sub-cortical dementia: progressive supranuclear palsy

Terms in this set (18)

which dementia is consider the "prototypical subcortical dementia?

PSP, because it help to bring to create the category of subcortical dementia

Whats the age onset of PSP and survival rate of PSP?

PSP is mostly a geriatric rare disease with a mean=65 age onset and a median survival of 6 years

How do we differentiate btw PSP and PD

Due to its similar symptoms, PSP is commonly misdiagnosed as PD, but if give PD treatment (L-DOPA) to PSP patients it wont work

Which parts of the brain does PSP affects and what symptoms does it cause?

-Subthalamic nuclues: Motor/visual disturbance
-Subtantia nigra: Motor deficits
-superior colliculus: Visual disturbance
-inferior colliculus: attention focus to auditory stimuli
-striatum: cognitive deficits

what are 2 of the classic symptom of PSP?

Vertical gaze palsy/down gaze abnormalities and we see greater impairment of Executive/prefrontal functions

Conjugate gaze palsies

-neurological disorders affecting the ability to move both eyes in the same direction. These palsies can affect gaze in a horizontal, upward, or downward direction

PSP Motor symptoms

-gait impairment (slowed wide based)
-Dysarthia
-bradykinesia
-rigidity and/or resting tremo
-swallowing difficulties
-postural instabillity with falling down (backwards)
-Downward gaze abnormalities

PSP Dementia syndrome

-forgetfulness
-bradyphrenia
-personality changes like apathy and depression
-pseudobulbar palsy

pseudobulbar palsy

an inability to control the muscles in the face; impacts a person's ability to speak and swallow; also causes uncontrollable crying or laughing at inappropriate times. Mostly unilateral

PSP Cognitive symptoms

-Prefrontal/executive (disproportionately impaired)
-perseveration
-grasping reflex
-utilization behavior
-Poor orientation knowledge of place in time
-Slowed or impaired reasoning, much more than PD
-Apathy
-inertia

utilization behavior

characterize extreme dependency on prototypical responses

Inability to stick to a task, often leaves without finishing. Ex: Goals, sentences etc

PSP cognitive symptoms: Language

-Processing and comprehension relatively spared
-Slowed speech
-dysarthria

difficult or unclear articulation of speech that is otherwise linguistically normal.

PSP cognitive symptoms: Memory

-Mildly impair, complain of forgetfulness
-Retrieval is more impair than recognition

What is the physiological brain hallmark of PSP?

"Pinguin sign" in the midbrain, on top of the pons

No effective treatment exist. Dopaminergic agents help some patients, but most are not. Main goal is to monitor the progress of the disease

Difference btw PD and PSP

PD
-Atrophy of substantia nigra
-mild/moderate Executive dysfunction
-usually responsive to L-DOPA
PSP
-atrophy of several subcortical areas
-severe executive dysfunctions
-poor response to drug therapies
-downward gaze palsy

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