L11 PD Flashcards
1
Q
what is the most common serious movement disorder
A
Parkinson’s disease
2
Q
what is parkinson’s disease
A
a progressive neurodegenerative disease of the basal ganglia, in which dopaminergic neurons in the substantia nigra degenerate
3
Q
what percentage of dopaminergic neurons have died before clinical symptoms of parkinson’s appear
A
70-80%
4
Q
what is the onset and progression of PD like
A
gradual
5
Q
is symptom presentation symmetrical or asymmetrical
A
asymmetrical
6
Q
what is the most common initial feature of parksinson’s
A
resting tremor in one hand
7
Q
synucleinopathy
A
neurodegenerative diseases characterised by the abnormal accumulation of alpha-synuclein protein in neurons, nerve fibres or glial cells
8
Q
Is Parkinson’s disease a synuclienopathy or a tauopathy
A
synuclienopathy
9
Q
estimated prevalence of PD
A
100–180 per 100,000 of the population
10
Q
prevalence of PD in 40-49 year olds
A
40 per 100,000
11
Q
prevalence of PD in >80-year-olds
A
1,900 per 100,000
12
Q
is there a higher prevalence of PD in males or females
A
males
13
Q
what is the estimated number of people living with PD globally
A
around 6 million people
14
Q
how many people does the Parkinson’s Association of Ireland estimate are affected by PD in Ireland
A
12,000
15
Q
is the amount of people living with PD in Ireland expected to rise or fall in twenty years
A
it’s expected to rise to double
16
Q
does PD reduce life expectency
A
yes
17
Q
what happens to untreated cases of PD
A
they progress to severe immobility within 7-10 years with risk of:
- Bronchopneumonia
- Septicemia
- Pulmonary embolism
18
Q
what percentage of patients with PD develop dementia
A
25-40%
19
Q
why do so many patients with PD develop dementia
A
due to Lewy bodies spreading to the cerebral cortex and limbic system.
20
Q
how much higher is the risk of developing dementia in people with PD compared to healthy individals
A
1.7–5.9 times higher
21
Q
risk factors for dementia for people with PD
A
- Age at onset
- Disease duration/severity
- APOE genotype
22
Q
what is the primary cause of reduced life expectancy in PD patients
A
dementia
23
Q
do current treatments cure PD
A
no, they are symptomatic but they do improve life expectancy
24
Q
how long can it take to receive a diagnosis of probable PD
A
5 years
25
what is secondary parkinsonism
parkison's symptoms caused by something else
26
secondary parkinsonism causes
- Drug-induced
- Post-encephalitis
- Post-head injury
27
examples of Atypical Parkinsonian Syndromes
- Progressive Supranuclear Palsy (PSP)
- Multiple System Atrophy (MSA)
- Corticobasal Syndrome (CBS)
- Dementia with Lewy Bodies (DLB)
27
what percentage of parkinsonism cases does idiopathic parkinson's disease account for
80%
27
at what age does prevalence of PD increase
after age 50 with a steep rise after age 60
27
what is the cause of PD
unknown but thought to involve environmental and genetic factors
27
in what percentage of PD cases is there a family history
20-30%
27
what does onset of PD before age 30 suggest
a hereditary form of the disease
28
what are the four cardinal features of IPD
- tremor
- rigidity
- akinesia/bradykinesia
- postural instability
28
tremor
Resting "pill-rolling" tremor, disappears with action/sleep.
29
rigidity
Increased muscle tone and resistance to movement.
29
akinesia/bradykinesia
Slowed movement initiation and execution.
29
what does postural instablility lead to
falls
29
what other symptom is common in PD
freezing episodes
29
other motor symptoms of PD
- Dysarthria, dysphagia, sialorrheoa.
- Decreased arm swing, shuffling gait, difficulty arising from chair, turning in bed.
- Micrographia, cutting food, feeding, hygiene, slow activities of daily living.
