there's four that I'd like you to know.

    00:53 Lewy body dementia, multiple system atrophy, progressive supranuclear palsy, and corticobasal or corticobasal ganglionic degeneration.

    01:03 And there are a number of things that I want you to remember about these syndromes.

    01:07 1. They're all Parkinsonisms.

    01:10 So all four of these conditions present with bradykinesia, rigidity, and postural instability, that's really, really important to remember.

    01:19 However, there are also some unique features of each of these conditions that are unique to the condition and help us to establish the diagnosis.

    01:27 With Lewy body dementia, we see early dementia as a prominent feature in those patients.

    01:33 So dementia begins within one year of the development of Parkinsonian symptoms.

    01:39 We can also see hallucinations in these patients and neuroleptics sensitivity is common.

    01:44 We use neuroleptics to manage some of the symptoms we can see when patients are hallucinating or agitated, and these patients will develop severe sensitivity and become agitated and confused and may need hospitalization as a result of initiation of neuroleptics.

    01:59 And those features are supportive of a diagnosis of Lewy body dementia.

    02:04 Next multiple system atrophy.

    02:06 The key features to this syndrome is early autonomic dysfunction.

    02:11 So we think about orthostasis, or erectile dysfunction and incontinence, and early findings any of those three findings early in the course of the Parkinsonism should prompt the consideration for multiple system atrophy.

    02:26 Next, let's talk about progressive supranuclear palsy.

    02:30 The key things we see there are twofold.

    02:32 One is downgaze impairment.

    02:34 So these patients have early vertical downgaze palsy, they can't look down.

    02:38 As a result they have frequent falls and we see falling In Parkinson's disease, falls or late.

    02:44 In PSP, falls or early.

    02:46 This is also an axial, akinetic rigid Parkinsonism.

    02:51 So patients have difficulty moving and they are very, very rigid, often in the axial musculature that controls our posture and axial tone.

    03:01 Patients may have other brainstem symptoms, dysphasia or oral dyskinesias or dysphonia and then lastly, corticobasal degeneration.

    03:10 This is a really unique and kind of strange, uniquely strange syndrome.

    03:15 Here we see that it's prominently asymmetric.

    03:18 Patients begin with very asymmetric symptoms.

    03:21 And we see a few things, apraxia, alien hand and cognitive deficits and this owes to its predilection for the parietal lobes.

    03:29 Apraxia is motor planning, so these patients are able to move, but they just can't form the plan for how to do it and what they need to do.

    03:37 Alien limb is an extreme variant of that where a patient's limb will be alien to them.

    03:44 It will shave on its own, it will cook on its own, it will write things that the patient doesn't want to write.

    03:49 It literally is alien, it does what it wants to do, and the patient does not appear to be in control.

    03:55 Those findings of early visual-spatial dysfunction and early asymmetric apraxia, or alien limb should raise suspicion for corticobasal degeneration.

    04:07 Let's walk through each of these and understand why each of these Parkinsonisms present differently and look at the areas of brain that are involved.

    04:16 First, I want to look at idiopathic PD.

    04:18 And the problem that we think about is in the substantia nigra pars compacta, but it actually begins way down deep in the brainstem early.

    04:28 So the development of Lewy bodies that degenerative pathology that's developing in the brain begins down in the caudal medulla.

    04:35 And that early caudal medulla dysfunction likely leads to early REM behavior disorder.

    04:42 Slowly over time we see increase in Lewy Body deposition, further higher up in the brainstem and the pons, and ultimately in the basal ganglia contributing to the motor development of symptoms.

    04:54 And then over time, into the cortex where patients will develop late dementia, Parkinson's disease, dementia and other cognitive dysfunction.

    05:02 So again, early in the disease for idiopathic PD, we see REM behavior disorder anosmia.

    05:07 In the typical presentation, we see the motor symptoms as the subcortical basal ganglia structures are involved.

    05:14 And then late in the course of the disease as Lewy bodies develop in the cortex, we see dementia and other cortical dysfunction in these patients.

    05:22 This is different from progressive supranuclear palsy.

    05:26 And the distribution of neurodegeneration.

    05:29 It differs for PSP and helps us to determine what symptoms we see when patients present and things we want to look out for.

    05:37 PSP is a tauopathy.

    05:38 So the structure that's developing within the neurons, the degeneration that's occurring is a result of buildup of tau and we see tau deposition in the brainstem.

    05:49 And you can see in the upper diencephalon here, there is frequent deposition of tau and this gives rise to the vertical gaze dysfunction, the vertical gaze center is in the upper midbrain, as well as prominent axial rigidity that we see in this condition.

    06:05 We can contrast that to multiple system atrophy.

    06:09 And you can see the brain regions involved in multiple system atrophy.

    06:13 It is many, many multiple brain regions.

    06:16 And we can see predominance in one area or the other.

    06:19 But we often see in this condition, deposition of abnormal protein in both the subcortex, the brainstem and in the brain.

    06:28 MSA is alpha-synuclein apathy.

    06:31 And so the deposition that we're seeing is alpha-synuclein.

    06:35 In some cases, the alpha-synuclein develops and builds up in the cerebellum, and we may see an MSA cerebellar type.

    06:42 In other cases, it primarily affects the subcortical basal ganglia, and we can see an MSA striatonigral or Parkinsonian type.

    06:51 And lastly, we can see problems in the deep brainstem.

    06:54 And this gives rise to an MSA a or autonomic type.

    07:00 And then lastly, let's look at corticobasal degeneration understand the brain regions that are affected by this condition.

    07:06 This is also a tauopathy.

    07:08 And so we're dealing with development of tau protein and deposition of tau and you can see here the prominent involvement of the parietal lobes giving rise to the the higher level cortical sensory findings in these patients including that alien limb syndrome.

    About the Lecture

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

    Included Quiz Questions

    1. Levodopa non-responsive Parkinsonism
    2. Levodopa-responsive Parkinsonism
    3. X-linked dominant disorder
    4. Amyloidopathy
    1. Lewy body dementia
    2. Multiple system atrophy
    3. Corticobasal degeneration
    4. Progressive supranuclear palsy
    5. Alzheimer dementia
    1. Multiple system atrophy
    2. Corticobasal degeneration
    3. Progressive supranuclear palsy
    4. Lewy body dementia
    5. Parkinson disease
    1. Brainstem
    2. Basal ganglia
    3. Thalamus
    4. Occipital cortex
    1. Parietal lobes
    2. Frontal lobe
    3. Temporal lobe
    4. Occipital lobe
    5. Corpus callosum
    1. Basal ganglia
    2. Amygdala
    3. Arcuate gyrus
    4. Brainstem
    5. Mammillary bodies

    Author of lecture Parkinson-Plus Syndromes: Introduction

     Roy Strowd, MD

    Roy Strowd, MD

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