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Pathology 2 Flashcards

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1
Q

What is the most common aminoacidopathy?

A

phenyketonuria

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2
Q

What is the inheritance of phenylketonuria?

A

AR

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3
Q

What are hallmark exam findings in PKU? How is it treated?

A

fair skinned blue eyed kid with musty odor

limit phenylalanine consumption

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4
Q

The aminoacidopathies are generally inherited in _____ fashion.

A

AR

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5
Q

What is the inheritance of homocystinuria?

A

AR

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6
Q

What is the deficient enzyme in PKU?

A

phenylalanine hydroxylase

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7
Q

What is the deficient enzyme in homocystinuria?

A

cystathionine B-synthase

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8
Q

What is the symptomatology of homocystinuiria?

A

similar to Marfan’s syndrome, but with mental retardation and increased incidence of stroke, lens dislocations, and arachnodactyly

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9
Q

What is a key finding in maple syrup urine disease?

A

burned sugar smell in the urine

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10
Q

What’s the inheritance of maple syrup urine disease?

A

AR

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11
Q

What is the deficient enzyme in maple syrup urine disease?

A

branched chain a-keto acid dehydrogenase

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12
Q

What is Hartnup disease?

A

aminoacidopathy, cannot absorb neutral amino acids

develop Niacin deficiency with pellegra like symptoms

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13
Q

The sphingolipidoses are inherited in ______ fashion except for ____, which is _____.

A

AR

Fabry, XR

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14
Q

What enzyme is deficient and what accumulates:

Nieman Pick

A

Sphingomyelinase

Sphingomyelin

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15
Q

What enzyme is deficient and what accumulates:

Gaucher

A

Glucocerebrosidase

Glucocerebreside

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16
Q

What enzyme is deficient and what accumulates:

Fabry

A

a-galactosidase

ceramides

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17
Q

What enzyme is deficient and what accumulates:

Tay Sachs

A

Hexosaminadase

AGM2 gangliosides

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18
Q

What enzyme is deficient and what accumulates:

Sandhoff

A

Hexosaminidase A

BGM2 gangliosides

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19
Q

What enzyme is deficient and what accumulates:

GM1 gangliosidosis

A

B-galactosidase

GM1 gangliosides

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20
Q

Which sphingolipidoses cause cherry red spots?

A

Niemann Pick
Tay Sachs
GM1 Gangliosidosis
Gaucher

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21
Q

Which sphingolipidoses affect Ashkenazi Jews?

A

Niemann Pick

Tay Sachs

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22
Q

What are key findings in Niemann Pick?

A

cherry red spot, vertical gaze palsy, MR, hepatosplenomegaly

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23
Q

What are Niemann Pick cells?

A

foamy cells; large vacuolated histiocytes and lymphocytes

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24
Q

What is the most frequent sphingolipidosis?

