Parkinson's Disease

Question 1

Definition of PD

Answer 1

Neurodegenerative disease and movement disorder caused by degeneration of dopaminergic neurons in the substantia nigra

Question 2

Area of NS affected

Answer 2

CNS: basal ganglia (substantia nigra) degeneration.
Extrapyramidal pathway affected

Question 3

Epidemiology of PD

Answer 3

• Incidence: 4 per 100,000 in 20-49 years. 196 per 100,000 in 85-89 years.
• Prevalence: increases with age. Overall 100-180 per 100,000. 40 per 100,000 in 40-49 years. 1,900 per 100,000 80+ years.
• Higher incidence/prevalence in males
• 6 million worldwide
• 12,000 in Ireland

Question 4

Cause of PD

Answer 4

• Synucleinopathy (neurodegenerative diseases characterised by the abnormal accumulation of aggregates of alpha-synuclein protein in neurons)
• Multifactorial: mix o genetic and environmental factors (pesticides, herbicides, MPTP)
• Family history in 20-30%

Question 5

Course of PD

Answer 5

Gradual onset and progression. Early signs include tremor, bradykinesia, rigidity, hypophonia.
1. Preclinical
2. Prodromal
3. Clinical phase

Question 6

Prognosis of PD

Answer 6

• PD reduces life expectancy (largely due to dementia)
• About 25–40% of the patients with PD eventually develop dementia
• Risk of dementia is 1·7–5·9 times higher in pts with PD than in healthy people
• Risk factors for dementia include: age at onset, disease duration or severity, APOE genotype.

Question 7

Cardinal features of PD

Answer 7

TRAP
*Tremor – usually a tremor at rest (pill
rolling). Typically disappears with
action and during sleep
*Rigidity – Increased muscle tone and
increased resistance to movement
*Akinesia/Bradykinesia – Slowness in
initiation and execution of
movements
*Postural instability – causes falls
*Other - freezing

Question 8

Motor symptoms of PD

Answer 8

• Tremor, bradykinesia, rigidity, postural instability
• Hypomimia (reduced faciala expression), dysarthria, dysphagia, sialorrhea
• Shuffling gait
• Micrographia

Question 9

Non-motor symptoms of PD

Answer 9

• Cognitive impairment, bradyphrenia, WFDs
• Depression, apathy
• Sensory: anosmia, ageusia, pain, parasthesias
• Dysautonomia
• Sleep disorders

Question 10

what is bradyphrenia?

Answer 10

Slowness of thought, delayed responses and lack of motivation

Question 11

what is anosmia?

Answer 11

loss of smell

Question 12

what is ageusia?

Answer 12

loss of taste

Question 13

Diagnosing PD

Answer 13

• Usually diagnosed by an experienced neurologist typically over at least a five
  year period

1. UK Brain Bank Criteria
2. MDS Clinical Diagnostic Criteria for Parkinson’s Disease
3. National Institute of Neurological Disorders and Stroke (NINDS) diagnostic criteria for PD

Consider:
1. Hallmark clinical features
2. Neuroimaging to exclude other conditions only
3. Response to drug treatment

Question 14

Rating Scales for PD

Answer 14

*Unified Parkinson’s Disease Rating Scale
*Hoehn and Yahr Scale

Question 15

Treatment for PD

Answer 15

• Symptom-management
• Aimed at restoring neurochemical balance either by anticholinergic agents or with drugs that enhance dopaminergic pathway
• Best delayed until symptoms warrant it due to drug side effects.

1. Pharmacological
2. Surgical
3. Rehabilitation

Question 16

Medication for PD

Answer 16

1. Levodopa: Gold Standard, chemical building-block that your body converts into dopamine.
2. Dopamine Agonist: act like dopamine to stimulate nerve cells
3. Enzyme Inhibitors: Prevent breakdown of dopamine.

Others:
* Anticholinergics and Amantadine – used for treatment of tremor
* Apomorphine (a strong subcutaneous/infusion dopamine agonist)
* Glutamate antagonist (e.g., Amantadine- mild effect)
* COMT inhibitors (e.g., Comtess- block enzyme breaking down levodopa)
* MAO-B inhibitors (e.g., Selegine, block an enzyme preventing breakdown of dopamine)
* Some PD medication (dopamine agonists & levodopa) can lead to impulsive/ compulsive behaviour (ICD)
e.g. excessive shopping, gambling

Question 17

How does L-Dopa work?

