ALS

Question 1

what is ALS?

Answer 1

degeneration of both UMNs and LMNs

Question 2

what is a diagnostic tool for ALS?

Answer 2

mm fasciculations (LMN sign)

Question 3

why do ppl initially go to the doctor to find out they have this?

Answer 3

dropping things, getting clumsy, slurring speech

Question 4

LMN signs

Answer 4

flaccidity, fasciculations, weakness, hyporeflexia

Question 5

UMN signs

Answer 5

spasticity, hyperreflexia, pseudobulbar symps

Question 6

what is the only treatment available for ALS? does it work?

Answer 6

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

Question 7

positive prognostic factors of ALS?

Answer 7

>getting diagnosed younger
>limb vs. bulbar onset
>rate of progression
>psycological well being
>multidisciplinary approach

Question 8

what is almost always spared in ALS?

Answer 8

sensory neurons

Question 9

Other ALS drugs

Answer 9

> NP001- reduces macrophage activity
Tirasemtiv- modulates ca/triponin interaction (atrophy sparing)
ceftriaxone and dexpramipelole just failed phase III trials

Question 10

respiratory support for ppl with ALS

Answer 10

CPAP and BIPAP

- ppl need BIPAP when their FVC <50%, pressure during inspiration and not expiration

Question 11

tracheostomy may extend life how long?

Answer 11

5-15 years, however, high potential for Locked in sydrome

Question 12

who low does FVC get to be considered resp. failure?

Answer 12

<30%, life expectancy 6 mo. at this point

Question 13

what is a dedicated vs. nondedicated augmentative, alternative communication (AAC) or known to medicare as (SGD: speech generating device)

Answer 13

dedicated= electronics used for speech only
non-dedicated= ipad or something thats also used for other purposes

Question 14

what are common causes of pain in ALS?

Answer 14

pain from sedentary posititioning or from cramping

Question 15

how to treat mm cramping

Answer 15

stretching, dietary, some meds in severe cases, ROM, posititioning

Question 16

how do u treat fasciculations?

Answer 16

caffeine restriction,

Ativan or morphine for severe symptoms

Question 17

activity and exercise

Answer 17

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!

Question 18

what should u tell pts to improve the most while they can?

Answer 18

aerobic and respiratory health

Question 19

who covers durable medical equipment?

Answer 19

medicare part B

Question 20

what does medicare cover?

Answer 20

> 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!

Question 21

orthotics

Answer 21

like carbon fiber Pre-tibial AFO- creates knee extension moment, very light weight

Question 22

what does the patient pay under medicare part B?

Answer 22

patient (or supplemental insurance plan) pays deductible ($147) + 20%

Question 23

what does assigned mean? under medicare?

Answer 23

no out of pocket cost to pt

Question 24

non-assigned (under medicare)

Answer 24

patient pays full cost up front, medicare reimburses

Question 25

Capped Rental (under medicare)

Answer 25

medicare covers 80% of rental cost for 13 consecutive months- most DME (hosp. beds, lifts, WCs, resp. equip.)

Question 26

speech problem that goes along with ALS?

Answer 26

dysarthria (prob with actual speech forming mm- not with cognition)

Question 27

other neuromm diseases: SMA, define types of SMA

Answer 27

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

Question 28

what areas are subject to degeneration in ALS?

Answer 28

motor cortex
corticospinal tract
corticobulbar tract
brainstem
anterior horn spinal neurons

Question 29

Diagnosing ALS

Answer 29

> 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)

Question 30

CNS regions of ALS

Answer 30

bulbar
cervical
thoracic
lumbar

Question 31

negative symptoms with ALS (to say, no this isn’t ALS, its something else)

Answer 31

• lack of progression
• sensory impairment
• visual decline
• bowel/bladder dysfunc.
• Imaging, EMG or other evidence of alt. disease

Question 32

sporadic ALS (sALS)

Answer 32

dont know why it happens, likely interactions of environmental and genetic factors

Question 33

familial ALS (fALS)

Answer 33

autosomal dominant

mutation of certain genes

Question 34

once initiated, what does cellular cascade produce?

Answer 34

• ox. stress
• glutamate induced excitotoxicity
• intracellular protein aggregation
• mitochondrial dysf.
• growth factor deficiency
• axonal transport failure
• capsase enzyme act.

Question 35

Degeneration of lower CNs (progressive bulbar palsy)

Answer 35

IX: glossopharyngeal (S&M)
X: vagus N (S&M)
XI: Accessory (M)
XII: hypoglossal (M)

Question 36

What is IBALS?

Answer 36

isolated bulbar ALS