A

Gaucher

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25
What are Gaucher cells?
wrinkled tissue paper appearing cells
26
What are key findings in Gaucher disease?
hypersplenism with anemia and thrombocytopenia
27
What are key findings in Fabry disease?
painful dysesthesias, vascular disease (Htn, renal, death by MI or stroke in adulthood)
28
What are key findings in Tay Sachs disease?
MACROcephaly, cherry red spot (NO organomegaly)
29
What are clinical findings in Sandhoff disease?
similar to tay sachs but has visceral storage
30
Mucopolysaccharidoses are all _______ inheritance except _____ which is _____.
AR Hunter, XR
31
What is the enzyme deficiency and urine sulfate metabolite for the following: Hurler
a-L-iduronidase Heparan and dermatan
32
What is the enzyme deficiency and urine sulfate metabolite for the following: Scheie
a-L-iduronidase Heparan and dermatan
33
What is the enzyme deficiency and urine sulfate metabolite for the following: Hunter
iduronidase sulfatase Heparan and dermatan
34
What is the enzyme deficiency and urine sulfate metabolite for the following: Morquio
B galactosidase Keratin
35
What are key features of Hurler's disease?
gargoyle face, MR, dwarfism, corneal clouding, skeletal abnormalities, thick meninges (can cause spinal cord compression)
36
What are Zebra bodies?
seen on electron microscopy with Hurler's disease
37
What are key features of Scheie?
milder Hurler, no MR
38
What are key features of Hunter?
milder Hurlery, no MR and less corneal clouding; classic skin pebbling and peripheral nerve entrapment (Carpel tunnel)
39
Which MPS has the most severe skeletal deformities and ligamentous laxity?
Morquio
40
Which MPS can have carpel tunnel syndrome?
Hunter, Scheie, Maroteaux Lamy
41
Which MPS classically has hydrocephalus?
Sly
42
The leukodystrophies are all _____ inheritance except for _____ which are ____ and _____ which is ____.
AR Adrenoleukodystrophy and Pelizaeus-Merzbacher, XR Alexander, sporadic
43
Which leukodystrophy is associated with Ashkenazi Jews?
Canavan
44
What is the enzyme deficiency and what accumulates: Krabbe
galactocerebroside β-galactosidase galactocerebroside
45
What is the enzyme deficiency and what accumulates: Metachromatic leukodystrophy
arylsulfatase A sulfatides
46
What is the enzyme deficiency and what accumulates: Adrenoleukodystrophy?
ABCD 1 long chain fatty acids
47
Leukodystrophies have cavitation or degeneration of ______ but spare _____ except for ______ which preferentially targets them.
white matter subcortical U fibers Canavan
48
Which leukodystrophy is associated with adrenal insufficiency and bronze skin?
adrenoleukodystrophy
49
What CT finding is unique to Krabbe disease?
hyperdense basal ganglia and thalamus
50
What is seen on histology for Krabbe disease?
Globoid cells; large macrophages around blood vessels
51
What is the treatment in adrenoleukodystrophy?
Lorenzo oil and diet low in long chain fatty acids
52
Which leukodystrophy has a Tigeroid pattern on MRI?
Pelizaeus Merzbacher
53
Alzheimer type II astrocytes are seen in what leukodystrophy?
Canavan
54
What are the key clinical features of MELAS?
encephalopathy, lacitc acidosis, stroke like episodes with cortical blindnesss
55
What are the key clinical features with MERRF?
myoclonic epilepsy with ragged red fibers on muscle biopsy
56
How is Kearns Sayre syndrome inherited?
mitochondrial
57
Which mitochondrial diseases have ragged red fibers on muscle biopsy?
MERRF and Kearns Sayre
58
What are key clinical findings in Kearns Sayre?
cardiomyopathy and ophthalmoplegia/retinal issues
59
What is the inheritance of Lebers hereditary optic neuropathy?
mitochondrial
60
What are the key clinical findings in levers hereditary optic neuropathy?
progressive painless loss of central visiosn
61
What is the inheritance of Menke Kinky hair disease?
XR
62
Menke kinky hair disease is due to a defect in ____ causing decrease in _____.
Copper transporting ATPase GI absorption of copper
63
What are the key clinical features of Menke Kinky hair disease?
tortuous vessels, metaphysial spurring, brittle colorless hair growth, seizures, MR
64
What is the inheritance of Leigh disease?
AR
65
What is the deficient enzyme in leigh disease?
cytochrome C oxidase
66
Leigh disease results in ______ leading to diffuse neurologic deficits such as _____.
bilateral symmetric spongiform degeneration and necrosis of the thalamus, basal ganglia, brainstem, and spinal cord with peripheral nerve demyelination decreased muscle tone and head control, seizures, myoclonus, ophthalmoplegia, and respiratory and swallowing problems
67
What's another name for Lowe syndrome? How is it inherited?
XR oculocerebrorenal syndrome (cataracts, MR, death by renal failure)
68
What is the inheritance of Lesch-nyhan syndrome? What is the deficient enzyme? What are its key clinical features?
