B7.019 Diffuse Weakness without Numbness Flashcards
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
