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Master the boards: Neurology > Neuromuscular Disorders > Flashcards

Neuromuscular Disorders Flashcards

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1
Q

What is Amyotrophic Lateral Sclerosis (ALS)?

A

Loss exclusively of upper and lower motor neurons

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2
Q

How does ALS commonly present?

A

Weakness of unclear etiology starting age 20-40: with a unique combination of upper an lower motor neuron loss

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3
Q

What is the most serious common symptom of ALS?

A

Difficulty in chewing and swallowing with a decreased gag reflex
-pooling of saliva in the pharynx and aspiration

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4
Q

What are poor prognostic signs in ALS?

A

Weak cough and loss of swallowing

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5
Q

How do Upper Motor Neurons signs present in ALS?

A

Weakness
Spasticity
Hyperreflexia
Extensor Plantar Responses

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6
Q

How do Lower Motor Neuron Signs present in ALS?

A

Weakness
Wasting
Fasciculations

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7
Q

How is ALS diagnosed?

A

Electromyography: loss of neural innervation in multiple muscle groups

Elevated CPK

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8
Q

How do you treat ALS?

A

Riluzole: reduce glutamate buildup in neurons

Edaravone: antioxidant: helps relieve oxidative stress that may damage neurons

baclofen: help spasticity

CPAP and BiPAP: help respiratory difficulites

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9
Q

What is the role of Riluzole in ALS?

A

Reduces glutamate buildup in neurons

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10
Q

What is the role of Edaravone in ALS?

A

Antioxidant: help oxidative stress believed to play a role in neuronal killing

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11
Q

What is Pseudobulbar Affect?

A

Condition of emotional lability or emotional incontinence with intermittent episodes of inappropriate laughter or crying

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12
Q

What condition is Pseudobulbar affect highly associated with?

A

ALS
-can also be causes by MS and stroke

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13
Q

How do you treat Pseudobulbar affect?

A

Dextromethorphan with Quinidine

SSRI help in some patients

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14
Q

What is Chartoc-Marie-Tooth-Disease?

A

Genetic disorder with loss of both motor and sensory innervation

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15
Q

How does Chartoc-Mario-Tooth-Disease present?

A

Distal weakness and sensory loss (Foot Drop)
Wasting in the legs
Decreased Deep tendon reflexes
Tremor

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16
Q

What foot deformity is common in Chartoc-Marie-Tooth Disease?

A

Pes Cavus: high arch

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17
Q

What is the most accurate test for Charcot-Marie-Tooth Disease?

A

Electromyography

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18
Q

What ist he most common cause of Peripheral Neuropathy?

A

Diabetes Mellitus

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19
Q

What are common causes of Ulnar Neuropathy?

A

Biking, pressure on palm of hands, trauma to the medial side of the elbow

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20
Q

How does Ulnar Neuropathy present?

A

Wasting of hypothenar eminence, pain in 4th and 5th fingers

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21
Q

What are common causes of Radial Neuropathy?

A

Pressure of inner, upper arm: falling asleep with arm over the back of chair (Saturday night palsy), Crutches with pressure toaxilla

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22
Q

How does Radial Neuropathy present?

A

Wrist drop

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23
Q

What are common causes of Lateral Cutaneous Neuropathy of the thigh?

A

Obesity, pregnancy, sitting with legs crossed

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24
Q

How does Lateral Cutaneous Neuropathy of the thigh present?