XR HGPRT accumulation of uric acid leading to choreoathetosis and self mutilation
69
What is Zellweger syndrome? What's another name for it?
XR Cererbrohepatorenal syndrome (cortical dysgensis, hepatorenal failure)
70
What neurons are affected first in Hungtinton's disease?
medium spiny type 1 neurons
71
What neurotransmitters are affected in Huntington's disease?
decreased GABA and ACh increased NE and somatostatin
72
What is the inheritance of Wilson's disease?
AR
73
What chromosome is associated with Wilson's disease?
Ch 13
74
What is seen on histology for Wilson's disease?
Alzheimer type II astrocytes in grey matter, Opalski cells (microglia) in the GP
75
What is seen on gross pathology of Wilson's disease?
spongy red degeneration and cavitation of the putamen and GP with occasional atrophy of the superior and middle frontal gyri
76
How is Wilson's disease treated?
limit copper food (liver and chocolate), penicillamine, copper chelate
77
What two disease have neurofibrillary tangles? What's the difference?
Alzheimers (flamed) and Progressive supranuclear palsy (globose)
78
What is an key history finding in patients with progressive supranuclear palsy?
multiple falls with poor response to L-DOPA
79
What is seen on gross pathology with striatonigral degeneration?
atrophic/brown putamen and depigmented SN
80
What is Hallorvorden Spatz disease?
AR, iron deposition disease atrophic GP and SN leading to extrapyramidal and corticospinal dysfunction
81
What is the effect of amantadine for Parkinson's?
increases DA release
82
Benztropine and trihexylphenidyl are both _____.
antiholinergics
83
What medication can be used for tremor in parkinson's disease?
propanolol
84
______ deep brain stimulation is best for contralateral bradykinesia and tremor.
Subthalmic
85
Shy Drager syndrome has loss of cells in the ______.
intermediolateral column of the spinal cord and putamen
86
What is the inheritance of friedreich's ataxia?
AR
87
What is the genetics of friedreich's ataxia?
Ch 9 trinucelotide repeat, Frataxin gene
88
What is the inheritance of Lafora body disease?
AR
89
What are the symptoms of Lafora body disease?
myoclonic seizures and dementia
90
What is seen with Baltic myoclonus? What is the inheritance?
AR myoclonic seizures with purkinje cell atrophy
91
Spinal muscular atrophy is defined by degeneration of _____ and sparing of _____.
anterior horn and hypoglossal nuclei corticospinal and bulbar nuclei
92
What is the most common SMA?
SMA type 1 (Werdnig Hoffman)
93
What is the genetics of SMA type 1? What is the inheritance?
AR Ch 5
94
Floppy infant syndrome is a result of ____.
SMA type 1
95
All SMA types have _____ weakness with sparing of the _____.
proximal muscle eyes
96
Most ALS cases are sporadic but ____ percent are____ on Ch ____ due to mutation in ______.
10% AD Ch 21 superoxide dismutase
97
What are Bunina bodies?
intracytoplasmic inclusions in ALS in anterior horn cells
98
How does diphtheria affect myelin?
inhibits Schwann cell myelin synthesis
99
MS is associated with HLA ___.
DR 2 and DR 4
100
What is Charcot's triad? Where is it seen?
nystagmus, scanning speech, intention tremor MS
101
B/l intranuclear ophthalmoplegia is pathognomonic for ______.
MS
102
MS plaques on gross pathology are ____ if older and _____ if acute.
grey pink
103
Lymphocytes that are seen in MS are predominantly _____.
T cells
104
In MS, steroids decrease ______, B-interferon decreases _____, and Copaxone decreases _____.
steroids - attack duration B-interferon - attack rate Copaxone - frequency of relapses
105
What is Margburg variant (acute) MS?
rapidly fatal variant with diffuse large plaques
106
What microscopic changes are seen at different time intervals in ischemic stroke?
Under 6 hrs: cell swelling and edema 6-12 hrs: shrinking of the cell, incrustations 24 hrs: PMNs accumulate 48 hrs: PMNs peak 3-5 days: macrophages arrive 2 weeks: vessels form around the periphery and enhancement begins
107
Ischemic penumbra is associated with blood flow between ______.
8-23 mL/100g/minute
108
What cerebral areas are most susceptible to ischemia?
hippocampus, cortex (parietooccipital deep sulci third, fifth, and sixth layers), basal ganglia (caudate and putamen), and cerebellum (Purkinje cells)
109
What areas of the hippocampus are most susceptible to ischemia? Most resistant?
CA1 (somner) and CA3 (endplate) CA2
110
What is hypoxic ischemic encephalopathy?
due to global hypoperfusion or hypoxia; watershed strokes especially parietooccpital area symptoms are "man in barrel" syndrome with proximal limb weakness
111
What is seen on pathology for hypoxic ischemic encephalopathy?
laminar necrosis of cortical layers 3,5,6 and putamen
112
What neurotransmitter has been associated with cellular necrosis after ischemia and with blockage can increase neuronal survival?
glutamate
113

