Neurodegenerative Diseases Quiz

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Questions and Answers

Which symptoms are associated with the swallowing difficulties in Huntington's Disease?

Improved oral stage efficiency

Cognitive clarity during eating

Choreic movements of the lips and tongue (correct)

Increased muscle strength in the jaw

What is a common issue related to the hereditary nature of Huntington's Disease?

Choosing to ignore genetic predispositions

Absence of family planning considerations

Immediate recovery after diagnosis

Detecting symptoms in older family members (correct)

How does Motor Neurone Disease (MND) primarily manifest at the neurological level?

Isolated weakness in cranial nerves only

Degradation of motor neurons in various regions (correct)

Only affects the lower motor neurons in the spinal cord

Increase in new motor neuron generation

What is one of the behavioral issues that may affect swallow safety in Huntington's Disease?

Eating inappropriate food consistencies (B) Signup and view all the answers

Which of the following symptoms is NOT typically associated with the progression of Huntington's Disease?

Heightened intellectual capabilities (A) Signup and view all the answers

What is a primary feature of neurodegenerative diseases?

They are progressive in nature. (C) Signup and view all the answers

Which symptom is NOT typically associated with neurodegenerative diseases?

Acute respiratory infections (C) Signup and view all the answers

Which of the following is associated with the onset of Parkinson's Disease?

It often starts in mid-to-later life. (D) Signup and view all the answers

What is one of the main reasons for the clinical symptoms of Parkinson's Disease?

Loss of dopamine-secreting neurons. (A) Signup and view all the answers

Which of the following describes Parkinson Plus syndromes?

They include multiple system atrophy. (D) Signup and view all the answers

What is the life expectancy after the onset of Parkinson's Disease symptoms typically?

Around 9 years. (C) Signup and view all the answers

Which part of the brain is primarily affected by Parkinson's Disease?

Substantia nigra. (A) Signup and view all the answers

Which of the following accurately describes the role of the basal ganglia?

They coordinate motor functions. (B) Signup and view all the answers

What is a common early intervention strategy for managing communication difficulties in MND?

Establishing a therapeutic relationship early (D) Signup and view all the answers

Which of the following is a potential symptom associated with swallowing problems in MND?

Coughing on thin liquids (C) Signup and view all the answers

What is the reason behind avoiding muscle-strengthening exercises in individuals with MND?

They stimulate excessive fatigue (B) Signup and view all the answers

What is considered a first sign of dysphagia in patients with MND?

Coughing on thin liquids (A) Signup and view all the answers

Which intervention can be beneficial during the early stages of dysphagia in MND?

Super supraglottic swallow manoeuvre (D) Signup and view all the answers

What aspect of treatment is emphasized in managing patients with oral difficulties?

Ensuring safety of oral intake (D) Signup and view all the answers

Which of the following is a characteristic of Progressive Supranuclear Palsy (PSP)?

It has an unknown cause (A) Signup and view all the answers

Which of the following cognitive problems is associated with PSP?

Reduced concentration (D) Signup and view all the answers

In managing patients' voice therapy, what should be reviewed regularly?

Changes and alteration of management (D) Signup and view all the answers

What form of dysarthria is commonly found in patients with Progressive Supranuclear Palsy?

Spastic dysarthria (B) Signup and view all the answers

Which of the following is NOT a physical issue related to PSP?

Increased muscle flexibility (D) Signup and view all the answers

What therapeutic approach is referenced for voice improvement?

Voice modulation therapy (B) Signup and view all the answers

What is a notable emotional behavior associated with PSP?

Irritability (B) Signup and view all the answers

What is a common characteristic of tremor seen in Parkinson's Disease?

Resting, pill-rolling motion (D) Signup and view all the answers

Which of the following is NOT a cognitive issue related to Parkinson's Disease?

Increased problem-solving skills (C) Signup and view all the answers

What symptom is associated with hypokinetic dysarthria in Parkinson's Disease?

Monopitch and reduced loudness (D) Signup and view all the answers

Which medication combination is commonly used in the treatment of Parkinson's Disease?

Levodopa and carbidopa (Sinemet) (C) Signup and view all the answers

What is a typical swallowing difficulty experienced in Parkinson's Disease?

Delay in swallow trigger (A) Signup and view all the answers

Which of the following is a non-motor symptom associated with Parkinson's Disease?

