B7.019 Diffuse Weakness without Numbness Flashcards Preview

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Flashcards in B7.019 Diffuse Weakness without Numbness Deck (19)
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1
Q

presentation of ALS

A
mixed UMN and LMN signs with normal sensation
asymmetric weakness
bulbar dysfunction
-dysarthria
-dysphagia
2
Q

epidemiology of ALS

A

3-8 / 100,000 prevalence
mean onset: 56-63
male: female 1.5:1
3 year mean survival

3
Q

etiology of ALS

A

unknown
free radical mediated oxidative stress
glutamate toxicity

4
Q

treatment of ALS

A

riluzone

edavarone

5
Q

management of ALS

A

multidisciplinary approach
feeding tube (PEG)
noninvasive ventilation

6
Q

presentation of dermatomyositis

A
symmetric proximal muscle weakness
normal reflexes
normal sensation
increased CK
myopathy on EMG
heliotrope rash
7
Q

treatment of dermatomyositis

A

prednisone
azathioprine
methotrexate
IVIg

8
Q

acquired myopathies

A
inflammatory
-dermatomyositis
-polymyositis
-inclusion body
medication (statins)
endocrine
9
Q

inherited myopathies

A

muscular dystrophy
congenital myopathies
metabolic

10
Q

inclusion body myositis pattern of involvement

A

knees

fingers

11
Q

features of myopathies

A
usually proximal weakness
pain is not a prominent feature
CK often elevated
EMG myopathic features
biopsy can be helpful
genetic testing
12
Q

presentation of spinal muscular atrophy

A
newborn w severe weakness
unable to feed
requires mechanical ventilation
ocular movements and sensation intact
DTRs absent
13
Q

genetic testing in SMA

A

deletion in SMN gene chromosome 5

14
Q

treatment of SMA

A

Neusinersen

15
Q

presentation of myotonic dystrophy

A
prox and dist weakness
upper and lower limbs
thin face
drooping eyelids
normal reflexes
normal sensation
elevated CK
16
Q

genetic testing in myotonic dystrophy

A

increased number of CTG trinucleotide repeats in DMPK gene chromosome 19

17
Q

diagnosis of myotonic dystrophy

A
most common muscular dystrophy in adults
proximal and distal weakness
frontal balding, temporal wasting, ptosis
muscle myotonia
cardiac arrhythmias
DM
cataracts
18
Q

presentation of MG

A

double vision and drooping eyelids
mild proximal weakness
normal sensation
preserved reflexes

19
Q

treatment of MG

A

edrophonium