Parkinson Plus Syndromes Flashcards
There are 2 predominant types of multiple system atrophy.
What are they? [2]
There are 2 predominant types of multiple system atrophy
1) MSA-P - Predominant Parkinsonian features
2) MSA-C - Predominant Cerebellar features
Describe the general presentation of MSA [3]
MSA is an adult-onset, rapidly progressive disease that is characterised by profound autonomic dysfunction leading to severe postural hypotension, urogenital dysfunction and a plethora of other features including cerebellar and corticospinal features. There is a poor response to treatment.
Features
* parkinsonism
* autonomic disturbance
* erectile dysfunction: often an early feature
* postural hypotension
* atonic bladder
* cerebellar signs
Describe the featurs of progressive supranuclear palsy [5]
What imaging result would indicate PSP? [1]
Early postural instability and frequent falls.
Impairment of vertical gaze (particularly DOWNGAZE).
Stiff, broad-based gait.
Parkinsonism (bradykinesia, rigidity).
An MRI of the brain may show a hummingbird sign, a characteristic shape of midbrain atrophy seen in PSP.
Describe the presentation of Corticobasal Degeneration (CBD) [4]
- Unilateral parkinsonism, typically affecting one limb.
- Alien limb phenomenon (patients feel that their limbs move involuntarily outside of their control
- Cortical sensory loss (patients cannot recognise objects by touch despite normal sensory input).
- Apraxia (difficulty with purposeful movement despite intact motor function).
Describe the presentation of dementia w/ Lewys bodies [3]
Dementia with Lewy bodies is a type of dementia associated with features of Parkinsonism. It causes a progressive cognitive decline.
There are associated symptoms of visual hallucinations, delusions, REM sleep disorders and fluctuating consciousness.
QM
Suspect dementia with lewy bodies in patients with parkinsonism and which features? [2]
Early (within 1 year of onset) cognitive dysfunction
Visual hallucination
QM
What are the mean features of multiple system atrophy type P? [1]
Multiple system atrophy type P is characterised by parkinsonian features, with profound central autonomic signs
Suspect multiple system atrophy in patients with parkinsonism and which features? [2]
Prominent autonomic dysfunction
Cerbellar or pyramidal features
In addition to typical Parkinsonian features, what is the cardinal sign of MSA? [3]
Early autonomic features (eg. postural hypotension, incontinence, impotence).
What are the main features of multiple system atrophy type C?
Multiple system atrophy type C is characterised by cerebellar features, with profound central autonomic signs
QM
In addition to typical Parkinsonian features, what is the cardinal sign of cortico-basal degeneration? [1]
Spontaneous activity by a limb, or akinetic rigidity of a limb.
Suspect progresive supranuclear palsy in patients with parkinsonism and which features? [3]
Eye movement disorder (vertical gaze palsy)
Early postural instability and axial rigidity
Early cogntive dysfunction
In addition to typical Parkinsonian features, what is the cardinal sign of Lewy body dementia? [2]
Early fluctuations in cognitive impairment and visual hallucinations.
Name 4 different classes of Parkinson’s Plus Syndromes [4]
State very basic manifestations of each
Progressive Supranuclear Palsy (PSP)
- often fall earlier in disease due to gaze restriction
Multiple Systems Atrophy (MSA) Early and more pronounced autonomic symptoms.
- MSA-P: more akin to PD but might have more autonomic symptoms than idiopathic PD. Often have dry eyes
- MSA-C: gait dysfunction; autonomic dysfunction required for dx
Corticobasal Degeneration (CBD)
- Apraxia, limb dystonia
Lewy Body Dementia (LBD)
- Fluctuating cognition, visual hallucinations, Parkinsonism, and sleep disorders.
Describe the difference between PD dementia and Dementia with Lewy body [1]
Parkinson’s disease dementia:
- dementia occurring in the setting of established Parkinson’s disease. This is defined as having Parkinson’s disease for more than one year before the onset of dementia.
Dementia with Lewy body:
- development of dementia and Parkinsonism concurrently. This is defined as developing dementia within one year of Parkinsonism features
How do you differentiate between PD and PSP? [2]
PSP is characterised by vertical gaze dysfunction, dysarthria and cognitive decline.
Tremor is rare in this condition.
How do you differentiate between PD and LBD? [3]
Lewy-body dementia, the dementia usually occurs concomitantly with, or prior to, the development of parkinsonism
DLB is characterised by early onset dementia (< 1 year)
Characterised by a triad of visual hallucinations, fluctuating cognition, and parkinsonism
Parkinsonism, autonomic disturbance, cerebellar signs - multiple system atrophy
MSA
