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Clin Med- Neuro > MS, MG, MD lecture > Flashcards

MS, MG, MD lecture Flashcards

1
Q

Which cells wrap around axons, insulating them

*Form myelin in the PNS

A

Schwann cells

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2
Q

What cells myelinated the CNS?

A

Oligodendrocytes

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3
Q

Form spider-like network in CNS

  • *Facilitate nutrient extraction from blood supply (capillaries)
  • *Disposal of cellular waste products
A

Astrocytes

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4
Q

Exclusive to CNS

Circulate nervous system helping with immunity by DESTROYING BACTERIA OR DEAD CELLS

A

Microglial cells

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5
Q

Line areas of the nervous system that have cerebrospinal fluid, and help circulate the fluid

A

Ependymal cells

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6
Q

Action potentials travel down axon to terminal bulb, where vesicles filled with Ach are released into synaptic cleft via _____ mediated mechanism

A

Calcium

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7
Q

After Ach binds to its receptor, ligand gates Na channels are activated. This allows Na to enter into the….

A

post synaptic membrane

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8
Q

When enough positive ions enter the post synaptic membrane, an excitatory AP causes Ca to be released from the SR, which causes….

A

Muscle contraction

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9
Q

What does Acetylcholinesterase (AchE) do?

A

Hydrolizes (inactivates) Ach

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10
Q

Neutrophils
Basophils
Eosinophils

..examples of?

A

Granulocytes

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11
Q

Antigen presenting cells with epitope (antigen fragment) interacts with…

A

Helper T cells

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12
Q

Autoimmune (?) disease which causes inflammatory demyelination of CNS

*demyelination of axons reduces neurons ability to function

A

Multiple Sclerosis (MS)

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13
Q

Is there a specific auto-antibody found in MS?

A

NO

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14
Q

What is the age of diagnosis in MS?

A

Usually young adult (under 55 at onset)

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15
Q
  • Age of onset usually mid 20s-30
  • women more common than men
  • likely autoimmune, so risk of other autoimmine dz.
  • MC in Northern Europe, Southern Canada, Northern US
A

MS

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16
Q

Less common in areas closer to equator

HLA-DR2? HLA-DRBI association

A

MS

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17
Q

In MS….

Inflammation –> demyelination –> ?

A

Axonal degradation

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18
Q 
Weakness
Fatigue
Numbness, tingling
Unsteadiness in a limb
Spastic paraparesis
Disequilibrium/vertigo
Pain
Sphincter disturbances (urinary urge/hesitancy)
Optic neuritis
A

MS!

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19
Q 
Blurred or diminished vision
Blind spots, esp with central vision
Pain with eye movements
HA
Sudden color blindness
Impaired night vision
Impaired contrast sensitivity
Diplopia
A

Retrobular neuritis/optic neuritis (in MS)

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20
Q
  • Relapses and remission
  • Onset age 15-50
  • Optic neuritis
  • Fatigue
  • Lhermitte’s sign
  • Internuclear ophthalmoplegia
  • Uhthoff’s phenomenon
A

Suggestive features for MS

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21
Q

What is Lhermitte’s sign?

A

Electrical shock extending down the spine, triggered by flexing the head forward

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22
Q

Internuclear ophthalmoplegia is impairment of _________ eye movement

A

horizontal

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23
Q

Uhthoff’s phenomenon is the worsening of neurologic symptoms when…

A

body gets overheated! (i.e. warm weather, exercise, fever, etc)

*generally the optic neuritis and visual symptoms, but can be anything

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24
Q

Can nystagmus be seen in MS pts?

A

YES

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25
Q

For diagnosis of MS…

Must show ____ or more different areas in central areas of white matter affected at different times

A

TWO

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26
Q

What if…..

Pt has multi focal disease on imaging but only 1 clinical attack or if pt has a history of at least 2 clinical attacks but signs of only 1 lesion

A

a probable MS diagnosis can be made

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27
Q

Diagnostic of choice for clinically suspected MS?

