PD and Movement Disorders

Question 1

What is PD?

Answer 1

Progressive neurodegenerative disorder

Question 2

Epidemiology of PD

Answer 2

Most common movement disorder

Incidence increases with age (mean age of Dx is 70 years)

More males affected than females

Question 3

Natural Hx of PD

Answer 3

Disability occurs at all stages of the disease but severity of disability increases with disease duration

Question 4

Mrs D, 70, living at home with her husband, admitted for 3/52 of worsening motor Sx of PD

Previously walked unaided and independent with ADLs but currently bedbound and needing help from husband to get up from bed/chair, brush teeth and bathe

Husband, 75, previously in good health, has been married to Mrs D for 50 years and they have a good relationship, but he is finding it more difficult physically as his wife needs much more help with personal care and mobility; his back is sore, especially after trying to lift his wife after a fall and he is much more tired, as well as being worried about her (he has been asking his daughter to assist with shopping and stopped going to bingo as he cannot get his wife into the car easily)

PD diagnosed 6 years ago after presenting with rigidity and bradykinesia (no tremor), on Madopar (levodopa and benserazide) 200/50 mg every 3.5 waking hours; sees neurologist in movements disorders clinic and has had multiple hospital admissions related to medication complications

What are the cardinal features of PD?

Answer 4

Tremor

Bradykinesia

Rigidity

Postural instability

Question 5

Mrs D, 70, living at home with her husband, admitted for 3/52 of worsening motor Sx of PD

O/E: normal orientation/attention/memory, normal oculomotor exam, masked facies, speech monotonous and low volume, no resting tremor, asymmetrical cogwheel rigidity and bradykinesia (R more affected than L), slow hesitant foot tapping, normal power and sensation in limbs but unable to fasten buttons or tie laces, no dyskinesia/dystonia

What gait abnormalities may be seen in PD?

What other special test can be done for PD?

Answer 5

Reduced speed and stride length

Narrow base of support

Forward trunk flexion

Reduced arm swing

Short, shuffling steps

Festination, freezing

Difficulty turning, changing direction, stepping over or moving around objects

Pull test: staggers backwards multiple steps when pulled (retropulsion)

Question 6

What is dyskinesia? How does it present and how is it treated?

Answer 6

Reversible levodopa-induced motor complication (present in 30-40% of pts on levodopa for 5 years, nearly 60% by 10 years)

Abnormal involuntary movements (choreic, dystonic, ballistic, myotonic)

Treat by adjusting levodopa dosing

Question 7

What is dystonia? How can it be distinguished from dyskinesia and what is the cause?

Answer 7

Involuntary muscle contraction involving abnormal movements and postures

More sustained abnormal posture than dyskinesia

Can be an undertreated PD motor symptom or a complication of levodopa treatment

Question 8

Mrs D, 70, living at home with her husband, admitted for 3/52 of worsening motor Sx of PD

Constant rigidity and bradykinesia, not relieved by 3 hourly oral Madopar, but no unpredictable “on-off”, no episodes of freezing and no dyskinesia

Rx: commenced on low dose Madopar (levodopa/benserazide) at Dx (1/4 tablet QID), but 2 years later became immobile before next dose (i.e. “wearing-off” but no unpredictable “off” periods) so neurologist increased dose and frequency to 3-hourly - this was tolerated well with improvement in Sx and no dyskinesia

What is levodopa? What is its mechanism of action and its adverse effects?

Answer 8

Mainstay of PD treatment: most effective for hypokinetic motor Sx, tremor and rigidity, but less effective for postural instability

Replaces DA via prodrug (levodopa)

Always combined with peripheral decarboxylase inhibitor (benserazide) to minimise peripheral conversion to DA and adverse effects resulting from this

AEs: nausea, abdominal cramping, diarrhoea, somnolence, dizziness and headache, confusion, hallucination, delusions, agitation, psychosis, orthostatic hypotension

Question 9

Complications of levodopa

Answer 9

50% of patients on levodopa for 5 years experience motor fluctuations and dyskinesia

Wearing-off: end-of-dose effect

On-Off syndrome: episodes of unpredictable “off”, alternating with “on” (+/- dyskinesia)

Failure to turn “on” (“no-on”): due to excessively prolonged or severe “off” period

Acute akinesia: sudden exacerbation of PD, akinetic state lasting for days and not responding to anti-Parkinson medications

Question 10

Sinemet/Kinson

Answer 10

Levodopa + carbidopa (inhibitor)

Question 11

PD medications

Answer 11

Levodopa (DA prodrug) + peripheral DA decarboxylase inhibitor

COMT inhibitors

Selective MAO type B inhibitors (MAOI)

DA agonists (DA)

Anticholinergics

Amantadine

Question 12

What are the non-motor features of PD?

