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NMSK Exam 3 > Chapter 26 Pt 2 > Flashcards

Chapter 26 Pt 2 Flashcards

1
Q

In what process are myelin ovoids formed?

A

Axonal neuropathies: Wallerian degeneration => Schwann cells will catabolize myelin => unravel and disintegrate into spherical structures.

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2
Q

What do we see on histology in demyelinating neuropathies?

A

Onion bulbs: excess proliferation of Swchwann cells => concentric layers of cytoplasm of Schwann cells and redundant BM wrapped around thinly myelinated axons, like an onion

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3
Q

Type 1 Muscle Fibers

  1. Color
  2. Action
  3. Strength
  4. Physiology
A
  1. Red
  2. Sustained force
  3. Weight-bearing
  4. Slow-twich
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4
Q

Type 2 Muscle Fibers

  1. Color
  2. Action
  3. Strength
  4. Physiology
A
  1. White
  2. Sudden movements
  3. Purposeful motion
  4. Fast-twitch
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5
Q

What causes Type 2 Fiber Atrophy (group atropgy)?

A
  1. Inactivity or disuse (limb fracture, degeneration of pyramidal tract)
  2. Neurodegenerative disease
  3. Corticosteroids => steroid myopathy
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6
Q

What type of reactions do muscle fibers undergo?

A
  1. Segmental necrosis
  2. Vacuolization
  3. Regeneration
  4. Hypertrophy
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7
Q

When a muscle fiber undergoes segmental necrosis, briefly describe the process?

A
  1. Necrosis of segment
  2. Myophagocytosis
  3. Replaced with collagen and fat
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8
Q

Characteristics of muscle fibers that are regenerating

A
  1. Centrally located nuclei
  2. Cytoplasm is red with trichrome stain because alot of RNA
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9
Q

Characteristics of muscle fibers that are undergoing hypertrophy

A

Muscle fiber splitting: cell membrane splits it down middle with adjacent nuclei

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10
Q

What can cause a neurogenic bladder? (6)

A
  1. MS
  2. Parkinsons
  3. DB
  4. Infection of brain/SC
  5. Spina bifida
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11
Q

Course of Bells Palsy

A
  • Occurs 48-72 hours after a URI/DM and resolves spontaneously in pts who are 15 - 60YO
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12
Q

Inflammatory neuropathies: how do we differentiate them?

A
  1. Guillian Barre Neuropathy
  2. Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Differentiate by: time course and whether htey respond to steroids.

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13
Q

MC AQUIRED Inflammatory Peripheral Neuropathy

A

Chronic Inflammatory Demyelinating Peripheral Polyradiculoneuropathy

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14
Q

2/3’s of Guillain-Barre Syndrome cases are preceded by what; which etiologies have been implicated?

A

Acute, influenza-like illness

  1. Campylobacter
  2. CMV,
  3. EBV,
  4. Mycoplasma penumoniae,
  5. Prior vaccination
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15
Q

How is CIDP and AIPD different from one another?

A

CIDP

  1. Chronic: persists more than 2 months and has remissions and relapses
  2. Responds to steroids
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16
Q

Pathophysiology of CIDP?

A

IgM and IgG Ab on myeling sheath

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17
Q

Infectious Polyneuropathies (5)

A
  1. Leprosy (Hansen Disease)
  2. Diptheria
  3. Varicella-Zoster Virus
  4. Lyme Diseae
  5. HIV
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18
Q

MC Viral Infection of the PNS

A
  1. Varicella Zoster
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19
Q

What is Leprosy, Hanson Disese?

A

Mycobacterium Leprea invades Schwann cells and other cells => symmetric polyneuropathy in COOL extrememties

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20
Q

In lepromatous leprosy (Hansen Disease) Schwann cells are invaded by Mycobacterium leprae and what pattern of peripheral nerve damage will be seen?

A
  1. Segmental demyelination and remyelination
  2. Loss of myelinated AND unmyelinated fibers
  3. Endoneurial fibrosis
  4. Multi-layered thickening of perineural sheaths.
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21
Q

What are the clinical symptoms of Leprosy (Hansen Disease)?

A
  1. Symmetric polyneuropathy of pain fibers COOL extremeties => loss of sensation in hands and toes, but nose is often affected first.
  2. Large, traumatic ulcers => pt does not feel it.
22
Q

Leprosy Findings

A
  • AFB (+): multuple uniform-staining acid-fast slender baccili
23
Q

More localized nerve involvement + granulomatous nodules in the dermis is characteristic of what form of leprosy?

A

Tuberculoid leprosy = TH1 (cell-mediated) response

24
Q

Peripheral nerve dysfunction as a result of Diptheria is due to what; what are the early sx’s and later findings?