A

MRI

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28
Q

Lesion is cerebral or spinal plaque

*region of demyelination and initially a preserved axon

A

MS

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29
Q
  • Periventricular
  • Juxtacortical
  • Infratentorial
  • Spinal cord
A

Locations of MS lesions

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30
Q

Increased Gadolinium enhancement may be indicative of….

A

an acute MS lesion

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31
Q

What labs should be done in a potential MS pt?

A

LUMBAR PUNCTURE!

also want to get things like B12 and lyme to rule out other causes of neuro symptoms

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32
Q
  • Mild lymphocytosis
  • IgG in CSF
  • Most likely normal opening pressure
  • Possible albumin in CSF (indicates disruption of BBB)
  • **Oligoclonal bands
A

Possible findings of lumbar puncture in MS patient

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33
Q

Oligoclonal bands in HIGHLY SUGGESTIVE of….

A

MS

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34
Q

A series of distinct bands found in the immunoglobulin of the CSF

A

Oligoclonal bands

**highly suggestive of MS

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35
Q

A test in which a pt sits before a screen which displays alternative checkerboard patterns

*used to ID impaired transmission along optic nerve pathways

A

Visual evoked response (VER)

*positive in 85% of MS pts

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36
Q
  • Visual Evoked Response
  • Brainstem Auditory Evoked Response
  • Somatosensory Evoked Potentials
  • CSF with oligoclonal banding
  • IgG index of CSF
  • CSF albumin
  • Brain MRI
A

Tests that can be done (and may be positive) in MS patients

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37
Q

Albumin in CSF indicates a disruption of…

A

the Blood Brain Barrier

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38
Q
  1. Relapsing Remitting
  2. Secondary Progressive
  3. Primary Progressive
  4. Progressively Relapsing
A

4 types of MS

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39
Q

An initial episode then months or years before new symptoms emerge or previous symptoms return

A

Relapsing-remitting MS (RRMS)

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40
Q

This cycle can lead to incomplete remissions and progressive disability with:

  • weakness
  • spasticity
  • ataxia of limbs
  • impaired vision
  • urinary incontinence
A

Relapsing-remitting MS (RRMS)

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41
Q

Acute exacerbation of symptoms lasting days to weeks (lasting a minimum of 24 hrs)

A

Relapse

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42
Q

Relapse triggers?

A

Infection
Trauma
Pregnancy

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43
Q

Which type of MS…

Starts out as relapsing remitting (RRMS) but then beings to progress without relapse. disease beings to progress more steadily

A

Secondary progressive MS (SPMS)

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44
Q

Which type of MS…

Symptoms progress from the beginning without relapse/attacks.

A

Primary progressive MS (PPMS)

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45
Q

Which type of MS….

Steady decline from the onset with additional superimposed attacks/relapses

A

Progressive relapsing MS (PRMS)

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46
Q

Is there a cure for MS?

A

NO

tx is aimed at improving quality of life

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47
Q

What do you treat acute MS attacks with?

A

Glucocorticoids (ie Methylprednisone IV for 3-7 days)

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48
Q

Disease modifying therapy is aimed at…

A

Reducing relapses

Slowing progression of disease

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49
Q

Cytokine that modulates immune responsiveness

A

Interferon-B

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50
Q

Interferons and Glatiramer are MS treatments given through….

A

injection

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51
Q

GLATIRAMER* is a mixture of amino acids antigenically similar to…

A

Myelin protein

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52
Q

Fingolimod is the first oral agent approved for MS and works by down regulating receptors and T cell sequestration in….

A

lymphoid tissue

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53
Q

Teriflunomide inhibits mitochondrial enzymes involved in DNA replication.

Teriflunomide also decreases T and B cell proliferation and response to ____________

A

Auto antigens

54
Q

Natalizumab decreases migration of immune cells into CSF, causing fewer….

