Neuromuscular

Question 1

Mononeuropathy Multiplex

Answer 1

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

Question 2

Does polyneuropathy affect more proximal or distal nerves?

Answer 2

DISTAL

Question 3

Myelopathy

Answer 3

d/o of spinal cord

Question 4

Define myopathies

Answer 4

• Muscle diseases w/proximal weakness
• Sensory bladder/bowel/cognitive function in tact
• Reflexes decreased in proportion to amount of weakness

Question 5

Myotome

Answer 5

innervation of the muscle, functions of a spinal root

Question 6

Dermatome

Answer 6

innervation of the sensory fans of spinal root

Question 7

Where is the pathophys of myopathies and muscular dystrophy

Answer 7

muscle tissue is the primary site of pathology

Question 8

What is Duchenne MD?

Answer 8

• Males
• Onset 3-5yo
• Progressive weakness
• Wheelchair by 12 yo

Question 9

Pathophys of duchenne?

Answer 9

Mutation of the gene that makes dystrophin (GOOD and necessary)

Question 10

What is metabolic myopathy?

Answer 10

• Error in energy metabolism d/t glycogen/lipid/mitochondria

- Exercise intolerance, progressive myopathy, isolated respiratory distress

Question 11

Subtypes of metabolic myopathy

Answer 11

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

Type 5: McArdle’s Dz (myophosphorylase deficiency)

Question 12

Sx of Glycogen storage dx (types 2-4)

Answer 12

Adults: ptosis, neck flexor weakness, pulmo weakness

bx with rimmed vacuoles

Question 13

Sx of Type 5 McArdles

Answer 13

high intensity exercise intolerance and painful muscle ramps

Question 14

Tx for glycogen storage type 2-4

Answer 14

enzyme replacement- Myozyme

Question 15

What are the muscle channelopathies?

Answer 15

Myotonia Congenita
Paramyotonia Congenita
Periodic paralysis

Question 16

Which channelopathies are abnl sodium?

Answer 16

Paramyotonia Congenita

Periodic paralysis P.S.

Question 17

What channelopathies are abnl chloride

Answer 17

Myotonia Congenita

hey, it’s already MC!

Question 18

Sx of myotonia congenita

Answer 18

• 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 19

Sx of paramyotonia congenita

Answer 19

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

Question 20

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

Answer 20

Periodic Paralysis

Carbonic anhydrase inhibitors

Question 21

Define fasciculations

Answer 21

• Muscle movements under the skin

- Common in nerve diseases

Question 22

Reflexes in nerve/muscle disorders vs. spine/brain disorders?

Answer 22

• LESS reflex activity in nerve/muscle disorders

- HYPER reflexes in CNS disorders

Question 23

What does Babinski’s sign show?

Answer 23

Pyramidal (motor) tract dysfunction

Question 24

Define plexus lesions

Answer 24

Complex mixtures - lost reflexes motor and sensory loss are key

Question 25

What is Fromet's sign?

Answer 25

- Weak OK sign | - Caused by AIN neuropathy (resulting in weak FPL muscle)

Question 26

Types of thoracic outlet syndrome

Answer 26

- Vascular | - Neurogenic

Question 27

Vascular thoracic outlet syndrome

Answer 27

- Lost arterial pulse | - Ischemic symptoms (worse w/exercise)

Question 28

Neurogenic thoracic outlet syndrome

Answer 28

Pain from neck-trap-medial-scapula-axilla-inside bicepts to ulnar forearm to hand

Question 29

What is "saturday night palsy"?

Answer 29

Radial neuropathy

Question 30

Seddons classification of nerve injury

Answer 30

- 1st degree: neurapraxia (conduction block from myelin disruption) - 2nd degree: axonotmesis (axon loss w/CT intact) - 3rd degree: neurotmesis (complete transection)

Question 31

What is sciatica?

Answer 31

L5-S1 radiculopathy

Question 32

Define coccydynia

Answer 32

- Pain in tailbone - Considered a neuralgia (no entrapment) - Direct hit, HSV2 are causes

Question 33

General anticonvulsant rules

Answer 33

- All may create sedation - Suicide risk (legal dilemma) - Most are liver metabolized - Beware of OCP interactions

Question 34

Describe Depakote

Answer 34

- Indicated for neuralgia and migraine | - Teratogenic

Question 35

Clinical presentation of Duchenne's MD?

Answer 35

- CPK over 3000 - Calf hypertrophy - Reflexes decreased in proportion to weakness - Increased lumbar lordosis

Question 36

Becker's MD

Answer 36

- Later onset than Duchenne's | - Milder version, slower progression

Question 37

FSH MD

Answer 37

- Rostral caudal spread of weakness - Asymm face - Autosomal dominant - Onset in teens and older - Eye muscles and swallowing NOT affected - Popeye look (preserved deltoid and forearms)

Question 38

How to diagnose oculopharyngeal MD vs. Myasthenia Gravis?

Answer 38

Myasthenia gravis is a/w fatigue

Question 39

What is MERRF?

Answer 39

- Myoclonic epilepsy and ragged red fibers | - Multisystem disorder a/w myoclonus, generalized epilepsy, ataxia, hearing loss

Question 40

What is MELAS?

Answer 40

- Mitochondrial encephalopathy, Lactic acidosis, Stroke like episodes - Young adults

Question 41

Define myotonia congenita

Answer 41

Chloride channel mutation - Dominant: Thompson disease - Recessive: Becker disease

Question 42

Define paramyotonia congenita

Answer 42

- Na channel defect | - Autosomal dominant

Question 43

Define periodic paralysis

Answer 43

- Na channelopathy - Attacks of paralysis with either high or low K during attack - Postpone attack with carbs - Treat w/carbonic anhydrase inhibitors

Question 44

Define malignant hyperthermia

Answer 44

Systemic reaction of fever to 106 and marked rhabdo in response to succinylcholine (paralytic) or halothane (anesthetic) exposure

Question 45

Describe polymyositis

Answer 45

- Onset over 20 yo - FEMALES - Proximal weakness LE/UE, neck flexors - Normal sensory, bowel, bladder, cognition - PAINLESS

Question 46

Criteria to diagnose polymyositis

Answer 46

1. Proximal weakness 2. Increased CPK 3. EMG denervation 4. Abnormal biopsy w/WBC in muscle

Question 47

Describe dermatomyositis

Answer 47

-Similar to polymyositis but add 5th criteria (skin rash)