A

Result of diptheria exotoxin

  1. Early loss of proprioception and vibratory sensation
  2. Bulbar and respiratory muscle dysfunction -> dealth or long-term disability due to suffocation.
25
Lesion seen in peripheral nerves due to **Diptheria**
1. **_Selective demyelination_** that extends to the **_anterior/posterior roots_** and into the **_mixed sensorimotor nerves_**
26
**Diptheria** is MC due to what?
1. **Incomplete immunization** 2. **Waning adult immunty**
27
MC viral infection of PNS
Varicella-Zoster Virus
28
What damage to nerves occurs in Varicella-Zoster?
1. **Loss of affected ganglia and neurons**: mononuclear infilrate, regional necrosis and hemorrohage 2. **Death of sensory neurons** =\> causes **death of axons of peripheral nerves** 3. Focal destruction of **large motor neurons in anterior horns** or **CN motor nuclei** at affected level
29
**_Lyme Disease_** 1. Causes what damage to peripheral nerves? 2. Clinical symptoms
1. **Polyradiculopathy** 2. **Unilateral/bilateral facial N palsy**
30
**_HIV_** 1. Causes what damage to peripheral nerves? 2. Clinical symptoms
1. **Mononeuritis multiplex** 2. Late stages =\> **demyelinating disorder** that resembles **GB** or **CIDP** 1. _Distal_ _sensory_ neuropathy
31
MCC of peripheral neuropathy
**DB**
32
**MCC** of _symmetrical_ neuropathy that involves _dista***l sensory + motor***_ nerves
**DB**
33
What is the most common pattern of **peripheral neuropathy** seen with **Diabetes**?
**Ascending** distal **symmetric** **sensorimotor** **polyn**europathy
34
Prevalence of peripheral neuropathy associated with **diabetes** is dependent on what?
**_Duration_** of the disease; 80% of ppl with DM for over 15 years
35
Clinical symptoms of Diabetees
1. **Ascending distal symmetric sensorimotor polyneuropathy =\>** 1. Numbing 2. Loss of pain 3. Paresthesias and dysthesias =\> pain 4. Balance 2. **ANS sx:** 1. Postural hypotension 2. Incomplete bladder emptying =\> IC risk of infections
36
**Lesion** in DB
1. **Segmental demyelination** =\> _thin_ myelinated fibers 2. **Loss** of _small_ myelinated and unmyelinated fibers 3. **Endoneurorial arterioles** =\> thick, hyalinzed and PAS +
37
* **Uremic neuropathy** seen in setting of **renal failure** is a causes what type of neuropathy? * Symptoms?
1. **Distal symmetric neuropathy** 2. Asymptomatic in some; or 1. **Muscle cramps** 2. **Distal dysthesias** 3. **DEC DTR**
38
Lesion is Renal failure (uremic syndrome)
1. **Axonal degeneration, t**hen **regeneration** after recovery after dialysis
39
**Thyroid dysfunction** can cause what type of neuropathy?
1. **HypOthyroidism** =\> carpal tunnel compression neuropathy 2. **HypERthyroidism** =\> GB
40
Other toxic neuroapthies
Pb (lead) Chemo
41
Which type of neuropathy is the **most common paraneoplastic syndrome** and which malignancy is it most commonly associated with?
**Small cell lung cancer =\> _Sensorimotor_ neuronopathy**
42
What is a a distinctive presentation of neuropathy associated with **monoclonal gammopathies (B-cell neoplasms)?**
* Paraprotein deposts in between non-compacted myelin lamalle * **POEMS:** Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes
43
**"Saturday night palsy"** is due to compression of which nerve?
**Radial nerve i**n the **upper arm**
44
Which nerve is affected in **Morton neuroma** and what histologic finding is seen?
**- Interdigital nerve** at **intermetatarsal sites** --\> **_metatsalgia_** =\> * =\> ball of foot is **painful/inflamed** and feels like "walking on a marble" - **Histologically** = _perineural fibrosis_
45
What is the **MC inherited peripheral neuropathy,** which should ALWAYS be in a DDX of someone with a peripheral neuoropathy?
**Charcot -Marie Tooth (CMT)**
46
What is **CMT**?
Hereditary demyelination of **_MOTOR_** + **_SENSORY_** nerves that is slowly progressive, presenting in early 20s.
47
What is the **most common subtype** of hereditary motor and sensory neuropathy (CMT) disease, what is the inheritance pattern, when and how does it present?
**CMT1** = _AD_ mutation of _PMP22_ (peripheral myelin protein 22) on _Chr 17._ * -Presents in 20s: w/ slowly progressive **_distal_** demyelinating motor and sensory neuropathy
48
Which structures are injured in the **CMT2** variant of CMT disease and when does it present?
_AD_ mutation of _MDN2_ gene _mitochondrial fusion_ **Axonal injury**; typically severe w/ early childhood onset
49
Clinical Sx of **Charcot Marie Tooth**
1. Presents in **20s** 2. **Peroneal neuropathy =\> clumsy child that has problems walking/runniing**: lower leg atrophy =\> deformed LE (looks like inverted bottle) 3. **Foot deformitie**s: pes cavus and hammer toe 4. **DEC or no DTR** 5. **Foot drop** 6. **EMG = slowing of motor nerve conduction velocity**
50
What is **Familal Amyloid Polyneuropathy?**
**Amyloid deposition** in the peripheran nerve
51
How do **traumatic neuromas** aka **pseudotumors** grow?
**Despite misalignment**, axons contineu to grow into small swirls that are randomly oriented, but surrouned by ORGANIZED layers of Schwaanc cells, fibroblasts and perineural cells

NMSK Exam 3 (17 decks)

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