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C3 > L9 Myopathy > Flashcards

L9 Myopathy Flashcards

1
Q

what are typical clinical features in myopathy

A

weakness: usually proximal (biggest muscles)
weakness>wasting
reflexes: normal (except severely wasted muscle)
sensory: normal
EMG: myopathic picture
CK: high

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2
Q

what does increased amplitude of motor units suggest in EMG

A

nerve problem

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3
Q

what does reduced amplitude of whole response suggest

A

axonal loss

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4
Q

what amplitude (microvolts) of normal, myopath, neuropathy can they reach

A

normal: 100
myopathy: 50
neuropathy: 300

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5
Q

what parts of muscle system are involved in myopathy

A

cranial, limb, cardiac, respiratory

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6
Q 
what are the 
symptoms
signs
tests
of cranial part
A

symptoms: swallowing difficulty, can’t whistle
signs: facial weakness, slurred speech
tests: EMG myopathic MRI - to rule out other causes

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7
Q 
what are the 
symptoms
signs
tests
of limb part
A

symptoms: difficulty getting up from the floor, lifting heavy objects
signs: proximal weakness
tests: EMG - myopathic, muscle biopsy

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8
Q 
what are the 
symptoms
signs
tests
of cardiac part
A

symptoms: heart failure, syncope (arrthymia), sudden death

signs :cardiomegaly, heart failure
tests: ECG, echo, 24hr tape

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9
Q 
what are the 
symptoms
signs
tests
of respiratory part
A

symptoms: orthopnoea, morning headaches
signs: reduced vital capacity, paradoxical diaphragm movement
tests: spirometry, overnight oximetry

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10
Q

what are typical patterns/organ systems involved

A 
limbic girdle muscular dystrophy
duchenne muscular dystrophy
mitochondrial myopathy
statin myopathy
polymyositis
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11
Q

what are specifc organ involvement /pattern

A 
facioscapulohumeral dystrophy
myotonic dystrophy
chronic progressive external ophthalmoplegia
distal myopathies
inclusion body myositis
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12
Q

what are multi organ involvement in myopathy

A 
myotonic dystrophy
duchenne muscular dystrophy
mitochondrial disease
HIV myopathy
dermatomyositis
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13
Q

when is myopathy acquired

A 
acute or sub acute
monophasic or relapsing remitting
exposure history
asymmetrical
adult onset
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14
Q

when is myopathy inherited

A 
longstanding
progressive
family history
symmetrical
childhood onset
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15
Q

when is the involvement of cranial, limb, cardiac, respiratory in duchenne muscular dystrophy

A

cranial: very late
limb: age 3-5, lose ambulation early teens
cardiac: teenager onwards, most common cause of death
respiratory: teenager onwards

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16
Q

are other systems involved in duchenne muscular dystrophy

A

scliosis

mild cognitive

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17
Q

is there an specific pattern of weakness in duchenne muscular dystrophy

A

no, typical proximal pattern

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18
Q

what investigations are done for duchenne muscular dystrophy

A

CK increased several thousand fold
biopsy - absent dystrophin staining
genetic testing dystrophin gene - frameshift mutation

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19
Q

what treatment is there for duchenne muscular dystrophy

A

supportive - prolonged lifespan
Prednisolone - proven efficacy, prolonged ambulation
experiemental - restoration of dystrophin, phase 3 trials negative. ataluren licensed. stop mutations

20
Q

how is cranial, limb, cardiac, respiratory involved in myotonic dystrophy

A

cranial: myopathic facies
limb: distal upper limb weakness and myotonia
cardiac: arrhythmias, sudden death common, low threshold ICD
respiratory: sometimes

21
Q

are other organ systems involved in myotonic dystrophy

A

frontal balding, cataracts, cognitive, hypersomnolence, diabetes, hypogonadism,

22
Q

is there a specific pattern of weakness in myotonic dystrophy

A

face and finger flexion

23
Q

what investigations were done in myotonic dystrophy

A

EMG shows myotonia, biopsy not needed

genetic testing DMPK gene

24
Q

what treatment is there in myotonic dystrophy

A

supportive - prophylactic arrhytmia management
genetic counselling
experimental: early phase block toxic RNA

25
Q

how are cranial, limb, cardiac, respiratory involved in facioscapulohumeral dystrophy

A

cranial: facial weakness early
limb: peri-scapular, distal lower limb
cardiac: 5% mild arrhythmias
respiratory: rare - restrictive secondary to kyphoscoliosis

26
Q

are other organ systems involved in facio. dystrophy

A

rare-deafnese, retinal vasulopathy

27
Q

is there a specific pattern of weakness in facio. dystrophy

A

face, scapular often asymmetric

28
Q

what investigations were done in facio.dystrophy

A

CK moderately elevated
biopsy generally not needed
genetic confirmation - d4z4 repeat 4q

29
Q

what treatment is there for facio. dystrophy

A

supportive - surgery to stablise scapula

experimental treatments: lab stage

30
Q

is cranial, limb, cardiac, respiratory involved in dermatomyositis

A

c - severe cases
limb - yes
cardiac - no
res - severe cases

31
Q

are other organ systems involved in dermatomyositis

A

rashes and associated with malignancies

32
Q

is there a specific pattern of weakness in dermatomyositis

A

no, usually classical proximal pattern

33
Q

is cranial, limb, cardiac, respiratory involved in polymyositis

A

c - severe cases
limb - yes
cardiac - no
res - severe cases

34
Q

are other organ systems involved in polymyositis

A

no

35
Q

is there a specific pattern of weakness in polymyositis

A

no usually classical proximal pattern

36
Q

what investigations were done in dermamyositis

A

CK moderately elevated

biopsy needed for diagnosis

37
Q

what treatment for dermamyositis

A

immunosuppression

steroids

38
Q

what investigations were done in polymyositis

A

CK moderately elevated

biopsy needed for diagnosis

39
Q

what treatment for dermamyositis

A

immunosuppression

steroids

40
Q

is cranial, limb, cardiac, respiratory involved in inclusion body myositis

A

cranial - swallowing
limb - quadriceps, finger flexors
cardiac: no
respiratory: no

41
Q

is other organ systems involved in inclusion body myositis

A

no

42
Q

is there specific pattern of weaknessinclusion body myositis

A

quadriceps, finger flexors, ankle dorsiflexors

43
Q

investigations for inclusion body myositis

A

CK slightly elevated

biopsy needed for diagnosis

44
Q

treatment for inclusion body myositis

A

immunosuppresion - ineffective

currently no effective therapy

45
Q

how is image used in

  • inclusion body myositis
  • polymyositis + derma
  • facio
  • myotonic
  • duchenne
A
  • ibm- specific pattern noted and may aid in diagnosis
  • poly + derma: may be useful to guide biopsy to increase yield
  • facio: STIR hyperintensity associated with inflammation on biopsy
  • myotonic: specific patterns reported on T1 images, correlation with ankle strength
  • duchenne: much recent research in quantitative MRI to assess new treatments

C3 (13 decks)

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