Dementia (A) Signup and view all the answers

Which factor is related to the typical oral-preparatory stage difficulties in Parkinson's Disease?

Increased mastication time (B) Signup and view all the answers

What type of dysfluency might be observed in patients with Parkinson's Disease?

Neurogenic dysfluency (A) Signup and view all the answers

Which of the following is a recommended approach for managing late-stage dysphagia in patients with MND?

Enteral feeding via PEG (C) Signup and view all the answers

What is a typical symptom of Multiple Sclerosis related to the brain stem?

Dysarthria and dysphagia (C) Signup and view all the answers

Which factor may contribute to the onset of Multiple Sclerosis?

Genetic predisposition (B), Viral infections (D) Signup and view all the answers

How should therapeutic exercises for dysarthria be approached?

Incorporating breath control and pacing (B) Signup and view all the answers

What should be regularly reviewed to manage dysphagia in MND patients?

Swallowing abilities (C) Signup and view all the answers

Which of the following symptoms is associated with cognitive issues in Multiple Sclerosis?

Memory and learning deficits (B) Signup and view all the answers

What type of texture modifications might be necessary for dysphagia management in MND?

Soft and pureed textures (D) Signup and view all the answers

Which aspect should be emphasized when providing support for clients and their families dealing with dysphagia?

Regular communication and advice (C) Signup and view all the answers

Study Notes

Neurodegenerative Conditions: Introduction and Parkinson's Disease

A series of mini lectures will cover neurodegenerative disorders, communication/swallowing difficulties related to them, and implications for speech-language therapy (SLT) management.
Key areas of focus will include listing neurodegenerative disorders, identifying common communication/swallowing problems, and discussing the SLT management implications.

What is Neurodegenerative?

Neurodegenerative diseases are neurological in origin.
They affect the central nervous system (CNS), peripheral nervous system (PNS), or both.
They are progressive in nature.

Neurodegenerative Disease Impacts

Neurodegenerative disorders can impact speech (dysarthria, AOS), language (aphasia), cognitive abilities, swallowing, behavior, physical abilities, mental health, and families/carers. This encompasses all four dimensions of the WHO's International Classification of Functioning, Disability and Health (ICF).

Parkinson's Disease (PD)

Idiopathic (unknown cause) is the most common form.
Secondary PD can have multiple causes, such as trauma in boxing.
Parkinson-plus syndromes like multiple system atrophy are also possible.
PD begins in mid-to-later life.
Prevalence is approximately 1 in 500 people.
Symptom onset to death is roughly 9 years.

What Causes PD?

PD is associated with a significant depletion of dopamine.
Dopamine-producing neurons in the substantia nigra (part of the basal ganglia) are lost.
A conclusive cause for the death of these neurons is currently unknown.
Recent evidence implies that harmful protein clumps within these neurons may be the cause of cell death.

Basal Ganglia Recap

Basal ganglia is a collection of subcortical nuclei.
These include the corpus striatum (caudate and putamen), globus pallidus, subthalamic nucleus, and substantia nigra.
They work together to control motor function.
They facilitate the initiation/termination of movements and suppress extraneous movements by selectively activating and suppressing learned motor programs.

Parkinson's Disease (PD): Salient Characteristics

Tremor (resting, "pill-rolling")
Bradykinesia (slowness of movement, difficulty initiating movement) leading to:
- Reduced facial expression ("mask-like facies")
- Micrographia (small handwriting)
- Dysarthria (speech difficulties)
Rigidity ("cogwheel rigidity"—increased muscle tone)
Other issues for SLTs: hypokinetic dysarthria, dysphagia, and associated cognitive issues (like dementia).

Non-motor Symptoms of PD

Understanding of Parkinson's disease is evolving, with recognition of numerous non-motor symptoms.
A comprehensive understanding of these symptoms is crucial for effective management and assessment.

