Flashcards in Hypokinetic Movement Disorders (18, part 1) Deck (47)
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31
What is apraxia of eye opening, another opthalmologic feature of PSP?
Difficulty opening closed eyes when asked to do so--> but can still do it spontaneously
32
What signs may be present in PSP that is not present in PD?
Pyramidal tract signs - like positive Babinski; indicates upper motor neuron damage
(Sleep disturbances may be present as well)
33
Grossly, what 2 regions of the brain show atrophy in progressive supranuclear palsy?
Midbrain and cerebral cortex
34
What protein is abnormally deposited in progressive supranuclear palsy? What structures are seen?
Tau protein--> seen in neurofibrillary tangles (unpIred straight filaments contained phospharylated tau protein)
35
What three structures in brain show neuronal loss and gliosis in PSP?
1. Globus pallidus
2. Pedunculopontine nucleus
3. Substantia nigra
36
What is the most prominent neural helical abnormality of PSP?
Nigrostratial dopaminergic deficiency
37
What is the age range of onset for Multiple System Atrophy?
50-55 (also younger than PD); similar to PSP however
*life expectancy ~ 5-10 years (maybe shorter than PSP)
38
What three different clinical patterns may be observed in Multiple System Atrophy?
1. Parkinsonism (eventually develops in ~90%)
2. Progressive autonomic failure
3. Cerebellar syndrome
*used to be considered 3 distinct diseases
39
What two neurological symptoms does multiple system atrophy share with PD? What features are similar to PSP?
Rigidity and Bradykinesia are typically seen
-like PSP, postural instability occurs early and also tremor is unusual
40
Although it doesn't affect all patients, what general features are considered a clinical hallmark of Multiple system atrophy?
Progressive autonomic failure - orthostatic hytension is perhaps the most widely recognized
*appears earlier and is more sever than that seen in PD; seen in 78% of pts with MSA
41
What are 4 symptoms that may be due to cerebellar dysfunction in MSA?
Ataxia, dysarthria, coulombs or abnormalities, exaggerated rebound
*may only be evident in 55% of patients
42
What pyramidal feature may be seen in MSA that is not seen in the others? What is one shared by PSP?
1. Hyperreflexia
2. Positive Babinski
43
What behavioral condition EXCLUDES a diagnosis of MSA? Which may be more characteristic for MSA than the others?
Dementia- not seen in MSA and may exclude the dx (seen only in PSP)
Personality changes - more characteristic of MSA
-also shares executive dysfunction as a feature, along with PD
44
What are two "other" features that are characteristic of MSA? Which one may increase the risk of sudden death?
1. Involuntary sighing
2. Respiratory stridor
*Raynaud's and postural myoclonus are additional characteristic features
45
What two areas of brain outside of brainstem, cerebellum, and spinal cord are characterized by cell loss and gliosis in Multiple System Atrophy?
Substantia nigra and posterior putamen
47
What is the typical age of onset for Progressive supranuclear palsy?Life expectancy?
50-60 ( so somewhat younger than Parkinson's; is also more rare)
Life expectancy is only about 10 years after dx- there is currently no effective treatment
48
