Rehab Unit 3 - Amyotrophic Lateral Sclerosis (ALS)

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KAFO

A _______ is not recommended because it is more fatiguing for the patient to ambulate with a heavy orthosis that to ambulate without the impairment being corrected.

50%

A ________ survival probability after the first symptom of ALS appears is slightly greater than 3 years unless mechanical ventilation is used to sustain breathing.

- Spinal cord - Brainstem - Motor Cortex - V, VII, IX, X, & XII - Anterior

ALS causes a progressive degneration and loss of motor neurons in the _____ _____, _______, and _____ _____, along with involvement of cranial nerves ____, _____, _____, ______, & _______ and the ______ horn cells.

- Progressive - Deteriorating

ALS is a _______ and _______ disease trajectory with the progression from pathology to impairments to activity limitations (functional limitations) to participation restrictions (disability) inevitable.

No

Can the current BCI technology allow the pt to just think of a word or phase and have appear on the screen, or control a w/c by thinking where they want to go?

3-5 years

Death usually occurs ________ after diagnosis and usually results from respiratory failure.

- Several months - 20 years

Disease course varies for each patient with time from onset to death ranging from ________ to _________.

Increasing lordosis

How do some patients compensate for the progressive cervical extensor weakness?

2-3 months at best

How long can riluzole extend survival?

Riluzole (Rilutek)

In 1995, the FDA approved ________, a glutamate inhibitor for ALS treatment.

Spelling Systems

Most ______ ______ display a series of letters, either one at a time or by highlighting letters in a grid, so when the letter you want lights up, your brain wave changes.

Noninvasive

Most _________ systems use electrodes placed on the scalp, usually held in place in a cap that looks like a fabric swimming cap.

35-40

Patients less than ________ at onset had better 5-year survival rates than older patients.

- Shoulder pain - Capsular patterns

Patients with ALS may develop _______ _______ or present with _______ _______ of restrictions.

Light weight

Patients with ALS need ______ ______ orthoses due to energy expenditure issues.

Bulbar

Patients with _______ have initial changes in voice, difficulty moving the tongue, or decreased ability to move the lips or open/close the mouth.

Bulbar-onset

Patients with ________ ALS are more likely to have cognitive impairments.

- 37-44% - 9-16%

Patients with limb-onset ALS have a better prognosis with survival rates of ________ than those with bulbar-onset survival rates of _________ at the 5-year mark.

Cervical Extensor Weakness

Progressive ______ ______ ______ will cause the head to fall forward, resulting in overstretching of the posterior muscles and soft tissues.

50%

Reinnervation can compensate for about _______ of the motor unit loss.

Abnormal Scapulohumeral Rhythm

Shoulder pain may be caused by ______ ______ ______ secondary to spasticity or weakness that causes muscle imbalances, leading to impingement; overuse of strong muscles; muscle strain; poor positioning; or a fall.

35%

Studies show that _______ of patients with ALS showed clinically significant cognitive impairment, and FTD is now being a component of the pathological spectrum of ALS.

True

T/F As motor neurons degenerate, they cannot control the muscles they innervate.

False - reinnervation cannot compensate for the rate of degeneration.

T/F As the disease progress, reinnervation can compensate for the rate of degeneration.

True

T/F Causes of ALS are unknown for the most part except for the small percentage of inherited cases, but multiple mechanisms are suspected that include internal factors and lifestyle factors, such as viral infections.

True

T/F Improvements in cognition have also been seen with NIV.

True

T/F Noninvasive systems cause little or no discomfort, although currently require the use of a conductive gel which must be wiped or washed out of the hair after use.

True

T/F There are 2 types of BCI systems: invasive and noninvasive.

True

T/F Usually, each letter must be 'selected' multiple times, so typing with a BCI is quite slow.

'Keystroke'

The computer of a BCI system looks for the change of brainwave and interprets it as a _________.

Reinnervate

The intact axons can sprout and _______ the partially denervated muscle, which helps preserve strength and function early in the disease process.

Frontotemporal Dementia (FTD)

The nerve cell damage caused by ______ ______ leads to loss of function in these brain regions, which variably cause deterioration in behavior and personality, language disturbances, or alterations in muscle or motor functions.

15

There are ______ new cases of ALS diagnosed every day with incidence at 0.4-2.4 per 100,000.