A

fewer LESIONS ON MRI and fewer RELAPSES!

55
Q

True or false…

Mitoxantrone is cytotoxic?

A

TRUE

56
Q

What type of vaccines should be avoided in MS patients?

A

Live attenuated

57
Q

Rare, autoimmune disease

MC disease of neuromuscular transmission

A

Myasthenia Gravis

58
Q

In myasthenia gravis, there are antibodies found against which type of Ach receptors at the neuromuscular junction?

A

Nicotonic receptors

59
Q

Some antibodies impair ability of Ach to bind to receptors

Some antibodies destroy Ach-receptors

A

Myasthenia gravis

60
Q

Proteins that mediate clustering of Ach-Receptor during neuromuscular junction formation and are associated with Ach-Receptor maintenance

A

MuSK (muscarinic tyrosine kinase)

61
Q

Antibodies are present against MuSK (muscarinic tyrosine kinase)

A

Myasthenia Gravis

62
Q

Antibodies against:

  • Nicotonic receptors at neuromuscular jxn
  • Muscarinic tyrosine kinase (MuSK)
A

Myasthenia Gravis

63
Q

2 possible manifestations of myasthenia gravis?

A
  1. ocular..limited to lids and EOM

2. generalized

64
Q

What percent of those with ocular myasthenia gravis will progress to generalized within 2 years?

A

50%

65
Q

Seen in both men and women
**Occurs at all ages (infant to elders)

Women, more likely to occur during child bearing years

Men, more likely to occur in 6th or 7th decade

A

Myasthenia gravis

66
Q

True or false…

Pregnancy and menstrual periods can exacerbate signs and symptoms of myasthenia gravis?

A

TRUE

67
Q

Genetic predisposition (HLA-DR3) is commonly found in myasthenia gravis patients with…

A

other autoimmune disorders

68
Q

Can there be placental transmission of maternal antibodies of myasthenia gravis?

A

YES.

*myasthenia gravis may be transient in neonates

69
Q

up to 75% of myasthenia gravis patients have ________ abnormalities

A

THYMIC

70
Q

Organ in upper anterior portion of chest cavity, just behind sternum

*main function is to provide area for T lymphocyte maturation

A

Thymus

71
Q
  • Ptosis (uni or bilateral)
  • Diplopia
  • Bulbar muscle weakness (chewing, swallowing, speech)
  • expressionless face
  • neck muscles (head drop)
A

Myasthenia gravis

*commonly associated with facial symptoms!

72
Q

Can myasthenia gravis progress to respiratory muscles and limb weakness?

A

YES

73
Q

Weakness may be focal or generalized

Weakness may fluctuate throughout day (may be worse at night or after exercise)

A

Myasthenia gravis

74
Q

Can remit and relapse

*Symptoms usually progress to become more frequent, may peak years after disease onset

A

Myasthenia gravis

75
Q

Fluctuate of symptoms in myasthenia gravis is helpful to distinguish from…

A

Myopathy and motor neuron diseases

76
Q
  • Fatigability of muscles
  • NORMAL sensation and reflexes!!!
  • May have frank ptosis, slack jaw
  • Positive Simpson Test
  • Positive Cogan Lid Twitch Sign
A

Myasthenia gravis physical exam

77
Q

Test where you…

look down, then up quickly
lid will lag

A

Simpson test

*positive in Myasthenia Gravis patients

78
Q

Test where you…

have prolonged downward gaze
look up, and upper eyelid twitches

A

Cogan Lid Twitch sign

*positive in Myasthenia Gravis patients

79
Q

The Tensilon test (endrophonium) can confirm a diagnosis of…

A

Myasthenia gravis

80
Q

Short acting AchE inhibitor

*works by inhibiting the enzyme that breaks down Ach

A

Tensilon test

81
Q

What are 2 contraindications of the Tensilon test when trying to diagnose Myasthenia Gravis

A

Cardiac disease

Asthma

82
Q

Increase in salivation
Abdominal cramping
Symptomatic bradycardia
Bronchospams

A

Potential side effects of Tensilon test

83
Q

What must you keep bedside during a Tensilon test incase the pt develops symptomatic bradycardia or bronchospasm?

