Parkinson's disease | Flashcards
What is the incidence of PD per year?
4-20 in 100,000
Approx how many people in the UK have PD?
120,000
Of the people with PD in the UK, what proportion:
- Are in hospital/residential care
- In the community requiring help
- Are independent, living in the community
- 25%
- 33%
- 50%
What are risk factors for PD?
- Definite (2)
- Highly likely (1)
- Probable (1)
- Possible (5)
- Possible protective effect (2)
Definite
- Age
- Men 1/3 higher risk than women
Highly likely
1. MZ co-twin with early-onset PD
Probable
1. Positive family history
Possible
- Herbicides / pesticides
- Heavy metals
- Proximity to industry
- Farming communities
- Repeated head trauma
Possible protective effect
- Smoking
- Caffeine
What are the pathological changes which occur in PD? (5)
- Dopaminergic cells in the substantia nigra (in basal ganglia) degenerate
- Leads to a reduced production of dopamine
- Striatal dopamine deficiency and PD symptoms
- Lewy bodies present at post mortem
- Midfolding of alpha-synuclein protein
What is the normal function of the basal ganglia?
2
- Controls the preparation, initiation, sequencing and timing of well learnt motor skills
- ‘Auto pilot’ facility
When dopamine production is depleted it
leads to hypokinetic disorders such as PD
What are the different types of Parakinsonism? (6)
- Idiopathic Parkinsons disease (IPD)
- Lewy body dementia
- Multiple systems atrophy
- Progressive supranuclear palsy
- Vascular parkinsonism
- Drug-induced Parkinsonism
What are the 4 main features of PD?
- Bradykinesia
- Rigidity
- Resting tremor
- Postural instability
What symptoms can predate motor symptoms of PD for several years (4)?
- Depression
- REM sleep disorder
- Anosmia
- Constipation
Around what % of dopamine producing cell loss occurs prior to diagnosis?
up to 80%
What are the motor symptoms in PD (9)?
3 main ones
6 others
- Tremor (resting) 70%
- more noticeable at rest, when anxious, angry or
excited. May decrease when the body part is used. - Bradykinesia
- harder to make movements and they take longer, slow movements. Reduced arm swing and small steps.
- Decrimental bradykinesia - Stiffness (rigidity)- trouble turning, getting out of a chair, difficulty with fine finger movement. Lead pipe/cog-wheeling
[2 is a must, +1 or 3]
- Postural Instability
- Dystonia- painful cramping often of the feet and legs and often worse at night. Off phase symptom.
- Freezing-often in confined spaces, on turning or sudden sensory input.
- Swallowing difficulties (dysphagia)
- Communication difficulties
- Dyskinesia
What % of people with PD have difficulty with some degree of swallowing difficulties?
50-80%
How do swallowing difficulties present (7)?
- Weight loss
- Drooling
- Anxiety at meal times
- Disturbed medication intake
- A ‘gurgly’ voice
- Decreased social contact
- Bronchopneumonia
What is the management of dysphasia (6)?
- Refer to SALT and dietician if necessary
Management focuses on:
- Positioning & posture
- Food consistency & temp
- Rate of eating
- Insight & cueing
- Saliva & drooling
What are the communication difficulties that people with PD can face (3)?
- Voice can become harsh, breathy & whispery, monotonous & low volume
- Speech can become slurred, festinant with word blocks, increased pauses and have sound repetitions
- Individuals may have reduced understanding, disrupted thought planning, inappropriate stopping of sentences and be less likely to initiate conversation
What is the management of communication difficulties (3)?
- Exercises and strategies such as speaking one
word at a time, pausing between words, use of metronome - Advice regarding external distractions, positioning, allowing for thought processing, non verbal communication
- Communication aids such as voice amplifiers, alphabet or word chart, pen and paper, portable keyboard with speech output.
What is the unpredictable ‘ON-OFF’ phenomena in PD (5)?
- Sudden and unpredictable switches in mobility from mobile to immobile
- Patients may switch ON or OFF in seconds or minutes
- ‘Freezing’ may occur
- Often occur when patient experiences dyskinesia
- Profoundly akinetic when OFF and dyskinetic when ON
How is the unpredictable ‘ON-OFF’ phenomena in PD treated?
Consider use of continuous dopaminergic stimulation
strategies such as longer acting oral agonists or apomorphine, duodopa or surgery
What is the management of freezing (5)?
- Avoid multi tasking
- Do not rush
- Use visual, auditory or internal cues
- Physiotherapy- advice regarding cueing techniques, falls, balance
- OT - review of home environment
How does dyskinesia present (2)?