Juvenile-onset ALS

There are also rare cases of ______ ______ which is inherited through autosomal recessive pattern.

ADLs

Weakness of the UEs greatly impacts a pt's ability to carry out _______.

- Glycopyrrolate (Robinul) - Benztropine (Cogentin) - Transdermal hyoscine (scopolamine) - Atropine - Artane

What are some examples of anti-cholinergic medications?

- Bath tub seats - Shower commode - Handheld shower head - Grab bars - Raised toilet seat - Electronic toothbrush or shaver - Strap-fitted hairbrush

What are some things to assist with self-care and bathing?

- Hyporeflexia - Decreased or absent reflexes - Decreased muscle tone or flaccidity - Muscle cramping (related to hyperexcitability of motor axons)

What are the clinical manifestations of ALS in LMN loss?

- Spasticity - Hyperflexia - Clonus - +Babinski - Hoffman Sign (flicking the nail of the middle finger with response of flexion of ipsilateral thumb and/or index finger) - Muscle weakness

What are the clinical manifestations of ALS in UMN loss?

- Low tech writing boards - Computers with voice synthesizers - Single switch scanning computerized system - Brain-computer Interface (BCI)

What are the interventions for dysarthria?

- Loss of respiratory muscle strength - Decreased vital capacity

What are the respiratory impairments related to?

- Fatigue - Dyspnea with exertion - Difficulty sleeping in supine - Frequent awakening at night - Recurrent sighing - Daytime sleepiness - Morning headaches due to hypoxia

What are the respiratory impairments?

- Liver toxicity - Asthenia - Nausea - Vomiting - Dizziness

What are the side effects of Riluzole (Rilutek)?

- Cognitive decline - Executive functioning impairments - Difficulties with planning, organization, and concept abstraction - Personality/behavior changes

What are the symptoms of FTD?

- Wheeled walker - Lofstrand crutches (rarely)

What assistive devices are utilized for ALS?

- Head and trunk control - Position in sitting - Reinforce strategies for eating/swallowing and use of any communication devices

What can PT (with the SLP) address to manage dysarthria and dysphagia?

- Deep breathing - Diaphragmatic breathing - Exercises to strengthen trunk extensors

What can be done for respiratory muscle weakness?

- Beta-blockers (propranolol or metopropol) - Botox injections into the parotid and submandibular gland - Low dose radiation (effective with medically refractory sialorrhea)

What can be good for patients for thick mucus production?

Benzodiazepines (Librium, Valium)

What can be prescribed for anxiety or patients who have depression with insomnia?

Tricyclic Anti-depressants (Amitriptyline or SSRIs)

What can be prescribed for patients with pseudobulbar affect?

BuSpar

What can be taken for anxiety for a patient who has respiratory difficulties?

- Zipper pulls or hooks - Button hooks - Long handled shoe horn - Velcro clothing closures

What can be use to aid in dressing?

- Foam tubing to increase the size of utensil handles - Long-levered jar opener - Plate guard - Mobile arm support

What can be used for aiding with feeding and eating?

- Foam tubing for pens - Triangular pencil grip - Book holders - Automatic page turner - Adjustable angle table or wheelchair tray

What can be used to aid in writing and reading?

- Soft foam roller - As weakness progresses a semirigid or rigid collar can be prescribed

What can be worn for cervical muscle weakness during specific activities for support?

Fixed dose combination of dextromethorphan/quinidine

What can help reduce the severity and frequency of crying and laughing behaviors?

Video fluoroscopy

What can swallowing exams be conducted with?

- Verbal fluency - Language comprehension - Memory - Abstract reasoning - Generalized impairments in intellectual function

What do ALS pts demonstrate a variety of cognitive impairments in without FTD?

- Frequent rest periods - Supportive seating with high back chairs or recliners - Tilt-in-space or reclining wheelchair - Elevating reading material on a slant board - Education on proper posture and head support

What do patients with cervical muscle weakness need?

Movement imagery

What do systems designed to control a computer cursor often rely on?

Intra-articular analgesic and anti-inflammatory cocktail injection followed by aggressive ROM exercises.

What do the treatments include for shoulder pain?

- Immobility - Adhesive Capsulitis - Spasticity - Contractures

What does pain occur secondary to since sensory pathways are usually spared with ALS?