A

Atropine

84
Q

Must be administered incrementally!

2 mg every 1 minute, with a max of 10 mg

A

Tensilon test (for diagnosing Myasthenia Gravis)

85
Q

In a patient with Myasthenia Gravis, what will the Tensilon test do?

A

Will improve muscle weakness

ie..if the pt has severe ptosis, it will improve during the test

86
Q

Neuromuscular transmission is improved at ______ temps

A

cooler

87
Q

The ice pack test can help diagnose…

A

Myasthenia Gravis

88
Q

The Ice pack test is not helpful in muscles that cannot be cooled, such as….

A

EOM (extra ocular)

89
Q

Eyelid muscles can easily be cooled! During the icepack test you place an icepack on them for…

A

2 minutes

90
Q

Can ptosis improve immediately after removal of ice pack?

A

YES

91
Q

What is the sensitivity of the ice pack test? Can there be false positives?

A

80% sensitivity

Yes, there can be false positives

92
Q

Which three antibodies can be seen (but not always) in myasthenia gravis?

A

Antibodies against:
nAchR
MuSK
anti striated muscle

93
Q

Which antibody is present in 80-90% of generalized MG

  • 3 types: binding, blocking, modulating
  • highly specific, almost no false positives
A

Antibody against nAchR

94
Q

Which antibody is seen in circulation of 3-7% of MG patients?

A

Antibody against MuSK

95
Q

Do levels of circulating antibodies correlate with severity of dz?

A

NO

96
Q

Which antibody is seen in 30% of MG patients, and 80% of MG patients who have thymoma

A

Antibody against striated muscle

97
Q

Can patients with MG be seronegative (without antibodies against nACHR or MuSK)?

A

Yes!

these pts have similar response to treatment and similar findings on electrophysiologic testing

98
Q

True or false…

Most MG patients have thymic abnormalities , especially those with AchR-antibodies

A

TRUE

99
Q

Hyperplasia of thymus or thymoma

A

Thymic abnormalities that can be seen in MG

100
Q

nAchR-antibodies
MuSK antibodies
Anti-striated muscle antibodies

A

3 types of antibodies that can be seen in MG

101
Q
  1. repetitive nerve stimulation studies

2. single fiber EMG

A

2 types of electrophysiologic testing in MG

102
Q

3 things used in diagnosing myasthenia gravis

A
  1. labs (Abs against nAchR, MuSK, anti striated muscle)
  2. thymus imaging
  3. electrophysiologic testing
103
Q

What is the use of Penicillamine and what is the importance with myasthenia gravis?

A

Penicillamine is an immunosuppressant used to treat RA

*it is associated with myasthenic syndrome and can progress to myasthenia gravis

104
Q

What drug can potentially cause myasthenia gravis (but symptoms usually resolve when DC’d)

A

Penicillamine

105
Q
  • AchE inhibitors
  • Immunomodulation
  • Surgery (esp if thymoma)
  • IV-Ig
  • Plasmapheresis (plasma exchange)
A

Txs for MG

106
Q

Respiratory weakness may lead to respiratory failure

can be precipitated by: 
Surgery
Infections
Meds
Tapering of immunosuppression
A

Myasthenia Gravis Crisis

worsening generalized weakness may precede crisis

107
Q 
Admit to ICU!!
May need to intubate
Withdraw AchE inhibitors 
IV Ig
High dose ummunomodulating therapy
A

Myasthenia Gravis Crisis

108
Q

What classification of MG…

Any ocular weakness, all other muscle strength normal

A

Class I

109
Q

What classification of MG…

MILD weakness affecting muscles other than ocular muscles (may also have ocular muscle weakness)

A

Class II

110
Q

What classification of MG….