A

Pain/numbness of the outer aspect of one thigh

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25
What is a common cause of Tarsal Tunnel Neuropathy (Tibial Nerve)?
Worse with walking
26
How does Tarsal Tunnel Neuropathy (Tibial Nerve) Present?
pain/numbness in ankle and sole of foot
27
What are common causes of Peroneal Neuropathy?
High boots, pressure on the back of the knee
28
How does peroneal neuropathy present?
Weak foot with decreased dorsiflexion and eversion
29
What are common causes of Median Neuropathy?
Typing, Carpenters, working with hands
30
How doe Median Neuropathy present?
Thenar wasting, pain/numbness in 1st 3 fingers
31
What are the treatment options for Peripheral Neuropathy?
Pregabalin or Gabapentin TCA Seizure Meds: Phenytoin, Carbamazepine, Lamotrigine
32
What is Complex Region Pain Syndrome (Reflex Sympathetic Dystrophy)?
Severe, excruciating pain in a limb with allodynia (pain elicited by normal stimuli)
33
How does Complex Region Pain Syndrome (Reflex Sympathetic Dystrophy) present?
Severe excruciating limb pain with allodynia, vastomotor symptoms, intermittent edema and skin changes
34
What is there always a history of in Complex Region Pain Syndrome (Reflex Sympathetic Dystrophy)?
Trauma damaging the myelin of peripheral nerves
35
How do you treat Complex Region Pain Syndrome (Reflex Sympathetic Dystrophy)?
NSAIDS TCA Gabapentin or Pregabalin
36
What are common causes of Facial (CN7) Nerve Palsy (Bell Palsy)?
Idiopathic Lyme Disease Sarcoidosis Herpes Zoster Tumors
37
What is the characteristic presentation of Facial (CN7) Nerve Palsy (Bell Palsy)?
Paralysis of the entire side of the face Difficulty closing the eye Inability to wrinkle the forehead Hyperacusis Taste distubrances
38
What is Hyperacusis in Facial (CN7) Nerve Palsy (Bell Palsy)?
Sounds are extra loud because the 7th CN normally supplies the stapedius muscle which acts as a shock absorber on the ossicles of the middle ear
39
Why are there taste disturbances in Facial (CN7) Nerve Palsy (Bell Palsy)?
CN 7 supplies taste sensation to the anterior 2/3 of the tongue
40
What is the most accurate test for Facial (CN7) Nerve Palsy (Bell Palsy)?
Electromyography and nerve conduction study
41
How do you treat Facial (CN7) Nerve Palsy (Bell Palsy)?
Prednisone: 60% recover without any intervention
42
What is Guillain-Barre Syndrome?
Acute Inflammatory Polyneuropathy: autoimmune damage to multiple peripheral nerves
43
What causes Guillain-Barre Syndrome?
Circulating antibody that attacks the myelin sheath of peripheral nerves removing their insulation
44
What infection is associated with Guillain-Barre syndrome?
Campylobacter Jejuni
45
How Does Guillain-Barre syndrome present?
Ascending weakness + loss of reflexes
46
What is the most specific test for Guillain-Barre Syndrome?
Nerve conduction study/electromyography: shows a decrease in propagation of impulses along the nerves over 1-2 weeks
47
What respiratory function changes are seen when the diaphragm is involved in Guillain-Barre Syndrome?
Decreased FVC and Peak inspiratory pressure
48
How do you treat Guillain-Barre Syndrome?
IVIG or Plasmapheresis: DO NOT COMBINE THEM PICK ONE
49
What is Miller Fisher?
Guillain-Barre variant with weakness descending from the top down
50
What is a key finding in Miller Fisher?
Oculomotor nerve involvement
51
What testing should be done for Miller Fisher?
GQ1b antibody testing
52
How do you treat Miller Fisher?
IVIG or Plasmapheresis
53
How does Tick paralysis present?
Ascending paralysis that resolves with tick removal
54
What is Myasthenia Gravis?
Muscular weakness from production of antibodies against ACh-receptors at the Neuromuscular junction
55
What antibodies are seen in Myasthenia Gravis?
Ab against ACh-receptors at the neuromuscular junction
56
How does Myasthenia Gravis typically present?
Double vision, difficulty chewing, dysphonia or weakness of limb muscles worse at the end of the day
57
What is seen on physical exam for Myasthenia Gravis?
Ptosis, weakness with sustained activity and normal pupillary responses
58
What is the best initial test for Myasthenia Gravis?
ACh-Receptor antibodies
59
What is the most accurate test for Myasthenia Gravis?
Electromyography: decreased strength with repetitive stimulation
60
What drug test can be used for Myasthenia Gravis?
Edrophonium: short acting AChe inhibitor: shows improvement in motor function
61
What is the best initial treatment for Myasthenia Gravis?
Neostigmine or Pyridostigmine
62
What is the mechanism of Neostigmine and Pyridostigmine?
Long-acting inhibitors of AChe
63
If a patient with MG does not respond to Neostigmien or Pyridostigmien and is <60 what do you do next?
Thymectomy
64
If a patient with MG does not respond to Neostigmine or Pyridostigmine and is >60 what do you do next?
Prednisone
65
What drugs can be added for Refractory and recurrent disease?
Rituximab: anti-CD20 Eculizumab: removes complement
66
How does Acute Myasthenic Crisis present?
severe, overwhelming disease with profound weakness or respiratory involvment
67
How is Acute Myasthenic Crisis treated?
IVIG or plasmapheresis
68
How does Lambert-Eaton Myasthenic Syndrome (LAMS) present?
muscle weakness in patients with small-cell lung cancer
69
What is the cause of LAMS?
decreased release of Ach at the neuromuscular junction due to anti-P/Q voltage gated calcium channel antibodies
70
What symptom are seen in LAMS?
Increased strength with increased use Increased DTR after exercise
71
What is the best initial test for LAMS?
anti-P/Q Voltage Gated calcium channel antibodies
72
What is the best initial treatment for LAMS?
Pyridostigmine
73
What is used to treat Acute, Severe LAMS?
IVIG Amifampridine: K channel blocker

Master the boards: Neurology (11 decks)

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