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Clin Med III - Neuro > Neuromuscular > Flashcards

Neuromuscular Flashcards

1
Q

Mononeuropathy Multiplex

A

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

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2
Q

Does polyneuropathy affect more proximal or distal nerves?

A

DISTAL

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3
Q

Myelopathy

A

d/o of spinal cord

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4
Q

Define myopathies

A
  • Muscle diseases w/proximal weakness
  • Sensory bladder/bowel/cognitive function in tact
  • Reflexes decreased in proportion to amount of weakness
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5
Q

Myotome

A

innervation of the muscle, functions of a spinal root

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6
Q

Dermatome

A

innervation of the sensory fans of spinal root

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7
Q

Where is the pathophys of myopathies and muscular dystrophy

A

muscle tissue is the primary site of pathology

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8
Q

What is Duchenne MD?

A
  • Males
  • Onset 3-5yo
  • Progressive weakness
  • Wheelchair by 12 yo
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9
Q

Pathophys of duchenne?

A

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

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10
Q

What is metabolic myopathy?

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

- Exercise intolerance, progressive myopathy, isolated respiratory distress

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11
Q

Subtypes of metabolic myopathy

A

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

Type 5: McArdle’s Dz (myophosphorylase deficiency)

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12
Q

Sx of Glycogen storage dx (types 2-4)

A

Adults: ptosis, neck flexor weakness, pulmo weakness

bx with rimmed vacuoles

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13
Q

Sx of Type 5 McArdles

A

high intensity exercise intolerance and painful muscle ramps

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14
Q

Tx for glycogen storage type 2-4

A

enzyme replacement- Myozyme

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15
Q

What are the muscle channelopathies?

A

Myotonia Congenita
Paramyotonia Congenita
Periodic paralysis

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16
Q

Which channelopathies are abnl sodium?

A

Paramyotonia Congenita

Periodic paralysis P.S.

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17
Q

What channelopathies are abnl chloride

A

Myotonia Congenita

hey, it’s already MC!

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18
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
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19
Q

Sx of paramyotonia congenita

A

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

20
Q

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

A

Periodic Paralysis

Carbonic anhydrase inhibitors

21
Q

Define fasciculations

A
  • Muscle movements under the skin

- Common in nerve diseases

22
Q

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

A
  • LESS reflex activity in nerve/muscle disorders

- HYPER reflexes in CNS disorders

23
Q

What does Babinski’s sign show?

A

Pyramidal (motor) tract dysfunction

24
Q

Define plexus lesions

A

Complex mixtures - lost reflexes motor and sensory loss are key

25
Q

What is Fromet’s sign?

A
  • Weak OK sign

- Caused by AIN neuropathy (resulting in weak FPL muscle)

26
Q

Types of thoracic outlet syndrome

A
  • Vascular

- Neurogenic

27
Q

Vascular thoracic outlet syndrome

A
  • Lost arterial pulse

- Ischemic symptoms (worse w/exercise)

28
Q

Neurogenic thoracic outlet syndrome

A

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

29
Q

What is “saturday night palsy”?

A

Radial neuropathy

30
Q

Seddons classification of nerve injury

A
  • 1st degree: neurapraxia (conduction block from myelin disruption)
  • 2nd degree: axonotmesis (axon loss w/CT intact)
  • 3rd degree: neurotmesis (complete transection)
31
Q

What is sciatica?

A

L5-S1 radiculopathy

32
Q

Define coccydynia

A
  • Pain in tailbone
  • Considered a neuralgia (no entrapment)
  • Direct hit, HSV2 are causes
33
Q

General anticonvulsant rules

A
  • All may create sedation
  • Suicide risk (legal dilemma)
  • Most are liver metabolized
  • Beware of OCP interactions
34
Q

Describe Depakote

A
  • Indicated for neuralgia and migraine

- Teratogenic

35
Q

Clinical presentation of Duchenne’s MD?

A
  • CPK over 3000
  • Calf hypertrophy
  • Reflexes decreased in proportion to weakness
  • Increased lumbar lordosis
36
Q

Becker’s MD

A
  • Later onset than Duchenne’s

- Milder version, slower progression

37
Q

FSH MD

A
  • 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)
38
Q

How to diagnose oculopharyngeal MD vs. Myasthenia Gravis?

A

Myasthenia gravis is a/w fatigue

39
Q

What is MERRF?

A
  • Myoclonic epilepsy and ragged red fibers

- Multisystem disorder a/w myoclonus, generalized epilepsy, ataxia, hearing loss

40
Q

What is MELAS?

A
  • Mitochondrial encephalopathy, Lactic acidosis, Stroke like episodes
  • Young adults
41
Q

Define myotonia congenita

A

Chloride channel mutation

  • Dominant: Thompson disease
  • Recessive: Becker disease
42
Q

Define paramyotonia congenita

A
  • Na channel defect

- Autosomal dominant

43
Q

Define periodic paralysis

A
  • Na channelopathy
  • Attacks of paralysis with either high or low K during attack
  • Postpone attack with carbs
  • Treat w/carbonic anhydrase inhibitors
44
Q

Define malignant hyperthermia

A

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

45
Q

Describe polymyositis

A
  • Onset over 20 yo
  • FEMALES
  • Proximal weakness LE/UE, neck flexors
  • Normal sensory, bowel, bladder, cognition
  • PAINLESS
46
Q

Criteria to diagnose polymyositis

A
  1. Proximal weakness
  2. Increased CPK
  3. EMG denervation
  4. Abnormal biopsy w/WBC in muscle
47
Q

Describe dermatomyositis

A

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

Clin Med III - Neuro (11 decks)

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