Additional BG Pathologies Flashcards
What’s the difference between primary and secondary parkinsonisms
Primary is Parkinson’s
Secondary: BG damage secondary to something else
What’s the difference between primary and atypical parkinsonisms
atypical: less effective response to meds, progress faster, higher mortality than primary
Etiology unknown, the pathophysiology behind PSP (progressive supranuclear palsy) involves accumulation of _. protein. Among others, brainstem structures, thalamus,and _,atrophy along with astroglial cells and neurons.
tau; basal ganglia
How is PSP diagnosed? What specific MRI findings are involved? (hint: (frontal lobe, subthalamic and putamen atrophy)
early clinical exam is primary and onset typically in 60s
MRI: morning glory sign, hummingbird sign
What is PSP commonly misdiagnosed as?
depression, dementia, Parkinsonisms
What are 5 signs and symptoms unique to PSP?
early gait disturbance
rocket sign and axial rigidity
pseudobulbar affect
Mona Lisa stare
loss of eye movement/lid ctrl (esp vertical)
How is PSP managed?
not really effectively managed–sometimes dopamine, botox and antidepressants
How is the prognosis for PSP?
poor- severe disability in 3-5 yrs, mortality in 5-8.
what serious complications may arise in PSP pts?
pneumonia and falls
Describe MSA (multiple system atrophy).
a rare neurodeg dx that also includes autonomic dysfunction
What are the two subtypes of MSA and how do they differ?
MSA-P (parkinsonian)
MSA-C (cerebellar)-later onset/slower progression
they are named for which s/s present first
How is MSA diagnosed? What specific MRI findings are involved?
usually in early 50s (rarely past 70s) via clinical exam
+PET, DaTscan, MRI (hot cross bun sign of pons atrophy)
What is MSA commonly misdiagnosed as?
PD> pts don’t respond to dopamine therapy
Etiology unknown, the pathophysiology behind multiple system atrophy involves accumulation of ____with the most involvement observed in ____ (cell type)
proteins in glial cells, oligodendrocytes
The signs & symptoms associated with premotor phase MSA are sexual dysfn, urinary incontinence, OH, and ____
inspiratory stridor, REM behavior
The signs & symptoms associated with MSA are sleep disorders, oculomotor disturbance (MSA-C), antecollis, dyskinesia, and (4)?
Autonomic symptoms (esp OH), symmetric PD s/s,, coat hanger pain, Pisa syndrome,
how is MSA managed?
PD meds (temp) and used w/ caution for OH effects
potentially surgical trach for feeding with later progression
symptom management
How is the prognosis for MSA?
poor-almost half are dep in 8 years w/ mortality within 5-10 years
what serious complications may arise in MSA pts?
cardiac and respiratory failure, urosepsis and sudden death
what s/s is rarely seen in pts with MSA?
cognitive deficits
What is the average age of onset of Corticobulbar Degeneration (CBD)?
60-80 years old
women> men
Etiology unknown, the pathophysiology behind CBD involves focal ____ and ____neuronal loss –specifically in glial cells such as ____.
cortical and substantia nigra lesions; astrocytes
An MRI image of CBD would provide the appearance of “brain shrinkage” via _____
over pronounced sulci
T/F: The Cognitive impairments related to corticobasal degeneration (Dementia, AD, FBSS) tend to present later than that of progressive supranuclear palsy.
False.
Aside from asymmetrical PD s/s, the signs & symptoms unique to CBD are visuospatial impairments, apraxia, and ____ (2)
myoclonus and progressive aphasia
T/F: There is currently no cure available for CBD and treatment is largely supportive (symptom based)
True.
CBD prognosis indicates an average survival of ____ years from symptom onset.
6-8 but there are cases of people surviving >10 years.
_____is not a “Parkinsonism” but a BG diagnosis characterized by involuntary muscle contractions >slow, repetitive movements/ abnormal postures
dystonia
the 5 classifications of dystonia are:
focal-1 body part
segmental (2+ continuous body parts)
multifocal (2+ noncontinuous parts)
generalized (trunk and 2+body parts)
hemi
Dystonia is typically onset at childhood and early adulthood and the difference in presentation is that
early onset presents w/ ____while adult onset presents with ____
limb involvement; neck/facial involvement
Dystonia is typically ____but can also be genetic or acquired and there aren’t any identifiable damages on imaging.
idiopathic
what are the 4 common initial s/s of dystonia? (hint: 3 in foot, 1 in hand). Generally speaking these may lead to contracture.
foot: cramping, toe drag, and/or excessive inversion @ swing
hand: cramping while writing
what are the effects of eyelid and neck/trunk dystonias?
eyelid: excessive blinking
neck/trunk: postural abnormality
T/F: Dystonia can be circumstantial and only impact specific movements.
true
What are the 4 pharmacologic treatment options for dystonia and which have the highest success rate?
