Parkinsonism and parkinsons disease Flashcards
CST/pyramidal tract leads to what kind of features
UMN - pyramidal weakness, spasticity
basal ganglia leads to what kind of symptoms [extra pyramidal]
hyperkinetic - dystonia, tics, myoclonus, chorea, tremor
hypokinetic - parkinsonism, parkinsons disease
cerebellum leads to what kind of symptoms
nystagmus
ataxia
intention tremor
what is the parkinsonian syndrome
rigidity, akinesia/bradykinesia and resting tremor
what is dystonia
prolonged muscle spasms and abnormal posture
what is chorea - ballismus
fragments of movements flow irregularly form one body segment to another causing a dance like appearance
ballismus: if amplitude of these movements is large
what motor symptoms does PD present with
tremor, muscle rigidity, akinesia, rest tremor and gait and postural impairment
what are motor features in PD divided into
what does a certain course have on the prognosis
tremor dominant - with relative absence of other motor symptoms
non-tremor dom - such as akinetic - rigid syndrome and postural instability gait disorder
mixed
tremor dom - slower rate of progression and less functional disability
clinical non motor features of PD
when are they common and what are they associated with
olfactory dysfunction, cognitive impairment, psychiatric symptoms, sleep disorders, autonomic dysfunction, pain and fatigue
common in early PD and before the onset of motor features and assoc with reduced health related quality of life
when is PD usually diagnosed how long can non motor symptoms be present before motor onset how is progression of PD worsened what is the honeymoon phase advanced stages characterised by what late stage PD
with the onset of motor symptoms
more than a decade
worsening of motor features
initially ^ responds to symptomatic therapies (honeymoon phase)
emergence of complications of long term symptomatic treatment
resistent motor and non motor features, postural instability, freezing of gait, falls, dysphagia, speech dysfunction
after 17 years of disease what happens
after 20 years
80% have gait freezing and falls, 50% report choking
dementia occurs in 83% of patients
what is rapid eye movement sleep behaviour
abnormal or disruptive behaviour which occur during rapid eye movement sleep and are often related to dream enactment
how is rapid eye movement disorder diagnosed
overnight polysomnography to document REM sleep without atone and to rule out mimics such as obstructive sleep apnoea, seizures
what is the treatment for rapid eye movement disorder
clonazepam or melatonin at bedtime
PD with RBD (rapid eye movement sleep behaviour disorder) has a disease subtype of what
characterised by more severe autonomic dysfunction, gait impairment and dementia
what do individual with isolated RBD have
increase risk f developing a neurodegenerative condition
in parkinsons disease what do sections through the brainstem show
pigment loss correlates
what is a neurohistological hallmark of PD
loss of normal dark black pigment in the substantia nigra and locus coeruleus
with dopaminergic cell loss
Lewy bodies
M:F ratio of risk for PD
other risk factor
what does early onset dictate
M: F 3:2
age greatest risk factor
<40 - genetic cause likely
what do symptomatic treatments do
enhance intracerebral dopamine concentrations or stimulate dopamine receptors
give symptomatic drugs
levodopa
dopamine agonists
monoamine oxydase type B inhibitors
amantadine
when is treatment initiated in PD
when symptoms cause disability or discomfort aiming to improve function and QOL
what responds to treatment early in the disease
bradykinesia and rigidity
what are MAO B inhibitors for
whats needed for more severe symptoms
only moderately effective
levodopa and dopamine agonists
how does tremor respond to treatment
inconsistently responsive esp in lower doses to dopamine replacement therapy
anticholinergics, trihexyphenidyl or clopazine can be effective for tremor
SE of dopamine agonists and levodopa
nausea, daytime somnolence and oedema
SE of dopamine agonists
impulse control disorders such as pathaoligcal gambling, hyper sexuality binge eating compulsive spending
who should dopamine agonists be avoided in
history of addiction
OCD
impulsive personality
