Amyotrophic Lateral Sclerosis (ALS)

Question 1

Though etiology is uncertain, increased levels of ____ is thought to clog up the synaptic cleft and suffocate what structure?

Answer 1

glutamate, alpha motor neurons

Question 2

what is the average mortality for individuals dx with ALS?

Answer 2

3-5 years

Question 3

the pathogenesis of ALS can be best described as a progressive degeneration/ loss of ____ in the SC, ____, and motor cortex.

Answer 3

loss of motor neurons, brain stem

Question 4

T/F: Because ALS targets the anterior horn cells and descending corticoobulbar/corticospinal tracts, it’s a solely LMN pathology.

Answer 4

false. it also impacts UMN cell bodies in the motor cortex. but LMN»UMN weakness.

Question 5

In addition to familial ALS that is autosomal dominant and makes up ___% of cases, additional RF include (5)

Answer 5

• 5-10%
• gender (M>F), age (55-75), race, geographic clusters, prior trauma/TBI

Question 6

ALS is largely a dx of exclusion, but what diagnostic tools might help lead to its discovery? (4)

Answer 6

clinical examination
EMG/NCV (LMN impact)
MRI
CSF/ blood urine tests (can help rule out other neurologic ocnditions)

Question 7

Clinical dx of ALS requires what 2 patterns?

Answer 7

-both UMN and LMN symptoms
-persistent decline in physical fn unexplained by other dx.

Question 8

The 2 Criteria systems used to dx ALS are ___ and __. The main difference between them is

Answer 8

El Escorial Criteria for Dx & The Gold Coast Criteria for Dx of ALS.

the latter has more broadly defined regions (blubar/UE/LE vs bulbar/spinal levels)&raquo_space; more sensitive and aids in IDing clinical trial candidates.

Question 9

El Escorial Criteral for Dx of ALS leads to ___

Answer 9

suspected, possible, probable, and definitive ALS

Question 10

The Gold Coast Criteria for Dx of ALS leads to

Answer 10

+ or - ALS

Question 11

T/F The cardinal sign of ALS is muscle weakness and we often see extraoccular muscle, bowel, and bladder involvement as early indicators.

Answer 11

False. These are normally spared until terminal stages.

Question 12

Fatigue, dyspnea on exertion, frequent sighing and morning HA are early s/s of respiratory involvement. They usually being to show up when VC reaches about __%. A decrease to ___% poses significant risk of repiratory failure or death

Answer 12

50%; 25-35%

Question 13

Primary impairments related to ALS are:
* UMN/LMN/ bulbar motor dysfunction.
* respiratory impairments
* cognitive deficit (esp bulbar)
* and_____(3)

Answer 13

pain
fatigue
and ANS symptoms

Question 14

The main clinical subtypes of ALS include classical ALS, ALS w/ frontotemporal dementia, familial ALS, and ___(2)

Answer 14

Primary Lateral Sclerosis
Progressive Spinal Muscular Atrophy (SMA)

Question 15

T/F: In cases of limb-onset classical ALS, we might expect motor s/s such as fasciculations, weakness/atrophy and spasticity to appear in the calf and progress to the toes (following the direction of motor signals) .

Answer 15

false. symptoms progress distal>proximal

Question 16

bulbar-onset subtype of classical ALS progresses __ (slower/faster) than limb onset and affects cranial nerves___. What poppulation has the highest prevalence

Answer 16

faster
9-12
older females

Question 17

What is the characteristic that distinguishes Primary Lateral Sclerosis from classical ALS

and improves prognosis relating to life expectancy!

Answer 17

LMN not affected within 2 years
(remains UMN in nature)

with spasticity and pseudobulbar findings

Question 18

“Man in barrel syndrome” aka falil/arm syndrome is characteristic of this ALS subtype. With it’s highly variable progression, mean survival is ___ with median time of about ___longer than classical ALS.

Answer 18

progressive spinal muscular atrophy
5-10 years
12 months

Question 19

1/5 of ALS patients develop frontotemporal dementia, presenting with deficits in ___ (3)

Answer 19

executive fn
language
personality

Question 20

T/F: There are no differences between famillal and sporadic ALS on a neuro exam.

Answer 20

true.

Question 21

ALS Stage 1

Answer 21

• early disease,
• mild focal weakness,
• assymetrical distribution,
• symptoms of hand cramping and fasciculations

Question 22

ALS Stage 2

Answer 22

• moderate weakness in GROUPS of muscles
• some atrophy
• Mod I w/ AD

Question 23

ALS Stage 3

Answer 23

• severe weakness of specific muscles
• inc FATIGUE
• mild-mod fn limits
• AMBULATORY

Question 24

ALS Stage 4

Answer 24

• severe weakness and wasting in LEs
• mildly weak UEs
• Mod A + AD
• w/c user

Question 25

ALS Stage 5

Answer 25

• weakness + inc fatigue/dec endurance/mobility
• mod-severe weakness of limbs and trunk
• spasticity/hyperreflexia
• loss of HEAD CTRL
• Max A

Question 26

ALS stage 6

Answer 26

• bedridden
• Dep w/ ADLs
• FMS
• progressive respiratory distress

Question 27

Because there is not yet an effective treatment for ,pharmaceutical interventions target ___(2)

Answer 27

symptoms and prolonging high function/survival

Question 28

The ALS Functional Rating Scale measures ___across 12 categories. It’s scored 0-48 with ___ scores signifying worse performance.

Answer 28

ADL and global function; higher

Question 29

What does the ALSFRS-R tell us?

Answer 29

assesses reponse to treatment/dx progression

Question 30

Mortality can be delayed by ___ as opposed to palliative or comfort care. Another positive prognostic indicator is ____

Answer 30

• home-based mechanical vent
• participation in multidisciplinary care