- Glabellar reflex, dystonia, striatal deformity, scoliosis.
29
other nonmotor symptoms of PD
- Cognitive impairment, bradyphenia, word-finding difficulties.
- Depression, apathy, anhedonia, fatigue, other behavioural and psychiatric problems.
- Sensory symptoms: anosmia, pain (shoulder, back), parasthesias.
- Dysautonmia (orthostatic hypotension, constipation, urinary and sexual dysfunction, abnormal sweating, seborrhoea), weight loss.
- Sleep disorders (REM behaviour disorder, vivid dreams, daytime drowsiness, sleep fragmentation, restless leg syndrome).
29
is there a definitive diagnostic test for PD
no (except autopsy)
29
diagnostic criteria for PD
1. UK Brain Bank Criteria
2. MDS Clinical Diagnostic Criteria for Parkinson’s Disease (this one is most commonly used).
29
considerations when reaching a diagnosis of PD
- Hallmark clinical features.
- Neuroimaging to exclude other conditions.
- Response to drug treatment.
30
in what percentage of PD cases, has a misdiagnosis been made
25%
30
common misdiagnoses
- Essential tremor
- Vascular parkinsonism
- Atypical parkinsonian syndromes
30
when should PD be suspected
when patients present with tremor, stiffness, slowness, balance problems and/or gait disturbance.
30
who should patients with suspected PD be referred to
a specialist (neurologist) with expertise in the differential diagnosis of this condition.
30
when should a diagnosis of PD be reconsidered
if atypical features develop
31
what does SPECT stand for
single photon emission computed topography
32
when should SPECT be considered for use
for patients with tremor where Essential Tremor cannot be differentiated from Parkinsonism.
33
what syndromes mimic PD
- Essential tremor
- Atypical Parkinsonian Syndromes
- Alzheimer’s disease
- Cerebrovascular disease
34
two examples of rating scales for PD
- Unified Parkinson’s Disease Rating Scale
- Hoegn and Yahr Scale
35
MDT members
- neurologist
- GP
- clinical nurse specialist
- SLT
- OT
- physio
- dietician
- social work
- neuropsychologist
- pharmacist
36
neurologist's role in MDT
diagnosis and management of neurological symptoms
37
clinical nurse specialist role on MDT
link between patient and other specialties
38
OT's role on MDT
- assessment and management of cognitive, physical and perceptual skills
- optimising functional ability
- - Supporting participation in relationships, roles, work, leisure and driving.
- Positioning and posture.
- Seating assessment.
- Moving and handling techniques.
39
physio role on MDT
- Maximise quality of movement.
- Improvement of general fitness.
- Minimise secondary complications.
- Optimising safety.
- Transfer training.
- Gait rehabilitation.
- Balance.
- Fall prevention.
- Manual activities.
- Respiratory function.
- Compensatory strategies.
40
dietician role on MDT
- Management of nutritional complications.
- Assessment for and mangagement of malnutrition.
- Self-feeding support.
- Nutritional supplementation.
- Involvement in process for consideration of gastrostomy insertion.
- Planning for home enteral feeding.
- Consideration of drug-nutrient interactions.
- Appropriate dietary advice.
41
neuropsychologist's role on MDT
- Assessment of mood and adjustment.
- Psychotherapeutic intervention for depression, anxiety, and psychosis.
- Interventions to promote adjustment and cognitive rehabilitation.
- Capacity assessment.
- Emotional support.
42
three main treatment approaches
1. Pharmacological
2. Surgical
3. Rehabilitation
43
aim of pharmacological treatment
Restore neurochemical balance via anticholinergic agents or dopaminergic drugs.
44
comment on delay of pharmacological treatment
Delay treatment until symptoms significantly impact function due to drug side effects.
45
three common medications used to treat PD
- Levadopa (L-DOPA)
- Dopamine antagonists
- Enzyme inhibitors
46
which of the three common medications are gold standard or PD
L-Dopa
47
hwo does L-dopa work
- Natural substrate for the synthesis of dopamine.