PD – Neuropsychiatric Symptoms

Memory and cognitive changes (including dementia)
Depression
Anxiety
Hallucinations

Hypokinetic Dysarthria

Speech features:
- Imprecise consonants
- Monopitch (single pitch)
- Monoloudness (single loudness)
- Reduced loudness
- Brief rushes of speech (potential acceleration of AMRs, such as /papapa/)
- Repetitive phonemes (possible neurogenic dysfluency)
- Palilalia (repetitions of words/phrases)
- Reduced vital capacity

PD: Cognitive/Language Issues

Distractibility
Impaired organizational and planning skills (executive functioning)
Shared attention difficulties
Memory problems
Difficulty learning new information
Problems with word retrieval
Dementia (approximately 30% of cases)
Lewy body dementia is sometimes seen or associated with PD

PD: Typical Swallowing Difficulties (Oral Prep/Oral Stages)

Increased mastication time
Limited mandible movement
Repetitive tongue pumping
Difficulty forming a cohesive bolus
Slow oral transit

PD: Typical Swallowing Difficulties (Pharyngeal Stage)

Delayed swallow trigger
Reduced hyolaryngeal excursion, thus decreased airway protection during swallowing
Piecemeal deglutition
Reduced pharyngeal constriction
Residue in valleculae and pyriform sinuses
Silent aspiration
Anterior saliva escape
Fewer spontaneous swallows

PD: Typical Swallowing Difficulties (Oesophageal Stage)

Feelings of oesophageal obstruction
Reflux
Chest pain
General gastrointestinal dysfunction

PD Medication

Levodopa (L-dopa)
Levodopa/carbidopa combinations (e.g., Sinemet)
"On-off" effects (impacts on assessment/intervention)
Peak-dose dyskinesia (overcorrection of dopamine)

Aims of SLT Management in PD

Maximising residual skills (e.g., LSVT, breath support)
Maintaining functional communication
Augmentative and alternative communication (AAC) systems (e.g., amplifiers)
Ensuring safety of oral intake
Reviewing for changes and adapting management accordingly
Providing support and advice as a holistic approach

Lee Silverman Voice Treatment (LSVT)

This therapy has a large and growing evidence base.
Other variations of the therapy exist, such as voice modulation therapy.

Progressive Supranuclear Palsy (PSP)

Now seen as distinct from Parkinson's disease (PD) but may be confused with it.
Unknown cause.
Rare (5 new cases per 100,000 people over 50 per year).
Onset to death is approximately 10 years.
Physical issues (balance, mobility, eye movements)
Behavioral issues (irritability, apathy)
Cognitive concerns (memory, reduced concentration)
Speech (often includes spastic dysarthria) and swallowing problems, often more severe than in PD.

Huntington's Disease (HD)

Inherited autosomal dominant neurodegenerative disorder.
Symptoms develop in the fourth decade.
Death occurs 15–20 years after onset.
Affects approximately 4-7 individuals per 100,000.
Equal male to female ratio.

Huntington's Disease (HD): Symptoms

Chorea (uncontrolled movements) and rigidity
Dystonia (sustained muscle contractions)
Incoordination
Eye movement abnormalities
Weight loss
Dysarthria (hyperkinetic—uncontrolled movements affecting speech)
Dementia
Dysphagia (swallowing difficulties)
Personality changes
Attention deficits
Dementia
Depression
Specific language deficits

Huntington's Disease (HD): Specific Language Deficits

Table listing language tasks, findings, and evidence related to language deficits, from Yorkston, Miller and Strand (1995).
- Connected Speech: Reduced number of words, decreased syntactic complexity, reduced phrase length, and increased paraphasic errors.
- Naming: More visually-based errors than normal, decreased confrontation naming.
- Auditory Comprehension: Decreased understanding of subtle prosodic aspects, reduced token test scores.

Huntington's Disease (HD): Hyperkinetic Dysarthria

Chorea impacting respiration, phonation, and articulation.
- Respiration: Intensity changes, sudden forced exhalation/inspiration.
- Phonation: Elevated pitch, harsh vocal quality.
- Articulation: Prolonged and distorted phonemes.

HD: Speech Management

Mild cases: use of relaxation techniques.
Moderate cases: using environmental approaches, facilitated communication.
Severe cases: AAC options, but note additional physical, cognitive, or behavioral issues may be present.

HD: Swallowing Issues

Physical challenges and positioning difficulties.
Whole body chorea.
Lip, jaw, tongue movements affect oral phase
Behavioral issues may impact swallow safety, including inappropriate consistencies.
Need for additional calories.

HD: Specific Management Issues

Awareness of the inherited nature of the disease.
Genetic testing options.
Early intervention is crucial.