Atherosclerotic plaques form most commonly at the ______.

ICA bifurcation and distal basilar artery

114

The formation of an atherosclerotic plaque is usually initiated by _____ leading to increase in ____.

subtle intimal injury permeability of lipoproteins

115

What percent of the population has a complete circle of willis?

20%

116

What is the most common cause of TIA?

platelet–fibrin embolus from an ulcerative atherosclerotic plaque

117

What is the most cause of death in stroke patients?

MI

118

What are Gitter cells?

lipid laden macrophages seen on day 5-7 following ischemic stroke

119

What is pseudolaminar cortical necrosis?

caused by generalized hypoxia; middle cortical layers are affected (layers 3, 5, and 6)

120

Lacunar strokes count for ______ percent of strokes.

20

121

Moyamoya in children usually presents with _____ and in adults with _____.

ischemia/transient weakness hemorrhage

122

Which NF is associated with multi-vascular abnormalities?

NF 1

123

Klippel Traneuy Weber is associated with what vascular abnormality?

spinal AVMs and carotid aplasia

124

What is seen on angiogram in fibromuscular dysplasia?

string of beads appearance

125

FMD most commonly affects which vessels?

cervical ICA (75%), vertebral artery (25%), and renal arteries

126

What does CADASIL stand for?

Cerebral autosomal dominant inherited arteriopathy with subcortical infarcts and leukoencephalopathy

127

What mutation is associated with CADASIL?

notch 3 gene on Ch 19

128

Where is the most common place for carotid dissection?

2 cm above the carotid bulb

129

What is the most common place for vertebral artery dissection?

between C2 and occiput

130

What is Raeder's syndrome?

Unilateral headache and face pain of the V1 and V2 distributions and Horner’s syndrome ICA may be narrowed by sinusitis, arteritis, or dissection

131

What is the most common necrotic vasculitis with CNS lesions?

polyarteritis nodosa

132

What is polyarteritis nodosa?

necrotic vasculitis; affects small and medium sized arteries causes polyneuropathy by obliteration of vaso nervosum, microaneurysms (70%), skin purpura, renal dysfunction

133

What is seen on pathology for temporal arteritis?

mononuclear cell inflammation of all three layers with multinucleated giant cells and resorption of the internal elastic lamina

134

Steroids in temporal arteritis has what outcome?

decreases risk of blindness

135

What is takayasu arteritis?

occlusive thromboaortopathy with giant cell arteritis affecting the aortaand its branches and the pulmonary arteries causing stenosis and aneurysms decreased peripheral pulses (pulseless disease)

136

How do you treat Wegner's granulomatosus?

cyclophosphamide

137

Which vasculitis is closely linked with smoking?

Buerger disease

138

What is Behcet disease?

recurrent inflammatory disease with male predominance that affects arteries and veins characterized by oral and genital ulcers, UC, aneurysm thrombophlebitis, erythema nodosum, polyarthritis

139

What is the triad of TTP? Pentad?

severe thrombocytopenia, microangiopathic hemolytic anemia, and neurologic dysfunction fever and renal dysfunction

140

What causes TTP?

deficiency in ADAMTS13, von-Willibrand factor protease

141

What are Charcot Bouchard aneurysms?

form on the lenticulostriate arteriesdilated perivascular spaces, état lacunaire (in the centrum semiovale), and état criblé (in the basal ganglia), which form lacunae with gliosis but no symptomscan cause hypertensive ICH

142

What is Binswanger disease?

form on the lenticulostriate arteries. There are dilated perivascular spaces, état lacunaire (in the centrum semiovale), and état criblé (in the basal ganglia), whichform lacunae with gliosis but no symptoms

143

What are the five most common areas for hypertensive hemorrhages?

putamen (60%), thalamus (20%), pons (10%), cerebellum (near the dentate, 5%), and subcortical white matter (2%)

144

Amyloid is ______ on birefringence when stained with _____.

yellow-green

 

congo red dye

145

What is seen on pathology for amyloid angiopathy?

Blood vessels become dilated with thick walls containing pink amorphous material

146

What percent of MI patients treated with thrombolytics develop intracranial hemorrhage? What's the mortality?

1%

 

60% mortality

147

Why does the germinal matrix hemorrhage in premature infactns?

involutes by week 36 usually,. hemorrhage from hypoxia/ischemia of the deep border zone that supplies the germinal matrix 

148

What is the grading for germinal matrix hemorrhages?

Grade 1: limited to the germinal matrix.

 

Grade 2: blood in the ventricles, but no increase in ventricular size.

 

Grade 3: blood in the ventricles with hydrocephalus.

 

Grade 4: intraparenchymal extension of the hemorrhage

149

What is seen on pathology with capillary telangiectasias?

multiple normal sized thin vascular spaces, without muscle in the wall, with normal brain in between 

150

In HHT, the mucocutaenous lesions are ______ and the brain lesions are _____. 

telangiectasia

 

AVM

151

What is the most common vasculature malformation?

venous malformations

152

What is the most common location for a venous malformation?

at the angle of the ventricle

153

Multiple vascular malformations are associated with what syndrome?

blue rubber nevus syndrome

154

Venous varixes are associated with _____. 