Answer 17

• Natural substrate for the synthesis of dopamine
• Able to cross blood brain barrier so can reach its site of action following oral administration
• Effect on speech/swallowing

Efficacy limited after 2-5 years by:
1. Motor fluctuations: “on-off” is unpredictable - switch from benefit from medication (“on”) to an akinetic-rigid state (“off”)
2. Dyskinesias: involuntary movements occurring in association with drug treatment

Question 18

Surgical tx for PD

Answer 18

• Deep Brain Stimulation: requires thorough case hx to determine if appropriate.
• Benefit of DBS on voice and speech quality varies between studies.
• Patients can report more severe symptoms such as: exacerbation of slurred speech, adverse impact on rhythm, intonation, articulation
  and intelligibility, interference with social interaction.

Question 19

Role of SLT

Answer 19

• Communication: speech, voice, language ax and tx.
• Eating, drinking, swallowing ax and tx
• AAC
• Capacity ax
• Ax for Deep Brain Stimulation
• Education , support and counselling

Question 20

Features of dysarthria in PD

Answer 20

Hypokinetic dysarthria:
* Low volume
* Imprecise articulation
* Dysphonia
* Monotone
* Monopitch
* Abnormal rate
* Palilalia (involuntary repetition of syllables)

Question 21

Speech ax tools for PD

Answer 21

• AIDS
• Frenchay AX
• UPDRS subsection
• DIP
• Speech sample

Question 22

Speech tx for PD

Answer 22

Traditional therapy:
*Pacing boards
*Rate control drills
*Increased respiratory support
*Increased vocal cord adduction
*Increasing stress
*Focus on subsystems
*LSVT

Question 23

Dysphagia in PD

Answer 23

• May involve oral, pharyngeal or
  oesophageal phases of swallowing
• May be present in every stage of the disease.
• Associated with increased risk of aspiration pneumonia and mortality
• Variability in rate of dysphagia reported in literature: 18.5% to 100%
• Pneumonia is a main cause of death in IPD (4- 30%)
• Age, disease duration and dementia all seem to contribute.

Question 24

Clinical evaluation for PD:

Answer 24

1. Case history
2. Presentation
3. Oro-facial
4. Swallow trials

Question 25

Case history

Answer 25

* Time since PD diagnosis * Rate of progression * Comorbid symptoms/conditions * Pharmacological Tx of PD * Response to medications * Surgical Tx * Chest status * ?weight loss * Time taken to complete meals? * Choking episodes? * Avoiding certain foods? * Swallowing tablets?

Question 26

Presentation

Answer 26

* Alertness * Posture/positioning * Mobility * Cognition/memory * Language (able to give report?) * Feeding ability (i.e. Tremor? Dyskinesia?) * Salivary control * Confusion * Hypokinetic dysarthria? * Impact of dysphagia on QOL

Question 27

Oro-facial

Answer 27

* Masked facial expression? * Open mouth posture? * Involuntary movements? * Dry oral muscosa * Drooling? * Tardive dyskinesia? * Dentition * Rate & range of lingual movement? * Rule out asymmetry * Voice quality * Cough response (vol/spon)

Question 28

Swallow trials

Answer 28

* Self-feeding ability? * Rate of feeding? * Anterior spillage? * Delayed oral phase? (tongue pumping) * Prompt pharyngeal swallow? * Weak hyo-laryngeal excursion? * Cough response? * Voice quality post swallow? * Oral residue? (ant/lat sulci) * Time taken to complete? * Effort involved? * Improvement with volume/taste

Question 29

Common VFS findings

Answer 29

Oral prep/oral phase * anterior spillage of fluids * repetitive tongue pumping * prolongation of oral transit time * difficulty with bolus formation * impaired mastication * difficulty initiating swallow * premature spillage of material into pharynx * residue post swallow on the tongue surface, in lateral sulci and in the vallecular sinuses Pharyngeal phase: * delayed pharyngeal initiation of swallow * reduced pharyngeal peristalsis * reduced posterior motion of the tongue base towards PPW, leading to residue both in the valleculae * reduced hyo-laryngeal excursion leading to residue in pyriform sinuses * silent aspiration on fluids before swallow due to pharyngeal reflex delay * Benefit from chin tuck/smaller volumes of fluid, taste/temperature changes

Question 30

Dysphagia tx for PD

Answer 30

* Timing of medication (L-Dopa) * Postural changes (e.g. chin tuck) * Compensation (e.g. volume change) * Lip, tongue & laryngeal elevation exercises?? (Logemann 1998) * Diet modification (e.g. thickened drink) * Biofeedback (sEMG) * Verbal cueing * Sensory stimulation (iced water, sour taste) * LSVT (El Sharkawi et al, 2003) * Expiratory Muscle Strength Training (EMST)

