ALS Flashcards
what is ALS?
degeneration of both UMNs and LMNs
what is a diagnostic tool for ALS?
mm fasciculations (LMN sign)
why do ppl initially go to the doctor to find out they have this?
dropping things, getting clumsy, slurring speech
LMN signs
flaccidity, fasciculations, weakness, hyporeflexia
UMN signs
spasticity, hyperreflexia, pseudobulbar symps
what is the only treatment available for ALS? does it work?
Rilutek, a glutamate inhibitor- it appears when glutamate is released and the neurons get excited, they die, the treatment may possibly at 2-3 months to the lifespan
positive prognostic factors of ALS?
>getting diagnosed younger >limb vs. bulbar onset >rate of progression >psycological well being >multidisciplinary approach
what is almost always spared in ALS?
sensory neurons
Other ALS drugs
> NP001- reduces macrophage activity
Tirasemtiv- modulates ca/triponin interaction (atrophy sparing)
ceftriaxone and dexpramipelole just failed phase III trials
respiratory support for ppl with ALS
CPAP and BIPAP
- ppl need BIPAP when their FVC <50%, pressure during inspiration and not expiration
tracheostomy may extend life how long?
5-15 years, however, high potential for Locked in sydrome
who low does FVC get to be considered resp. failure?
<30%, life expectancy 6 mo. at this point
what is a dedicated vs. nondedicated augmentative, alternative communication (AAC) or known to medicare as (SGD: speech generating device)
dedicated= electronics used for speech only non-dedicated= ipad or something thats also used for other purposes
what are common causes of pain in ALS?
pain from sedentary posititioning or from cramping
how to treat mm cramping
stretching, dietary, some meds in severe cases, ROM, posititioning
how do u treat fasciculations?
caffeine restriction,
Ativan or morphine for severe symptoms
activity and exercise
more reps, less intensity, moderate aerobics and/or resistance training may improve fxn and QOL–> think energy bank, don’t spend it all in one place!
what should u tell pts to improve the most while they can?
aerobic and respiratory health
who covers durable medical equipment?
medicare part B
what does medicare cover?
> walkers: up to $130 every 5 years
commodes: 5 years
hoyers: capped rental
lift chairs: only reimburse for lift mechanism ($280)
hospital beds- capped rental
wheel chairs: medicare pays 80% every 5 years, make medicare buy u the cadilac, not the pinto, think progression!
orthotics
like carbon fiber Pre-tibial AFO- creates knee extension moment, very light weight
what does the patient pay under medicare part B?
patient (or supplemental insurance plan) pays deductible ($147) + 20%
what does assigned mean? under medicare?
no out of pocket cost to pt
non-assigned (under medicare)
patient pays full cost up front, medicare reimburses
Capped Rental (under medicare)
medicare covers 80% of rental cost for 13 consecutive months- most DME (hosp. beds, lifts, WCs, resp. equip.)
speech problem that goes along with ALS?
dysarthria (prob with actual speech forming mm- not with cognition)
other neuromm diseases: SMA, define types of SMA
Type 1: (Werdnig Hoffman) Infantile onset, lifespan <2 years
Type 2: (linds) (Juvenile/Chronic) Onset 6-18 months, often survive into adulthood but with significant motor impairment
Type 3: (Wolhlfart-Kugelberg-Welander): onset in toddlerhood or adolescence, usually remain ambulatory but with increased risk of resp. compromise
what areas are subject to degeneration in ALS?
motor cortex corticospinal tract corticobulbar tract brainstem anterior horn spinal neurons
Diagnosing ALS
> LMN sings cranial to UMNsigns (possible)
LMN+UMN in 1 region (possible)
UMN signs cranial to LMNsigns (probable)
LMN + UMN in 2 regions (probable)
LMN + UMN in 3 regions (clinically definite ALS dx)
CNS regions of ALS
bulbar
cervical
thoracic
lumbar
negative symptoms with ALS (to say, no this isn’t ALS, its something else)
- lack of progression
- sensory impairment
- visual decline
- bowel/bladder dysfunc.
- Imaging, EMG or other evidence of alt. disease
sporadic ALS (sALS)
dont know why it happens, likely interactions of environmental and genetic factors
familial ALS (fALS)
autosomal dominant
mutation of certain genes
once initiated, what does cellular cascade produce?
- ox. stress
- glutamate induced excitotoxicity
- intracellular protein aggregation
- mitochondrial dysf.
- growth factor deficiency
- axonal transport failure
- capsase enzyme act.
Degeneration of lower CNs (progressive bulbar palsy)
IX: glossopharyngeal (S&M)
X: vagus N (S&M)
XI: Accessory (M)
XII: hypoglossal (M)
What is IBALS?
isolated bulbar ALS