AVMs

155

What is a sinus pericranii?

A large communication between intracranial and extracranial veins; congenital or traumatic

 

soft mass that changes with head position

156
What is McCune Albright syndrome?
unilateral polyostotic disease with endocrinopathy
157
What is the McGregor Line?
line from the hard palate to the most caudal portion of the occipital curve, odontoid tip should be < 4 mm above the line
158
What is the McRae line?
foramen magnum diameter 35 ± 4 mm
159
What is the Chamberlin line?
diagonal line from the hard palate to the posterior foramen magnum, the odontoid should not have ⅓one third of its length above it
160
What is platybasia?
Flattened skull base with an increased angle of the clivus to the spine or clivus to the anterior fossa > 135 degreesassociated with basilar invagination and Chiari malformation
161
What are wormian bones? When are they seen?
Small intrasutural bones, usually in the lambdoid sutureseen with cleidocranial dysostosis, cretinism, osteogenesis imperfecta, chronic hydrocephalus, and as a normal variant
162
Foramen magnum lesions typically cause weakness in what pattern?
ipsilateral UE -> ipsilateral LE -> contralateral LE -> contralateral UE
163
Honeycomb pattern with radiating spicules in the skull is associated with ____.
hemangioma
164
What is the most common cause of tethered cord?
spinal lipoma
165
What percent of spinal lipomas are lipomyelomeningoceles?
85%
166
Intradural lipoma is most commonly found in the _____.
cervical and thoracic spine
167
What filum thickness suggests possible tethered cord?
Filum > 1.5 mm in diameter
168
What constitutes caudal regression syndrome?
consists of lumbosacral agensis, imperforate anus, genital malformations, renal dysplasia, and fused legs (sirenomelia)
169
Anterior sacral meningocele is associated with what syndromes?
NF1 and marfan's syndrome
170
What is the most common presacral mass in children?
sacrococcygeal teratoma
171
What is split notochord syndrome?
persistent connection exists between the gut and the dorsal ectoderm; may be caused by an adhesion between the endoderm and ectoderm
172
What is diastematomyelia?
split cord with fibrous, bony (50%), or cartilaginous septum
173
What percent of patients with diastematomyelia have a single dural tube?
50%
174
What causes enterogenous cysts?
failure of the notochord and foregut to separate
175
What is the most common location for enterogenous cysts? Where within the spinal canal are they located?
Thoracic followed by cervicalintradural, extramedullary
176

Spinal bone marrow before the age of 7 contains more _____ marrow and therefore ____ on MRI.

redenhances

177

Osteoid osteomas are most commonly found in the spine at _______.

lumbar neural arch (rarely in the bodies)

178

What is usually seen on CT with osteoid osteomas?

dense sclerotic bone around a lytic lesion with a central calcified nidus of osteoid and woven boneless than 2 cm (if bigger, think osteoblastoma)

179

Which bone tumor pain responds to ASA?

osteoid osteoma

180

What is the most common location for an osteoblastoma in the spine?

cervical spine, posterior elements

181

Giant cell tumors are rare in the spine but if present, the most common location is the ____. 

Sacrum

182

Where is the most common location for osteochondroma in the spine?

C2 spinous process or transverse processes at other levels

183

Where do osteochondromas arise from and how do the appear on imaging?

lateral displacement of the epiphyseal growth cartilage and have a bony projection with a medullary cavity contiguous with the parent bone and covered with cartilage

184

Where is the most common location for aneurysmal bone cysts in the spine?

posterior elements of cervical and thoracic spine

185

When is seen on imaging of aneurysmal bone cysts?

multiloculated, expansile, lytic, vascular, and surrounded by eggshell cortical bone and no calcifications

186

What is seen on microscopic evaluation of aneurysmal bone cysts?

reveals thin-walled blood cavities without endothelium or elastic lamina and frequent multinucleated giant cells

187

Eosinophilic granuloma is in the _______ group of diseases and classically is a cause of ______ in the spine. 

Langerhans cell histiocytosis

 

vertebrae plana

188

What is the key phrase for chordoma on pathology?

physaliphorous cells 

189

What is the most common primary sacral tumor?

chordoma

190

What is seen on CT with osteosarcoma?

matrix calcifications with a sunburst pattern

191

What are the three most common metastatic tumors to the epidrual space in the spine in adults? In children?

breast, lung, prostate

 

Ewing's sarcoma, neuroblastoma

192

Which mets to the spine can be sclerotic/blastic?

breast and prostate

193

Epidural lipmatosis is associated with _____. 

obesity and steroid use

194

What is the most common spinal tumor?