- Crosses blood-brain barrier to increase dopamine levels.
48
how do dopamine antagonists work
they mimic domapine to stimulate receptors
49
how do enzyme inhibitors work
prevent dopamine breakdoqn
50
other medications used
- anticholinerics & amantadine
- apomorphine
- glutamate antagonists
- COMT inhibitors
- MAO-B inhibitors
51
what are anticholinergics used for
treatment of tremor
52
what is apomorphone
a strong domapine angonist (subcutaneous)
53
example of a COMT inhibitor
entacapone
54
how do COMT inhibitors work
pronlong levodopa effects by blocking the enzyme that breaks down levadopa
55
example of a MAO-B inhibitor
selegiline
56
what do MAO-B inhibitors do
Slows dopamine breakdown (by blocking the enzyme which breaks it down).
57
medication side effects
Some dopamine agonists & levodopa can cause impulsive/compulsive behaviors (e.g., gambling, excessive shopping).
58
why is L-dopa's effectiveness limited
limited by motor complications and dyskinesias after 2-5 years
59
what motor fluctuations can occur with L-Dopa use
- **Wearing off** – Shorter effects between doses.
- **On-off phenomenon** – Unpredictable fluctuations between medication’s benefit to an akinetic-rigid state.
60
what are dyskinesias
involuntary movements occurring in association with drug treatments
61
what kind of dyskinesias occur with L-Dopa use
Peak dose dyskinesias – Involuntary twisting movements at high dopamine levels.
62
what kind of dystonias occur with L-Dopa use
Wearing off dystonias – Painful muscle contractions at low dopamine levels.
63
surgical treatment option of PD
- Deep Brain Stimulation (DBS)
- - Does not halt disease progression but can control movement symptoms.
- Strict eligibility criteria.
64
Deep Brain Stimulation
- Electrodes implanted in the brain.
- Reduces tremors, stiffness, and dyskinesias.
- Does not improve speech or swallowing issues.
65
Possible adverse effects of DBS on speech and swallowing
- Exacerbation of slurred speech
- Adverse impact on rhythm, intonation, and articulation.
- Reduced intelligibility.
- Impact on social interaction.
- Interference with daily experiences due to voice and speech difficulties.
66
what areas of focus are there for SLT management in PD
- Speech
- Voice
- Language
- Augmentative and Alternative Communication (AAC)
- Swallowing
- Drooling
67
what type of dysarthria occurs with PD
hypokinetic dysarthria
68
characteristics of hypokinetic dysarthria in PD
- Low volume
- Imprecise articulation
- Dysphonia (hoarse voice)
- Monotone & monopitch
- Abnormal speech rate (increased in some segments)
- Palilalia – rapid repetition of words/syllables
69
examples of assessment tools for dysarthria used in PD
- AIDS (Assessment of Intelligibility of Dysarthric Speech)
- Frenchay Dysarthria Assessment
- Unified Parkinson’s Disease Rating Scale (UPDRS) Speech Subsection
- Dysarthria Impact Profile (Walshe, Peach & Miller, 2009)
- Recording a speech sample.
- LSVT assessment.
70
traditional therapy methods for dysarthria in PD
- Pacing boards
- Rate control drills
- Increased respiratory support
- Increased vocal fold adduction
- Emphasizing stress patterns
- Focus on multiple speech subsystems
71
what is the gold standard dysarthria treatment for PD
Lee Silverman Voice Treatment (LSVT LOUD)
72
when was LSVT developed
1987
72
main goal of LSVT
Increase vocal loudness ("Think LOUD").
72
key principles of LSVT
- High effort, intensive treatment (4x/week for 4 weeks).
- Simple approach focusing on phonation.
- Focused on one subsystem to minimise cognitive load.
- Knock-on effects on respiration, articulation, resonance.