- Uncontrollable wriggling or writhing movements
- Choreiform, usually painless, first appears on most affected side
- Can significantly interfere with gait, balance and
quality of life
What is usually the cause of dyskinesia?
Increased risk from high doses and long duration of
treatment with L-dopa
How is dyskinesia managed?
Careful management with combination of therapies is
often required
What are the non-motor symptoms that can occur in PD (11)?
- Olfactory deficiencies 98%
- Depression and anxiety
- Apathy
- Fatigue
- Bladder and bowel problems
- Pain
- Skin and sweating problems
- Sleep and night time problems REM sleep behaviour disorder 80%
- Sexual dysfunction
- Cognitive dysfunction and dementia
- Psychosis and hallucination
What is the management of depression and anxiety in PD?
Anti-depressants/b-blockers
Psychotherapy, CBT, relaxation techniques
How does incontinence usually present in PD (3)?
- Bladder dysfunction increases with disease progression
- Mainly detrusor over activity& bladder sphincter
bradykinesia - Worse in off state- frequency, urgency, urge
incontinence, incomplete emptying, nocturia
What is the management of incontinence in PD (7)?
- Individual and specialist assessment
- Bladder retraining
- Pelvic floor exercises
- Indwelling or supra pubic catheter
- Appliances
- Fluid intake
- Clothing
What are the features of constipation in PD (2)?
- Often precedes diagnosis
- Caused by reduced bowel motility, reduced physical mobility, inadequate fluid and fibre intake, problems emptying the bowel and some medications
What is the management of constipation in PD (5)?
- fluid intake
- diet
- medications
- exercise
- position and habit
What are the problems with saliva control in PD?
Caused by reduced swallowing, stooped
or poor posture and reduced lip seal
What is the medical management of saliva problems in PD (6)?
- Atrovent inhaler
- Atropine eye drops
- Hyoscine patch (with caution)
- Botox
- Sage
- SALT: effective swallowing, posture and lip seal
What are some night time problems that occur in PD?
- REM behaviour disorder- act out dreams which are vivid and often frightening.
- Restless legs- Unpleasant sensation with uncontrollable urge to move when at rest.
- Dystonia
- Wearing off
- Nocturia
How is REM behaviour disorder managed?
Clonazepam
How is restless legs managed (3)?
- Reducing alcohol, caffeine and tobacco intake
- Check Iron, folate
and magnesium levels. - Dopamine agonists
How do you manage PD medication wearing off at night time?
Take last dose of PD medication as close to bedtime as possible
What is the incidence of falls in people with PD every year?
45-68%
What are some risk factors for falls specific to PD (5)?
- Cognitive impairment
- Postural instability/gait impairment
- static
- dynamic - Reduced lower extremity strength/power
- Motor fluctuations and dyskinesia
- Freezing of gait/gait impairment
What are negative consequences of falls in PD?
- Fractures, injuries and fear of falling
- > - Immobilisation
- > - Osteoporosis, social isolation, muscle weakness
- > - “Malignant Parkinson disease”, reduced quality of life, increased mortality
In newly diagnosed untreated PD patients, what % have cognitive impairment with a MOCA of less than 26?
What are these people are at risk of?
22%
Developing dementia
How much more likely are you to get dementia with PD compared to a healthy person?
6x
According to Queen Square Brain Bank criteria, what are the core features for the diagnosis of PD dementia?
UK brain bank criteria
A dementia syndrome with insidious onset and slow progression, developing within the context of established Parkinson’s disease and diagnosed by history, clinical, and mental examination, defined
as:
1. Impairment in more than one cognitive domain
2. Representing a decline from premorbid level
3. Deficits severe enough to impair daily life (social, occupational, or personal care), independent of the impairment ascribable to motor or autonomic symptoms.
Step 1: Bradykinesia and at least one of the following: -muscular rigidity rest tremor postural insatbilty
Step 2
Exclusion
Step 3: supportive criteria
What are the associated cognitive clinical features for the diagnosis of PD dementia (5)?
- Attention
- Impairment in spontaneous and focused attention, may fluctuate during the day/from day to day - Executive functions
- Impairment in tasks requiring initiation,
planning, concept formation, rule finding, set shifting or set maintenance; impaired mental speed (bradyphrenia) - Visuo-spatial functions:
- Impairment in tasks requiring visualspatial orientation, perception, or construction - Memory:
- Impairment in free recall of recent events or in tasks requiring learning new material, memory usually improves with cueing, recognition is usually better than free recall - Language:
-Core functions largely preserved. Word finding difficulties and impaired comprehension of complex sentences
may be present
What are the associated behavioural clinical features for the diagnosis of PD dementia (5)?