Nerve sheath tumors, particularly schwannomas

195

What percent of patients with nerve sheath tumors have NF?

40%

196

Within the spinal canal, which compartment do nerve sheath tumors lie in?

intradural/extramedullary (70%), extradural (15%), dumbbell (15%), and intramedullary (1%)

197

Meningiomas account for what percent of spinal tumors?

25%

198

In what compartment do meningiomas lie within the spine?

intradural (90%), extradural (5%), and dumbbell (5%)

199

Paragangliomas in the spine are rare but if found, are most commonly in ____. 

cauda equina

200

Neurenteric cysts in the spine are generally located in the _____ spine and are ____ to the cord. 

throacic

 

ventral

201

Intradural metastatic spinal cord tumors (including primary CNS tumors) have a mortality rate of ____ in _____. 

80% in 4 months

202

What is the most common intramedullary tumor in adults?

ependymoma (60%)

203

In the spnie, cellular ependymomas are most commonly in the _____ while myxopapillary are most commonly in the ____. 

cervical

 

conus/filum

204

What is the most common intramedullary spinal tumor in children?

astrocytoma

205

What subtype of astrocytoma is most commonly in the spine?

fibrillary

206
What is the most common location for aneurysms in the spine?
ASA in the cervical or thoracic spine
207
What is the difference between an AVM and an AVF?
AVM has true nidus, AVF does not
208
What is the most frequent spinal AVM?
AVF
209
Where is the most common location for type 1 spinal AVMs?
dorsal lower thoracic or upper lumbar spine
210
What defines a type 1 spinal AVM?
actually a dural AVF; single transdural arterial feeder that goes to an intradural arterialized vein over multiplesegmentsusually rostral venous drainagenidus is in or adjacent to the dura around a nerve root
211
Symptoms from type 1 spinal AVMs are generally caused by _____.
venous hypertension
212
A good surgical outcomes is achieved in _____ percent of type 1 spinal AVMs.
88%
213
What are the pressures and flows for all types of spinal AVMs?
Type 1: low pressure, low flowType 2: high flow, high pressureType 3: high flow, high pressureType 4: low pressure, high flow
214
Which type of spinal AVM is associated with aneurysm?
type 3 juvenile
215
What defines a type 2 (glomus) spinal AVM?
congenital; intramedullary with multiple feeders draining into a venous plexus around the cord
216
Where are type 2 (glomus) spinal AVMs usually located?
dorsal cervicomedullary
217
What defines a type 3 (juvenile) spinal AVM?
congenital; involves entire cross section of the cordlarge intramedullary and extramedullary malformation with multiple extrapinal feeders; bidirectional venous drainage
218
A good surgical outcomes is obtain in _____ perfect of type 3 (juvenile) spinal AVMs.
50%
219
What defines a type 4 spinal AVM?
intradural/extramedullary AVF; congenitalanterior to spinal cord fed by ASA
220
Where are type 4 spinal AVMs usually located?
near the conus
221
Which spinal AVMs are acquired? Which are congential?
Acquired: type 1Congenital: types 2, 3, 4
222
What is Foix Alajouanine syndrome?
Subacute necrotizing myelitis, especially in the gray matter, usually with a type 1 AVM, and caused by venous hypertensionpresents as spastic and then flaccid paraplegiawith an ascending sensory loss and loss of sphincter control
223
What is Klippel Trenaunay Weber syndrome?
spinal cord AVM with a cutaneous vascular nevus and an enlarged finger or upper limb (if cervical)
224
Cav mal in the spine is usually located in the _____ spine.
thoracic
225
Which region of the spinal cord is most often affected in spinal cord stroke?
mid thoracic
226
What is decompression sickness? What is the classic neurologic spinal presentation?
Intravascular accumulation of N2 with vessel obstructionfrequently causes spinal cord dysfunction in the posterior columns of the thoracic cord
227
What are the most common causes of pyogenic spinal infections?
Staph aureus (60%) and Enterobacter (30%)
228
What is the most common route of spread of infection to the spine?
hematogenous
229
The spine is involved in ____ of TB cases.
6%
230
In the acute stage of transverse myelitis, the MRI is normal in _____ of patients.
50%
231
Radiation myelopathy usually occurs following radiation to ______.
nasopharyngeal carcinoma in the cervical spine
232
What is pathologically seen in radiation myelopathy?
