ALS

Question 1

first described by charcot, a relentlessly progressive, incurable degenerative disorder that causes muscle weakness disability and eventually death

Answer 1

ALS

Question 2

aka lou gehrigs disease

Answer 2

ALS

Question 3

peak age distribution for ALS

Answer 3

seventh or eighth decades, however can occur in people in their 20’s

Question 4

ALS is most commonly _______

Answer 4

sporadic no thnice o racial predispositions/ genetic or familial accounts for 10%

Question 5

what are the hallmark upper motor neuron findings of ALS? why?

Answer 5

weakness with slowness, hyperreflexia, and spasticity result from degeneration of frontal motor neurons located in the motor strip (brodman area)

Question 6

what can you find at autopsy of a patient with ALS?

Answer 6

the dorsolateral area of the spinal cord, the region containing the lateral corticospinal tract, is gliotic and hardened or sclerotic to palpation

Question 7

what are the hallmark lower motor neuron findings of ALS? why?

Answer 7

weakness, atrophy or amyotrophy, and fasciculations are a direct consequence of degeneration of lower motor neurons in the brain stem and spinal cord producing muscle denervation

Question 8

inclusions within both upper and motor neurons and glia stain ________

Answer 8

positively for ubiquitin; many also for TAR DNA binding protein (TDP-43); and less stain for fused in sarcoma

Question 9

ALS was once presumed to be a pure motor disorder, it has become increasingly apparent that degeneration of other brain regions such as _________________ may also occur as part of the clinicopath spectrum .

Answer 9

frontal and temporal cortical neurons

Question 10

what disease do some experts believe to believe to be a form a ALS, this disease remains confined to the lower motor neuron and survival may be prolonged compared to classic ALS

Answer 10

progressive muscular atrophy

Question 11

what disease in some individuals never develops clinical upper motor neuron signs, and may develop them later in the clinical course

Answer 11

progressive muscular atrophy

Question 12

a progressive isolated upper motor neuron disorder, charcterized by slower progression, lack of weight loss, may develop lower motor neuron signs later in clinical course.

Answer 12

Primary lateral sclerosis

Question 13

Pure primary lateral sclerosis and upper motor neuron dominant ALS appear to have a more _______ than typical ALS

Answer 13

benign

Question 14

progressive upper and lower motor neuron disorder of cranial muscles

Answer 14

progressive bulbar palsy

Question 15

progressive bulbar palsy may be isolated to the bulbar segment, but when upper and lower motor neuron signs and symptoms spread to involve other segments it is then called ______

Answer 15

bulbar onset ALS

Question 16

aka brachial amyotrophic diplegia

Answer 16

flail arm syndrome

Question 17

characterized by progressive lower motor neuron weakness and wasting that predominantly affects the proximal arm

Answer 17

flail arm syndrome

Question 18

flail arm syndrome usually begins _________ and spreads _______ to the point where where arm and hand functions ________

Answer 18

proximaly
distally

are severely impaired

Question 19

flail arm syndrome is often ________ and have a ________ rate of progression to spread in other body segments

Answer 19

asymmetric

slower rate of prepossession

Question 20

what syndrome can patients can have all clinical features of ALS along with features of other disorders such as frontotemporal dementia (FTD), autonomic insufficiency, parkinsonism supranuclear gaze paresis, and or sensory loss

Answer 20

ALS-plus syndrome

Question 21

how can ALS vary between individuals

Answer 21

differences in site and segment of onset, pattern and speed of spread and the degree of upper and lower motor neuron dysfunction

Question 22

what is the most common presentation of ALS

Answer 22

asymmetric limb weakness

Question 23

what is spit hand syndrome

Answer 23

a frequent pattern of weakness and atrophy in ALS that predominantly involves the median and ulnar innervated lateral (thenar) hand intrinsic muscles with sparing of the medial (hypothenar muscles)

Question 24

how does ALS in lower extremities most often begin

Answer 24

with weakness of foot dorsiflexion (foot drop)

Question 25

what are the symptoms of ALS involving the upper motor neurons

Answer 25

slowness of movement incoordination and stiffness with little overt weakness

Question 26

in ALS , leg upper motor neurons manifest as _______

Answer 26

spastic gait****

with poor balance and may include spontaneous leg flexor spasms and ankle clonus

Question 27

_______ and ________ are the most common bulbar upper motor neuron symptoms in ALS

Answer 27

dysarthria and dysphagia

Question 28

__________ produces a characteristically strained vocal quality with low speech in patients with bublar ALS

Answer 28

spastic dysarthria

Question 29

in ALS upper motor neuron symptoms their may be involuntary _______ with biting of the sides of the tongue and cheeks

Answer 29

jaw clenching

Question 30

axial upper motor neuron dysfunction may contribute to ________ and ______

Answer 30

stiffness and imbalance

Question 31

what is the pseudobulbar affect (i think effect) may occur in early manifestation s of ALS

Answer 31

inappropriate laughing, crying, or yawning

Question 32

____________ dysfunction may result in laryngospasm, increased masseter tone and difficulty opening mouth

Answer 32

upper motor neuron bulbar dysfunction

Question 33

incomplete eye closure in the lower face the result may be a poor lip seal that may contribute to drooling or sialorrhea especially in patietns with associated swallowing difficulty.

upper or lower motor neuron affected?

Answer 33

lower

Question 34

lower motor nueron weakness of the diaphragm produces progressive dyspnea with decreased ___________________

Answer 34

amounts of effort culminating in dyspnea at rest and with talking along with reduced vocal volume

Question 35

In ALS patients who choose long term mechanical ventilation have a longer clinical course that can include ________

Answer 35

progressive difficulty with ocular motility

Question 36

what is a locked in state

Answer 36

A clinical condition in which there is an inability to move any voluntary muscles

Question 37

what are some common behavioral changes in ALS

Answer 37

apathy, loss of sympathy, changes in eating behaviors,

Question 38

what are some cognitive problems in a patient with ALS

Answer 38

executive function, language, and letter fluency with sparing of memory and visuospatial function

Question 39

what are some autonomic symptoms of ALS

Answer 39

symptoms of early earlt satiety and bloating consistent with delayed gastric emptying in disease progression

also excessive sweating

Question 40

extrapyramidal symptoms and signs of ______ may precede or follow the upper and lower motor neuron symptoms such as facial masking, tremor, bradykinesia and postural instability

Answer 40

parkinsonism

Question 41

what drugs are used for treatment for ALS?

Answer 41

riluzole, edaravone, experimental therapy (arimoclomol)

Question 42

what criteria is used to identify ALS?

Answer 42

escorial criteria