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Citow Review Book > Pathology > Flashcards

Pathology Flashcards

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1
Q

The Nissl stain is for ________ and binds ______.

A

neuronal bodies

nucleic acid

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2
Q

Lipofuscin accumulates in neurons due to ____.

A

aging

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3
Q

True melanin is made by _______ and is located in the _______.

A

tyrosinase

leptomeningeal melanocytes of the ventral medulla and cervical cord

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4
Q

Cytotoxic edeme is caused by ________.

A

impaired Na/K ATP pump

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5
Q

What is Ferruigination?

A

when dead neurons become encrusted with Fe and Ca

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6
Q

When does central chromatolysis occur?

A

injury to an axon near the cell body

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7
Q

What occurs in chromatolysis?

A

Nissl substance disappears, the nucleus becomes eccentric, and the cell body enlarges

cells may recover or progress to death

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8
Q

Silver stain stains ______.

A

cellular processes

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9
Q

Neurofibrillary tangles can be seen on what stain?

A

sillver stain

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10
Q

Subacute pansclerosing encephalitis is caused by _______.

A

measles

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11
Q

What viral infections have intranuclear inclusion bodies?

A

HSV 1, CMV, measles (the last two also have cytoplasmic inclusion bodies)

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12
Q

What are negri bodies? When are they seen?

A

intracytoplasmic inclusion bodies

rabies

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13
Q

Metabolic/degnerative inclusion bodies are all _______.

A

intracytoplasmic

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14
Q

Lewy bodies are seen in ______ and have a characteristic ______. They are located in the ______.

A

Parkinson’s disease

halo

cytoplasm

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15
Q

Lafora bodies can be stained with ______.

A

PAS

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16
Q

Hirano bodies are seen in _______ and are most commonly seen in the brain in the _______.

A

alzheimer’s disease

hippocampus

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17
Q

What are Bunina bodies?

A

intracytoplasmic inclusion bodies seen in ALS

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18
Q

What is a hamartoma?

A

disorganized cells located in the proper location

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19
Q

What is a choristoma?

A

properly organized cells in the wrong location

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20
Q

What causes duret hemorrhages during herniation?

A

arteriole stretching

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21
Q

What causes the Kernohan’s notch presentation?

A

contralateral cerebral peduncle compressed against the incisura

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22
Q

<p>What are rosenthal fibers?</p>

A

<p>eosinophilic masses in the astrocytic processes</p>

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23
Q

<p>Where are rosenthal fibers seen?</p>

A

<p>Pilocytic astrocytomas, Alexander disease, and reactive (secondary) astrocytosis</p>

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24
Q

<p>What are alzheimer type II astrocytes? When are they seen?</p>

A

<p>type of primary astrocytosis; large vesicular gray nuclei with glycogen inclusions in the cytoplasm</p>

<p></p>

<p>hepatic encephalopathy</p>

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25

When ependymal cells are injured, what replaces them?