coagulative necrosis affecting the white matter more than the gray matter and thrombosed hyalinized vessels
233
On MRI, what do post radiation changes to vertebral bodies look like? Why?
T1 hyperintensityincreased fat content
234
What's another name for ankylosing spondylitis?
Marie-Strumpell disease
235
Ankylosing spondylitis is associated with HLA ___.
B27
236
The bamboo spine in AS is due to ______.
syndesmophytes and zygapophyseal joint fusion
237
Thoracic discs account for ____ percent of herniated discs and ____ percent are assymptomatic.
1%15%
238
What percent of herniated discs are foraminal? Far lateral?
3%4%
239
What is a Schmorl's node?
disc herniation through the endplate; seen in 75% of normal population
240
Spondylosis is present in ____ percent of the population over 50 years old.
70%
241
What are Sharpey fibers?
where the annulus is connected to the bone
242
Lumbar stenosis is most commonly due to hypertrophy of _____.
Superior articulating process
243
What is the normal cervical spine diameter?
18 mm
244
Congenital spinal stenosis is seen in what two syndromes?
achondorplasia and Morquio syndrome
245
What are the most common places for degenerative spondylolisthesis?
L4-L5 (66%) and L5-S1 (30%)
246
What are tarlov cysts?
nerve root cysts most common in sacral spine along dorsal nerve roots
247
A Charcot shoulder joint is highly suspicious for what neurologic finding?
cervical syrinx
248
Cord compression in Morquio syndrome is often do to a ______.
hypoplastic dens
249
In trauma, anterior cord syndrome is secondary to ____.
hyperflexion injury
250
In trauma, central cord syndrome is generally secondary to ______.
hyperextension injury
251
What is the role for steroids in nonpenetrating spinal cord injury?
methylprednisolone 30 mg/kg over 1 hour, followed by 5.4 mg/kg/hour for 23 hours if started within 3 hours of the injury or for 47 hours if started between 3 and 8 hours of injury to help decrease secondary injury
252
In AO dislocation, what is the Power's ration? Dens-basion distance?
> 112 mm
253
What is the Power ratio?
distance of the basion (B) to the posterior arch of the atlas (C) divided by the distance of the anterior arch of the atlas (A) to the opisthion (O)
254
What is a Jefferson's fracture? Stable or unstable?
b/l fx through the anterior and posterior arches of C1stable unless transverse ligament disrupted
255
Rotary atlantosubluxation is when ______ and is associated with _______.
C1 rotated over 45 degrees over C2flexion injuries, RA, Tonsilitis/pharyngitis (Grisel's syndrome)
256
Hangman's fracture is _____ and is caused by ______.
C2 traumatic spondylolisthesis with b/l pars fxshyperextension
257
Wallerian degeneration occurs (proximal/distal) to the site of injury.
distal
258
What are the Bands of Bungner?
proliferation of Schwann cells under the old basal lamina of a nerve with axons growing inside
259
What is segmental demyelination?
Scattered demyelination with replacement by thinner myelin and shorter variable internodes (normally the nodes of Ranvier have a set internodal length)relative axonal sparing
260
What kind of demyelination is seen with diptheria?
segmental
261
Porphyria causes what type of neuropathy?
Rapid, severe, symmetric, motor > sensory loss, bilateral brachial weakness
262
Uremia causes what type of neuropathy?
painless, symmetric, sensorimotor, lower > upper limbs
263
In GBS, pathologic examination reveals _____.
perivascular mononuclear infiltrates and segmental demyelination
264
When is the protein peak seen in GBS on CSF?
5 weeks
265
What do nerve conduction studies show in GBS?
decreased velocity and amplitude
266
What percent of GBS patients have severe/permanent weakness?
10%
267
Experimental allergic neuritis is caused by _____.
T cell mediated attack of P2 protein
268
What is the inheritance of Dejerine Sottas? What type of disease is it?
ARhereditary/hypertrophic (onion bulb) neuropathy
269
What is the inheritance of Refsum's disease?
AR
270
What is Refsum's disease?
a type of hereditary/hypertrophic (onion bulb) neuropathydeficiency in phytanic acid oxidase
271
What is neuropraxia?
functional but no structural damage (nerve concussion) with temporary loss of function that may last 6–8 weeks
272
What is axonotemesis?
interruption of axons and myelin with intact perineurium and epineuriumspontaneous regeneration may occur at 1–2 mm/day
273
What is neurotemesis?