Motor Neuron Disease (MND)

Characterized by the loss of motor neurons in the motor cortex (UMN), brainstem (UMN & LMN), cranial nerves (LMN), and spinal nerves (LMN).
Affects corticonuclear (corticobulbar) and corticospinal tracts.
Most people are diagnosed after 40, with the highest incidence between 50 and 70 years old.
Men are diagnosed approximately twice as often as women.
Incidence: about two people in every 100,000 will develop MND yearly.

MND Types

Amyotrophic Lateral Sclerosis (ALS)
Progressive Bulbar Palsy (PBP)
Primary Lateral Sclerosis (PLS)
Progressive Muscular Atrophy (PMA)

MND: Symptoms

Table showing upper motor neuron and lower motor neuron symptoms (weakness, reduced movement, increased muscle tone, strained/strangled voice, muscle atrophy, fasciculations, breathy voice).

SLT Diagnoses in MND

Dysarthria, including anarthria and AOS.
Cognitive issues (affect communication)
Dysphagia (swallowing difficulties)

MND: Communication, SLT Role

Early intervention to address communication difficulties early.
Therapeutic relationship with the patient and their family.
Support and understanding to help with the changes.
Maintaining functional communication.

MND: Swallowing Problems

Speech and swallowing often co-occur.
Consistent pattern of decline.
Reduced breath support/reduced airway protection (can be a cause of apnoea during swallowing)
Reduced respiratory support may impact surgical intervention.
Weakness impacting mastication, oral manipulation, and lipseal.
Issues with thin liquids can be an initial sign of dysphagia (e.g., coughing).
Saliva texture changes (xerostomia, escape)

MND: Early Stage Dysphagia Intervention

Chin tuck maneuvers
Specific swallow maneuvers, like supraglottic swallows.
Dietary changes (avoiding foods that require extensive chewing, using thickened fluids)
Advice on distractions and how to respond to choking episodes.
Enteral feeding (PEG tube) discussions.

MND: Later Stage Dysphagia Management

More intensive texture modifications (purees).
Thickening fluids (for smoother swallowing)
Increasing sensory awareness (e.g., temperature, taste).

MND: Late-Stage Dysphagia Management

Enteral feeding potentially indicated (PEG).
Consideration of multiple choking, aspiration episodes, and patient aversion.

MND: Dysphagia Management

Regular review of swallowing.
Discussion of eating/drinking options.
Liaison with relevant medical teams.
Supportive advice.
Ongoing support for the patient and family.
Supervision and support given to the SLT.

Multiple Sclerosis (MS)

Progressive or remitting-relapsing autoimmune disease.
Demyelination impacting the CNS.
Myelin being stripped away by macrophages.
Scar tissue formation (sclerosis).
Nerve conduction block.
Remitting-relapsing starts between ages 18 and 40, and progressive type starts later.
Genetic as well as viral potential causation is proposed.

MS: Symptoms

Symptoms vary depending on the CNS site affected.
Symptoms include impairments to cognitive and behavioral domains.
Symptoms are categorized according to different anatomical regions affected, including the cerebrum, spinal cord, brain stem, cerebellum, and optic nerve.

MS: Communication

AOS is rare.
Dysarthria (spastic, ataxic, mixed) is present in about 50% of instances.
Language impairments include aphasia but are less common in comparison to the prevalence of the other symptoms.
Executive/higher-order language problems like Word-finding difficulties are possible.
Cognitive impairments such as memory, learning, problem-solving, and affect (e.g., depression).
Fatigue experienced with exertion.

MS: Communication Management

Dysarthria management involves breath control exercises for phonation and volume. Be aware of and address fatigue.
Rate and pacing are management tools for equal and excessive stress patterns and fatigue.
Assistive communication options (AAC), such as an alphabet board, can be considered.
When considering AAC options, consider existing tremors, visual issues, spasticity, or ataxia, as these are all factors to consider.

MS: EDS Management

Lesions often found in the brainstem.
Symptoms are often associated with delayed swallow reflex and reduced pharyngeal peristalsis.

Neurodegenerative Disorders Summary

PD, HD, MND, MS, Friedreich's ataxia.
Progressive supranuclear palsy.
Dementia.

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Description

Test your knowledge on the symptoms and characteristics of neurodegenerative diseases such as Huntington's Disease and Parkinson's Disease. This quiz covers various aspects including genetic factors, motor functions, and behavioral issues related to these conditions. Challenge yourself to see how well you understand these complex disorders.