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Clinical Conditions 2 > Motor Neuron Disease > Flashcards

Motor Neuron Disease Flashcards

1
Q

biggest difference between progressive bulbar palsy and pseudobulbar palsy

A

progressive: LMN lesion
pseudo: UMN

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2
Q

progressie & pseudo bulbar palsy age of onset

A

about 50

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3
Q

difference of progressie & pseudo bulbar palsy sx/sx

A

progressive: weakness & ATROPHY of facial muscles, dysarthria, dysphagia, aspiration, drooling dyspnea
pseudo: weakness & SPASTICITY of facial mm, dysarthria, dysphagia, aspiration, drooling

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4
Q

prognosis for progressie & pseudo bulbar palsy

A

poor, 3 years

- also rare diagnoses

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5
Q

PSMA & PLS affects what muscles

A

spinal musculature & limbs

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6
Q

PSMA is a ____ lesion whereas PLS is a ____ lesion

A

PSMA: LMN

PLS: UMN

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7
Q

PSMA onset & freqeuncy

A

30-60 years

variant of ALS

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8
Q

PLS onset & frequency

A

30-60 years

RARE variant of ALS

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9
Q

sx/sx of PSMA: what is happening to the muscles

A 
weak & flaccid muscles of extremities/face
muscle twitching/fasciculations
ataxia
incoordination 
atrophy
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10
Q

sx/sx PLS: what is happening to the muscles

A 
weak & SPASTIC muscles of extremities/face
clonus 
\+ babinski 
muscle twitching/fasciculations
ataxia
atrophy (not as severe as PSMA)
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11
Q

ALS affects what muscles

A

bulbar and spinal muscular atrophy

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12
Q

what MN affected with ALS

A

UMN, LMN, or both

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13
Q

most common onset of ALS

A

30-60 yo

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14
Q

sx/sx ALS

A

more variable

  • weak muscles of extremities/face
  • muscle twitching/fasciculations
  • ataxia
  • incoordination
  • atrophy
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15
Q

prognosis of ALS

A

3-5 years
20% live > 5 years
10% live > 10 years

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16
Q

etiology of ALS include mutations oin what

A

C90RF72
SOD
- multi factorial
- involves cell accumulations, autophagy, and death of SC or bulbar neurons

17
Q

ALS affects what columns

A

lateral

18
Q

who is most affected by ALS

A

males > females
40-70
caucasian

19
Q

what muscles are spared with ALS? there is no ___ changes seen

A

ocular

- no cognitive changes seen

20
Q

etiology of botulism

A

toxin in food or from an infection leads to impaired CA mediated release of Ach at NMJ

21
Q

botulism affects what area of the nervous system

A

LMN

NMJ

22
Q

groups most affected by botulism

A

< 5 yo

anyone eating contaminated food

23
Q

sx/sx of botulism

A

sudden onset of muscular paralysis including

  • double vision
  • blurred vision
  • drooping eyelids
  • slurred speech
  • difficulty swallowing
  • dry mouth
  • muscle weakness
24
Q

Lambert eaton syndrome etiology

A

AI mediated block of voltage gated Ca channels at NMJ

decrease in # of channels or they become nonfunctional leading to a decrease release in Ach

25
Q

LES affects what part of the nervous system

A

LMN

NMJ

26
Q

botulism affects what muscles

A

respiratory, facial, and eyes

27
Q

who is most at risk to get LES

A

anyone with cancer

28
Q

initial sx/sx of LES

A

LE & UE weakness
difficutly walking
double vision
blurred vision

29
Q

what things are seen later in LES

A 
slurred speech 
diff swallowing 
dry mouth 
constipation
urinary urgency
30
Q

in LES, strength/power ___ with repeated effort

A

increase

31
Q

MG etiology

A

AI dx associated with thymic tumor, RA, lupus, or thyrotoxicosis
affects # or function of Ach receptors on NMJ

32
Q

MG affects what area of the nervous system

A

LMN

NMJ

33
Q

MG most commonly affects who

A

females > males

middle age, but typically women < 40 and males >60

34
Q

sx/sx of MG

A

slowly progressive fluctuating course
diplopia & ptosis, but pupillary reflex not affected
dysarthria, dysphagia, generalized weakness

35
Q

with MG, you see generalized weakness especially with

A

sustained effort

strength/power decrease with repeated effort

36
Q

how to define probable ALS

A
  1. UMN & LMN signs in 2+ regions

2. some UMN signs must be rostral to LMN deficits

37
Q

how to define probable lab supported ALS

A
  1. UMN & LMN signs in 1 region
    or
  2. UMN signs in 1 region and LMN signs found by EMG in at least 2 limbs
    and
  3. neuroimaging and electrodiagnostic studies exclude other causes
38
Q

how to define possible ALS

A 
1. UMN & LMN signs in only 1 region 
or 
1. UMN signs alone in 2+ regions 
or 
1. LMN signs rostral to UMN signs
39
Q

how to define suspected ALS

A
  1. lower but not upper MN signs in at least 2 regions

Clinical Conditions 2 (1 decks)

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