Motor disorders/ Demyelinating diseases Flashcards

(71 cards)

1
Q

Acute onset motor disorders can be what

A

vascular vs toxic/metabolic

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2
Q

Subactue onset motor disorders are what

A
  • neoplastic
  • infectious
  • inflammatory
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3
Q

Chronic onset motor disorders are what

A
  • neoplastic
  • hereditary
  • endocrine
  • degenerative
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4
Q

Disorders of what are not accompanied by altered sensation

A
  • anterior horn cells
  • neuromuscular junction
  • muscles
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5
Q

Myasthenia gravis

A

neuromuscular disorder characterized by weakness and fatigability of skeletal muscles

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6
Q

Defect in MG

A

decrease in the number of available AChRs at neuromuscular junctions d/t anti-body mediated autoimmune attack

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7
Q

Is ACh normally released in MG

A

YES but the decreased number of receptors causes failure to trigger muscle action potentials

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8
Q

What plays a role in the autoimmune response of MG

A

thymus

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9
Q

Peak incidence of MG

A

women in 20s and 30s

men in their 50s and 60s

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10
Q

Cardinal features of MG

A

weakness and fatigability of muscles

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11
Q

What can “unmask” MG

A
  • menses
  • pregnancy
  • antibiotics
  • CCB
  • phenytoin
  • lithium

exacerbates symptoms

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12
Q

What muscles are involved early in MG

A

cranial muscles, particularly lids and extraocular muscles

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13
Q

First presenting complaints on MG

A
  • diplopia

- ptosis

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14
Q

What is especially prominent in MuSK antibody- positive MG

A

bulbar weakness

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15
Q

If weakness remains restricted to the extraocular muscles for 3 years—>

A

it will most likely not progress to the limbs (ocular MG)

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16
Q

Are deep tendon reflexes presevered in MG

A

YES

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17
Q

Proximal or distal muscle weakness in MG?

A

mostly proximal

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18
Q

Sx’s of MG

A
  • diplopia
  • ptosis
  • dysarthria
  • lower extremity weakness
  • generalized weakness
  • dysphagia
  • upper extremity weakness
  • masticatory weakness
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19
Q

PE for MG

A
  • quantitative testing of muscle strength
  • forward arm abduction time
  • vital capacity (breathing)
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20
Q

Labs for MG

A
  • anti AChR radioimunoassay (neg doesnt not r/o MG)
  • repetitive nerve stimulation
  • single fiber electromyography
  • Edrophonium chloride
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21
Q

Mainstay of MG treatment

A

Pyridostigmine

30-120mg every 4 hrs

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22
Q

What can overmedication in MG cause

A

increase in weakness that will not be effected by edrophonium

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23
Q

When would you use a long acting anticholinesterase drug for MG?

