Neuropathy Flashcards

1
Q

notes

A

mononeuropathy
multiplex - isolated , but more than one.

connective tissue disease - e.g sacrodosis

polyneuropathy - disease of mu;tiple periphearal nerves
tend t be bilateral , symmetrical , motor and sensory impact.

distal part loses function first - proximal part spared in early times , as progress damage spreads t proximal part

aeitolgy - is it linked to cancer etc , cause

radiculopathy - compression of nerve root

pain along nerve - weakness , sensory lost , dysfuntion of what the nerve innervates.
doral plus vebtral roye form spinal nerv.

motor neurone d - with cns - cortex to

amytrophic musclar atropy - upper , lower , brainstem motr neurones impacted — whole thing affected

PLS - Primary laterl SCLEROSIS - damage to brainstem neurones

Muscular dystrophy - motor neurones in spinal cord damage.

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2
Q

nerve damage

A

compression

damage to myelin - central & peripheral mylein are the same.
central neurones - less myelin vs peripheral

peripheral - whole series of shwann cells wrapping around peripheral nerve - thick covering - the outer layer has a cytoplasm which contains nuclues

central - oligodenendrocyte - many branches - single layer - cover given by OLigodendrocytes are sparse vs cshwann cells - so in the case of muscular sytropy - so impacts on central nervous system vs peripheral

classification

  • Transient block - damage to myelin sheath ,
  • Axonotmesis - damage to axon
  • Neurootmesis - damage to axon , endoneurtum , perineuritum , epineuritum
    ( can be entire axon )

progressively it gets worse.

segemental demyeination - segments of myelin gone.

axonal demyelination - myelin sheath in tact , but problem with the axon itself

Wallerian degeneration -
axoplasmic flow - directly connected to cell body.

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3
Q

Nerve vs Neurone ?

A

Nerve - collection of neurones

Neurone - single neurone.

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4
Q

What is a Spinal nerve made out of ?

Structure

A

Ventral & Dorsal route join together to form spinal nerve.

Spinal nerve - part of peripheral NS.

Structure -

epineurium - sheath surrounding spinal nerve
Contains :
0 BV - Veins , Arteries
0 fascicle - clusters of bundles of neurone.
o each
fascicle
surrounded
by
perineurium
0 Neurones - contained in fascicle - either myelinated / unmyelinated
( endoneurium - surrounds this )

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5
Q

Parts of the neurone ?

A

0 Axon

0 Cell body - ( Soma) -( if this is damage - neurone cannot regenerate )

0 Dendrites

0 Terminal bulbs - (if this is damaged regeneration can occur. )
neurotransmitter released from here.

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6
Q

Why don’t most CNS fibres regenerate while PNS fibres can ?

A

0 Oligodendocytes which surround CNS fibres - inhibit regeneration ( shwann cells cover PNS fibres - so dont have this problem. Shwann cells actually assist in regeneration.)

0 Clean up is slow ( faster in PNS by macrophages)

0 environment not very optimal

Time is important - need to clean up damage - to allow the fibres that can regenerate to regenerate.

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7
Q

Classification of Nerve injury ?

A

Neuropraxia - myelin sheath damage - demyelination ( reversible conduction block )

0 can happen as a result of compression - axon ,endoneurium ( surrounds neurone intact ) but myelin sheath compressed .
e.g radial nerve compression. (wrist drop )

Axonotmesis - Demyelination + Axon loss

Axon and corresponding segment of myelin sheath loss - but endoneurium still intact so it can grow back.
( However , distal end - terminal bulb will undergo Wallerian degeneration )- commoly seen in crash inhries - fractures.

Neurotmesis - Demyelination , Axon loss + ( one or all of following - endoneurium , perineurium , epineurium damage )

IF DAMAGED
o endoneurium - can grow back / fair growth.
o perineurium - poor regrowth o epineurium - no growth

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8
Q

What is Wallerian degeneration ?

A

Damage to neurone interrupts connection to cell body - or damages cell body itself.

Part of axon is disconnected from the soma of neuron.

Disconnected portion degenerates & debris is cleared aided by glial cells.

CNS - causes

0 Tumour
0 Haemorrhage
0 head injury with shearing of fibres.

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9
Q

Types of Neuropathies ?

  • Mononeuropathy
    Mononeuropathy mutiplex

WHAT IS IT ?
CAUSES?
EXAMPLES

A

Mononeuropathies - damage to single peripheral nerve - sensory disturbances ( paranthesia , numbness , pain ) & weakness in one isolated area - supplied by the peripheral nerve.

CAUSES -

TRAUMA

  1. compression
  2. violent muscular activity / overextension of joint.

