Peripheral nerve system pathology Flashcards
1
Q
Upper motor neurons decussate at this location
A
Medulla
2
Q
Is the alpha motor neuron an upper or lower motor neuron?
A
Lower
3
Q
This is a lower motor neuron that goes to the NMJ
A
Alpha motor neuron
4
Q
Does this describe upper or lower motor neuron lesion:
Muscles are innervated but not regulated
A
UMN
5
Q
Does this describe upper or lower motor neuron lesion:
Result is spastic paralysis
A
UMN
6
Q
Does this describe upper or lower motor neuron lesion:
Lack of muscle innervation
A
LMN
7
Q
Does this describe upper or lower motor neuron lesion:
Result is flaccid paralysis
A
LMN
8
Q
Does this describe upper or lower motor neuron lesion:
No atrophy
A
UMN
9
Q
Does this describe upper or lower motor neuron lesion:
Atrophy present
A
LMN
10
Q
Does this describe upper or lower motor neuron lesion:
Increased reflexes
A
UMN
11
Q
Does this describe upper or lower motor neuron lesion:
Decreased reflexes
A
LMN
12
Q
Does this describe upper or lower motor neuron lesion:
Increased tone
A
UMN
13
Q
Does this describe upper or lower motor neuron lesion:
Decreased tone
A
LMN
14
Q
Does this describe upper or lower motor neuron lesion:
Positive Babinski
A
UMN
15
Q
Does this describe upper or lower motor neuron lesion:
No fasciculations
A
UMN
16
Q
Does this describe upper or lower motor neuron lesion:
Fasciculations present
A
LMN
17
Q
Does this describe upper or lower motor neuron lesion:
Spastic weakness
A
UMN
18
Q
Does this describe upper or lower motor neuron lesion:
Flaccid weakness
A
LMN
19
Q
Spontaneous motor unit firing when muscle is at rest
Involves individual muscle fibers
A
Fasciculations
“twitch”
20
Q
This part of the neuron is present in ganglion or cord
A
Cell body
21
Q
This part of the neuron is present in peripheral nerve
A
Axon
22
Q
Connective tissue that encases entire nerve
A
Epineurium
23
Q
Connective tissue around fascicle of fibers
A
Perineurium
24
Q
Connective tissue around individual axon fiber
A
Endoneurium
25
Myelin sheath is made up of these type of cells
Schwann cells
26
What is the result of the nerve conduction study in axonal injury?
Loss of signal amplitude
27
What is the result of the nerve conduction study in demyelinating injury?
Loss of conduction velocity
28
Nerve conduction study will show loss of signal amplitude in this type of peripheral nerve injury
Axonal
29
Nerve conduction study will show loss of conduction velocity in this type of peripheral nerve injury
Demyelinating
30
Conduction velocity is a function of this
Nerve diameter
31
Signal amplitude is a function of this
Number of fibers and synapses
32
Wallerian degeneration is seen after this type of peripheral nerve injury
Axonal
33
Does an axonal injury have better prognosis if it is due to a crush injury or transection?
Crush
34
After axonal injury, what happens to the nucleus of the cell body?
Becomes eccentric
35
This is a process that occurs in a neuron's cell body when it is damaged
Characterized by the redistribution of the Nissl substance, which is made up of rough endoplasmic reticulum and ribosomes
Chromatolysis
36
Inability to successfully re-innervate after axonal injury results in this
Traumatic neuroma
37
This is a process that occurs when a nerve fiber (axon) is injured or severed
Leads to the gradual disintegration and breakdown of the axon and its myelin sheath distal to the injury site
Wallerian degeneration
38
Wallerian degeneration involves this part of the neuron degenerating due to lack of axonal transport
Axon
39
Chromatolysis occurs during axonal regeneration, and is seen in this part of the neuron
Cell body
40
Do attempts at regeneration occur simultaneously as degeneration after traumatic or non-traumatic injury to axon?
Non-traumatic
41
Is non-traumatic injury to axon length dependent?
Yes
Results in stocking glove type of deficit
42
Stocking and glove deficit results from this type of peripheral nerve injury
Axonal injury (non-traumatic)
43
Nodular collection of nerve twigs which persists after attempted regeneration
Traumatic neuroma
44
Lack of this makes attempted regeneration less likely in traumatic neuroma
Epineurial approximation
45
This is a tangled mass of nerve endings in fibrous scar
Traumatic neuroma
46
Is CNS or PNS injury more likely to regenerate?
PNS
(CNS has no connective tissue sheath to follow)
47
Do Schwann cells have the ability to proliferate?
