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MJ- F2 E4- Neuro > Neuromuscular Disorders- MJ > Flashcards

Neuromuscular Disorders- MJ Flashcards

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)–> MC
  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
A

MG

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
Q

What is the 1st line tx for MG?

A

Anticholinesterase inhibitors (Pyridostigmine)

(Slows the degradation of ACh which prolongs its effect)

26
Q

What is a Myasthenic crisis in a patient with MG?

A
  • Respiratory failure from wekness of resp muscles
  • Precipitated by infection
  • (will be on exam)
27
Q

Myasthenia Gravis:

What treatment can be helpful in hastening recovery after a Myasthenic Crisis?

(will be on exam)

A

Plasmapheresis

28
Q

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
A

Lambert-Eaton Myasthenic Syndrome (LEMS)

(very similar to MG- differences are highlighted)

29
Q

Which cancer is the most commonly associated tumor in patients with Lambert-Eaton Myasthenic Syndrome (LEMS)?

(will be on exam)

A

Small cell lung cancer

30
Q

What is the hallmark presentation of LEMS?

A

Postexercise facilitation

(recovery of DTRS or improvement of muscle strength with vigorous movement)

31
Q
  • Which diagnostic study is important in diagnosing LEMS, especially in differentiating it from MG?
  • What will this study show?
A
  • Repetitive Nerve stimulation (RNS)
  • LEMS will have increased amplitude w/ stimulation (MG would be decreased)
32
Q

How do you treat LEMS if there is moderate to severe weakness? (3 options)

A
  1. Amifampridine (enhances Ca entry at the presynaptic terminal)
  2. Guanidine
  3. Pyridostigimine + above Rx
33
Q

LEMS or MG?

Antibodies against the nerve where ACh is released

A

LEMS

34
Q

LEMS or MG?

Antibodies against the muscle receptor for ACh

A

MG

35
Q

LEMS or MG?

  • _________starts at eyes and moves down
  • __________ starts at extremities and moves up
A
  • __\__MG_____starts at eyes and moves down
  • ___\_LEMS___ starts at extremities and moves up
36
Q

LEMS or MG?

  • ________= Weakness improves upon activity
  • ________= Weakness worsens upon activity
A
  • ___LEMS___= Weakness improves upon activity
  • \___MG___= Weakness worsens upon activity
37
Q

LEMS vs. MG:

  • _____ is associated w/ small cell lung cancer
  • _______ is associated w/ thymoma
A
  • \__LEMS___ is associated w/ small cell lung cancer
  • _\_MG_____ is associated w/ thymoma
38
Q

Which neuromuscular disorder?

  • Acute immune-mediated polyneuropathy
  • Provoked by preceding infection by 2-4 weeks (Campylobacter jejuni)
A

Guillain- Barre Syndrome (GBS)

39
Q

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
A

GBS

40
Q

T/F: There is only one form of GBS

A

False. There are several different forms of Guillain-Barre

41
Q

Which GBS variant is MC?

(Variants= Acute motor axonal neuropathy, Acute inflammatory demyelinating polyneuropathy, Acute motor and sensory axonal neuropathy, Miller Fisher syndrome)

A

Acute inflammatory demyelinating polyneuropathy (AIDP)

  • This is progressive symmetric weakness accompainied by absent or depressed DTRs and parasthesias in hands/feet
42
Q

What is the hallmark presentation of GBS?

(will be on exam)

A
  • Progressive symmetric ascending muscle weakness (starts in LE and works its way up)
  • diminished DTRs
43
Q

Which neuromuscular disorder should be considered in any child w/ an acute gait disturbance?

A

GBS

44
Q

T/F: Everyone with GBS can be treated outpatient

A

FALSE

everyone w/ GBS requires hospitalization b/c respiratory failure can develop

45
Q

Which diagnostic test is essential for diagnosis of GBS? What does this show?

(will be on exam)

A
  • LP → CSF studies are essential for diagnosis
  • If GBS, will show elevated protein with normal WBC count
46
Q

What are the 2 most important treatment components for GBS?

A
  • Supportive care in the hospital
  • Plasmapheresis
    *
47
Q

T/F: It is extremely important to give Corticosteroids to patients w/ GBS

A

False

Corticosteroids are not beneficial

48
Q
  • How long after symptom onset does a patient w/ GBS begin to spontaneously recover?
  • Do symptoms ascend or descend as they resolve?
A
  • Spontaneous recovery begins after 3-4 weeks
  • Descends on recovery

MJ- F2 E4- Neuro (19 decks)

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