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CM 3- Neuro > Neuromuscular > Flashcards

Neuromuscular Flashcards

1
Q

Mononeuropathy

A

involves single nerve (may have motor and sensory fibers)

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2
Q

Mononeuropathy Multiplex

A

simultaneous or sequential inflamm. of several nerves in unrelated parts of the body (noncontiguous nerve trunks)

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3
Q

Polyneuropathy

A

generalized, usually homogenous process affecting many peripheral nerves,
distal MOST affected

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4
Q

Does polyneuropathy affect more proximal or distal nerves?

A

DISTAL

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5
Q

Radiculopathy

A

irritation of nerve root: cervical, lumbar or thoracic

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6
Q

Plexopathy

A

injury to a nerve plexus: brachial, lumbosacral

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7
Q

Myelopathy

A

d/o of spinal cord

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8
Q

Myopathy

A

disease of muscle

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9
Q

Myotome

A

innervation of the muscle, functions of a spinal root

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10
Q

Dermatome

A

innervation of the sensory fans of spinal root

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11
Q

What is the pathophys of peripheral neuropathy?

A

impaired fxn of periphery nerves, sensory, motor or autonomic

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12
Q

In peripheral neuropathy when are the reflexes lost?

A

reflexes are lost EARLY!!!

peripheral early : physical exam

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13
Q

What causes peripheral neuropathy?

A 
genetic
traumatic
infx
toxins
autoimmune
nutritional 
vascular
metabolic (renal/liver failure)
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14
Q

Are peripheral neuropathies inside or outside of the brain and spinal cord?

A

affects nerves OUTSIDE!

dying back/ demyelinating/ axonal

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15
Q

What causes neuromuscular junction d/o?

A

autoimmune (myasthenia gravis)

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16
Q

What is the pahtophy of neuromsk jxn?

A

hinder production, release or uptake of ACh

postsynaptic autoimmune attach doesn’t recognize the ACh

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17
Q

What happens the reflexes in neuromsk jxn?

A

reflexes are NORMAL

18
Q

Where is the pathophys of myopathies and muscular dystrophy

A

muscle tissue is the primary site of pathology

19
Q

What causes myopathies/ MD?

A

genetic
meds (statins)
autoimmune-inflamm.
hypothyroid

20
Q

Reflexes in myopathies/MD?

A

Reflexes proportional to weakness!!

dec. muscle, dec. reflexes

21
Q

What is motor neuron disease?

A

degenerative disorder

progressive weakness/wasting of muscle without sensory changes

22
Q

What are the reflexes in MND?

A

HYPERreflexic with ATROPHY of the muscle!

counterintuitive

23
Q

What labs do you run for these?

A 
CBC & CMP
HbAIc
UA
B12/MMA
ESR, RF, ANA, SPEP
Hep C, HIV
TSH (no longer)
24
Q

Muscular dystrophy presentation:

A

symmetric proximal weakness
not painful d/t dystrophy, but painful d/t posture
intact sensory function
intact bladder/bowel

25
Q

What is progressive, degenerative and can be genetic?

A

Muscular dystrophy

26
Q

What is Duchenne MD?

A

Affects Males the worst, don’t live long enough to reproduce
onset 3-5yo, progressive weakness
wheelchair by 12 yo

27
Q

Pathophys of duchenne?

A

caused by mutation of the gene that makes dystrophin (GOOD and necessary serves many fxn inc. linking the contractile apparatus to the sarcomere

28
Q

Concurrent conditions with Duchenne

A 
scoliosis
cardiomyopathies
arrhythmias
pulmo insufficiency 
contractures
cognitive slowing
cataracts
high CPK > 3000, ALT enzymes elevated 
Calf HYPERtrophy (but WEAK)
29
Q

How are the reflexes in duchennes?

A

proportional to the weakness

30
Q

Tests for duchenne?

A

EMG- confirm muscular dx

Muscle bx– DNA to confirm

31
Q

What is metabolic myopathy?

A
  • error in energy metabolism d/t glycogen/lipid/mitochondria

- think metabolic w/ exercise intolerance, progressive myopathy, isolated respiratory distress

32
Q

Labs for metabolic myopathy

A 
Increase CPK
resting lactate abnl
urine abnl- organic acids
serum ischemic exercise test
EMG
muscle Bx
abnl serum acytcarnitine labs
33
Q

Subtypes of metabolic myopathy

A

Types 2-4: MC Pompe’s Dz (acid maltase)

Type 5: McArdle’s Dz (myophosphorylase deficiency)

34
Q

Sx of Glycogen storage dx (type 2-4)

A

Adults: ptosis, neck flexor weakness, pulmo weakness

bx with rimmed vacuoles

35
Q

Sx of Type 5 McArdles

A

high intensity exercise intolerance and painful muscle ramps

36
Q

Tx for glycogen storage type 2-4

A

enzyme replacement-

Myozyme

37
Q

What are the muscle channelopathies?

A

Myotonia Congenita
Paramyotonia Congenita
Periodic paralysis

38
Q

Which channelopathies are abnl sodium?

A

Paramyotonia Congenita
Periodic paralysis

P.S.

39
Q

What channelopathies are abnl chloride

A

Myotonina Congenita

hey, it’s already MC!

40
Q

Sx of myotonia congenita

A

myotonia present in second decade w/ improvement on myotonia w/ exercise and VERY muscular build
muscle stiff, large and do not fatigue but NOT weak

41
Q

Sx of paramyotonia congenita

A

muscle pain, myotonia, stiffness, paralysis episodes and phenotypic types, cold intolerance

42
Q

What is an attack of paralysis w/ either high or low K+ during attack?
Tx?

A

Periodic Paralysis

Carbonic anhydrase inhibitors

CM 3- Neuro (1 decks)

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