Answer 12

Orthostatic hypotension and falls

Urinary frequency

Constipation

Question 13

Mrs D, 70, living at home with her husband, admitted for 3/52 of worsening motor Sx of PD

Constant rigidity and bradykinesia, not relieved by 3 hourly oral Madopar, but no unpredictable “on-off”, no episodes of freezing and no dyskinesia

DDx for Mrs D’s deterioration?

Answer 13

Reduced levodopa response and motor fluctuations: “wearing off” and/or “failure to turn on”, ?acute akinesia

Underlying systemic infection or intercurrent medical problems (?UTI)

Malnourishment

Dehydration

Deconditioning

Question 14

Mrs D, 70, living at home with her husband, admitted for 3/52 of worsening motor Sx of PD

Constant rigidity and bradykinesia, not relieved by 3 hourly oral Madopar, but no unpredictable “on-off”, no episodes of freezing and no dyskinesia

Also bothered by orthostatic hypotension, urinary frequency and constipation

Treatment goals?

Answer 14

Motor Sx Mx: several options (in this case apomorphine, a DA agonist, infusion is commenced, together with the anti-emetic domperidone), keep movement chart to monitor motor response as an inpatient

Hydration and nutrition

Dx and treat infection

Treat constipation (bowel program and aperients)

Reconditioning, improve mobility and function (PT and OT involvement, recommend inpatient rehabilitation)

Psychosocial support

Dietitian involvement: meal supplements, regular small meals, avoid large high-fat meals (which slow gastric emptying), schedule protein intake away from levodopa dose

Question 15

What is the difference between GEM and rehabilitation?

Answer 15

GEM (geriatric evaluation and Mx): “slow stream”, multidisciplinary rehab under geriatrician

Rehabilitation: “fast stream” rehab with daily intensive multidisciplinary therapy under rehab physician

Question 16

What are the criteria for admission to “fast stream” rehab?

Answer 16

Wants to participate: gives consent, motivated

Can participate, can learn: tolerates 3 hours of therapy per day, medically and psychiatrically stable, cognitive ability to learn and carry over

Has goals: working towards something, functional gains in set time-frame

Question 17

What are the main goals of rehabilitation?

Answer 17

Prevent complications

Maintain/optimise function, mobility and self-care skills

Educate patient and carers, provide counselling and support

Facilitate community reintegration

Question 18

What is the evidence for rehabilitation in PD?

Answer 18

General consensus is that PD patients benefit from multidisciplinary rehabilitation and an exercise program focussing on improving ROM, endurance, balance and gait

Gains are not sustained after therapy is discontinued (rehabilitation does not alter the underlying pathology of chronic neurological disorders)

Possible role for long term rehab programs or regular “booster” therapy sessions?

Question 19

Who may be involved in the rehabilitation team for a pt with PD?

Answer 19

Patient and family

Rehab physician

OT

Social worker

RDNS

PD support group

Psychologist/neuropsychologist

Neurologist

Gastroenterologist

Neurosurgeon

Speech pathologist

PT

Dietician

PD and rehab nurse

Question 20

How can mobility in PD be improved?

Answer 20

Individual and group PT focussing on:

Flexed, stooped posture

Gait/walking and transfers

Balance

General fitness (address deconditioning)

Setting up a home exercise program to maintain gains

Question 21

How can flex, stooped posture in PD be improved?

Answer 21

Teach proper posture and trunk extension, breathing exercises

Stretching and ROM exercises (may also help with pain/dystonia)

Question 22

How can gait/walking and transfers of PD patients be improved?

Answer 22

Widen the stance, increase base of support (walking frame may be needed)

Conscious strategies to initiate gait and maintain cadence (mental rehearsal, counting and singing, marching to rhythm)

Strategies such as rocking to and fro in the chair before rising

Question 23

How can ADLs of PD patients be improved?

