Week 4 Flashcards

(47 cards)

1
Q

What does a motor unit comprise of

A

A a-motoneuron
All extrafusal skeletal muscle fibres it supplies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the a-motoneurone

A

A specific subset of neurones of the nervous system through which all commands from the brain are relayed to skeletal muscles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is a neuromuscular junction

A

The site of communication between the motoneurone and muscle

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Theres 2 variants to the classification of motoneurone

A

Upper and lower motoneurones
Arranged strictly in series, damage to either of these leads to motoneurone signs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Where are upper motoneurones found

A

Reside in the brain in the cerebral cortex and travel down to the brain stem or spinal cord

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Where are lower motoneurons’ cell bodies found

A

In the ventral horn of spinal cord grey matter or cranial nerve motor nuclei in the brain stem

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is meant by innervation

A

The term used to describe the normal state of nerve supply to a muscle of other target
Act of stimulating

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Denervation

A

Depriving muscle of its nerve supply

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Re-innervation

A

Re-growth of the nerve to re-supply the muscle
Re-innervation of the original effector organ is not always successful, most nerves reinnervate an effector organ that is different from its original target
This leads to emergence of all sorts of unexpected results

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What leads to death of motoneurones

A

Loss or damage to the motoneurones cell body
Theres a class of diseases that target cell bodies of motoneurones
This would result in the denervation of muscles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is poliomyelitis

A

Communicable infection that targets cell bodies of lower motoneurones of body
Also known as infantile paralysis
Caused by infection by polio virus
Leads to toxic infection of cell bodies of neurones of ventral horn
Any motoneurones of spinal cord are susceptible to virus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What does death of motoneurones lead to

A

Denervation hence paralysis of muscles they supply
Toxic infection of cell bodies of neurones of ventral horn can lead to death of dorsal horn too

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is motoneuron disease

A

Rare condition affecting brain and nerves
2 variants
Disease simultaneously kills both lower and upper motoneurones. Known as progressive supranuclear palsy
The disease targets only lower motoneurone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is programmed cell death known as

A

Apoptosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Which motoneurones dont undergo apoptosis under MD

A

Motoneurones supplying extraocular muscles
Motoneurones supplying anal sphincter

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Implications of disorders of motoneurones axon and LMN signs

A

Loss or damage to motoneurones axon doesn’t necessarily lead to death of motoneurone
However it leads to the removal innervation to muscle- known as denervation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Complete transaction of axons of motor nerve

A

Exemplified by crushing injuries to limbs, stabbing injuries
Usually accidental
Often inevitable involvement of nerves supplying affected part of body
Results in denervation of muscles involved

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is Guillain-Barré syndrome

A

G-B syndrome is acquired as a complication following a viral infection such as common cold
Rare and serious condition mainly affects feet, hands, limbs causing numbness, weakness and pain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Demyelination of axons of motoneurones

A

Diabetic neuropathy leads to demyelination of axons of both sensory and motor axons
Clinical signs reverse when condition is cured or goes into remission
Clinical motor signs can return with relapse
Vision loss, muscle weakness, muscle stiffness and spasms, loss of coordination, changes in bladder and bowel function, walking problems

20
Q

What is peripheral neuropathy

A

Type of nerve damage that can cause pain, weakness, numbness
Nerves located outside Brian and spinal cord

21
Q

Implications of disorders of neuromuscular junction and LMN signs

A

Neuromuscular junction is necessary element to integrity of motor unit
Theres a class of diseases that target NMJ in its own right
LMN damage include weakness, muscle atrophy (wasting), and fasciculations (muscle twitching), decreased reflexes

22
Q

What is the NMJ

A

It’s the tissue interface between motoneurone and muscle
It’s synaptic interface
Special synapse
Also called end plate

23
Q

How does NMJ work

A

Motoneuron forms presynaptic membrane, releases acetylcholine as neurotransmitter
The muscle forms post synaptic membrane, expresses Nicotinic receptors that convert chemical messages carried by Ach
The synaptic cleft expresses enzyme acetylcholine esterase that destroys any Ach that isn’t bound to postsynaptic membrane

24
Q

What does botulinum toxin do

A

Acts to deplete the presynaptic terminal of NMJ of its neurotransmitter
Effect of botulinum toxin is dry mouth and difficulty swallowing