Question 31

Drooling in PD

Answer 31

* Involuntary loss of saliva- social embarrassment * 56% (32-74% range) prevalence of drooling in PD based on 8 studies in a systematic review Management options include: * Swallow reminder device * Sour sweets * Chewing gum * Dysphagia Treatment * Postural changes * Hyoscine patch * Botox to parotid glands * Radiation to salivary glands

Question 32

Atypical Parkinsonian Syndromes (APS)

Answer 32

* Encompass a collective of rare neurodegenerative diseases including: * Progressive supranuclear palsy (PSP) * Multiple system atrophy (MSA) * Corticobasal syndrome (CBS) * Dementia with Lewy Bodies (DLB) * Characterised by rapid disease progression and decreased life expectancy (Roach et al., 2020) * Can be particularly debilitating and are not yet well understood * Dysarthria or dysphagia within 1 year of disease onset was a distinguishing feature for APS (specificity, 100%) (Muller et al 2001)

Question 33

Progressive Supranuclear Palsy

Answer 33

* Rare progressive disease causing degeneration of neurons in the brainstem, basal ganglia and cerebellum Typically characterised by: * Postural instability – tend to fall backward * Supranuclear vertical gaze palsy leading to impaired vision * Dementia * Dysarthria * Dysphagia Two subtypes: Richardson syndrome (PSP-RS) and PSP-parkinsonism (PSP-P) * Poor prognosis: Rapidly progressive, mean duration of survival of 10 years * Cause: Tauopathy * Average age of onset in the mid-60s * Disease duration approximately 6-9 years

Question 34

PSP vs PD

Answer 34

PD: *Cause: dopamine loss in substantia nigra *Tremor: present *Eye movement: normal *Cognitive decline: gradual *Response to Levodopa: good response *Prognosis: slow progression PSP: *Cause: tau protein accumulation *Tremor: absent *Eye movement: vertical gaze palsy *Cognitive decline: early executive dysfunction and dementia *Response to Levodopa: poor *Prognosis: rapid

Question 35

Dysphagia in PSP

Answer 35

* PSP is associated with early onset of dysphagia when compared with IPD * Aspiration pneumonia is the principal cause of death

Question 36

Speech in PSP

Answer 36

* Hypokinetic and mixed hypokinetic–spastic dysarthria observed most frequently * AOS sometimes present (19.5%)

Question 37

Main features of PSP

Answer 37

Onset in 6th to 7th decade * More men than women affected * Initial features loss of balance, gait disorder & falling * Akinetic-rigid state with symmetrical signs & prominent axial rigidity follows * Trunk & neck hyperextended * Wide-eyed stare * Furrowing of forehead * Deepening of other facial creases * Pseudobulbar palsy * Dysarthria * Dysphagia * Frontal lobe features * Dementia

Question 38

What is multiple systems atrophy?

Answer 38

* Synucleinopathy * Affects balance, movement and the autonomic nervous system. * REM, muscle stiffness and behaviour changes * Prevalence ranging from 31%-78% * Mixed dysarthria with combinations of hypokinetic, ataxic, and spastic components

Question 39

Dementia with Lewy Bodies

Answer 39

* Synucleinopathy * Fluctuating cognition with pronounced variations in attention and alertness * Recurrent visual hallucinations * REM sleep behaviour disorder which may precede cognitive decline * One or more spontaneous cardinal feature of parkinsonism – bradykinesia, rest tremor, or rigidity * Hypophonic/monotonous speech predominated in DLB

Question 40

Corticobasal Syndrome (CBS)

Answer 40

* Tauopathy * 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

Question 41

Dual Tasking/Divided Attention in Parkinson's Disease (PD)

Answer 41

Challenges in Dual-Tasking in PD *Motor Impairment *Cognitive Dysfunction *Basal Ganglia Dysfunction *Decreased Automaticity: more conscious effort, competing with cognitive tasks. Effects of Dual-Tasking in PD *Motor Task Impairment: Slower walking, poor coordination, and increased falls when combining motor and cognitive tasks *Cognitive Task Impairment: worsens during simultaneous motor tasks. *Freezing of Gait (FOG): Episodes of freezing are more likely when multitasking. *Increased Cognitive Load Research Findings *Slower Gait: Slower walking speed when performing cognitive tasks simultaneously. *Worsened Gait/Balance *FOG Triggers *Levodopa Effects: improves motor function but may not significantly improve dual-tasking performance; cognitive function may remain impaired or worsen. Strategies to Improve Dual-Task Performance *Physical Therapy *Cognitive Training *Compensatory Strategies: Use task simplification, prioritization, and external cues to aid multitasking. *Assistive Devices: Use canes, walkers, or specialized shoes to reduce falls and support dual-tasking. *Controlled Dual-Task Training: Structured practice in a safe environment to improve multitasking abilities.