14. Neuromuscular Disease Flashcards Preview

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Flashcards in 14. Neuromuscular Disease Deck (40)
1

neuropathy

damage or disease involving fibers of the nervous system. may affect sensation, movement, gland or organ function.

2

myopathy

a muscular disease[1] in which the muscle fibers do not function for any one of many reasons, resulting in muscular weakness.

3

neuromuscular junction

connects the nervous system to the muscular system via synapses between efferent nerve fibers and muscle fibers, also known as muscle cells.

4

"dying back" neuropathy

neuropathy affecting distal neurons first; toxins or metabolic deficiencies can affect longest nerve fibers first because longer nerve fibers require more energy to maintain functin, and because there is a greater opportunity to injure a longer nerver fiber

5

Wallerian degeneration

a process that results when a nerve fiber is cut or crushed, in which the part of the axon separated from the neuron's cell body degenerates distal to the injury

6

epineurium

The epineurium is the outermost layer of dense irregular connective tissue surrounding a peripheral nerve.[1] It usually surrounds multiple nerve fascicles as well as blood vessels which supply the nerve. Smaller branches of these blood vessels penetrate into the perineurium.

7

perineurium

In the peripheral nervous system, nerve fibers are each wrapped in a protective sheath known as the endoneurium. These are bundled together into groups known as fascicles, each surrounded by a protective sheath known as the perineurium

8

endoneurium

The endoneurium is a layer of delicate connective tissue made up of endoneurial cells that encloses the myelin sheath of a spinal cord nerve fiber.

9

schwann cells

glial cells of the PNS

10

entrapment neuropathy

compression of a nerve resulting in myelin disruption. could be due to mechanical abnormalities or swelling of tissues

11

where is a common site for an entrapment neuropathy?

carpal tunnel

12

lateral femoral cutaneous neuropathy

aka meralgia paresthetica, entrapment between ilium and inguinal ligament.

13

other sites of compression neuropathies?

ulnar passing behind the epicondyle, deep branch of the ulnar passing wrist, radial nerve at spiral groove of upper arm, peroneal nerve at head of fibula, lateral femoral cutaneous.

14

what nerves make up the lateral femoral cutaneous?

L2, L3

15

what is a result of lateral femoral cutaneous entrapment?

numbness, burning pain on lateral thigh.

16

polyneuropathy

Polyneuropathy is a serious, unpredictable, occasionally progressive, and life threatening neurological disorder[1] that occurs when many nerves throughout the body malfunction simultaneously.

17

Charcot Marie Tooth disease

Charcot–Marie–Tooth disease (CMT), also known as Charcot–Marie–Tooth neuropathy, is a genetically and clinically heterogeneous group of inherited disorders of the peripheral nervous system characterised by progressive loss of muscle tissue and touch sensation across various parts of the body.

18

Lambert-Eaton myasthenic syndrome: cause?

due to antibodies against the VG calcium channels in the terminals of motor nerves. Ca entry into these terminals is needed for release of Ach. produces weakness, diminished reflexes and autonomic dysfunction

19

paraneoplastic sx

associated with cancer. consequence of cancer being in body but not directly related to local cancer cells.

20

example of a paraneoplastic sx?

Cushing's, Lambert-Eaton

21

myasthenia gravis

autoimmune sx in which antibodies exist against the Ach receptor. means that nerve is unable to activate the muscle.

22

nerve conduction studies do what?

evaluate the speed of nerve conduction and amount of nerve signal that gets through. can test both sensory and motor.

23

electromyography

assesses electrical activity of muscle fibers, evaluates health of muscles, can detect muscle fibers that have been denervated.

24

large peripheral nerve fibers convey what?

localized touch, pressure, vibration, joint pressure sense

25

small nerve fibers convey what?

pain, touch, temp, autonomic

26

what are a few causes of polyneuropathy?

infection, diabetes, nutritional prob, alcohol, toxins, meds, hereditary, inflammatory

27

what is a hereditary cause of polyneuropathy

Charcot-Marie Tooth

28

with polyneuropathy, what reflex is detected early

ankle jerk reflex

29

what is a common pattern of polyneuropathy?

distal, symmetrical sensory loss (stocking/glove)

30

a few causes of myopathies?

metabolic, infectious, inflammatory, genetic, drug, toxin

31

myopathies usually result in what?

proximal and symmetrical weakness (hip, shoulder) and then move on to more distal muscles

32

reflexes in myopathies?

reflexes are preseved until very late in disease

33

sensation in myopathies?

no sensory loss. muscles may be tender/cramp

34

myasthenia gravis: most commonly what population?

young females, old males

35

myasthenia: sx?

fatigued muscles, due to damage to Ach receptors by antibody

36

myasthenia: how affect resp?

may affect diaphragm, other resp muscles

37

MG: treatmetn?

AchE inhibitors, immunosuppressives

38

Lambert-Eaton: weakness where?

hip girdle muscles, decr patellar reflexes

39

Lambert-Eaton: cause

sometimes small cell cancer, can also be idiopathic

40

Lambert-Eaton: strength pattern

strength increases with sustained contraction