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38 Cards in this Set
- Front
- Back
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What does AMYOTROPHY mean?
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Loss of muscle - the LMN involvement in ALS
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What does LATERAL mean?
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Involvement of the LATERAL corticospinal tracts (UMN) in ALS
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What does SCLEROSIS refer to?
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The secondary gliosis from UMN axon loss in the Lat corticospinal tracts
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So what distinguishes ALS?
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BOTH umn and lmn involvement
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In what race is ALS esp UNcommon?
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Blacks
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What is the peak age of onset of ALS?
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55-75 yrs old
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What type of ALS is esp underdiagnosed in elderly?
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Progressive bulbar palsy - PBP type
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What is the incidence of ALS per 100,000 in the USA? Prevalence?
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Incidence is 3-5/100000
Prevalence 6-8/100000 |
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How many years do patients with ALS usually survive?
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Only 3-5 yrs
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What are the rates of progression and patterns of onset of ALS like in general?
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Highly variable
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What are 3 'patterns' of onset of ALS? How does each differ in how its manifested??
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-LMN onset - spinal mm atrophy
-UMN onset - primary lateral sclerosis -Bulbar onset - progressive bulbar palsy |
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Which type of onset of ALS has a better chance of going on to some other disease and not full-blown ALS?
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LMN onset
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In what population is Progressive Bulbar palsy onset (PBP) of ALS more common?
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Particularly elderly
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What symptoms are seen in the PBP type of ALS?
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-Swallowing difficulty
-Speech difficulty |
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What happens in the large majority of patients with either PBP or primary lateral sclerosis type onset?
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Progression to full blown ALS
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What % of cases of ALS are familial? Inheritence?
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5-10% - autosomal dominant
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What is the mutation associated with 20% of familial ALS cases?
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SOD-1 superoxide dismutase
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Can we do genetic testing for SOD1?
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No - too many different mutations exist
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What are some common symptoms of ALS?
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-Weakness/cramps (lmn)
-Muscle twitches (lmn) -Spasms/hyperreflexia (umn) -Speech slurred, swallowing, drooling, SOB, impaired speech |
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What is a common sign of Pseudobulbar palsy?
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Emotional lability
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What is decreased cognitive function a sign of in patients with ALS?
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Frontotemporal dementia!
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What are some common signs of LMN involvement on PE of patients with ALS?
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-Interossei, tongue, triceps wasting
-Camptocormia |
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How many LMN are usually lost by the time CLINICAL weakness is evident in ALS?
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30-40% of LMNs!
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Why does it take so much loss before any weakness is noticed?
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Compensatory REINNERVATION of the orphan muscle fibers
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What happens with progressive loss of LMNs however?
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Reinnervation fails -> Paralysis
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What is NOT affected in ALS?
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Extraocular function - left intact!
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What is the only officially sanctioned medication for ALS?
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Riluzole/Rilutek
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What is the mechanism of Riluzole/Rilutek?
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Glutamate inhibitor - bc of its cytotoxic effects
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What is a pretty common mimic of ALS?
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Polyneuropathy
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What is a treatable autoimmune disease that can also mimic ALS?
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Multifocal motor neuropathy
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What 3 symptoms must be present for the diagnosis of ALS?
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-LMN degeneration evidence
-UMN degeneration evidence -Progressive spread within at least 3 regions, or to other regions |
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What are the 'regions'?
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bulbar, cervical, thoracic, or lumbar
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What sort of approach is taken to ALS Management?
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TEAM - MD, RN, PT, OT, Social work, respiratory/speech therapy
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At what intervals are f/u visits usually for ALS patients?
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8-12 weeks
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Why is the team multidisciplinary approach to ALS management better?
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Survival is longer
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What type of care commences when ALS is diagnosed?
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Palliative care
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What is the goal of palliative care?
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Relief from pain and distress
Supportive for both patient/family |
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Do all patients with ALS have decreased quality of life and depression
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no
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