- Proven long-term carryover effects.
72
at what stages of PD is dysphagia present
all disease stages
72
what does dysphagia increase the risk of in PD
- Aspiration pneumonia (4-30%).
- Malnutrition & dehydration.
72
what are the contributing factors to occurence of swallow disturbance
- Age
- Disease duration
- Dementia
72
what components should be in a swallow assessment of a person with PD
- comprehensive case history
- observation of the individual at rest
- oral examination
- clinical swallow evaluation
72
what information should be sought in a swallow ax case history of a person with PD
- Time since PD diagnosis
- Disease progression rate
- Comorbid symptoms/conditions
- Medications & response
- Chest health (pneumonia risk)
- Meal duration
- Avoidance of certain foods
- Weight loss, choking episodes
- Ability to swallow tablets
- Impact of dysphagia on QOL
72
what information should be sought during an observational assessment of a person with PD
- Alertness, posture, mobility
- Cognitive function, memory, language
- Salivary control & oral reflexes
- Tremor/dyskinesia affecting feeding
72
what information should be sought in an oral examination of a swallow ax
- Masked facial expression
- Open mouth posture
- Tardive dyskinesia
- Dry oral mucosa
- Drooling
- Dentition & lingual movement
- Cough response
72
what information should be sought in a swallow trial of a person with PD
- Self-feeding ability
- Rate of feeding
- Anterior spillage
- Delayed oral phase (tongue pumping)
- Pharyngeal swallow delay
- Weak hyo-laryngeal excursion
- Cough response
- Voice quality post swallow
- Oral residue
- Silent aspiration (no cough reflex)
- Effort involved
- Improvement with volume/taste
72
what impairments may be found in the oral and oral prepatory phases of the swallow may be found on videoflouroscopy
- Anterior spillage
- Repetitive tongue pumping
- Prolonged oral transit
- Bolus formation difficulty
- Impaired mastication
- Difficulty with swallow initiation
- Premature spillage of material into pharynx
- Presence of residue on tongue surface, in lateral sulci and vallecular sinuses
72
what impairments may be found in the pharyngeal phase of the swallow may be found on videoflouroscopy
- Delayed swallow initiation
- Weak pharyngeal peristalsis
- Reduced posterior motion of tongue base → residue in valleculae
- Reduced hyo-laryngeal elevation → residue in pyriform sinuses
- Silent aspiration on fluids before swallow due to pharyngeal reflex delay
- Benefit of chin tuck/smaller fluid volume/changes to taste or temp
73
what rehabilitative technique can be used in the treatment of dysphagia in people with PD
Expiratory Muscle Strength Training (EMST)
73
what management strategies can be used in the treatment of dysphagia in a person with PD
- Medication timing (L-Dopa before meals)
- Postural adjustments (e.g., chin tuck)
- Compensatory techniques (e.g., smaller volume sips)
- Diet modification (e.g., thickened liquids)
- Biofeedback training (sEMG)
- Verbal cueing for swallowing effort
- Sensory stimulation (e.g., iced water, sour taste)
73
what percentage of PD patients does drooling affect
56%
73
what is drooling in PD caused by
- Reduced automatic swallowing frequency
- Impaired lip closure
73
what management strategies can be used in the treatment of excessive drooling in people with PD
- Swallow reminder devices
- Chewing gum/sour sweets
- Dysphagia treatment
- Postural changes
- Pharmacological: Hyoscine patch, Botox to parotid glands
- Radiation therapy to salivary glands for severe cases
73
what are atypical parkinsonian sydromes charactersied by
- Rapid progression & worse prognosis (life expectancy) than PD.
- Dysarthria/dysphagia onset within 1 year highly specific to APS.
74
what is progressive supranuclear palsy
Rare, progressive disease causing degeneration in brainstem, basal ganglia, cerebellum.
