Neuromuscular

Question 1

Mononeuropathy

Answer 1

involves single nerve (may have motor and sensory fibers)

Question 2

Mononeuropathy Multiplex

Answer 2

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

Question 3

Polyneuropathy

Answer 3

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

Question 4

Does polyneuropathy affect more proximal or distal nerves?

Answer 4

DISTAL

Question 5

Radiculopathy

Answer 5

irritation of nerve root: cervical, lumbar or thoracic

Question 6

Plexopathy

Answer 6

injury to a nerve plexus: brachial, lumbosacral

Question 7

Myelopathy

Answer 7

d/o of spinal cord

Question 8

Myopathy

Answer 8

disease of muscle

Question 9

Myotome

Answer 9

innervation of the muscle, functions of a spinal root

Question 10

Dermatome

Answer 10

innervation of the sensory fans of spinal root

Question 11

What is the pathophys of peripheral neuropathy?

Answer 11

impaired fxn of periphery nerves, sensory, motor or autonomic

Question 12

In peripheral neuropathy when are the reflexes lost?

Answer 12

reflexes are lost EARLY!!!

peripheral early : physical exam

Question 13

What causes peripheral neuropathy?

Answer 13

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

Question 14

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

Answer 14

affects nerves OUTSIDE!

dying back/ demyelinating/ axonal

Question 15

What causes neuromuscular junction d/o?

Answer 15

autoimmune (myasthenia gravis)

Question 16

What is the pahtophy of neuromsk jxn?

Answer 16

hinder production, release or uptake of ACh

postsynaptic autoimmune attach doesn’t recognize the ACh

Question 17

What happens the reflexes in neuromsk jxn?

Answer 17

reflexes are NORMAL

Question 18

Where is the pathophys of myopathies and muscular dystrophy

Answer 18

muscle tissue is the primary site of pathology

Question 19

What causes myopathies/ MD?

Answer 19

genetic
meds (statins)
autoimmune-inflamm.
hypothyroid

Question 20

Reflexes in myopathies/MD?

Answer 20

Reflexes proportional to weakness!!

dec. muscle, dec. reflexes

Question 21

What is motor neuron disease?

Answer 21

degenerative disorder

progressive weakness/wasting of muscle without sensory changes

Question 22

What are the reflexes in MND?

Answer 22

HYPERreflexic with ATROPHY of the muscle!

counterintuitive

Question 23

What labs do you run for these?

Answer 23

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

Question 24

Muscular dystrophy presentation:

Answer 24

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

Question 25

What is progressive, degenerative and can be genetic?

Answer 25

Muscular dystrophy

Question 26

What is Duchenne MD?

Answer 26

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

Question 27

Pathophys of duchenne?

Answer 27

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

Question 28

Concurrent conditions with Duchenne

Answer 28

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

Question 29

How are the reflexes in duchennes?

Answer 29

proportional to the weakness

Question 30

Tests for duchenne?

Answer 30

EMG- confirm muscular dx | Muscle bx-- DNA to confirm

Question 31

What is metabolic myopathy?

Answer 31

- error in energy metabolism d/t glycogen/lipid/mitochondria | - think metabolic w/ exercise intolerance, progressive myopathy, isolated respiratory distress

Question 32

Labs for metabolic myopathy

Answer 32

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

Question 33

Subtypes of metabolic myopathy

Answer 33

Types 2-4: MC Pompe's Dz (acid maltase) | Type 5: McArdle's Dz (myophosphorylase deficiency)

Question 34

Sx of Glycogen storage dx (type 2-4)

Answer 34

Adults: ptosis, neck flexor weakness, pulmo weakness | bx with rimmed vacuoles

Question 35

Sx of Type 5 McArdles

Answer 35

high intensity exercise intolerance and painful muscle ramps

Question 36

Tx for glycogen storage type 2-4

Answer 36

enzyme replacement- | Myozyme

Question 37

What are the muscle channelopathies?

Answer 37

Myotonia Congenita Paramyotonia Congenita Periodic paralysis

Question 38

Which channelopathies are abnl sodium?

Answer 38

Paramyotonia Congenita Periodic paralysis P.S.

Question 39

What channelopathies are abnl chloride

Answer 39

Myotonina Congenita hey, it's already MC!

Question 40

Sx of myotonia congenita

Answer 40

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

Question 41

Sx of paramyotonia congenita

Answer 41

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

Question 42

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

Answer 42

Periodic Paralysis Carbonic anhydrase inhibitors