NEUROMUSCULAR DISORDERS Flashcards
NEUROMUSCULAR DISORDERS
These diseases can affect:
____ directly
——— indirectly
Or ————
muscles
Nerves
N-M junction
Generally ,
spasticity is——— motor neuron disorder)
-some degree of paralysis is ——-motor neuron disorder)
an upper
a lower
The following occur more in women or men??
Hungtington’s disease
Myasthenia gravis
Muscular dystrophy
Women
Women
Men
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.
spontaneously; inherited; transmitted by contact with infected tissue
transplant ; contaminated meat
tiny holes
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.
lower
become weak ; waste away
*———(ALS;——— disease) is ——-motor neuron condition
Amyotrophic lateral sclerosis
lou Gehrig’s
mixed upper and lower
NEUROMUSCULAR DISEASES CAN BE CAUSED BY some form of collagen disorder like ——-disorders
Ehler-danlos
failure of the myelin around nerves is seen in certain deficiency diseases such as failure of the body to——-
absorb vitamin B12
Myasthenis gravis: form of muscle weakness due to——-
- occurs in every 1 in—— people and no one knows why
- causes——
antibodies against acetylcholine receptors
200k
paralysis
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——
neostigmine; anticholinesterase; accumulation of larger amounts of Ach
immune system ; glucocorticoids
antibodies; 50%
plasmapheresis; antibodies
In lambert eaton myasthenic syndrome
-muscle strength—— as—— calcium is released
increases; more
tetanus and botulism are—— infections in which—— toxins cause——- or——— muscle tone respectively
bacterial; bacterial
increased; decreased
Poliomyelitis, fungal, viral, or bacterial
Viral
Poliomyelitis:
- destroys motor NEURONES
- results in—— of skeletal muscle
- may result in death due to——-
paralysis; respiratory failure
(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——
tetanic contraction
abnormally high ; more; maximal voluntary contraction
electrolyte imabalance; over exercise; persistent dehydration; induce APs directly
chemical imbalance; sudden stretch
reflexly; spinal cord
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
tetanic contraction
ECF’s calcium falls to about 40% of normal value
Na; depolarization
Cramping
plasma; sarcoplasmic
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
males; females; 3500 males
skeletal; cardiac
absence; defect; costameres
actin; myosin; dystrophin; striated
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
structural and regulatory; Z-disk
sarcolemma; extra cellular matrix
lateral transmission of force
sarcolemma; muscle contraction or stretch
intracellular
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
missing
X; dystrophin
non functional or missing
hip girdle ; trunk; 2-6; 20
Normal gene; dystrophic
Duchenne dystrophy progresses with age and use
T/F
T
Dystrophin
- normally forms a bridge between—— and overlying——
- in its absence, the fibers are subjected to ——during contraction and makes them susceptible to——-?
contractile filaments; sarcolemma
repeated structural deformation
rupture or cell death
Dystrophin. Extremely large or extremely small?
Extremely large
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——
duplicate gene
insulating sheath
hand; feet
slowly progressive
tendon reflexes
electromyography; nerve conduction velocity; mutation
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———
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
Godfather suffered from —- disease
Myotonic dystrophy
Godfather experienced——- in his—-; daughter experienced a ———weakness ; grandkid suffered———
mild weakness; arm
more noticeable arm and leg
severe muscle impairment
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
tiny glitch
40s; 50; 5
tiny genetic ; actin
nucleotide base; actin; Z line
enlarging
Hereditary idiopathic dilated cardiomyopathy
very rare or common form of—— failure?
heart
Rare
spinal muscular atrophies is not a genetic neuromuscular disease.
T/F
F. It is