Yes
Can remyelinate axon after demyelinating injury
48
The result of recovery after this injury is shortened internodal distance/more internodes
Demyelinating injury
49
"Onion bulb" morphology is seen in this
Demyelinating injury
50
Is regeneration possible after neuronal injury?
No
51
Are deficits from neuronal injury length dependent?
No
(NO stocking and glove like in axonal injury)
52
Spinal muscular atrophy is an example of this type of peripheral nerve injury
Neuronal
53
Pattern of PNS injury that favors trauma, entrapment syndromes
Mononeuropathy
54
Pattern of PNS injury that favors a systemic, metabolic, genetic factor
Polyneuropathy
55
Pattern of PNS injury that favors compression due to disk disease or DJD of spine
Radiculopathy
(involves spinal nerve root)
56
This condition is also known as Acute inflammatory demyelinating polyradiculopathy
Guillain Barre Syndrome
57
Most of Guillain Barre Syndrome cases are preceded by this
Recent infection
(Campylobacter especially)
58
Guillain Barre Syndrome is often preceded by recent infection, especially with this organism
Campylobacter
59
Guillain Barre Syndrome involves autoantibodies to this
Myelin proteins
60
Condition characterized by autoantibodies to myelin proteins which mimic foreign antigens
Causes demyelination of axons
Guillain Barre Syndrome
61
Is the effect of demyelination in Guillain Barre Syndrome more pronounced in short or long axons?
Longer
62
Does remyelination occur in Guillain Barre Syndrome?
Yes, is typical
63
Term that describes abnormal sensations that can feel like tingling, prickling, burning, or numbness in the skin
Paresthesias
64
A patient with a recent history of immunization and progressive leg weakness may have this condition
Guillain Barre Syndrome
65
Can Guillain Barre Syndrome involve cranial nerves?
It can; causes difficulty swallowing
66
Is areflexia seen in Guillain Barre Syndrome?
always
67
Is atrophy seen in Guillain Barre Syndrome?
NO
68
Most patients with Guillain Barre Syndrome require hospitalization for this
Respiratory muscle weakness
69
Does Guillain Barre Syndrome affects arms or legs more?
Legs
70
CSF high in protein, cells not increased, and glucose normal are lab results seen with this condition
Guillain Barre Syndrome
71
Laboratory results of CSF in Guillain Barre Syndrome will be high in this
Protein
72
A patient with progressive, symmetrical leg weakness and difficulty swallowing may have this condition
Guillain Barre Syndrome
73
Is nerve conduction normal in Guillain Barre Syndrome?
No, is slowed due to demyelination
74
What will be seen morphologically in Guillain Barre Syndrome?
Lymphocytic infiltration of peripheral nerve
75
Lymphocytic infiltration of peripheral nerve is seen morphologically in this condition
Guillain Barre Syndrome
76
This is a proposed chronic form of Guillain Barre Syndrome
Chronic inflammatory demyelinating polyneuropathy
77
Chronic inflammatory demyelinating polyneuropathy is a proposed chronic form of this
Guillain Barre Syndrome
78
Chronic inflammatory demyelinating polyneuropathy has the same CSF findings (high protein, cells normal) and nerve conducting findings (slower) as this condition
Guillain Barre Syndrome
79
Is there usually preceding infection in Chronic inflammatory demyelinating polyneuropathy?
Usually no
80
Chronic inflammatory demyelinating polyneuropathy responds to this type of drug
Steroids
81
Chronic inflammatory demyelinating polyneuropathy lasts this long
Longer than 2 months
82
Is a patient with Guillain Barre Syndrome or Chronic inflammatory demyelinating polyneuropathy more likely to have respiratory compromise?
Guillain Barre
83
Mononeuritis Multiplex is the classic pattern of this condition
Vasculitic neuropathy
84
What is the classic pattern of Vasculitic neuropathy?
Mononeuritis Multiplex
85
What size of arteries of vasa nervorum are affected by vasculitis neuropathy?
Small-medium
86
Vasculitis neuropathy produces ischemic damage and this type of peripheral nerve injury
Axonal injury
87
Antibody that may be positive in Vasculitis neuropathy
ANCA (anti-neutrophil cytoplasmic antibody)
(usually MPO-ANCA)
88
ANCA may be positive (usually MO-ANCA) in this condition
Vasculitis neuropathy
89
What are the levels of ESR in Vasculitis neuropathy?