Botox
but also anticholinergics, GABAergic and dopaminergic agents have mixed reviews.
___,___, ____,and____ are surgical intervention options for dystonia that aren’t usually a go-to due to invasiveness. Of them___is the most common.
Deep Brain stimulation, thalamotomy, pallidotomy, and anterior cervical rhizotomy
DBS
_____has no direct mortality prognosis or commonly fatal secondary complications
Dystonia
_____is a drug induced dx that involves uncontrolled stiff and jerky movements of the face/body. The primary culprits are____ and ______
tardive dyskinesia; amphetamine/meth/haloperidol and phenothiazines (for schizophrenia)
risk factors for TD are the amount/long term use of the drug, drug. abuse, and __(3 demographic RFs)
age >55, women>men, race (Black and Asian)
TD is dx via clinical exam and med review. Its treated via Deutetrabenzine, Valbenzine or ____
Cessation!
Huntington’s Disease is a ____autosomal trait that results in ___activity of basal nuclei.
Dominant
hyper-
The pathophysiology of Huntington’s involves severe loss of neurons in the ___ and putamen, among other lobes, that resembles ______on imaging.
caudate,
dilation of lateral ventricles
In HD, the indirect “no-go” pathway is hit first causing ______. Next, the direct “go” pathway is hit and we see more widespread loss and s/s similar to ____
disinhibition of thalamus (runnin amuck)
PD
HD is characterized by unique s/s including mood changes, cognitive impairment (including dementia and OCD) , and ____(3)
choreiform movements, motor impersistence, and PD s/s in later stages.
Pharmacologic treatment for Huntington’s is typically introduced when ….
chorea interferes w/ fn
How is the prognosis for Huntington’s?
mortality: 15-25 after onset
causes: often respiratory failure, suicide common
Our treatment plan for PSP and MSA would likely favor (compensation/remediation). Additionally, emphasisis should be placed on maintaining (distal/proximal) strength, stretch and positioning.
compensation, proximal
PT treatment considerations for dystonia and chorea
can be remembered as R.I.S.C.S. This stands for____
-relaxation techniques (may refer to psych)
-ID triggers and tricks
-Serial casting for severe cases
-Cog and Sensory training
T/F Conservative treatment (PT) using postural ctrl, balance reactions and biofeedback are typically the first line of defense for tardive dyskinesias.
False. We can do these things and should if the damage is permanent. But stopping the causative drugs is the first-step.
neck extensors, ____ and muscles of the hand/feet are common areas of weakness in people with HD.
postural trunk muscles
Early stage HD exercise recs include:
aerobic exercise, strengthening, stretch/ROM, coordination and advanced balance activity
Middle/Late stage HD exercise recs include:
shorter and more frequent activity bouts
salient practice of functional activity
Fall prevention
In addition to characteristic s/s of the dx, these common gait deviations make HD patients more of a fall risk (and hence can direct your treatment):
-narrow BOS
-variable step length
- frequent/drastic trunk displacement
-difficulty w/ dual tasks
What are the major problems associated with early stage HD?
chorea
gait
balance/flexibility
fine motor
What are the major problems associated with middle stage HD?
early stage +
falls
joint hypomobility
weak postural stabilizers
What are the major problems associated with late stage HD?
previous stages +
overall mobility
respiratory limitations (pneumonia risk)
Dysphagia is a common s/s in these dx: (4)
PSP
MSA
CBD
HD
Dystonia/Ataxia are common s/s in:
All of them!
HD
TD
dystonia
CBD
MSA
PSP
What makes up the clinical triad of fronto-behavioral-spatial syndrome
Executive dysfunction, behavioral/personality changes, Visio spatial deficits
Infection/ post-encephalitis, atherosclerosis, and _____ are common etiologies behind secondary Parkinsonisms.
toxicity/ drugs