- Apathy:
- Decreased spontaneity; loss of motivation, interest, and effortful behaviour - Changes in personality and mood including
depressive features and anxiety - Hallucinations: mostly visual, usually complex,
formed visions of people, animals or objects
4. Delusions: usually paranoid, such as infidelity, or phantom boarder (unwelcome guests living in the home) delusions
- Excessive daytime sleepiness
What % of patients with PD experience hallucinations at some point of disease?
80%
What is the management of hallucinations and delusions in PD (8)?
- Avoidance of triggers
- Withdrawal of potentially contributing medications
- Adjustment of antiparkinsonian medications
- Introduction of cholinesterase inhibitors in patients with PD and dementia
- Treatment with antidepressants if psychosis is mood congruent in concomitant depression
- Atypical antipsychotics, clozapine or, possibly, quetiapine
- Clozapine more effective than placebo for the management of psychosis in PD but limited by its potential for severe side effects
- No large RCT has shown quetiapine to significantly improve psychosis in PD although it continues to be used empirically in clinical practice
There is a higher prevalence of hallucinations and delusions in PD-D, what is the %
45-65%
What are the characteristics of hallucinations in PD-D?
- Usually visual, majority are complex, formed hallucinations
- Most common are anonymous people, but
they may also be family members, body
parts, animals, or machines.
• They tend to be in colour, static and
centrally located, and occur with similar
frequency and severity in PD-D and DLB
When is palliative care sought in PD?
A situation where it is recognised that PD is going to be life-limiting, that death from PD or it’s complications can be anticipated, and that the focus of our therapy goals should be on the short term alleviation of symptoms
What are the prognostic determinants in PD (4)?
- Age
- Genetic polymorphisms
- Poor semantic fluency
- Co-morbidities/frailty
What are the causes of death in PD?
- Mechanisms of immobility
- Falls
- Chest and urinary infections
- Exhaustion
- Weight loss
When do you begin treatment for PD?
As soon as you get first onset of symptoms
What are the pharmacological treatments of PD (6)?
- Dopamine replacement
Levodopa -> most effective symptomatic treatment - Dopamine agonists e.g. ropinerole, pramipexole, rotigotine
- Selective irreversible inhibitor of monoamine oxidase-B e.g. Selegeline/Rasagaline
- MAO-B inhibitor with additional activity at non-MAO-B targets e.g. Safinamide
- Antiviral agent with models antiparkinsonian effect e.g. amantadine
- COMT inhibitors that block the metabolism of L-dopa e.g. entacapone, stalevo
What is the most effective medication for PD?
Levodopa
What is usually the response to levodopa over time?
Initial smooth response to l-dopa but complications arise after 4-6 years
What is the benefit of a dopamine agonist (ropinerole, pramipexole, rotigotine) over levodopa?
Longer duration of action than l-dopa thus reduced pulsatility
When would Selegeline (selective irreversible inhibitor of MAO-B) be used in PD (2)?
- Can delay introduction of l-dopa by aprox one year
- Can increase extent and duration of response to l-dopa
in adjunct therapy
When would amantadine (antiviral with modest antiparkinsonian effect) be used in PD?
Occasionally used in early disease to delay l-dopa but now more frequently for
anti-dyskinetic properties
When would Rasagaline (potent, selective irreversible MAO-B inhibitor) be used in PD?
Provides symptomatic benefit in both early and advanced disease
When would Safinamide (MAO-B inhibitor with non-MAO-B targets) be used in PD?
Add on treatment to other therapies in mid to
late stage PD
When would COMT inhibitors in PD be used?
To improve ADL’s
What is the advanced therapy - continuous levodopa infusion?
- Gel suspension (duodopa) for continuous enteral
administration. - Administered via a portable pump directly into the duodenum via a gastrostomy or jejunostomy
- Reduced variability in plasma concentrations of l-dopa clinically reflected as more on time per day, less
parkinsonism and less l-dopa induced hyperkinesia
What are some negative effects of dopamine agonists (2)?
- Can contribute to Compulsive tendencies including excessive gambling, punding, hobbyism, dietary changes and hypersexuality
- Compulsive use of dopaminergic medications
escalated by the patient driven by an attempt to avoid
the dysphoria of ‘off’ periods
What are principle side effects of dopamine agonists (5)?
- Nausea & vomiting
- Postural hypotension
- Dyskinesia
- Neuropsychiatric symptoms - hallucinations
- Somnolence
What are tests for PD?
- No definite test
- Brain imaging
-CT/MRI - stroke/tumours/other
DaT scan - dopamine depletion (if negative, you can exclude PD)