Fine motor movements such as: - Difficulty buttoning - Pinching - Writing - Foot slapping and tripping while walking.

What does the initial onset usually cause difficulty with?

Degeneration and loss of motor neurons in the spinal cord, brainstem, and brain resulting in a variety of both UMN & LMN clinical signs and symptoms.

What is ALS characterized by?

US: Lou Gehrig's Disease Europe: Charcot's Disease

What is ALS known as in the US and Europe?

Brain-Computer Interface (BCI)

What is a system that allows a person to control a computer or other electronic device using only a person's brainwaves with no movement required?

- Dietary modifications - Patient education for maximizing calories and maintaining adequate hydration - Adaptations for swallowing (tucking the chin down during swallowing or performing a clearing cough after each swallow)

What is dysphagia treatment directed towards?

- Exaggerated articulation - Decreasing rate of speech

What is initial speech therapy working on?

Between 27-43 months

What is the average duration of ALS?

- Spastic bulbar palsy - Dysarthria - Dysphagia - Sialorrhea - Pseudobulbar affect

What is the bulbar pathology in an UMN pattern?

- Flaccid bulbar palsy - Dysarthria - Dysphagia - Sialorrhea - Pseudobulbar affect

What is the bulbar pathology of the LMN pattern?

Muscle Weakness

What is the cardinal sign of ALS that can be caused by an UMN or LMN lesion?

Decreasing the pt's dependence on speech as the primary method of communication.

What is the management focuses as dysarthria progresses?

Contagious Manner

What is the manner of ALS progression?

Focal, asymmetrical muscle weakness

What is the most frequent impairment beginning in the LE or UE bulbar muscles?

- Pain - Anterior muscle tightness

What is the result of the cervical extensor weakness?

Age at time of diagnosis

What is the strongest predictor of a prognosis?

Prescription of anti-cholinergic medications that decrease saliva production

What is the treatment of sialorrhea directed towards?

PEG - Percutaneous endoscopic gastrostomy, a procedure in which a flexible feeding tube is placed through the abdominal wall and into the stomach.

What may a patient need as dysphagia progresses?

Sling

What may a pt with a painful shoulder subluxation need?

Splinting of the wrist or hand

What may be needed to prevent contractures or to improve function with UE muscle weakness?

- Prozac - Zoloft - Elavil - Trofranil

What medications can be used for depression?

- External Ocular - Pelvic Floor

What muscles are usually spared in ALS?

- LMN signs - UMN signs - Progression of the disease within a region or to the other regions

What must a diagnose of ALS require a PRESENCE of?

- Electrophysiological and pathological evidence of other diseases that explain UMN & LMN signs - Neuroimaging evidence of other disease processes

What must a diagnose of ALS require the ABSENCE of?

- Invasion ventilation with tracheostomy - Hospice care to address late-stage respiratory symptoms

What must be decided between when NIV is no longer effective?

Ones with psychological well-being

What patients have shown longer survival times?

50%

When vital capacity decreases to ______, then positive-pressure noninvasive ventilation is recommended.

Bulbar-onset ALS

Which type is more common in middle-aged women?

- Nutritionist - SLP

Who can address early and mild dysphagia?

It affects men more than women but after age 65, the ratio evens out.

Who does ALS affect more?

SLP

Who primarily manages dysarthria impairments?

Amyotrophic Lateral Sclerosis (ALS)

_____ _____ _____ is the most common and devastatingly fatal motor neuron disease among adults.

Palliative Care

______ ______ is an approach that improves the quality of life of patients and their families associated with life-threatening illness, through the prevention and relief of suffering by means of early identification and impeccable assessment and treatment of pain and other problems, physical, psychosocial, and spiritual.

Orthoses

______ may be needed to support weak muscles, decrease the stress on compensatory muscles, conserve energy, or minimize muscle fatigue.

- 70% - 20-30%

______ of patients develop limb-onset ALS with initial involvement in the extremities while _______ develop bulbar-onset ALS with initial involvement in the bulbar muscles.

Invasive

______ systems require surgery to implant electrodes on or near the surface of the brain.

Psychosocial

_______ aspects can greatly affect a pt's quality of life and coping mechanisms.

Knee

_______ impairments can often be achieved through use of AFO.

NIV

________ can decrease symptoms of hypoventilation and increase survival time by several months.


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