Weakness Flashcards

(82 cards)

1
Q

What is the most common immune mediated inflammatory demyelinating disease of the CNS?

A

Multiple Sclerosis

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2
Q

T/F MS has the largest incidence in young adults?

A

28-31 yrs

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3
Q

What is the patho of MS?

A

auto-reactive lymphocytes- inflammatory T cells, B cells, and macrophages are typically seen on
histopathologic examination of MS lesions

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4
Q

What is the characteristic neuropathologic feature of MS?

A

Focal demyelinated PLAQUES with inflammation, gliosis, and axonal damage within the CNS

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5
Q

What is the role of macrophages?

A

pick up and degrade myelin debris

Myelin fragments > protein and lipids

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6
Q

Inactive plaque is seen in what stage?

A

Burned out stage

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7
Q

What is seen in the burned out stage?

A
  • Gliosis develops

- Demyelinated axons traversing glial scar tissue

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8
Q

What symptoms occur with MS?

A

all depends on where the plaque forms

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9
Q

What is Lhemitte sign?

A

electric shock like sensation that runs down the back with flexion of the neck

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10
Q

What disease is Marcus Gunn pupil seen in?

A

Multiple Sclerosis

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11
Q

What disease is Relative afferent pupillary defect (RAPD) seen in ?

A

Multiple Sclerosis

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12
Q

What is Marcus Gunn pupil?

A

In a dark room, consensual response of the affected eye will appear to dilate

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13
Q

What is internuclear ophthalmoplegia seen in?

A

Multiple Sclerosis

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14
Q

What is internuclear ophthalmoplegia?

A

injury of the right medial longitudinal fasciculus causing the affected eye to stay in the middle

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15
Q

“Pt says they have double vision or blurred vision, making them dizzy when they look left”

A

Internuclear ophthalmoplegia (MS)

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16
Q

Relapse of exacerbation

A

MS attack

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17
Q

What qualifies as a MS attack?

A
  • current or historical LASTING 24hrs

- CANNOT have fever or infection

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18
Q

What are 3 most common initial attacks?

A
  • Sensory disturbances
  • Motor weakness
  • Visual complaints
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19
Q

How is a MS attack confirmed?

A

MRI
Visual evoked response
Neuro exam

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20
Q

What is the most common form of MS?

A

Relapsing-remitting

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21
Q

What happens during relapsing-remitting?

A

reactivation of old plaques or from the formation of new ones

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22
Q

What occurs after the pt has gone through relapsing-remitting?

A

Secondary progressive (steady deterioration)

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23
Q

What is the least common form of MS?