subependymal astrocytes 

26
The brain and spinal cord are embryologically derived from _____ during weeks ______.
neuroectoderm 3-8
27
Primary neurulation occurs at ______.
3-4 weeks
28
What induces the primitive streak to form the neural plate?
notochord
29
The primitive streak forms POD ____.
POD 13
30
The notochord forms POD _____.
POD 17
31
The neural folds fuse on ______ to form _____.
POD 22 neural tube
32
The primitive streak forms the _____ which forms the _____ which forms the ______.
neural plate neural groove neural folds
33
The anterior neuropore closes on ______ forming the _______.
POD 24 lamina terminalis
34
Posterior neuropore closes on _____.
POD 26
35
What is dysjunction?
separation of the ectoderm from neuroectoderm after the neural tube forms
36
What happens when dysjunction occurs to early?
mesenchyme can enter the neural tube and form lipomas and lipomyelomeningoceles
37
Focal failure of dysjunction causes _______.
epithelial lined dermal sinus tract
38
Widespread failure of dysjunction can cause ______.
myelocele or myelomeningocele
39
Secondary neurulation occurs at _______ (days/weeks)
4-5 weeks
40
Problems with secondary neurulation cause _____.
spinal dysraphism below L1/2
41
What is ventral induction? When does it occur?
5-10 weeks PO; primary vesicles form from the neural tube
42
Abnormalities during ventral induction cause ________.
holoprosencephaly, septic optic dysplasia, and dandy walker malformation
43
Neuronal proliferation, differentiation, and migration occur around _______.
2-5 months
44
When does myelination start? When does it end?
5 months prenatalby 2 years post natal
45
What is the progression of myelination during CNS development?
caudad to cephalad, dorsal to ventral, central to peripheral, and sensory before motor
46
The prosencephalon divides into ________.
telencephalon and diencephalon
47
What structures arise from the telencephalon?
hemispheres, caudate, putamen, fornices, anterior commissure, corpus callosum, and hippocampus
48
What structures arise from the diencephalon?
thalamus, globus pallidus (GP), posterior hypophysis, infundibulum, optic nerve, retina, posterior commissure, and habenular commissure
49
The mesencephalon forms the ______.
midbrain
50
The rhombencephalon divides into ________.
metencephalon and myelencephalon
51
The metenecephalon forms the ______.
pons, fourth ventricle, and cerebellum
52
The myelencephalon forms the ______.
medulla
53
The germinal matrix forms at _______ and involutes at _______.
7 weeks GA 30 weeks GA
54
What is the directionality of the formation of the corpus callosum?
front to back except for the rostrum which forms last
55
What are the basal and alar plates?
prelimary structures to the brainstem
56
The basal plate contains somatic, special, and visceral _______ while the alar plate contains _______.
efferent afferents
57
Which subset of nuclei lie the most medial on the basal and alar plates?
somatic efferents/afferents
58
What is formed from the neural crest cells?
leptomeninges, Schwann cells, sensory ganglia of the CNs, dorsal root ganglia (DRG), autonomic nervous system ganglia, the adrenal medulla, melanocytes, and amine precursor uptake and decarboxylation (APUD) cells
59
What is derived from the ectodermal placodes?
olfactory epithelium, CN V and CN VII to CN X ganglia
60
Chordomas are believed to embryologically derive from _______.
notochord remnants
61
The dura is derived from what embryological layer?
mesoderm
62
The pia/arachnoid are derived from what embyrological layer?
neuroectoderm
63
When does the metopic suture close?
around 1 year
64
When does the anterior fontanelle close?
around 2.5 years
65
When does the posterior fontanelle close?
2-3 months
66
When does the sphenoid fontanelle close?
2-3 months
67
When does the mastoid fontanelle close?
around 1 year
68
What is a malformation?
when an organ is not formed properly
69
What is dysplasia?
when tissues are not formed properly
70
What is the most common congential malformation?
anencephaly
71
In anencephaly, what is increased in the amniotic fluid?
AFP and acetylcholinesterase
72
What is craniorachischisis?
complete exposure of the brain and spine (think extreme anencephaly)
73
What two drugs are associated with an increased risk of myelomeningocele?
valproic acid and carbamazepine
74
What is the most frequent type of encephalocele in Southeast Asian communities?
Sincipital (frontoethmoidal) encephalocele
75
Which type of encephalocele is associated with dermoids and epidermoids?
Sphenoehtmoidal (nasal) encephalocele
76
