Neuromuscular Disorders- MJ Flashcards

(48 cards)

1
Q

Which neuromuscular disorder?

•Immune-mediated (autoimmune) disorder of the CNS associated with destruction of myelin and nerve fibers.

A

Multiple Sclerosis

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2
Q
  • Is MS more common in men or women?
  • Age of onset?
A
  • Women
  • 15-50yrs
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3
Q

Is there a higher or lower incidence of MS as you move towards the equator?

A

lower incidence

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4
Q

On LP, what is seen in the CSF only in MS patients and can be used as a diagnostic marker?

(this will be on exam)

A

Oligoclonal bands

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5
Q

What are the 3 types of MS? Which one is most common?

(this will be on exam)

A
  1. Relapsing-remitting MS (RRMS)- Most common
  2. Secondary progressive MS (SPMS)
  3. Primary progressive MS (PPMSP)
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6
Q

The following describes which type of MS?

•Unpredictable attacks which may or may not leave permanent deficits followed by periods of remission

A

Relapsing-remitting MS

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7
Q

The following describes which type of MS?

  • Initial relapsing-remitting MS followed by gradual worsening with or without occasional relapses, minor remissions, and plateaus
  • Occurs 10-20 years after disease onset
A

Secondary Progressive MS

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8
Q

The following describes which type of MS?

•Steady increase in disability without attacks

A

Primary progressive MS

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9
Q

What are the 5 main signs and sxs of MS?

(will be on exam)

A
  1. Internuclear ophthalmoplegia (delayed adduction and horizontal nystagmus of abducting eye)
  2. Optic neuritis (painful, monocular vision loss w/ blurring/scotoma)

3. Lhermitte’s sign (electic shock when flex neck)

  1. Uhthoff phenomenon (heat sensitivity)
  2. Fatigue (sleep 22hrs/day, very difficult to wake up)
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10
Q

T/F: Signs and sxs of MS can be isolated or in combination. Attacks usually last days to weeks

A

True

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11
Q

What are the 2 main components of the MS work up?

A
  1. Brain and spinal cord MRI (gadolinium enhanced)
  2. LP- look for _oligoclonal bands in CSF ***_
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12
Q

What are the 3 parts of the McDonald criteria for diagnosing MS?

A
  1. Uses MRI +/- CSF to diagnose
  2. Dissemination in space
  3. Dissemination in time
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13
Q

How do you treat acute exacerbations/attacks of MS?

(will be on exam

A

IV glucocorticoids- high dose Methylprednisolone

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14
Q

What is one of the MC disease modifying therapies (for RRMS)

A

Interferon

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15
Q

Which neuromuscular disorder?

  • Variable combo of weakness due to antibody-mediated attack against muscle receptor for acetylcholine
  • fatigable weakness
A

Myasthenia Gravis

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16
Q

Epidemiology of which neuromuscular disorder?

  • Bimodal distribution: F: 2-3rd decade, M: 6-8th decade
  • W > M
  • Thymoma common
A

Myasthenia Gravis

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17
Q

Which muscle receptor antibody is most common in MG- AChR receptor Ab or MuSK receptor Ab?

A

AChR receptor Ab

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18
Q

In MG, when does weakness increase and when does it improve?

A
  • Increases during repetitive use and as day progresses
  • Improves during rest/sleep
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19
Q

What are the 2 types of MG and which one is more common?

A
  1. Ocular (weakness limited to eyelids and extraocular muscles)
  2. Generalized (weakness of ocular mm., bulbar, limb, and resp mm.)
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20
Q

What 3 initial presentation symptoms are most commonly seen in MG?

(will be on exam)

A

1. Ptosis

2. Diplopia

3. Blurred vision

21
Q

The following are the second most common sxs of which neuromuscular disorder?

  • Drooling
  • Fatigable chewing
  • Facial weakness
  • Dysarthria
  • Dysphagia
22
Q

How is MG diagnosed? (5 things)

A
  • Based on hx and typical exam findings
  • Repetitive nerve stimulation
  • Single-fiber electromyography
  • Serologic testign: AChR-Ab, MuSK-Ab

(will be on exam)

23
Q

In comparison to LEMS, what does Repetitive nerve stimulation (RNS) show in MG?

A

In MG, amplitude decreases over time with the more mm that are stimulated

24
Q

What are the 4 possible treatment options for MG?