elderly with cognitive impairment as it can be associated with hallucinations
SE of levodopa
long term use is associated with motor complications such dyskinesia and motor fluctuations
give the 4 long term complications of dopaminergic therapy for PD
motor fluctuations
non motor fluctuations
dyskinesia - involuntary choreiform or dystonic movements
drug induced psychosis - hallucinations, illusions, delusions
pharma stuffs to reduce dopamine fluctuations
add a dopamine agonist
add a MAO B inhibitor
add a COMT inhibitor
other ways of managing the long term complications of dopaminergic therapies
stable levodopa - carbidopa gel
sub cut infusion of apomorphine
non - dopaminergic treatments such as amantadine and clozapine
how is psychosis in PD managed
with clozapine
alternat quetiapine
visual hallucinations and delusions in PD patients with dementia
rivastigmine
depression assoc with PD
treated with anti depressants
the clinical syndrome of parkinsonism - motor symptoms
bradykinesia
rest tremor
rigidity
postural and gait impairment
what is bradykinesia
how can it be assessed
slowness of movement with progressive loss of amplitude or speed during attempted rapid alternating movement of body segments - hypomimia, hyophonia, micographia
asking the px to perform some repetitive movements as quickly and as widely as possible or global assessment to spontaneous movements
resting tremor is what
when does it vanish and reappear
frequency
hand tremor and PD
involuntary
active moment and reappears after a few sec when arms held outstretched
3-6Hx
hand tremor not typical for PD
rigidity is what
how to distinguish rigidity from spasticity owing to UMN lesion
rigidity and resting tremor leads to what
positive froments test is what
increased muscle tone
no increase with higher mobilising speed
cog wheel rigidity
rigidity increases in examined body segment by voluntary movement of other body parts
describe postural and gait impairment
stopped posture slow shuffling steps decreased arm swing slow turning with multiple small steps freezing
investigations for PD
rule out hypothyroidism, anaemia
structural brain imaging
PET, DAT
positive levodopa or SC apomorphine challenge
red flags in PD ( a lot of them)
symmetrical symptoms severe axial or lower limb involvement frequent falls fast disease progression eye movement disorder tics, myoclonus, chorea pyramidal or cerebellar dysfunction bulbar or psuedobulbar features parietal associate sensory disturbances apraxia severe cognitive deterioration or psychosis marked autonomic dysfunction
what is vascular parkinsonism rest tremor? other signs? levodopa response ix
parkinsonism in lower limbs
rest tremor uncommon
spasticity, hemiparesis, pseudo bulbar palsy
poor levodopa response
structural brain imaging
drug induced parkinsonism
tremor
presence of what
treatment
symmetrical
coarse postural tremor
orolingual dyskinesia, akathisia
gets better a few months after drug withdrawel
essential tremor describe seen at rest? genetic and onset responds to what hand tremor
symmetric, postural higher frequency up to 12 Hz infrequently observed at rest autosomal dom - 15 years alcohol responsiveness hand tremor is present is mild
multi system atrophy is a common cause of what when triad of what signs levodopa response MRI
degenerative parkinsonism
60/70s
dysautonomia, cerebellar features and parkinsonism
severe dysarthria, dysphonia, marked antecollis, inspiratory sighing, orofacial dystonia
suboptimal and short-lived response in 1/3
cerebellar and pontine atrophy - hot cross bun sign
progressive supra nuclear palsy is what
signs
leveodopa
symm akinetic rigid syndrome with predominant axial involvement gait and balance impairment vertical gaze supranuclear palsy retrocollis staring
no respose
FXTAS when genetic symptoms progression milder in who MRI dx children can have what
> 50
abnormal number of CGG repeats in the FMR1 gene
cerebellar gait ataxia, postural/intention tremor, parkinsonism, dysautonomia, cognitive decline, peripheral neuropathy
slow disease progression
in females often POF and menopause
MRI with T2 hyper intensities in the middle cerebellar peduncles
confirmation by molecular testing
children may have classical fragile X syndrome