0 Compression —–>1. entrapment neuropathy - narrows passageways , nerve compressed e.g carpal tunnel syndrome.

  1. Compression - by tumour , cast , crutches , prolonged cramped postures —–> nerve compression syndrome - can cause paralysis.
  2. compression - by hemorrhage.
Compression can be :
o  transient (eg, caused by an activity) 
o  fixed (eg, caused by a mass or anatomic abnormality).
Mononeuropathy multiplex / Mononeuritis Multiplex - damage to more than two isolated  peripheral nerve .   
     o Sensory 
        disturbance 
        & weakness 
        in more than 
        2 isolated 
        areas - 
     corresponding 
     to peripheral 
     nerves. 
CAUSES -
0 Connective tissue disorders 
  o polyarteritis nodosa,      
  o systemic lupus 
    erythematosus [SLE], 
  o other types of 
     vasculitis, 
  o Sjögren syndrome, 
  o rheumatoid arthritis 

0 Sarcoidosis

0 Metabolic disorders
o diabetes,
o amyloidosis)

0 Infectious disorders (eg, Lyme disease, HIV infection, leprosy)

neuropathy - can either be sensry , motor or mixed.
o sensory - sensory disturbance - no weakness
o motor - weakness , no sensory D.

EX. of mononeuropathy
- Carpal tunnel syndrome - compression of median nerve.

Peroneal nerve palsy - compression of peroneal / fibular nerve against fibular neck.
(FOOTDROP)

Radial nerve palsy - compression of radial nerve against humerus.
(WRIST DROP - weakness of wrist & finger extensors ) - C7 radiculopathy - compression of C7 nerve root can cause similar symptoms

Ulnar nerve Palsy - compressed under medial condyle - cubital tunnel syndrome . ( SENSORY DEFICIT IN 5TH , 4TH DIGIT - WEAKEND / ATROPHIEDINTEROSSEUS MUSCLE OF HAND - found by metatarsal bones - help control fingers )

POLYNEUROPAHTY - extensive involvement of many nerves.

RADICULOPATHY - damage due to compression of spina nerve root. -radiating pain , weakness , sensory disturbance down dermatome.

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10
Q

Types of Neuropathies ?

0 Polyneuropathy

  • Pattern of presentation
  • causes
  • things to consider
A

Extensive involvement of many nerves

Bilateral , symmetrical
CAUSES

0 damage to myelin sheath - demyelination - infectious causes

0 Damage to BV supplying nerve - causes nerve infarction
(Chronic arteriosclerotic ischemia, vasculitis, infections, hypercoagulable states )

0 Axon damage - can be symmetric or asymmetric

CAUSES

0 Nutritonal deficiences - B12 , B6 , E

0 Alcohol

0 Infection

Common
causes of symmetric
o Diabetes mellitus

o Chronic renal insufficiency

o Adverse effects of chemotherapy drugs (eg, vinca alkaloids)

THINGS TO CONSIDER

  • can affect;
  • sensory vs motor vs autonomic
  • distribution of nerve injury - stocking glove syndrome
  • nerve compartment affected - BV , Myelin or Axon]
  • Pattern of inheritance - acquired vs congenital
  • Cause
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11
Q

What is the Stocking glove syndrome ?

  • Peripheral neuropathy.
A

Early stages of peripheral neuropathy -

sensory disturbances , muscle weakness - present distally at first ———————–> as disease progresses it moves proximally.

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12
Q

Types of Motor neurone disease ?

A

ALS - Amyotrophic Lateral Sclerosis

A - No

myo - muscle

Trophic - nourishment

( NO MUSCLE NOURISHMENT)

Neurogenerative disorder - damage to neurones - upper , lower & brainstem motorneurones ( ALL THREE )

  • upper motor neurone - from brain to spinal cord
  • lower motor - spinal cord / brainstem to sketetal m. - cause movement
    DAMAGE HERE - IS MORE DISABLING WEAKNESS VS UPPER

Motor neurone stop working —-> neurone & muscle atrophies
progressive weakening of muscles
( start to lose hand and arm fuction ————-> difficulty walking, speaking —————–> difficulty breathing )

0 respiratory failure often cause of death - death usually 3 -5 yrs after diagnosis.

CAUSES

O Accumulation of dysfunctional proteins in neurones ( cell body )————————-> degeneration of neurone —— muscles die / atrophy due to no activation ——> impair function.

SYMPTOMS

Early
- asymmetricc hand weakness - dropping objects

  • cramping of upper extremities
  • dysarthria , dysphagia , dysphonia

Intermediate
- Atrophy ——————-> can’t ambulate ( walk without any assistance ) ————> wheelchair use.

Late

  • respiratory weakness —–> dyspnea ——> respiratory infection
  • recurrent cough , fever , chill , pneumonia

-

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13
Q

What is Progressive muscular atrophy ?

A

Subtype of Motor neurone disease

  • only affects mainly Lower motor neurones - Slow , progressive damage .

no UMN signs (but most people will progress to showing these ) - prognosis better than ALS

SYMPTOMS

0 Weakness in hands ——————-> spreads into the lower body- can be severe.

0 muscle wasting (shrinking),

0 clumsy hand movements, twitches, and muscle cramps.

0 Torso muscles and breathing may be affected.

*Exposure to cold can worsen symptoms.

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14
Q

What is Primary Lateral Sclerosis ?

