peripheral neuropathy Flashcards

1
Q

What peripheral neuropathy did the man in the video have and with what autoantibodies?

A

CANOMAD

With IgM ab against disialosyl

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2
Q

typical features of how peripheral neuropathy affects the somatic nervous system?

A
muscle weakness and loss of muscle tone.
Loss of dexterity
sensory loss tingling/pain or numbness temperature. 
imbalance
suppression of reflexes
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3
Q

features of peripheral nueropathy affecting the autonomic nervous system?

A

postural hypotension
lack of sinus arrythmia (no HR changes with inspiratio/n/expiration)
impaired pupillary reflexes to light.
abnormal sweating (glands under autonomic control)
stomach and bowel contraction and emptying issues.

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4
Q

3 ways that Peripheral nerves are damaged.

A

axon injury if in centre can extend proximally and distally
Or axon damage distally will start to die away from muscle.

Or segmental demylination- unable to contract despite intact axon and muscle connection. because of loss of conduction.

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5
Q

Order of peripheral nerves head to bottom of spine/

A

cranial, thoracic, lumbar, sacral.

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6
Q

What sensory and motor inputs do the cranial nerves supply?

A

To face and neck as well as through vagus nerve: gut, innervates the lungs and heart

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7
Q

what are the differences in how the CNS and PNS nerves are myelinated?

A

PNS myelinated by schwann cells and CNS myelinaed by oligodendrocytes.

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8
Q

What are the subdivisoin of the PNS?

A

sensory (afferent: somatic sensory and visveral sensory.

motor efferent: somatic nervous system and autonomic nervous system

autonomic: sympathetic and parasympathetic.

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9
Q

Effect of parasympathetic on HR and glucose release , airway smooth muscles and stomach and pupils?

A
slows HR
inhibits glucose release
constricts ariway
stimulates stomach activity.
constricts pupils
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10
Q

Effect of sympathetic on HR and glucose release , airway smooth muscles and stomach and pupils?

A
Increases HR
stimulates glucose releae
relaxes airways
inhibits stomach activity
dilates pupils
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11
Q

Nerves from spinal cord come in pairs, drsal root and ventral root.
Where do sensory and motor nerves belong and their cell bodies?

A

dorsal route has sensory neurones with cell body in dorsal root ganglion.

Ventral root contains motor neurones, cell bodies in anterior horn of cord.

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12
Q

What categories of myelination are C IV and Aa?

A

Aa has the greatest myelination and diamter- the fastest conduction for skeletal muscles.

C IV has least myelination and diameter for pain an temperature receptors.

Skeletal muscle > mechanoreceptors > pain and temperature.

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13
Q

Which will demyelination affect more? C IV neurones or Aa neurones?

A

More heavily myelinated will be more affected.

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14
Q

What is a classical presentation of axonal sensory-motor peripheral polyneuropathy? What can cause this?

A

distal extremities to proximal spread - some numbness in the middle chest.

A whole host of things can cause this chronic axonal loss, drugs infections diseases, endocinre, etc.

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15
Q

How can you distinguish axonal from demyelinating pathology clinically?

A

axonal has a lenght dependent pattern with weakness proportional to wasting.

Demyelinating: proximal and distal weakness, without the wasting (can have rapid recovery)

use electrodiagnositic testing:

axonal: decrease in amplitude
demyelinating: decrease in conduction.

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16
Q

What are examples of acute and chronic demyelinating neuropathies?

A

acute: Guillian barre syndrome (caused by an infectious. vaccine/trauma trigger)

Chronic: CIDP (Chronic inflammatory demyelinating polyneuropathy)

Paraprotein related.

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17
Q

Differences in progression to worse symptoms in GBS vs CIDP?

A

GBS: 4 weeks till peak

CIDP: 8 weeks +

18
Q

differences in the disease course in GBS and CIDP?

A

GBS: monophsic, will resolve.

CIDP: mostly chronically progressive- relapsing remitting or monophasic.

19
Q

Is there often cranial, respiratory muscle, autonomic involvement in GBS vs CIDP?

A

often for GBS

rarer for CIDP

20
Q

Pattern of progression GBS vs CIDP?

A

GBS: starts distally and progresses proximally.

CIDP: variable can start with upper limb onset.

21
Q

Differences in pathological subtypes in GBS vs CIDP?

A

GBS: axonal, demyelinating, nodal/paranodal

CIDP: nodal/paranodal and demyelinating

22
Q

Differences in the general treatment of GBS and CIDP?

A

GBS: IVg and plasma exchange. NO steroids

CIDP: steroid and plasma exchange, rituximab

23
Q

5 different pathologies of immune-mediated neuropathies.

A

Ab targeting components of myelin sheath/ nodes of ranvier (complement activation and MAC damage)

peripheral deposition of immune complexes

amyloidosis

infiltrating immune cells (could be macrophages MMPs) and autoimmunity (cancer?)

cytokine production causing inflammation

24
Q

Components of the myelin sheath that could be targeted?

A

MAG/SGPG

25
Q

What kind of protein can cause molecular mimicry and GBS in infection?

A

C. jejuni, LPS coating mimics GM1 and GD1a antignes- AMAN GBS subtype.

Or mimicry of GD3 GQ1b and GT1 (MFS subtype).

26
Q

What kind of neuropahy is AMAN?

A

axonal target GM1, GD1a antigens.

27
Q

What kind of neuropathy is MFS and AIDP?

A

MFS is regional: GD3 and GT1 GQ1b

AIDP is demyelinating but ukown antigen targeted.

28
Q

What antigens are targeted in chronic neuropathies CANOMAC? Antig-MAG/ DADS?

A

CANOMAC: GD1b, GD3 GQ1b GT1b.

Anti-MAG/DADS: myelin associated glycoproteins.

29
Q

What neuropathy has antibodies against NF155, NF140 CNTN1 and CASPR1?

A

CIDP

30
Q

By what mechanism can Ab mediated targeting of nodes of ranvier lead to axonal degradation?

A

gangliosides in nodes of Ranvier targeted,complement acivation and MAC formation.

Ca2+ will simulate axon degradation which means difficult to repair.

31
Q

Where can protein targets also be found that aren’t in the nodes of ranvier?

A

paranode, juxtaparanode, internode.

CASPR1/2 in paranode can bind to myelin sheath,

32
Q

Is NF140/186 in node or paranode?

A

Node

33
Q

Are CASPR1, NF155 and CNTN1 in nodes or paranodes?

A

paranodes.

34
Q

How might IgG4 be pathogenic in neuropathies?

A

they will block e.g. paranode proteins mediating myelin sheath adhesion

35
Q

What does PDN stand for and different types?

A

painful diabetic neuropathy.
IgG/IgA indistinguishable from CIDP.
IgM PDN: particularly associated with tremor, slow progression and anti MAG ab.

36
Q

Features of PEOMS

A

clonal expasion of plasma cell leading to elevated cytokine levels- mixed axonal/demyelinating neuropathy with poor outcome.

37
Q

treatment for CIDP or IgA/IgG PDN?

A

steroids, or IVig

Plex, immunosuppressants

38
Q

treamtent for GBS and MMN/pure motor CIDP?

A

no steroids, IVIg, PLEX

39
Q

treatment for DADS/IgM PDN?

A

IVIg or rituximab

40
Q

POEMS treatment?

A

chemotherapy and steroids, radiation and SCT.