NEUROMUSCULAR DISORDERS Flashcards

1
Q

NEUROMUSCULAR DISORDERS

These diseases can affect:
____ directly

——— indirectly

Or ————

A

muscles

Nerves

N-M junction

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2
Q

Generally ,

spasticity is——— motor neuron disorder)
-some degree of paralysis is ——-motor neuron disorder)

A

an upper

a lower

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3
Q

The following occur more in women or men??

Hungtington’s disease
Myasthenia gravis
Muscular dystrophy

A

Women
Women
Men

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4
Q

Creutzfeldt-Jakob disease ; may occur——-, be——- or be——-, such as during a ——or from eating——-. CJD gradually destroys brain cells and causes —-to form in the brain. People with CJD experience difficulty controlling body movements, changes in gait and speech, and dementia.

A

spontaneously; inherited; transmitted by contact with infected tissue

transplant ; contaminated meat

tiny holes

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5
Q

spinal muscular atrophies are disorders of the—— motor neuron.
- causes muscles to —-and——. People with SMA lose a specific type of nerve cell in the spinal cord (called motor neurons) that control muscle movement.

A

lower

become weak ; waste away

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6
Q

*———(ALS;——— disease) is ——-motor neuron condition

A

Amyotrophic lateral sclerosis

lou Gehrig’s

mixed upper and lower

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7
Q

NEUROMUSCULAR DISEASES CAN BE CAUSED BY some form of collagen disorder like ——-disorders

A

Ehler-danlos

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8
Q

failure of the myelin around nerves is seen in certain deficiency diseases such as failure of the body to——-

A

absorb vitamin B12

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9
Q

Myasthenis gravis: form of muscle weakness due to——-

  • occurs in every 1 in—— people and no one knows why
  • causes——
A

antibodies against acetylcholine receptors

200k

paralysis

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10
Q

Therapy to myasthenia gravis

  • administer——- or some——- that allows the——- in the synaptic space
  • you could also try blunting the ——-with—-
  • a thymectomy reduced the production of—— and reverses symptoms in ——-of the cases
  • ____: replacing plasma that contained the offending——
A

neostigmine; anticholinesterase; accumulation of larger amounts of Ach

immune system ; glucocorticoids

antibodies; 50%

plasmapheresis; antibodies

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11
Q

In lambert eaton myasthenic syndrome

-muscle strength—— as—— calcium is released

A

increases; more

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12
Q

tetanus and botulism are—— infections in which—— toxins cause——- or——— muscle tone respectively

A

bacterial; bacterial

increased; decreased

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13
Q

Poliomyelitis, fungal, viral, or bacterial

A

Viral

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14
Q

Poliomyelitis:

  • destroys motor NEURONES
  • results in—— of skeletal muscle
  • may result in death due to——-
A

paralysis; respiratory failure

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15
Q

(2) Muscle cramps
- involuntary—-of skeletal muscles
- action potentials firing at ——rates, even—- than what occurs at——
- Cause is unknown , but, theories:
* _____ in ECF surrounding both nerve and muscle caused by—— or—— (these 2 factors normally— in motor neuron and muscle fibers)
* ____ within muscle or—— of muscle stimulates sensory receptors in the muscle like mechano,stretch and noiceceptors leading to motor neurons being activated—— when these sensory signals reach the——

A

tetanic contraction

abnormally high ; more; maximal voluntary contraction

electrolyte imabalance; over exercise; persistent dehydration; induce APs directly

chemical imbalance; sudden stretch

reflexly; spinal cord

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16
Q

Hypokalemic tetany

  • involuntary—— of skeletal muscle
  • occurs when———-
  • this leads to increase—- channel opening causing membrane—— and spontaneous firing of APs
  • kinda similar to——

Note that the Effect of Ca changes is on the— membrane and not—— membrane

A

tetanic contraction

ECF’s calcium falls to about 40% of normal value

Na; depolarization

Cramping

plasma; sarcoplasmic

17
Q

MUSCULAR DYSTROPHY

  • affects more—— than—— (1 in every——)
  • progressive degeneration of—— and—— muscle
  • caused by—— or—— in one or more protein that make up the——(__,__,___) in——- muscle
A

males; females; 3500 males

skeletal; cardiac

absence; defect; costameres

actin; myosin; dystrophin; striated

18
Q

costameres

  • clusters of—— proteins that link the—— of the outermost myofibril to the—— and——
  • helps in——- from sacromeres to extra cellular matrix and neighboring muscle fibers
  • also helps to stabilize—— against physical force during——
  • could also initiate—- signals that link contractile activity with regulation of muscle cell remodeling
A

structural and regulatory; Z-disk

sarcolemma; extra cellular matrix

lateral transmission of force

sarcolemma; muscle contraction or stretch

intracellular

19
Q

DUCHENNE’s Muscular dystrophy

  • ____proteins
  • sex linked disorder caused by a defect in a gene on the__ chromosome that codes for the protein——
  • leading to a—- protein
  • ____and___ are the first to show symptoms of weakness at about age—— and most affected individuals don’t survive past age—-
  • attempts are being made to cure this by inserting a—— into ——muscle cells
A

missing

X; dystrophin

non functional or missing

hip girdle ; trunk; 2-6; 20

Normal gene; dystrophic

20
Q

Duchenne dystrophy progresses with age and use

T/F

A

T

21
Q

Dystrophin

  • normally forms a bridge between—— and overlying——
  • in its absence, the fibers are subjected to ——during contraction and makes them susceptible to——-?
A

contractile filaments; sarcolemma

repeated structural deformation

rupture or cell death

22
Q

Dystrophin. Extremely large or extremely small?

A

Extremely large

23
Q

Charcot -Marie tooth disease

  • caused by a____ that impairs the—— around the affected nerve
  • causing a ——weakness of the muscles of the—- and——
  • also a decrease in——- of these parts
  • you can test for it by—— ,——, test for gene——
A

duplicate gene

insulating sheath

hand; feet

slowly progressive

tendon reflexes

electromyography; nerve conduction velocity; mutation

24
Q

Myotonic dystrophy:

  • caused by ——- gene
  • delayed—— following——
  • caused by inheriting either of 2—- that—- with each——
  • as———, the symptoms——
  • these bad genes are transcribed unto rnas that are——— and therefore———
A

explanded

relaxation; contraction

expanding genes; grow; generation

genes enlarge; start at an earlier age

too big to leave the nucleus; don’t produce the protein they are to code for

25
Q

Godfather suffered from —- disease

A

Myotonic dystrophy

26
Q

Godfather experienced——- in his—-; daughter experienced a ———weakness ; grandkid suffered———

A

mild weakness; arm

more noticeable arm and leg

severe muscle impairment

27
Q

Hereditary idiopathic dilated cardiomyopathy

  • caused by a—-
  • starts in your—- and is lethal in—% of the cases—-years after unless transplant is done
  • condition is caused by a ——error in a form of—— found only in cardiac muscle
  • a change in a single——- disturbs—- ability to anchor to the—— in cardiac muscle cells
  • leads to gradual ——-of heart chambers and eventual failure
A

tiny glitch

40s; 50; 5

tiny genetic ; actin

nucleotide base; actin; Z line

enlarging

28
Q

Hereditary idiopathic dilated cardiomyopathy

very rare or common form of—— failure?

A

heart

Rare

29
Q

spinal muscular atrophies is not a genetic neuromuscular disease.
T/F

A

F. It is