A

Affects only upper motor neurone ( supply hands , legs , face )

  • neurodegenerative disease - progressive stiffness & weakness

SYMPTOMS

PAINLESS
- slow & difficult movement in hands , legs ,face

caused by spasticity - spasm

Legs first —————–> torso ————–> arms & hands ——-> muscles used for swallowing , speaking , chewing in face (less common).

difficult to walk , speak , fine hand coordination.

( distal to proximal )

Progresses more slowly than ALS.
if they develop LMN symptoms - diagnosis change to ALS.

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15
Q

What is Spinal musclar atrophy (SMA) ?

A

Affects LMN only.

most common cause of genetic infant mortalilty .

alpha LMN (responsible for innervation of voluntary skeletal muscle ) from spinal cord (anterior horn ) die prematurely -
( LMN can die or the whole LM nerve )

————> motor unit ( neurone / nerve + muscle fibre ) - stops working———————–>
IF ENOUGH MUSCLE FIBRES STOP WORKING
muscles weakness & flaccid -low tone paralysis ———————> Muscle atrophies ( lack of innervation ).

0 Fasciculations (Spontaneous involuntary muscle contraction ) — If a lot of muscle fibres affected

0 Diminished reflexes - deep tendon reflexes

CHRONIC SYMPTOMS

0 Scoliosis - poor support of spine
0 extremely thin limbs - muscle wasting.

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16
Q

Difference btw Upper motor neurone damage vs lower motor neurone ?

A

UMN - Increased muscle tone & spasticity (spasm )

LMN - Muscle weakness ——-> Flaccid / low - tone paralysis ——–> Muscle atrophy

17
Q

Types of SMA - spinal musclar atrophy ?

A

TYPES

0 Type 1a - congenital - before birth
(MOST SEVERE)
- mothers might notice reduced fetal movement

0 Type 1b -infantile
Normal at birth ———————> low muscle tone (hypotonia ) few weeks after
(MOST COMMON ) - normally don’t survive after a few years.

o progressive weakness - worse proximally than distally - more obvious in legs
( ex - worse in the top part of leg than bottom or the top part of arm vs bottom ) - DIFFICULT TO SIT UP

  • difficulty talking milk , swallowing their own secretions e.g saliva - weakness in muscle responsible for sucking , chewing , swallowing.
    o can cause aspiration

0 Weakness in chest wall all , diaphragm
o cause breathing difficulties.

Type II
Type III
Type IV
(these are progressively milder - IV - mildest , with later age of onset.)

18
Q

Why are there different subtypes of SMA ?

A

Subtype / severity determined by the number of copies of SMN 2.

  • Reason

SMN proteins needed for development of alpha LMN.
Two types of SMN genes than encode for protein - SM1 & 2 .
SM1- 1 is more functional - produces more viable proteins .
SMN -2 - produces non - functional + functional proteins.

SMA patients - have no copy of SMN-1
( SO THE MORE COPIES OF SMN 2 - THE MORE FUNCTIONAL PROTEINS PRODUCED – the less severe SMA is )

19
Q

Why does muscular dystrophy etc. affect either CNS or the PNS but not the other - only one ?

A

CNS - oligodendrocyte ( has many branches) - covers the neurone in single layer - so cover given is
sparse

vs PNS - Schwann cells wrap around axon multiple times ( thick covering )

so in the case of muscular dystrophy - demyelination will affect the CNS more

20
Q

How does the structure of CNS & PNS neurones affect the impact of allergic recation etc. on their neurones ?

A

CNS & PNS neurones - 30 % Protein , 70 % lipid

CNS & PNS - have similar lipids
but proteins have important differences.

differences in proteins - means an allergic reaction in one against proteins - may not affect the other.

However , lipids similar - so will affect both.

21
Q

What is Distal axonopathy ?

A

Degneration of axon / myelin begins at most distal part of axon - moves distally to proximally ———————— if it persists then axon dies back. – Stocking & glove .

CAUSES

Stagnation in Axoplasmic flow - so organelles & neurofillaments remain in degenerated part of axon ( cannot get back to cell body ) —- eventually axon becomes atrophic , breaks down.

0 Dysfunction in cell body means metabollic demands of neurone cant be met —–> distal part will be affected first (furthest away)
(THIS IS ALSO WHY LARGE AXONS - MOST SEVERELY AFFECTED - HIGHER METABOLLIC DEMAND )

0 Can be caused:

  • drugs and industrial poisons :
o pesticides, 
o acrylamide,
o organic 
   phosphates,
o industrial 
   solvents

AXOPLASMIC TRANSPORT
- cellular process moves organelles e,g mitochondria , vesicles , proteins etc - from neuronal cell body to distal end via axoplasm (axon cytoplasm ).
Responsible for transport of molecules destined for degradation back to cell body to be degraded by lysozymes.

22
Q

What is Segmental Demyelination ?

A

Breakdown & loss of myelin over a few segments

Axon remain intact & no change to neuronal body.

CONSQUENCES

0 Loss of saltatory conduction —————–> decreased conduction velocity and conduction block.

Deficts happen rapidly - but reversible as schwann cells make new mylein.

However , many cases - demyelination leads to axons & permanent deficits.