A

used at bedtime for pts with persistent severe weakness upon awakening

*Mestinon

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24
Q

Cholinergic crisis

A
  • pallor
  • sweating
  • nausea
  • vomiting
  • salivation
  • colicky abd pain
  • miosis
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25
Glycopyrrolate, propantheline, or hyscyamine are what? What do they cause?
anticholinergic drugs | decrease bowel motility and salivation
26
When should a thymectomy be used for treatment of MG
in all pts with thymoma - pts younger than 60 - pts older than 60 with weakness not restricted to extraocular muscles
27
When is Azthioprine used
severe of progressive disease despite thymectomy and tx w/ AChIs and corticosteriods
28
How long does it make azithioprine to work
up to 1 year
29
When is plasmapheresis used for MG
in pts that are deteriorating rapidly or in crisis special circumstances--> before surgery that will produce respiratory compromise
30
Instead of plasmapheresis what could you use in a sick pt with MG
IV immunoglobulins
31
Mycophenolate
inhibits proliferation of T and B cells in pts with MG which allows steroid dose to be lowered
32
Cerebral palsy
chronic static impairment of muscle tone, strength, coordination, movements that happens around birth
33
Signs and symptoms of CP
- spasticity of the limbs - ataxia - dyskinetic movement disorder - persistent hypotonia without spacitiy
34
What other neurological diseases are associated with CP
- seizures - mild/severe retardation - varying degrees of language, vision and sensory disorders
35
PE findings with CP
- spasticity - hyperreflexia - ataxia - involuntary movements - microcephaly - hemiplegia-->affected limb smaller - cataracts, retinopathy
36
Treatment of CP
- PT, OT, ST - botulism/meds --> spasticity of medication - tx of seizures can improve with age if mild
37
Mean age of onset of Tourettes
5 years old, males more than females
38
What is a Tic
sudden, brief, uncontrollable repetitive, non rhythmic, stereotyped, purposeless movements or vocalizations
39
What can precede tics
premonitory sensations or unpleasant somatosensory sx
40
What can trigger tourettes or make it worse
- worsened by anxiety or excitement | - can be triggered by physical experiences
41
Do tics go away while sleeping
no
42
Diagnosing tourettes
vocal and motor tics for 1 year MRI or EEG to r/o other conditions
43
Tx of tourettes
no real treatment - can use neuroleptics to suppress tics - alpha adrenergic agonists - ADHD Rx - behavioral treatments
44
Multiple sclerosis
autoimmune disorder in which the body attacks and destroys the fatty tissues that insulate nerves/axon
45
What part of the NS does MS affects
central NS and causes inflammation of white matter in the brain that forms plaques
46
MS is a ___ disease
demyelinating
47
Myelin is rich in what
lipid and proteins
48
Symptoms of MS
- fatigue - depression - memory changes - pain, spascitiy - vertigo, dizziness - tremor - double vision/vision loss - weakness - numbness/tingling, ataxia
49
What causes remission of MS
remodeling of the demyelinated axonal plasma membrane allowing for more sodium channels to permit conduction despite myelin loss
50
Diagnosis of MS
- need to have 2 episodes of symptoms that occur in different points in time - absence of other treatable causes for the sx's - LP, MRI - blood tests - evoked potentials
51
What do evoked potentials detect
slow or abnormal conduction in response to visual or auditory stimuli
52
LP in MS
mild pleocytosis and mild elevated total protein
53
What lab finding can be found in patients with MS
IgG
54
Primary diagnostic tool for MS
MRI (look for plaques)
55
Disease modifying rx for MS
- interferon beta 1a - interferon beta 1b - glatiramer acetate *used for relapsed remitting MS
56
Side effects of interferon
flu like symptoms, skin irritation
57
Side effects of glatiramer acetate
skin erythema, rash, dyspnea, transient chest pain
58
Tx for secondary progressive MS
INF-B1b and mitoxantrone
59
When is mitoxantrone bad
if a patient has a low EF, Cardiotoxic!
60
What is given for an MG flare
- plasma exhange | - steriods
61
Tx for systemic spasticity in MS
- baclofen | - cyclobenzaprine
62
Tx for optic neuritis in Ms
- methylprednisolone | - oral corticosteriods
63
Tx for tremor in MS
- propranolol | - primidone
64
Guillain-Barre syndrome is
an acute inflammatory demyelinating polyneuopathy
65
Precipitating factors for Guillain-Barre
- bacteria - CMV - HIB - EBV - surgery - trauma - malignancy
66
Most common infection causing Guillain-Barre in the US
campylobacter jejuni
67
Two subtypes of GBS
- antibody injury to myelin sheath | - antibody injures axonal membrane
68
Sx of GBS due to Zika
- symmetric muscle weakness in lower limbs - incapacity to walk - areflexia - facial palsy - trouble swallowing - paresthesia
69
What features need to be present for diagnosis of GBS
- progressive weakness in legs and arms | - areflexia in weak limbs
70
Diagnostics for GBS
- LP | - nerve conduction studies
71
Tx for GBS
- IV IG | - plasmapheresis