What is the most common type of encephalocele in the West?
occipital encephalocele
77
What is Meckel Gruber syndrome? What is a risk factor?
characterized by cystic dysplastic kidneys, cardiac anomalies, orofacial clefting, and cephaloceles maternal hyperthermia on days 20–26 of gestation
78
What percent of dermal sinus tracts end in epidermoids or dermoids?
50%
79
What are the most common locations for dermal sinus tracts?
lumbar followed by occipital
80
What is arrhinencephaly? What diseases is it associated with?
absence of the olfactory bulbs and tracts with normal cortex and gray matter in place of the corpus callosum Kallman's syndrome and holoprosencephaly
81
What's another name for de Morsier's syndrome?
septooptic dysplasia
82
What is septooptic dysplasia?
mild lobar holoprosencephaly, absence of the septum pellucidum, schizencephaly, and hypoplastic optic nerves associated with seizures, visual symptoms, hypothalamic–pituitary dysfunction (precocious puberty), enlarged ventricles, and hypotelorism
83
What is cleidocranial dysostosis?
occurs with retention of mandibular teeth, delayed closure of fontanelles, wormian bones, and midline defects
84
Posterior fossa arachnoid cyst (will/will not) fill with intrathecal contrast.
will not fill
85
What is Lhemitte Duclos disease?
hypertrophied cerebellar granular cell layer and increased myelin in the molecular layer of the cerebellum with thick folia; considered a hamartoma
86
What syndrome is Lhemitte Duclos associated with ?
Cowden syndrome
87
What is the presentation of Cowden syndrome?
facial trichilemmomas, fibromas of the oral mucosa, hamartomatous polyps of the gastrointestinal (GI) tract and breast, and thyroid tumors (as well as cerebellum)
88
What is the genetic abnormality in Cowden syndrome?
PTEN mutation on Ch 10 (ten on ten)
89
What is syringobulbia?
fluid filled cavity in the brainstem
90
What is the inheritance of Crouzon syndrome?
AD or sporadic
91
What is the mutation in Crouzon syndrome?
fibroblast growth factor receptor 2
92
What is the presentation of Crouzon syndrome?
shallow orbits, exophthalmos, midface hypoplasia, malformed ears, agenesis of the corpus callosum, less severe mental retardation than with Apert syndrome, and increased incidence of hydrocephalus. More than one suture is involved, and they may have oxycephaly,turricephaly, or dolichocephaly
93
What is the mutation in Alpert syndrome?
fibroblast growth factor receptor
94
What is the presentation of Alpert syndrome?
more severe Crouzon; many whole body abnormalities; ONLY THE CORONAL SUTURE IS INVOLVED (turricephalic)
95
What is Klippel Feil syndrome?
congenital fusion of the upper cervical vertebrae
96
What is Klippel Feil syndrome associated with?
Sprengel’s deformity (elevation of the scapula) and Chiari I malformation
97
Which of the Trisomies is associated with rocker bottom feet?
18 (edwards)
98
Which of the trisomies is assoociated with polydactyly?
13 (Patau)
99
What is the mutation in cri-du-chat?
5p deletion
100
What cerebellar abnormality is seen in Fragile X syndrome?
vermian hypoplasia
101
What is the mutation in Fragile X syndrome?
trinucleotide repeat in FMR1 gene
102
What causes ataxia telengectasia?
defective DNA repair
103
What is the presentation of ataxia telengectasia?
characterized by cerebellar atrophy, lentiform nucleus calcifications, pachygyria, impaired immune system, increased lymphoreticular carcinoma
104
What is caput succedaneum?
cutaneous hemorrhagic edema in the skin over the calvarium caused by pressure during birth with vascular stasis. It crosses sutures and resolves in 48 hours
105
What is a cephalohematoma?
Subperiosteal blood that does not cross suture lines. It is usually parietal and may calcify
106
What is occpital osteodiastasis?
perinatal injury traumatic separation of the squamous and lateral occipital bone with tearing of the occipital sinus and bleeding in the posterior fossa
107
What percentage of premature births have PIVH?
40%
108
What percent of full term births have PIVH?
3-7%
109
Where does PIVH originate from?
germinal matrix
110
What increases the risk of PIVH?
prematurity and ARDS
111
What is the morbidity and mortality of PIVH?
15-40%, 20-60%
112
What is colpocephaly?
dilated occipital horns associated with agenesis of the corpus callosum and periventricular leukomalacia, mental retardation, and seizures
113
What is status marmoratus?
thalamus, neostriatum, and cortex develop irregular intersecting bands of myelin and astrocytic fibers that grossly resemble marble caused by cell loss followed by remyelination
114
