ALS Flashcards
(41 cards)
What is ALS (2)?
- Amyotrophic Lateral Sclerosis (Lou Gehrig’s Disease)
- degeneration and loss of motor neurons in the spinal chord, brainstem, and brain (CNS).
Who does ALS’s affect (4)?
- avgerage onset 55 y/o (can occur at any age)
- 20% more common in men than women
- 5-10% of cases are inherited
- large majority have no family history of disease
What are the early symptoms of ALS (7)?
- painless, PROGRESSIVE MUSCLE WEAKNESS (most common)
- tripping
- dropping things
- abnormal fatigue in arms and/or legs
- slurred speech
- muscle cramps and twitches
- and/or uncontrollable periods of laughing or crying
Since ALS attacks only motor neurons, what is not affected (2)?
- the 5 senses (sight, touch, hearing, taste, smell)
- for many people, muscles of eye’s and bladder
For most patients what is the prognosis with ALS?
- for most 3-5 years after diagnosis
- death usual result of respiratory failure
What characteristic make for a better prognosis for patients with ALS?
- if < 35-40 y/o have better 5 year survival rates
- if limb onset rather than bulbar (mouth and throat) onset
Signs and symptoms of ALS _____ _______ within a ______ before moving to other regions.
spread locally; region
regions include bulbar, cervical, thoracic, lumbosacral
Clinical presentation of muscle weakness with ALS (4)?
- focal, asymetrical muscle weakness beginning in LE (feet), UE (hands), or bulbar muscles (face)
- initially isolated muscles distally and progress proximal
- cervical extensor weakness is typical
- weakness leads to 2° impairments (decr ROM, subluxation, joint contractures, adhesive capsulitis)
Why does the clinical presentation of fatigue happen in ALS (3)?
- as motor neurons die, remaining or sprouted neurons have to work harder
- weak muscle have to work at a higher % of max strength to perform same activities
- could also be from sleep disturbance, respiratory impairments, hypoxia, depression.
What other LMN signs can/will a patient have with ALS (3)?
- hyporeflexia, decreased or absent reflexes, decreased tone or flaccidity
- muscle cramping
- fasciculations (random twitching seen through skin)
Are fasciculation a usual ‘initial’ symptom with ALS?
- no, common but rarely an initial symptom
What UMN sign and impairments are seen with ALS (4)?
- spasticity
- hyperreflexia
- clonus
- babinksi or hoffman
What happens to UMN signs as ALS progresses?
- may decrease
What bulbar impairments are seen with ALS (4)?
- dysarthria (difficult speech)
- dysphagia (difficult swallowing)
- sialorrhea (excessive saliva and drooling)
- pseudobulbar affect (poor/impaired emotional control)
What respiratory impairments are seen with ALS (6)?
- loss of respiratory muscle strength
- decreased vital capacity
- early signs of fatigue, dyspnea with exertion, diff sleeping supine, walking at night, daytime sleepiness, morning headache d/t hypoxia
- progressing to truncated speech, orthopnea, dyspnea at rest, paradoxical breathing, accessory muscle use, weak cough
If vital capacity reaches < 25-30% and the patient doesn’t do on a ventilator whats gonna happen?
- CO2 retention will lead to acidosis, coma, and death d/t respiratory failure
If an ALS patient has no muscle support what are they at high risk of getting?
- pneumonia
What % of patient with ALS get cognitive impairments?
- 35.6%
What cognitive impairments are seen with ALS?
- ALS associated frontotemporal dementia (FTD)
- patients with bulbar onset are more likely to have cognitive impairments
How is ALS diagnosed?
The presence of:
- >/= 1 LMN sign (by clinical electrophysiological or neuropathological exam)
- >/= 1 UMN sign (by clinical exam)
- 1 region (progression within a region or to other regions of body)
The absence of:
- electrophysiological or neuropathological evidence of other disease
- neuroimaging evidence of other diseases.
What does medical management of ALS consist of (2)?
- symptomatic management (palliative care)
- only current drug approved is riluzole (Rilutek) and may extend life 2-3 months.
Goals of medical management in ALS (7)?
- inform patient/family of diagnosis and prognosis
- diagnosis and cognitive concerns
- drug therapies
- multidisciplinary management and palliative care
- manage communication problems (sign and symptoms list)
- nutrition management PEG decisions (feeding tube)
- respiration management and ventilation devices
What ALS specific quality of life measure are there (5)?
- ALSFRS (functional rating scale)
- SF-36
- SEIQoL-DW
- sickness impact scale
- ALSAQ-40 (assessment questionnaire)
How often is an ALS patient evaluated by the entire clinic team?
- every 6 months