74
what is tauopathy
group of neurodegenerative disorders characterised by abnormal neuronal and/or glial accumulations of the protein tau.
74
is PSP a synucleinopathy or tauopathy
tauopathy
74
key features of PSP
- Postural instability – backward falls.
- Supranuclear gaze palsy – difficulty looking downward.
- Dementia.
- Dysphagia & dysarthria (Viscidi et al., 2021).
- Onset in 6th to 7th decade.
- More men than women affected.
- Trunk and neck hyperextended.
- Wide-eyed stare.
- Furrowing of forehead
- Deepening of other facial creases
74
two subtypes of PSP
- Richardson Syndrome (PSP-RS)
- PSP-P (Parkinsonism subtype)
75
median survival of PSP
10 years
76
prevalence of PSP-RS
up to 6.4/100,000
77
average age of onset of PSP
mid sixties
78
does L-Dopa have an effect on PSP
no
79
differentiating symptoms between PSP and PD
- PSP is symmetrical.
- PSP presents with axial rigidity and PD presents with limb rigidity.
- PSP does not present with tremor.
- PSP presents with vertical paresis.
- Poor response to levodopa.
80
81
how long post-symptom onset does it usually take to get a diagnosis of PSP
3 years
82
prevalence of dysphagia in people with PSP
78-89%
83
which has an earlier onset of dysphagia: PD or PSP
PSP (42 months vs. 130 months)
84
what is the leading cause of death in PSP
aspiration pneumonia
85
what is Multiple Systems Atrophy
a degenerative disease which affects autonomic function, movement and balance
86
is MSA synucleinopathy or tauopathy
synucleinopathy
87
what sypmtoms does MSA present with
rapid eye movement, muscle stiffness and behaviour changes
88
prevalence of dysphagia in people with MSA
31-78%
89
three types of MSA
- MSA-P
- MSA-C
- MSA-A
89
onset of dyaphagia in people with MSA
67 months
90
dysarthria onset in people with MSA
24 months
90
type of dysarthria present in people with MSA
Mixed hypokinetic-ataxic-spastic type
91
what is MSA-P
MSA with parkinsonian features
92
MSA-C features
- Parkinsonism with prominent cerebellar signs
- Especially wide-based ataxic gait
- Variable autonomic signs
- Autonomic failure
- Incontinence in women
- Impotence in men
- Orthostatic hypotension
92
MSA-P symptoms
- Symmetrical parkinsonism often without
tremor
- Short-lived response to L-Dopa
- Midline dystonia
- Autonomic signs- orthostatic hypotension; incontinence and impotence
92
what is MSA-C
MSA with cerebellar ataxia dominant
93
is dementia with Lewy Bodies (DLB) synucleinopathy or tuaopathy
synucleinopathy
93
what is MSA-A
MSA with autonomic failure dominant
94
symptoms of MSA-A
- Variable autonomic signs
- Autonomic failure
- Incontinence in women
- Impotence in men
- Orthostatic hypotension
95
symptoms of LBD
- Fluctuating cognition with pronounced variations in attention and alertness.
- Recurrent visual hallucinations that are typically well formed and detailed.
- REM sleep behaviour disorder which may precede cognitive decline.
- One or more spontaneous cardinal feature of parkinsonism – bradykinesia, rest tremor, or rigidity.
96
dysphagia onset in DLB
34 months
97
dysarthria onset in DLB
42 months
98
type of speech predominated in DLB
Hypophonic/monotonous speech
99
is cortico-basal syndrome (CBS) synucleinopathy or tauopathy
tauopathy
100
symptoms of CBS
- Characterised by involuntary movements including rigidity, tremor, dystonia and myoclonus.
- Often associated with apraxia, cortical sensory deficits, and alien limb phenomena.
- Condition typically affects one side of the body more than the other and makes it difficult for patients to see, and navigate through space.
101
dysphagia onset in CBS
64 months
102
dysarthria onset in CBS
40 months
