in this talk let's review some of the Parkinson's plus syndromes these are parkinsonian syndromes patients present with a parkinsonism but they're not idiopathic PD first let's talk a little bit about parkinsonisms there are two basic parkinsonisms one is a levodopa responsive parkinsonism and that's idiopathic PD those patients have bradykinesia rigidity postural instability often with Tremor and they're readily responsive to levodopa challenge the second is levodopa non-responsive parkinsonisms and these are our PD plus syndromes that we'll review in this talk 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 Lewy Body dementia multiple system atrophy Progressive supernuclear policy and corticobasal or corticobasal ganglionic degeneration and there are a number of things that I want you to remember about these syndromes number one they're all parkinsonisms so all four of these conditions present with bradykinesia rigidity and postural instability that's really really important to remember 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 with Lewy Body dementia we see early dementia as a prominent feature in those patients so dementia begins within one year of the development of parkinsonian symptoms we can also see hallucinations in these patients and neuroleptic sensitivity is common 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 and those features are supportive of a diagnosis of Lewy Body dementia next multiple system atrophy the key features to this syndrome is early autonomic dysfunction so we think about orthostasis 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 next let's talk about Progressive supranuclear palsy the key things we see there are twofold one is down gaze impairment so these patients have early vertical down gaze palsy they can't look down as a result they have frequent Falls and we see falling in Parkinson's disease Falls are late and PSP Falls or early this is also an axial a kinetic rigid parkinsonism so patients have difficulty moving and they are very very rigid often in the axial musculature that controls our posture and axial tone patients may have other brain stem symptoms dysphagia or oral dyskinesias or dysphonia and then lastly corticobasal degeneration this is a really unique and kind of strange uniquely strange syndrome here we see that it's prominently asymmetric patients begin with very asymmetric symptoms and we see a few things apraxia alien hand and cognitive deficits and this owes to its predilection for the parietal lobes 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 alien Lin is limb is an extreme variant of that where a patient's limb will be alien to them it will shave on its own it will cook on its own it will write things that the patient doesn't want to write it literally is alien it does what it wants to do and the patient does not appear to be in control those findings of early visioospatial dysfunction in early asymmetric apraxia or alien limb should raise suspicion for corticobasal degeneration let's walk through each of these and understand why each of these parkinsonisms presents differently and look at the areas of brain that are involved first I want to look at idiopathic PD and the problem that we think about is in the substantia Paris compacta but it actually begins way down deep in the brain stem early so the development of Lewy bodies that degenerative pathology that's developing in the in the brain begins down in the caudal medulla and that early caudal medulla dysfunction likely leads to early REM behavior disorder slowly over time we see increase in Lewy Body deposition further higher up in the brain stem and the pons and ultimately in the basal ganglia contributing to the motor development of symptoms and then over time into the cortex where patients will develop a late dementia Parkinson's disease dementia and other cognitive dysfunction so again early in the disease for idiopathic PD we see REM behavior disorder anosmia in the typical presentation we see the motor symptoms as the subcortical basal ganglia structures are involved and then laid in the course of the disease as Lewy bodies develop in the cortex we see dementia and other cortical dysfunction in these patients this is different from Progressive super nuclear palsy and the distribution of neurodegeneration differs for PSP and deter helps us to to determine what symptoms we see when patients present and things we want to look out for PSP is a talopathy so the um the structure that's developing within within the neurons the degeneration that's occurring is a result of buildup of Tau and we see Tau deposition in the brain stem 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 we can contrast that to multiple system atrophy and you can see the brain regions involved in multiple system atrophy it is many many multiple brain regions and we can see predominance in one area or the other but we often see in this condition deposition of abnormal protein in both the subcortex the brain stem and in the brain MSA is an alpha synucleinopathy and so the deposition that we're seeing is Alpha synuclein in some cases the alpha synuclein develops and builds up in the cerebellum and we may see an MSA cerebellar type in other cases it primarily affects the subcortical basal ganglia and we can see an MSA striatonigral or parkinsonian type and lastly we can see problems in the deep brain stem and this gives rise to an msaa or autonomic type and then lastly let's look at corticobasal degeneration understand the brain regions that are affected by this condition this is also a tallopathy 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 um the higher level cortical sensory findings in this in these patients including that allium limb syndrome [Music] thank you