A

Primary progressive

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24
Q

Primary progressive

A

symptoms steadily progress from the onset, leading to early disability

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25
Relapsing remitting?
symptoms present for days to weeks, then asymptomatic for mo to yrs after 1st presentation
26
What criteria is used fo MS?
McDonald
27
What is dissemination in space?
two or more different regions of the central white matter affected
28
What is dissemination in time?
attacks at different times
29
What can be used as evidence of dissemination in time?
MRI
30
What criteria for Clinically isolated Syndrome (CIS)?
- follow closely @ risk for MS | - Repeat MRI in 6-12 mo
31
What is the DOC for MS?
MRI of brain and/or spinal cord
32
What is the clinical diagnosis for MS?
McDonald criteria | MRI
33
What lab can be used for MS?
CSF evaluation- elevated IgG
34
Best results achieved of txt in MS?
- Combining drug therapies - Rehabilitation - Adjusting pts lifestyle
35
T/F Half of patients HAVE significant disability 10 yrs after first symptoms?
FALSE, they don't
36
Txt for acute exacerbations?
IV methylprednisolone for 5d w/o taper
37
Txt for reduction of attack frequency?
Interferon beta 1b injection Dimethyl fumarate (Tecidera) oral
38
Txt for exacerbations while on interferon?
Natalizumab (Tysabri) | Alemtuzumab (Lemtrada)
39
Txt for severe or progressive dx?
Immunosuppressive thx | Plasmapheresis
40
When is plasmapheresis used?
severe relapses unresponsive to steriods
41
Symptom management in MS for?
fatigue, spasticity, gait, disturbance, depression
42
Life is reduced by ? in MS?
7-14 yrs
43
50% of deaths directly related to MS ??
complications
44
What is the most common cause of acute neuromuscular paralysis
Guillain Barre Syndrome
45
A symmetric, ascending and paralyzing illness caused by autoimmune inflammation of peripheral nerves
Guillain Barre syndrome
46
What is Guillain Barre often preceded by?
infection (URI, GI-symptoms resolved)
47
What bacteria is most commonly associated with Guillain Barre
Camphylobacter jejuni enteritis
48
What is GBS patho?
unknown
49
What is the clinical presentation of GBS?
- fine paresthesias in toes/fingers - Progressive lower extremity symmetric or asymmetric weakness - ASCENDING weakness - Gait disturbance
50
What is the first noticeable symptom of MS?
Gait disturbance | preschooler refuse to walk
51
What are the classic physical exam of GBS?
- Symmetric weakness - Diminished or absent reflexes - Minimal sensation loss
52
T/F Symptomatic nadir for 90% of pts is within 2-4 wks of symptom onset
True
53
What are the 2 mandatory diagnostics for all suspected GBS?
- Lumbar puncture | - Neurophysiology studies
54
In GBS, CSF will be?
elevated protein w/ NORMAL WBC and albuminocytological dissociation
55
What is the most specific and sensitive test for GBS diagnosis?
Electrodiagnostic studies
56
What are 4 Differentials for GBS?
- Poliomyelitis - Botulism - Tick paralysis - Focal cord lesion
57
Txt for GBS?
Vigilant supportive care including psychological support
58
What is plasmapheresis?
removes circulating antibodies
59
What does IVIG do?
neutralizes antibodies/ inflammation
60
T/F Combining IVIG and plasmapheresis is beneficial?
FALSE
61
T/F Glucocorticoid txt is recommended?
FALSE
62
TXT of GBS should be started... when?
Nonambulatory adult pt within 4 wks of onset Ambulatory adult pt not yet recovering within 4 wks of onset
63
How long does it take a adult to recovery from GBS?
85 day | Children 43-52 dy
64
Most common disorder of neuromuscular transmission?
Myasthenia Gravis
65
MG is a ?
autoimmune disorder
66
What happens in MG?
autoantibodies attack the acetylcholine receptor (AChR) fixing complement resulting in reduction of the # of AChR over time (Seropositive)
67
When does Myasthenia Gravis improve or disappear after?
Thymectomy
68
What is the most common form of MG?
70% of pts have antibodies against the muscle specific receptor tyrosine kinase (MuSK) (Seronegative)
69
What most common cardinal feature of MG?
Fluctuating skeletal muscle weakness (true muscle fatigue)
70
2 clinical forms of MG?
Ocular | Generalized
71
Diagnosis of MG?
Complete history and physical!
72
How is MG diagnosed?
pts have fatiguability and weakness of muscles plus abnormal diagnostic tests
73
Hallmark of Myasthenia Gravis?
fluctuating weakness on physical exam
74
Bedside test for ptosis?
Ice pack test- improved neuromuscular @ low temps
75
What is seen in labs for Myasthenia Gravis?
MuSK antibodies | AChR antibodies
76
1st line symptomatic therapy for MG?
oral anticholinesterase | Pyridostigmine (Mestinon)
77
2nd line symptomatic therapy for MG?
chronic immunosuppressive agents Azathioprine (Imuran) Oral glucocorticoid
78
What should be watched for when given 1st line txt for MG?
cholinergic crisis (due to excessive dosing)
79
What are two rapid txt for MG?
``` Plasmapheresis IVIG (intravenous immune globulin) ```
80
What must be sufficiently controlled first before a thymectomy?
Weakness
81
In Lambert-Eaton Syndrome (Myasthenic Syndrome) weakness of proximal limb muscles..
improves w/ activity (power increases w/ sustained contraction)
82
What is botulism?
Clostridium botulinum prevents release of Ach at the neuromuscular junctions and autonomic synapses