PARKINSONISM Flashcards
Role of BASAL GANGLIA
- initiation of movements
- modulation of movement
(relays info recieved from the cerebral cortex BACK to the cerebral cortex)
What occurs with basal ganglial disease?
- HYPOKINETIC or HYPERkinetic movement disorders
DIsease of the corticospinal/pyradmidal tract results in __________ and ______
- SPASTICITY
- PYRAMIDAL weakness
(UMN fts)
Disease of the cerebellum results in ________
ATAXIA
Disease of Basal Ganglia
- Hyperkinetic MD (dystonia/ Tics/ myoclonus/ chorea/ tremor)
- Hypokinetic MD
- parkinsonism
- parkinson’s disease
Pathological hallmark of parkinson’s?
- loss of dark pigment in the substantia nigra and locus ceruleus
- pigment loss correlates with dopaminergic cell loss
- LEWY bodies on histology
Loss of how many percent of dopaminergic neurones is requires for symptoms to become clinically apparent.
50-60% of neurones from the PARS COMPACTA of the SUBSTANTIA nigra
What are lewy bodies
- misfolded a-synuclein : insoluble and aggregated will form intracellular inclusions
- —LEWY bodies may also be seen in spinal cord and the peripheral nervous system
What are the motor symptoms of parkinson’s?
Tremor
Rigidity
Bradykinesia
postural instability
Non-motor symptoms of Parkinson’s?
- sleep d.o
- hallucinations
- GI dysfxn
- depression
- cognitive impairment/ dementia
- anosmia
According to BRAAK staging of LEWY pathology what are the first abnormalities seen?
- olfactory bulb
- enteric nervous system
What are the 2 subtypes of PD?
Which subtype has a small rate of progression?
- Tremor Dominant (relative absence of the other motor symptoms) —SLOWER progression (less functional dysability)
- Non-tremor dominant PD (akinetic-rigid syndrome and postural instability gait d.o)
- MIXED
Name a few prodromal symptoms of PD?
- constipation
- REM sleep Behavious d.o
- EDS/ HYPOSMIA/ DEPRESSIOn
What are additional fts which help in dx of PD?
- Bradykinesia AND one or MORE of the following: RESTING tremor, rigidity, postural instability
- ADDITIONAL motor fts (STOOPED, fixed posture, DYSTONIC postures, hypomimia, shuffling, short-stepped gait
To confirm dx of parkinson’s, what should the pts NOT present with?
- esrly onset bulbar problems, hallucinations, dementia, preferential involv. of lower limbs
- prominent eye movement
- intrusive early autonomic problems
If dx tests are needed, what invx are available?
- structural brain imaging
- SPECT (DaTSCAN)
What is seen on DaTSCAN to confirm PD dx?
- period-shaped
What may be ddx for parkisons?
- MPTP exposure
- NEGATIVE response to large doses of levodopa (malabsorpt. excluded)
- babinski sign
- cerebellar signs
- supranuclear gaze palsy
- cerebral tumor/ communicating hydrocephalus on MRI/CT
- early autonomic involvement
What must be ruled out when considering postural instability as a PD sign?
- should not be caused by primary visual, cerebellar, vestibular, proprioceptive dysfxn
Who is most likely to get PD?
- men
- of advancing age
- w. FAMILY HX
What suggests genetic cause of PD?
- early onset (< 40 y.o)
What gives rise to idiopathic PD?
- susceptible genes+ env. triggers+ AGE
Name a few env. triggers.
- pesticide and manganese exposure
- prior head injury
- rural living
- beta-blocker use
- well water drinking & agricultural job
- mining, welding jobs
What reduces the risk?
- tobacco smoking
- coffee drinking
- alcohol
- calcium channel blocker
- NSAID use
How many genes are involved in PD?
- mutations in 11 genes
Name 2 important monogentic forms of PD?
- LRRK2
- PARKIN