How is elevated bilirubin in neonates treated?
phototherapy using ultraviolet light, but if the bilirubin is > 20 with associated sepsis, prematurity, acidosis or low albumin, treat with an exchange transfusion
115
What lines the cavity in porencephaly?
gliotic white matter
116
What lines the cavity in schizencephaly?
grey matter
117
What is hydraencephaly?
cortex is mostly replaced by CSF (water head)
118
The most frequent pathogen overall for meningitis is _____.
H Flu
119
The most frequent cause of meningitis in adults is _______.
Strep pneumo
120
The three most common causes of meningitis are ________ and they normally colonize the ______.
H Flu, Strep pneumo, Neisseria meningitidis nasopharynx
121
Most common cause of shunt infections is ______.
S. epidermidis
122
What is the mortality rate of the most common types of meningitis?
10% (H. influenzae and N. meningitidis), 25% (S. pneumoniae), and 50% (neonatal, with 50% of survivors having permanent sequelae
123
In the neonate period (<4 weeks), what are the three most common causes of meningitis?
Group B strep, E coli, and Listeria
124
What is the most common cause of meningitis in the 4-12 week period?
Strep pneumo
125
What is the most common cause of meningitis in the 3 month to 3 year period?
H flu
126
B/l subdural effusions is associated with what meningitic infections in infants? What is it due to? What can it cause?
H flu mostly and Strep pneumo increased permeability of blood vessels seizures
127
Steroids in meningitis has been show to do what?
decrease incidence of deafness in children
128
What is the most common cause of meningitis in children and young adults?
N meningitidis
129
What is the most common cause of meningitis in the elderly?
Strep pneumo
130
What is the most common pathogen for brain abscess?
streptococcus
131
What is the most common cause/source of cerebral abscess?
ear or sinus infection spreading along valveless venous channels (40%)
132
What percent of cerebral abscesses are secondary to hematogenous spread?
33%
133
What's the incidence of brain abscess in cyantoic heart disease? What causes it?
5% due to decreased pulmonary blood filtration and lower oxygen tension in the brain
134
What's the incidence of brain abscess in hereditary hemorrhagic telangiectasia? What is the reason?
5% presence of pulmonary AVMs
135
What are the most common pathogens of brain abscess in neonates?
Citrobacter, Bacteroides, Proteus, and Gram-negative bacilli
136
In trauma, what is the most frequent pathogen in brain abscesses?
Staph
137
What percent of subacute bacterial endocarditis develop infectious aneurysms?
10%
138
What are the most common pathogens in encephalitis?
Legionella (CSF culture is usually negative), Mycoplasma, Listeria (in neonates and immunosuppressed patients, CSF culture is usually positive), and Brucella
139
What are the most common pathogens for subdural empyema?
Streptococcus and Bacteroides from an adjacent infection
140
What is Gradenigo syndrome?
petrous apex osteomyelitis with CN VI palsy and retro-orbital pain and may occur in children from extension of severe otitis
141
Pathologic examintion in TB meningitis shows _____.
demonstrates granulomas with caseating necrosis, lymphocytes, and Langerhans giant cells
142
What is the morbidity and mortality of TB meningitis?
80% and 30%
143
The nervous system is involved in ______ of cases of sarcoidosis.
5%
144
________ levels are elevated in sarcoidosis.
serum ACE
145
What is Whipple disease? What is seen on pathology?
chronic multisystem disease caused by Tropheryma whippelii characterized by weight loss, abdominal pain, diarrhea, lymphadenopathy, arthralgia, and Alzheimer’s disease-like neurologic symptoms (10%) demonstrates foamy macrophages with PAS-positive granules
146
What percent of cases of syphillis involve the CNS?
25%
147
What are the four types of syphilis?
- Meningovascular syphilis - general paresis of the insane - tabes dorsalis - Congenital syphilis
148
Which type of syphilis is associated with multiple ischemic strokes?
meningovascular
149
Which type of syphilis is associated with Argyl robertson pupils?
general paresis of the insane and tabes dorsalis
150
Which type of syphilis is associated with hutchinson's triad? What is hutchinson's triad?
congential and meningovascular notched teeth, deafness, and interstitial keratitis
151
What are the features of tabes dorsalis?
