DPT 860 – Neurological Physical Therapy II Guided Notes – Amyotrophic Lateral Sclerosis

This set of flashcards covers key concepts from the lecture on amyotrophic lateral sclerosis, including its pathophysiology, diagnostic criteria, prognosis, symptoms, and therapeutic management strategies.

What is the typical age of onset for amyotrophic lateral sclerosis (ALS)?

Mid to late 50s, increasing with each decade of life.

What percentage of ALS cases are familial due to genetic predisposition?

5-10%.

True or False: ALS is a static disease.

False, ALS is a progressive neurodegenerative disease.

Where are upper motor neuron cell bodies located?

In the cortex, corticospinal tracts, and brainstem nuclei tracts.

Where are lower motor neuron cell bodies located?

In anterior horn cells within lateral and medial motor nuclei of the spinal cord and brainstem nuclei for cranial nerves.

Name  cranial nerves that are preferentially affected by ALS.

Cranial nerves V, VII, X, or XII.

What are 5 non-motor areas that can be affected by ALS?

1. Autonomic nervous system 2. Basal ganglia 3. Cerebellum 4. Frontotemporal region 5. Oculomotor and sensory systems.

What happens to denervation and reinnervation in ALS?

Neighboring motor neurons can reinnervate muscle fibers until degeneration reaches 50%.

True or False: There are definitive tests and biological markers to diagnose ALS.

False, there are no definitive tests.

What are the three factors required for ALS diagnosis?

1. LMN signs 2. UMN signs 3. Disease progression within a region or to others

What is the most common cause of death from ALS?

Respiratory failure.

What is the average time from symptom onset to death in ALS?

Several months to 20 years.

What are the two primary phenotypes of ALS?

1. Limb onset 2. Bulbar onset.

What initial symptoms are associated with limb onset ALS?

Initial symptoms involve the extremities.

What initial symptoms are associated with bulbar onset ALS?

Difficulty speaking, chewing, and swallowing.

What are the cardinal signs of LMN damage?

Atrophy, hyporeflexia, hypotonia, fasciculations.

What are the cardinal signs of UMN damage?

Hyperreflexia, spasticity, pathological reflex.

True or False: All patients with ALS will eventually develop bulbar symptoms.

True.

What is sialorrhea?

Excessive saliva and drooling.

What vital capacity percentage indicates significant risk of impending respiratory failure in ALS patients?

Less than 25-30% of predicted.

What percentage of ALS patients demonstrate cognitive impairment?

35.6%.

Name three areas of cognition that may be affected in ALS.

Planning, organization, and verbal fluency.

What is palliative care?

Improves quality of life for patients facing life-threatening illnesses by preventing and relieving suffering.

List the FDA-approved drugs currently on the market to treat ALS.

Riluzole, Tiglutik, Exservan, Relyvrio, Radicava, Qalsody.

True or False: Disease-modifying pharmacotherapy can extend survival time for a few months.

True.

What is the purpose of the multidisciplinary care plan for ALS patients?

Includes a neurologist and at least four other disciplines, updated at least annually.

What LMN sign is characterized by involuntary muscle twitching visible under the skin?

Fasciculations.

What LMN sign refers to a decrease or absence of reflexes?

Hyporeflexia.

A reduction in muscle tone, or 'floppiness,' is a sign of LMN damage known as what?

Hypotonia.

What UMN sign is characterized by exaggerated or overactive reflexes?

Hyperreflexia.

The presence of stiffness and increased muscle tone, often velocity-dependent, is a characteristic UMN sign known as what?

Spasticity.

What UMN sign are abnormal reflexes such as the Babinski sign considered?

Pathological reflex.

Difficulty with sequencing tasks and initiating appropriate actions is a cognitive impairment in ALS related to which executive function?

Planning.

Which cognitive impairment in ALS affects a patient's ability to structure and manage their thoughts and actions effectively?

Organization.

Which cognitive domain, involving the generation of words within certain categories, can be affected in ALS?

Verbal fluency.

Can the frontotemporal region be affected in ALS, potentially leading to cognitive and behavioral changes?

Yes, it is one of the non-motor areas that can be affected.

Can the autonomic nervous system be affected in ALS, leading to issues such as orthostatic hypotension or bladder dysfunction?

Yes, it is one of the non-motor areas that can be affected.

Which of the following is considered to be associated with a better prognosis of ALS?

No symptoms of dyspnea at onset

LMN weakness in ALS begins in isolated muscles and progresses in what fashion?

distal to proximal

TRUE or FALSE: Patients with ALS demonstrate a linear rate of loss of respiratory strength and decrease in vital capacity.

true

List 7 sx of respiratory compromise

fatigue, dyspnea on exertion, orthopnea, insomnia, recurrent sighing, excessive daytime sleepiness, morning headaches

Vital capacity of _________% of predicted indicates significant risk of impending respiratory failure or death

<25-30%

True or false: you should monitor body weight closely with ALS

true: weightloss can be a sign of impaired nutrition or a sign that further nutritional assistance via a tube may be needed

At which value of vital capacity is it safest to place a PEG tube?

>50%

A LOW score on the braden scale for pressure sores indicates

HIGH risk of pressure sores

TRUE or FALSE: Sensation is often spared in ALS or affected to a much lesser extent than the motor system.

true

Which disease-specific test and measure is used to quantify ADL and global function in people with ALS

ALSFRS-R

What are the primary roles of PT in ALS management?

1. Promote independence and maximize function throughout disease

2. Promote health and wellness in early to middle stages

3. Minimize or prevent complications

4. Provide education, psychological support, and recommendations for equipment/resources

Which cervical muscle group is most prone to weakness with ALS?

cervical extensors

TRUE or FALSE: Cervical collars may be recommended to help support head position during certain activities but are not always well tolerated.

true

What is a common shoulder pathology with ALS?

frozen shoulder/adhesive capsulitis