MODERATE weakness affecting muscles other than ocular muscles

A

Class III

111
Q

What classification of MG…

Intubation with or without mechanical ventilation

A

Class V

112
Q

What classification of MG

SEVERE weakness affecting muscles other than ocular muscles

A

Class IV

113
Q

Inherited disorders causing progressive muscle weakness and atrophy due to genetic defect

*multiple types

A

Muscular dystrophy

114
Q

Muscular dystrophies are disorders of the…..

A

muscles

115
Q

Nerve sends signal to muscle, muscle is unable to respond in __________

A

myopathies

116
Q

Defect gene on X chromosome
Lack of dystrophin (a protein that protects muscles)
Occurs in males

A

Duchenne Muscular Dystrophy (DMD)

117
Q

Age of onset: 2-3 yo
Wheelchair by ~age 12
Survival into late teens/20s (usually pass away due to respiratory infections, cardiomyopathy)

A

Duchenne Muscular Dystrophy (DMD)

118
Q

Which type of muscular dystrophy DOES make some dystrophin

  • Enzymatic muscle breakdown
  • Later onset, milder symptoms
  • Survival into 40s, cardiomyopathy less common
A

Becker Muscular Dystrophy (BMD)

119
Q

Which 2 types of MD…

Occurs in males
Asymptomatic female carriers (or mild dz)
Increase in CK
Starts centrally/trunk, moves to legs first

Tx with corticosteroids will increase strength but will NOT change prognosis

A

DMD and BMD

120
Q

Cardiomyopathy
Scoliosis
Fractures
Cognitive impairments

A

Duchenne Muscular Dystrophy (DMD)

121
Q

Dx made with:

Weakness on exam
Family hx
CK, AST, ALT
Genetic testing
EMG
Muscle biopsy
A

DMD and BMD

122
Q

Tx:

Steroids
Ca/Vit D
ACE inhibitor
Beta blockers
Pacemaker/defib
Pulm support
Immunizations
A

DMD and BMD

123
Q

Caused by number of different inheritance patterns
*Seen in MALES and FEMALES

Weakness usually begins in ARMS in teen years, then progresses to legs and face

A

Emery-Dreifuss Muscular Dystrophy (EDMD)

124
Q
  1. contractors of elbows, ankle, plantar flexors and spine EARLY
  2. later onset of humeroperoneal weakness
  3. cardiac abnormalities (arrhythmias, cardiomyopathies)
A

Classic triad of EDMD

125
Q

Uncommon but distinctive
Weakness usually being in ARMS in teen years

Dx:
CK normal or slightly elevated
EMG
Genetic testing
**Often still ambulatory 20 years after dx***
A

Emery Dreifuss Muscular Dystrophy (EDMD)

126
Q

MC form of muscular dystrophy in whites
affects MALES and FEMALES
has 2 genetic subtypes

A

Myotonic dystrophy

127
Q

Muscle stiffness
Inability to relax muscle after contraction
Muscle loss and weakness: face, arms, legs
Cardiac complications
Cataracts
Change in intellectual fxn
Excessive daytime somnolence

A

Myotonic dystrophy

128
Q

Multiple subtypes

  • affects shoulder girdle and or hip girdle
  • dz progressive is SLOW

Tx= stretching to prevent contractures

A

Limb-Girdle Muscular Dystrophy

129
Q

Females and males
autosomal dominant

Reversal of anterior axillary folds
Scapular winging
Decreased hearing
Myotonic EMG pattern

A

Fascioscapulohumeral muscular dystrophy (FSHMD)

130
Q 
Ptosis
Dysphagia
Leg weakness
Tongue atrophy
Fascial muscle weakness
A

Oropharyngeal muscular dystrophy

131
Q

“floppy baby”

seen right at birth

A

Congenital muscular dystrophy

132
Q

MS image of choice?

A

MRI!!

Clin Med- Neuro (10 decks)

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