transection of the nerve and nerve sheathaxonal regeneration may lead to neuroma formation
274
Acute ophthalmoplegia can be seen in what metabolic disease? What is the mechanism?
diabetesischemia of the vaso nervosum
275
What is a Morton neuroma?
traumatic neuroma that forms on the digital nerve between the toes
276
What is Brachial Plexitis?
Idiopathic onset of upper limb pain and weakness that usually resolves in 6 to 12 weeks; there is no fever, leukocytosis, or elevated ESR
277
In carpal tunnel syndrome, what causes the compression?
transverse carpal ligament
278
What causes cubital tunnel syndrome?
caused by compression of the ulnar nerve under the two heads of the flexor carpi ulnaris
279
What is posterior interosseous syndrome?
Causes weakness of the radial-innervated forearm and hand muscles (supinator, extensor digitorum, extensor carpi ulnaris, and abductor pollicis longus). No sensory loss. It causes a finger drop without a wrist drop because of sparing of the extensor carpi radialis longus
280
What is anterior interosseous syndrome?
Pure weakness without sensory loss caused by compression of the anterior interosseous branch of the median nerve in the deep forearm. It involves the pronator quadratus, flexor pollicis longus, and flexor digitorum profundus 2 and 3 (FDP 4 and 5 are innervated by the ulnar nerve). Patients are unable to form the “okay” sign and demonstrate the “pinched” sign
281
What is meralgia paresthetica?
Compression of the lateral femoral cutaneous nerve (L2, 3) under the inguinal ligament. It causes anterolateral thigh numbness and dysesthesia. It is associated with obesity, pregnancy, and diabetes.
282
What is tarsal tunnel syndrome?
Compression of the tibial nerve with paresthesias of the sole of the foot without motor changes
283
Patients with b/l CN VII nerve palsies are suspicious for ______.
GBS and Lyme disease
284
On clinical exam, what distinguishes botulism from Myesthenia gravis?
botulism has unreactive pupils, MG has reactive
285
What are the differences between type 1 and type 2 muscle fibers?
type 1: red, slow, aerobic metabolismtype 2: white, fast, anaerobic
286
In the sarcomere unit, which bands shorten with contraction?
H and I bands
287
Steroids cause atrophy of what muscle fibers?
type 2
288
What is the inheritance of Duchenne and Becker's MD?
XR
289
What is seen on biopsy for Duchenne MD?
muscle fiber necrosis and regeneration
290
What is the inheritance of Facioscapulohumeral dystrophy? What chromosome is affected?
ADCh 4
291
What is the only MD with chronic inflammatory cells in the muscle?
Facioscapulohumeral dystrophy
292
What is the most common MD in adults? Whats the inferitance?
Myotonic MDAD
293
What is the genetics of myotonic MD?
trinucleotide repeat on Ch 19
294
What is the treatment for myotonic MD?
quinine or procainamide or phenytoin
295
What are some key clinical characteristics of myotonic MD?
muscles are unable to relax after contraction (myotonia), dysrhythmias, decreased intelligence, cataracts (90%), endocrine dysfunction (with testicular atrophy), temporalis and masseter atrophy, and frontal balding in both sexes
296
The inheritance of the glycogen storage diseases is _____.
AR
297
What is Pompe disease?
infantile form of acid maltase deficiency
298
What is the deficiency in McArdle's disease?
myophophorylase deficiency
299
What are the symptoms of McArdle's disease?
muscle cramps with activity
300
Phosphofructokinase is similar to what disease?
McArdle's disease
301
What type of disease is Lafora's disease?
glycogen storage disease
302
Which two diseases have lipid storage in vacuoles?
carnitine deficiency and Fabry disease
303
What is the inheritance of malignant hyperthermia?
AD
304
What is the mechanism of action of dantrolene?
reducing the Ca2+ release from the sarcoplasmic reticulum
305
What is the defect in malignant hyperthermia?
defect in a Ca2+ release channel (ryanodine receptor) with increased Ca2+ release from the sarcoplasmic reticulum
306
What is the cause for bacterial myositis?
staph aureus
307
What is the most frequent acquired inflammatory myopathy in adults?
polymyositis
308
What is seen on biopsy in polymyositis?
T cells and macrophages in the muscle fibers; inflammation in the endomysium
309
What percent of dermatomyositis is associated with cancer?
15%
310
Inclusion body myositis has what type of inclusion?
intranuclear inclusion

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