Answer 23

Functional UL training to improve fine motor skills, motor control and dexterity (most effective when task specific)

Provide adaptive aids (e.g. large-handled cutlery, plate guards, built-up pens, velcro instead of buttons and laces)

Teach compensatory strategies

Teach fatigue Mx strategies, relaxation and breathing exercises

Discuss OT driving assessment and retraining of driving skills

Home assessment: removal of floor rugs, proper lighting at night, non-skid surfaces in the bathroom, grab rails, raised chair and toilet seat, commode by bedside

Question 24

List 5 ways in which falls risk of PD patients can be addressed

Answer 24

Improving posture, gait and balance

Teaching how to break a fall and technique to rise from floor

OT home modifications and assistive equipment

Optimising medications

Addressing orthostatic hypotension

Question 25

How can orthostatic hypotension in PD be managed non-pharmacologically?

Answer 25

Teaching profiling strategies (rise slowly from a supine/sitting position)

Avoid sudden postural chanes, prolonged recumbence

Elevate head of bed

Use of compressive garments, elastic stockings or abdominal binders

Avoid excessive EtOH and large meals, warm temperature and excessive straining

Question 26

How can orthostatic hypotension in PD be managed pharmacologically?

Answer 26

Salt supplements

Fludrocortisone

Question 27

In what % of PD patients does orthostatic hypotension occur?

Answer 27

58%

Occurs late

Question 28

How can bowel and bladder dysfunction in PD be managed?

Answer 28

Constipation: small regular meals with adequate fibre and water, regular bowel program with aperients/suppository

Urinary frequency: treat any underlying UTI, check post-void residual volume, timed voiding, fluid regulation, commode by bed

Question 29

How can sexual dysfunction in PD be managed? What is it caused by?

Answer 29

Sexual function affected by PD autonomic dysfunction and/or psychological factors

Intimacy training, sexual counselling and strategies (posture, timing of meds)

Question 30

Speech therapy assessment of Mrs D found mild oropharyngeal dysphagia, no overt aspiration concern and can tolerate pre-morbid soft/thin diet, mild hypokinetic dysarthria with mild dysphonia but communication is functional

Mx?

Answer 30

Modified food texture (soft), optimise head and neck position when swallowing

Speech exercises: emphasise breath and rate control, improving (exaggerating) articulation, and improving control

Ongoing dietitic input: nutrition

Question 31

When does cognitive dysfunction and dementia occur in PD?

Answer 31

41% prevalence; occurs late, and might not be apparent on a social level in early disease

Question 32

What kind of dementia is seen in PD?

Answer 32

Subcortical dementia: psychomotor retardation, memory difficulty, executive dysfunction, visuospatial defects

Question 33

When is neuropsychology assessment recommended for PD patients?

Answer 33

Return to work

Safety to continue independent living or driving (will also need OT driving assessment)

To teach compensatory cognitive techniques (e.g. memory aids) and educates patient and carers

Question 34

What psychological problems are commonly encountered in PD patients?

Answer 34

Depression (50% of pts; can trial SSRI)

Anxiety

Apathy and abulia (absence of willpower or inability to act decisely): loss of motivation, diminished speech/motor activity and emotional expression, ascribed to frontal lobe dysfunction and basal ganglia lesion

Sleep disturbance

Question 35

What social problems are often encountered in PD patients?

Answer 35

Social isolation

Inability to drive decreases social access (e.g. to doctors’ appointments, gym, church group)

Loss of role (decreased ability to participate in home chores)

Personal relationship breakdown

Fear of increasing physical incapacitation and dependence on others

Negative stigma about the illness in society

Question 36

How can psychological and social aspects of PD be managed?

Answer 36

Counselling

Carer support

Services: home help, shopping assistance, half price taxi card

Information RE PD support group to allow interactions with other patients or families with similar experiences, provide access to useful information

Question 37

What kind of FU might a PD patient require?

Answer 37

Movement disorder clinic

Rehab clinic

LMO

RDNS (to monitor/maintain apomorphine pump if relevant)

Contact number for PD nurse

Dietician

Outpatient community-based PT and exercise group to maintain gains