25
What is myasthenia gravis
Rare genetic disease of neuromuscular junction Nicotinic receptors of postsynaptic membrane of NMJ are selectively destroyed by persons immune system, blocked by antibodies. Reduced transmission of ACh, symptoms worsen then get better when rest It affects any neuromuscular junction of body in affected individuals. Not localised Droopy eyelids, Ptosis Ophthalmoplegia- weakness or paralysis of eye muscles Brings about flaccid weakness of affected muscles. Fatigability, proximal weakness Can cause respiratory failure if muscle of respiration affected
26
Duchenne muscular dystrophy- genetic disorder
Genetic Progressive muscle weakness Defects in muscle proteins Death of muscle tissue Eventual death of affected individual
27
Lower motoneuron signs
Lesions of lower mn lead to characteristic impairments of movements Theres a series of emergent clinical presenting signs known as LMN signs LMN signs are very different from upn signs
28
Why are NMJ specialised for rapid transmission
Lots of places where the neurotransmitter is released Lots of active zones where vesicles release contents More than other synapses in CNS Contain junctional folds which contain postsynaptic densities
29
Role of Ca2+ in excitation-contraction coupling
Calcium conc greater outside- concentration gradient Action potential in post synaptic terminal Depolarisation opens voltage gated calcium channels, Ca enters presynaptic terminal So intracellular conc increases Fusion of vesicles with presynaptic membrane, neurotransmitter (ACh) released
30
Ca2+ induced vesicle fusion
Calcium entry into presynaptic terminal triggers vesicle fusion Calcium sensor is synaptotagmin (calcium binds to this and it changes shape) Change in conformation on ca binding triggers vesicle fusion Exocytosis of neurotransmitter Exact mechanism is unclear
31
ACh transmitter end plate
ACh binds to Nicotinic ACh receptors on postsynaptic end plate membrane 2 molecules of ACh needed to bind Ionotropic receptor/ ligand gated ion channel
32
NMJ- specialised synapse
Presynaptic- multiple quanta release- many vesicles releasing contents in CNS single vesicle only Postsynaptic- junctional folds, high density of nACh receptors ( allow sodium in end plate potential), high density of voltage gated Na+ channels- action potential, end plate potential
33
Why is End plate potential so high
So many receptors activated by so much neurotransmitter Large depolarisation
34
ACh transmitter at end plate
ACh binds to nACh receptors Channel opens-permeable to Na+ and K+ Em muscle cell= -90mV Na+ influx>>> K+ efflux because Em<
35
How does the end plate potential work
EPP initiates an AP in muscle, not action potential itself EPP decays as it moves away from end plate- Nicotinic receptors not expressed anywhere else other than end plate AP then travels through muscle cell
36
Properties of EPP
Timing presynaptic AP to EPP ~1msec EPP generated by ligand gated channels Very large in comparison to most synaptic potentials- ,many ACh vesicles, high density of nAChRs Threshold for AP generation easily passed- high density Na+ voltage gated channels at end plate
37
The postsynaptic action potential
AP invades T tubules system Allows transmission of AP deep into muscle fibre to separate myofibrils Propagate along muscle fibre
38
Where is calcium ions released from
Sarcoplasmic reticulum
39
Fate of ACh
ACh binds to nAChR for ~ 1ms Released into synaptic cleft- hydrolysed by acetylcholinesterase AChE Concentration ACh decreased rapidly Choline taken back up to make new ACh Choline acetyl transferase catalyses synthesis ACh in cholinergic neurones
40
Myesthenia gravis
Autoimmune disease which affects expression of receptors so synapse fails. Not enough nAChRs postsynaptically to get reliable transmission at this synapse which should never fail Caused by failure of NMJ Do not get large enough EPP to generate AP Muscle weakness during sustained activity
41
What muscles first affected in myesthenia gravis
Skeletal muscle of the eye fatigue most quickly so symptoms seen here first Eyes most active muscles in body
42
Treatment of myesthenia gravis
AChE inhibitors -prolongs signal -neostigmine-acetylcholinesterase inhibitor Allows ACh to last longer so receptors can get enough ACh to stimulate them
43
Disorders of the motor unit
Death or dysfunction of the muscle gives symptoms similar to lower motoneuron signs -characteristic impairments of movement -flaccid muscle weakness -hypotonia (low level of muscle tone) or atonia (lack of muscle tone) -hyporeflexia (skeletal muscles have a decreased or absent reflex response) or areflexia (absence of deep tendon reflexes) -denervation of muscle atrophy -fasciculations (acute phase) -muscle wasting
44
Duchenne muscular dystrophy
A genetic progressive muscle weakness. Defects in muscle protein dystrophin causes death of muscle tissue
45
UPN signs
Weakness Spasticity- a condition in which there is an abnormal increase in muscle tone or stiffness of muscle, which might interfere with movement speech or be associated with discomfort or pain Clonus Hyperflexia
46
Clinical sign that would be seen in acute phase of LMN lesion before muscle atrophy
Fasciculations- caused by increased receptor concentration on muscles to compensate for lack of innervation
47
Consequence of denervating a skeletal muscle fibre
If a muscle cell is not reinnervated it may ultimately die