Elevated
90
RF, anti-dsDNA, anti–SS etc. may be present in this condition
Vasculitis neuropathy
(if associated w/ autoimmune disorder)
91
This is the most common form of diabetic neuropathy
Distal symmetric polyneuropathy
92
Does Diabetes distal symmetric polyneuropathy begin with short or long axons?
Longest axons, then more proximal
Toes and feet, fingertips and hands = "stocking glove" deficit
93
Charcot joint (foot loses its arch) is seen in this condition
Diabetes (distal symmetric polyneuropathy)
94
What effect does Diabetic (distal symmetric polyneuropathy) have on the arch of the foot?
Can cause Charcot joint (foot loses its arch)
95
In addition to distal symmetric polyneuropathy, diabetes can also cause this type of neuropathy
Autonomic
96
A patient with orthostatic hypotension and frequent UTIs may have this condition
Diabetes (autonomic dysfunction/neuropathy)
97
Does ethanol associated neuropathy affect proximal or distal musculature?
Distal
Is mostly a symmetric distal sensory neuropathy
98
Ethanol associated neuropathy is very similar to diabetic neuropathy, with paresthesias, decreased proprioception, and weak distal musculature, but does not have this type of neuropathy
Autonomic
99
Ethanol associated neuropathy is multifactorial, possibly due to a deficiency in this
thiamine
100
This condition is also known as Hereditary motor sensory neuropathy
Charcot Marie Tooth Disease
101
Charcot Marie Tooth Disease is due to many genetically distinct disorders, mostly with this inheritance pattern
Autosomal dominant
102
Most disorders that cause Charcot Marie Tooth Disease are due to problems with this protein
PMP22 (peripheral myelin protein 22)
103
PMP22 (peripheral myelin protein 22) is involved in this condition
Charcot Marie Tooth Disease
104
When do patients with Charcot Marie Tooth Disease usually present?
Childhood or early adulthood
(variable rates of progression and severity)
105
Pes Cavus and anterior calf atrophy are typical of this condition
Charcot Marie Tooth Disease
106
What effect does Charcot Marie Tooth Disease have on the foot?
Can result in Pes Cavus (high arch)
107
Mononeuropathy due to focal compression or mechanical distortion within an anatomic compartment (or adjacent mass, bone, etc)
Entrapment syndromes
108
Entrapment of this nerve causes claw hand, sensory loss at 4/5 fingers
Ulnar
109
Elbow leaning and trauma can cause entrapment of this nerve
Ulnar
110
Entrapment of this nerve causes wrist drop
Radial
111
Entrapment of this nerve causes dorsal hand sensory loss
Radial
112
Tricep weakness occurs with entrapment of radial nerve at this location
Axilla
113
Sparing of triceps occurs in entrapment of the radial nerve at this location
Spiral groove
114
Entrapment of this nerve causes nocturnal paresthesia and pain hypesthesia
Median
115
Inability to regenerate and reinnervate after severing injury increases likelihood of this forming
Traumatic neurom
116
After severing injuries, chance of reinnervation is directly correlated with preservation of this
covering layers
117
Benign tumor of Schwann cells
Schwannoma
118
Schwannomas grow loosely attached to this
Peripheral nerve
119
Are Schwannomas easy to surgically resect?
Usually yes
(grows loosely attached to nerve)
120
This is the NF2 gene product that is often lost with Schwannomas
Merlin
121
Is the nerve incorporated in Schwannomas?
No
122
Loss of Merlin, the NF2 gene product, can cause this tumor
Schwannoma
123
Loss of this protein can lead to schwannoma
Merlin (NF2 gene product)
124
Alternating zones of cellularity (hypercellular and hypocellular regions) are seen in this benign tumor
Schwannoma
125
Acellular zones filled with cytoplasmic processes; palisading nuclei
Seen in Schwannoma
Verocay bodies
126
IHC marker for Schwannoma
S100
127
Antoni A-hypercellular and Antoni B-hypocellular zones are seen in this tumor
Schwannoma
128
Is the Antoni A pattern (seen in Schwannomas) hypercellular or hypocellular?
Hypercellular
129
Is the Antoni B pattern (seen in Schwannomas) hypercellular or hypocellular?
Hypocellular
130
What are Verocay bodies?