occurs after 15–20 years from syphilis infection characterized by myelopathy from meningealfibrosis; mainly dorsal root and posterior column involvement, W-shaped demyelination in the thoracic and lumbar spinal cord from the posterior horns inward, lightning-like pains, sensory ataxia, urinary incontinence, decreased lower limb deep tendon reflexes, decreased proprioception and vibratory sense, positive Romberg’s test, Argyll Robertson pupils (90%),ptosis, optic atrophy, and Charcot joints of the hip, knee, and ankle
152
What cranial nerve is most commonly affected in Lyme disease?
CN VII
153
What is the pathogen in Lyme disease?
Borrelia burgdorferi
154
What is the pathogen in syphilis?
Treponema pallidum
155
All fungi stain with ________.
methenamine silver
156
What is the most frequent CNS fungal infection?
Candida
157
Blastomycosis is associated with what part of the US?
eastern
158
Histoplasmosis is associated with what part of the US?
Ohio, Mississippi, and St Lawrence river valleys
159
What is the most frequent fungal meningitis?
Cryptococcus
160
Cryptococcus stains with ______ forming the distinct ______.
india ink halos
161
What part of the US is Coccidioidomycosis from?
southwest
162
What is the pathogen in Cysticerosis?
Taenia solium
163
What is the most frequent brain mass in AIDS patients?
Toxoplasmosis
164
Congential toxoplasmosis lesions include ______.
brain necrosis, periventricular calcification, hydrocephalus, hydranencephaly, chorioretinitis, and hepatosplenomegaly.
165
What is the mortality in overall viral encephalitis?
5-20%
166
What is the mortality in HSV encephalitis?
50%
167
What is the incidence of permanent sequelae in patient's who survive encephalitis?
20%
168
What is seen on pathology for viral encephalitis?
perivascular mononuclear infiltrates
169
HSV is found in the trigeminal ganglion in percent of adults?
50%
170
What is the most frequent cause of sporadic encephalitis?
HSV
171
What are Cowdry type A inclusions?
intranuclear eosinophilic masses with a surrounding halo found in neurons, oligodendrocytes, and astrocytes seen in HSV and CMV
172
What are the pathologic findings in CMV?
Cowdry type A and intracytoplasmic inclusions
173
What findings are seen in congenital CMV infection?
microcephaly, hydrocephalus, chorioretinitis, and microphthalmia
174
What percent of shingles involve trigeminal nerve? which branch?
15% at V1
175
Herpes infection of the geniculate ganglion is called ______.
Ramsey Hunt Syndrome
176
What are the pathologic findings in Varicella?
Intranuclear inclusions in the DRG and the posterior horn gray matter, posterior roots, and meninges
177
What are Negri bodies? Where are they usually seen?
Intracytoplasmic eosinophilic collections of ribonucleoproteins rabies infection in cerebellum (Purkinje cells), brainstem, and hippocampus
178
What are the symptoms of rabies infection?
Anxiety, dysphagia, and spasms of the throat when attempting to swallow (hydrophobia)
179
Which cells are most affected by polio virus?
anterior horn cells
180
What virus is associated with Progressive multifocal leukoencephalopathy (PML)?
JC virus
181
What inclusions are seen in PML?
intranuclear inclusions of “stick-and-ball” viral particles
182
What are the clinical findings in CJD?
myoclonus, pyramidal, and extrapyramidal degeneration, dementia, ataxia, and visual deterioration
183
What are the EEG findings with prion disease?
bilateral sharp waves of 1–2 wave/s that resembleperiodic lateralized epileptiform discharges, but are reactive to painful stimuli
184
What is seen on pathologic changes in prion disease?
spongiform changes with astrocytosis but no inflammation most prominently in the cortex, putamen, and thalamus
185
What is unique on pathologic examination of HIV encephalitis?
the presence of multinucleated giant cells
186
What is Rasmussen chronic encephalitis?
occurs in childhood and causes progressive deficits and seizures (classically complex partial status epilepticus) with unilateral atrophy. It may be caused by CMV infection or antibodies to glutamate receptors
187
What is the most frequent congenital CNS infection?
CMV
188
In the CNS, CMV has an affinity for ______ and causes ________.
germinal matrix perivascular necrosis and calcifications
189
What is the triad of congenital toxoplasmosis infection?
hydrocephalus, bilateral chorioretinitis, and cranial calcifications
190
What is the most common late manifestation of congenital toxoplasmosis?
deafness
191
Congenital rubella syndrome consists of what clinical presentation?
chorioretinitis, cataracts, glaucoma, microphthalmia, microcephaly, mental retardation, and deafness
192
In congenital syphilis, what is hutchinson's triad?
Dental disorders, bilateral deafness, and interstitial keratitis
193