A
  1. Symptomatic tx- Anticholinesterase agents
  2. Chronic tx- Glucocorticoids (prednisone and other immunosuppresive drugs)
  3. Rapid tx- plasmapheresis and IVIG
  4. Thymectomy (if indicated)
25
What is the 1st line tx for MG?
Anticholinesterase inhibitors (**Pyridostigmine**) (Slows the degradation of ACh which prolongs its effect)
26
What is a Myasthenic crisis in a patient with MG?
* Respiratory failure from wekness of resp muscles * **_Precipitated by infection_** * (will be on exam)
27
Myasthenia Gravis: What treatment can be helpful in hastening recovery after a Myasthenic Crisis? (will be on exam)
**_Plasmapheresis_**
28
**Which neuromuscular disorder?** * Autoimmune dz where **reduced ACh is released** from the presynaptic nerve terminals * Proximal muscles of lower **limbs MC affected** * **Ocular involvement not common**
**Lambert-Eaton Myasthenic Syndrome** (LEMS) (very similar to MG- differences are highlighted)
29
Which cancer is the **most commonly associated tumor** in patients with Lambert-Eaton Myasthenic Syndrome (LEMS)? (will be on exam)
**Small cell lung cancer**
30
What is the hallmark presentation of LEMS?
**_Postexercise facilitation_** (recovery of DTRS or improvement of muscle strength with vigorous movement)
31
* Which diagnostic study is important in diagnosing LEMS, especially in differentiating it from MG? * What will this study show?
* Repetitive Nerve stimulation (RNS) * LEMS will have **_increased amplitud_**e w/ stimulation (MG would be decreased)
32
How do you treat LEMS if there is moderate to severe weakness? (3 options)
1. **Amifampridine** (enhances Ca entry at the presynaptic terminal) 2. Guanidine 3. Pyridostigimine + above Rx
33
LEMS or MG? Antibodies against the **nerve** where ACh is released
LEMS
34
LEMS or MG? Antibodies against the **muscle recepto**r for ACh
MG
35
LEMS or MG? * \_\_\_\_\_\_\_\_\_starts at eyes and moves down * __________ starts at extremities and moves up
* \_\_\__**\_MG\_**_\_\_\_\_starts at eyes and moves down * \_\_\_\__**LEMS\_**_\_\_ starts at extremities and moves up
36
LEMS or MG? * \_\_\_\_\_\_\_\_= Weakness **improves** upon activity * \_\_\_\_\_\_\_\_= Weakness **worsens** upon activity
* \_\_\_**_LEMS_**\_\_\_= Weakness **improves** upon activity * \__**\_\_MG**_\_\_\_= Weakness **worsens** upon activity
37
LEMS vs. MG: * _____ is associated w/ **small cell lung cancer** * _______ is associated w/ **thymoma**
* \__**\_LEMS\_**_\_\_ is associated w/ **small cell lung cancer** * \_\__**MG\_**_\_\_\_\_ is associated w/ **thymoma**
38
**Which neuromuscular disorder?** * Acute immune-mediated polyneuropathy * Provoked by preceding infection by 2-4 weeks (**_Campylobacter jejuni)_**
Guillain- Barre Syndrome (GBS)
39
**The following is the pathophys behind which neuromuscular disorder?** * infection--\> immune response--\> antibody production--\> Ab attaches to peripheral nerve cells--\> **molecular mimicry**--\> macrophages attack peripheral nerves--\> **acute polyneuropathy**
GBS
40
T/F: There is only one form of GBS
False. There are several different forms of Guillain-Barre
41
**Which GBS variant is MC?** (Variants= Acute motor axonal neuropathy, Acute inflammatory demyelinating polyneuropathy, Acute motor and sensory axonal neuropathy, Miller Fisher syndrome)
**Acute inflammatory demyelinating polyneuropathy (AIDP)** * This is progressive symmetric weakness accompainied by absent or depressed DTRs and parasthesias in hands/feet
42
What is the hallmark presentation of GBS? (will be on exam)
* Progressive **_symmetric ascending muscle weakness_** (starts in LE and works its way up) * diminished DTRs
43
Which neuromuscular disorder should be considered in any child w/ an acute gait disturbance?
GBS
44
T/F: Everyone with GBS can be treated outpatient
FALSE everyone w/ GBS requires hospitalization b/c respiratory failure can develop
45
Which diagnostic test is essential for diagnosis of GBS? What does this show? (will be on exam)
* LP → CSF studies are essential for diagnosis * If GBS, will show **_elevated protein with normal WBC count_**
46
What are the 2 most important treatment components for GBS?
* **Supportive care** in the hospital * **_Plasmapheresis_** *
47
T/F: It is extremely important to give Corticosteroids to patients w/ GBS
False Corticosteroids are not beneficial
48
* How long after symptom onset does a patient w/ GBS begin to spontaneously recover? * Do symptoms ascend or descend as they resolve?
* Spontaneous recovery begins after **3-4 weeks** * **Descends** on recovery