Acellular zones filled with cytoplasmic processes
Between palisading nuclei
(seen in Schwannoma)
131
Palisading nuclei surrounding acellular zones are seen in this tumor
Schwannoma
132
Schwannoma of the vestibular branch of CN VIII
Acoustic neuroma
133
Acoustic neuroma are this type of tumor of the vestibular branch of CN VIII
Schwannoma
134
Acoustic neuroma is a Schwannoma of this
Vestibular branch of CN VIII
135
Acoustic neuroma involves this cranial nerve
CN VIII
136
Acoustic neuroma involves this branch of CN VIII
Vestibular
137
Acoustic neuroma often arises here
At cerebellopontine angle
138
Acoustic neuroma is very common with this condition
Neurofibromatosis type 2
139
Benign tumors of CN VIII that can cause tinnitus and hearing loss
Acoustic neuroma
140
Condition caused by a NF2 gene deletion, leading to absence of Merlin protein
Neurofibromatosis, type 2
141
NF2 gene is found on this chromosome
22
142
Inheritance pattern of Neurofibromatosis, type 2
Autosomal dominant
143
Bilateral acoustic neuromas is the hallmark of this condition
Neurofibromatosis, type 2
144
Bilateral acoustic neuromas and cutaneous schwannomas are characteristic of this condition
Neurofibromatosis, type 2
145
Does neurofibromatosis type 1 or 2 involves schwannomas?
Type 2
146
Neurofibromatosis, type 2 involves a NF2 gene deletion, leading to absence of this protein, causing schwannomas
Merlin
147
Condition characterized by benign Schwannomas, but also deafness, tinnitus, balance issues, weakness due to spinal tumors, and may also develop cataracts
Neurofibromatosis, type 2
148
Benign tumor of Schwann cells with accompanying neurites, perineural cells, fibroblasts, others
Neurofibroma
149
Does Neurofibroma incorporate the nerve?
yes
150
3 forms of neurofibroma
Localized cutaneous
Diffuse
Plexiform
151
Neurofibroma is a benign tumor of these cells
Schwann cells
152
Type of neurofibroma:
Small circumscribed dermal/subcutaneous nodule
Sporadic tumor
Localized cutaneous
153
Type of neurofibroma:
Forms large dermal/subcutaneous plaques
Grows along plane between fascia and dermis
Sporadic or associated with NF1
Diffuse
154
Form of neurofibroma:
Pathognomonic of Neurofibromatosis, type 1
Develop during childhood
Affect large segments of nerves
Produce diffuse swelling
Plexiform
155
Plexiform neurofibromas are pathognomonic of this condition
Neurofibromatosis, type 1
156
This condition is also known as von Recklinghausen disease
Neurofibromatosis, type 1
157
This is a plexiform neurofibroma that involves the entire limb, with overlying skin redundancy, skin pigmentation and may produce bone thickening (due to increased tumor blood flow)
Elephantiasis Neuromatosa
158
Can plexiform neurofibroma become malignant?
Yes
159
Neurofibromatosis, type 1 is also known as this disease
von Recklinghausen disease
160
What is the inheritance pattern of Neurofibromatosis, type 1?
Autosomal dominant
161
NF1 gene is lost in Neurofibromatosis, type 1, and is found on this chromosome
17
162
NF1 is part of this pathway
Ras
163
This is the gene product of NF1
Neurofibromin
164
Condition characterized by many neurofibromas of all types, cafe au lait spots, Lisch nodules of iris and optic nerve gliomas
Neurofibromatosis, type 1
165
Does Neurofibromatosis, type 1 or 2 involves cafe au lait spots?
1
166
Does Neurofibromatosis, type 1 or 2 involves Lisch nodules of iris?
1
167
This is a sarcoma of Schwann cells, perineural cells, fibroblasts of nerve sheath
Malignant peripheral nerve sheath tumor
168
Malignant peripheral nerve sheath tumor are common in patients with this condition
Neurofibromatosis, type 1
169
Malignant peripheral nerve sheath tumor arise in this tissue layer
Deep soft tissue or subcutaneous
170
Histology of this condition shows spindled cells similar to nerve tissue
Malignant peripheral nerve sheath tumor
171
IHC marker for Malignant peripheral nerve sheath tumor
S100
172
Malignant peripheral nerve sheath tumor is a sarcoma of these
Schwann cells, perineural cells, fibroblasts of nerve sheath
173
Sarcoma that is common in neurofibromatosis, type 1
Malignant peripheral nerve sheath tumor
174
Malignant peripheral nerve sheath tumor is common in neurofibromatosis type 1 or 2?
1
175
Does neurofibromatosis type 1 or 2 involve hearing loss?
2
176
Does neurofibromatosis type 1 or 2 involve spinal tumors?
2
177
What chromosome is involved in neurofibromatosis, type 2?
22
178
What chromosome is involved in neurofibromatosis, type 1?
17
179
Elephantiasis Neuromatosa is commonly seen in patients with this condition
Neurofibromatosis, type 1
