Neuro of the PNS Flashcards

1
Q

Axonal Neuropathies are due to _______ and ______ insults

A

toxic, metabolic

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2
Q

During Axonal Neuropathies, the axons often degenerate in a __________, resulting in a __________ pattern

A

length-dependent fashion, “dying-back”

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3
Q

Which types of axons in Axonal Neuropathies are most susceptible?

A

Longest axons

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4
Q

What is the Axonal Neuropathy Electrophysiologic hallmark ?

A

reduction in signal amplitude owing to the dropout of axons from affected peripheral nerves with relative preservation of conduction velocity.

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5
Q

In Demyelinating Neuropathies, what are the primary targets of the damage?

A

Schwann cells with their myelin sheaths

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6
Q

T/F are axons relatively preserved during Demyelinating Neuropathies?

A

True

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7
Q

During Demyelinating Neuropathies, individual myelin sheaths degenerate in a __________ resulting in _______ damage of myelin segments

A

seemingly random pattern, discontinuous

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8
Q

During Demyelinating Neuropathies, in response, ________ proliferate and initiate repair through the formation of new myelin sheaths, but now _____ and _______ than the original ones

A

Schwann cells
shorter
thinner

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9
Q

What is the electrophysiologic Demyelinating Neuropathy hallmark?

A

is slowed nerve conduction velocity, reflective of the loss of myelin.

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10
Q

What do Neuronopathies result from and what do they lead to?

A

destruction of neurons, leading to secondary degeneration of axonal processes.

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11
Q

What are examples of neuronopathy insults ?

A

Infections like herpes zoster and toxins like platinum compounds

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12
Q

In neuronopathies, because the damage is at the level of the neuronal cell body, the peripheral nerve dysfunction is equally likely to affect the ________ and ________ parts of the body

A

proximal, distal

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13
Q

In peripheral axonopathies, it ONLY affects the __________ extremities

A

distal

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14
Q

What are Mononeuropathies?

A

single nerve and result in deficits in a restricted distribution dictated by nerve anatomy ex: Trauma, entrapment, and infections

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15
Q

Polyneuropathies affect ________ nerves and is usually __________

A

multiple nerves, usually symmetric

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16
Q

What are Polyneuropathies?

A

axons are affected in a length-dependent fashion leading to deficits that start in the feet and ascend with disease progression. The hands start when deficits at knee, resulting in a characteristic “stocking and glove” distribution of sensory deficits.

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17
Q

What are Mononeuritis multiplex?

A

individual nerves in a haphazard fashion. right wrist drop from involvement of the right radial nerve and a left foot drop from peroneal nerve damage. Vasculitis is a common cause.

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18
Q

What are Polyradiculoneuropathies

A

nerve roots as well as peripheral nerves, leading to diffuse symmetric symptoms in proximal and distal parts of the body.

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19
Q

Guillain-Barré Syndrome is an acute inflammatory__________

A

Demyelinating Polyneuropathy

20
Q

In Guillain-Barré Syndrome, where does the weakness begin?

A

in the distal limbs that rapidly advances to affect proximal muscle function (“ascending paralysis”)- respiratory compromise

21
Q

What are the histologic features of Guillain-Barré Syndrome

A

inflammation and demyelination of spinal nerve roots and peripheral nerves (radiculoneuropathy)

22
Q

What are 2/3 of causes of Guillain-Barré Syndrome preceeded?

A

by an acute, influenza-like illness convalescing by the time the neuropathy becomes symptomatic

23
Q

What are the inciting agents of Guillain-Barré Syndrome ?

A

Campylobacter jejuni,CMV, EBV, andMycoplasma pneumoniaeas well as prior vaccination

24
Q

What are Guillain-Barré Syndrome’s main clinical feature?

A

Ascending paralysis and areflexia

25
Why are nerve conduction velocities slowed in Guillain-Barré Syndrome?
because of multifocal destruction of myelin segments in many axons within a nerve.
26
What is the most common chronic acquired inflammatory peripheral neuropathy?
Chronic Inflammatory Demyelinating Poly(radiculo)neuropathy-CIDP
27
Chronic Inflammatory Demyelinating Poly(radiculo)neuropathy-CIDP has _________ mixed with _________ polyneuropathy that persists for 2 months or more
symmetric, sensorimotor
28
What are examples of systemic autoimmune disease associated neuropathy
rheumatoid arthritis, Sjögren syndrome, or systemic lupus erythematosus
29
What are examples of infections neuropathies ?
1. Leprosy : Schwann cells invaded by Mycobacterium leprae 2. Lyme Disease: uni/bilateral facial palsies 3. HIV infection: distal sensory neuropathy that is often painful. 4. Varicella-Zoster Virus: most common viral infection of PNS. latent infection persists within neurons of sensory ganglia virus is reactivated-> transported along the sensory nerves to the skin -> infects keratinocytes-> painful, vesicular skin eruption in a distribution that follows sensory dermatomes (shingles).
30
What is the most common cause of peripheral neuropathy?
diabetes
31
How does Distal symmetric diabetic polyneuropathy typically present?
with sensory symptoms like numbness, loss of pain sensation, difficulty with balance, and paresthesias or dysesthesias
32
Which disease is the most common inherited peripheral neuropathy?
Charcot- Marie Tooth-
33
What is the neuromuscular junction ?
the interface of motor nerve axons and skeletal muscle that serves to control muscle contraction
34
What symptoms would you see in a patient with a disease in the neuromuscular junction
painless weakness, fatigue
35
How does the nueromuscular junction stimulate muscle contraction?
Upon depolarization, presynaptic nerve terminals release acetylcholine (ACh) into the synaptic cleft--> postsynaptic sarcolemma contains localized clustering of ACh receptors > initiate signal leading to muscle contraction.
36
Myasthenia Gravis (NMJ) is an autoimmune disease with autoantibodies directed against
postsynaptic Ach receptors
37
Who does Myasthenia Gravis affect more?
young females, older males
38
How does Myasthenia Gravis typically present?
with fluctuating weakness that worsens with exertion and often over the course of the day. Diplopia and ptosis (extraocular muscles) are common generalized weakness severity can require mechanical ventilation
39
What are toxins that affect the NMJ?
1. Botulism condition caused by exposure to a neurotoxin (botulinum toxin, popularly known as Botox) produced by the anaerobic gram-positive organism Clostridium botulinum Botox acts by blocking the release of ACh from presynaptic neurons 2. Curare is the common name for plant-derived muscle relaxants that block ACh receptors, resulting in flaccid paralysis. Poison on arrow tips by indigenous people in the Amazon rain forest
40
Majority of benign and malignant neoplasms of peripheral nerve sheaths are composed of cells that show evidence of __________ differentiation. 
Schwann cell
41
What are the 3 common types of peripheral nerve sheath tumors?
1. schwannoma 2. neurofibroma 3. malignant peripheral nerve sheath tumor (MPNST).
42
What are peripheral nerve sheath tumors associated with?
familial tumor syndromes, including neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis.
43
What are Shwannomas?
benign tumors that exhibit Schwann cell differentiation local compression of the involved nerve or adjacent structures (e.g., brainstem or spinal cord)
44
Shwannomas typically arise from ________
peripheral nerves I
45
What are acoustic neuroma?
schwannomas occurring at the cerebellopontine angle, attached to the vestibular branch of 8th CN. Tinnitus and hearing loss.
46
What are Neurofibromas?
benign nerve sheath tumors more heterogeneous than schwannomas admixed with perineurial-like cells, fibroblasts, mast cells, and CD34+ spindle cells