What percent of brain tumors are infratentorial in children?

70%

194

What are the most common infratentorial brain tumors in children?

cerebellar astrocytoma (33%), brainstem glioma (25%), medulloblastoma (25%), and ependymoma (12%)

195

What are the most frequent supratentorial brain tumors in children?

low-grade astrocytomas (50%), craniopharyngiomas (12%), and optic gliomas (12%)

196

In neonates and infants, are tumors more commonly supratentorial or infratentorial? What are the most common types?

supratentorial most common is teratoma, followed by primitive neuroectodermal tumors (PNETs), highgradeastrocytoma, and choroid plexus papilloma
197

In older children, are tumors more commonly supratentorial or infratentorial? What are the most common types?

infratentorial astrocytoma (50%), PNET (15%), craniopharyngioma (10%), ependymoma (10%), and pineal tumor (3%)

198

What is the genetic mutation in Turcot syndrome?

APC mutation on Ch 5

199

What is the triad of Turcot syndrome?

familial polyposis, colorectal cancer, and primary brain tumors

200

Synaptophysin stains tumors with ______.

neurons

201

Which phases of the cell cycle are sensitive to radiation? Which are resistant?

G1 and M phase S phase

202

Which tumors stain positive for bHCG?

choriocarcinoma and the syncytiotrophoblastic variant of germinomas

203

Which tumors stain positive for desmin?

rhabdosarcoma, teratoma

204

Mitotic index measures _____.

which cells are in the M phase (misleading as cells can be dividing but not in M phase)

205

What does flow cytometry measure?

stains DNA and measures how many cells have double DNA

206

Ki67 is expressed in all stages of the cell cycle except______.

G0

207

Burkitt lymphoma is associated with which oncogene?

c-myc

208

What oncogene is associated with neuroblastoma?

n-myc oncogene 9

209

What percent of astrocytomas have p53 mutations?

33%

210

What chromosome is p53 located on?

17p

211

What are Anti-Yo antibodies? What are they associated with?

paraneoplastic cause cerebellar degeneration associated with breast and ovarian

212

What are Anti-Hu antibodies? What are they associated with?

paraneplastic sensory neuropathy, encephalitis, and cerebellar degeneration oat cell pulmonary carcinoma and lymphoma

213

What are Anti-Ri antibodies? What are they associated with?

paraneoplastic cause ospoclonus associated with breast ca

214

What is a paraneoplastic cause of Stiff man syndrome?

antibodies to glutamic acid decarboxylase

215

What are radiation doses for metastatic tumors generally?

30 Gy over 2 weeks

216

What are radiation doses for gliomas generally?

6,000 cGy in 200 cGy daily fractions

217

What is seen pathologically with radiation necrosis?

White matter coagulation necrosis or demyelination associated with arterioles with hyalin intimal thickening, fibrinoid necrosis, and thrombosis

218

When do radiation necrosis symptoms usually manifest?

Symptoms usually start between 3 months and 3 years (average 15–18 months) after radiation

219

Radiation myelopathy risk is reduced if dosing kept under _____ daily, ____ weekly, and _____ total.

200 cGy 900 cGy 6,000 cGy

220

What are the three types of astrocytes?

Fibrillary, Protoplasmic, and Gemistocytic

221

What WHO grade is a Juvenile Pilcytic Astrocytoma?

WHo grade I

222

What are the most common locations for a JPA?

cerebellum, brainstem, optic pathway, and infundibulum

223

What is the most common place for a JPA in an adult?

near the third ventricle

224

What are key features for JPAs radiographically?

60% are cystic with a mural nodule (red-tan nodule) 

225

What is seen on pathology for JPAs?

biphasic pattern of loose cells and microcysts and also dense elongated hair-like astrocytes with Rosenthal fibers and eosinophilic granular bodies

226

What is the survival rate for JPAs?

86–100% at 5 years, 83% at 10 years, and 70% at 20 years

227

What are the key radiographic findings in plemorphic xanthoastrocytoma?

predilection temporal lobe (seizures), cystic with mural nodule

228

PXAs are WHO grade ______. 

Grade II

229

What is seen on pathology in PXAs?

bizarre pleomorphic astrocytes with xanthomatous fat cells, spindle cells, and multinucleated cells, frequent mitoses, calcifications, a rich reticulin network, and no necrosis

 

230

What are the key radiographic findings in subependymal giant cell astrocytoma?

located near the foramen of Monro, enhance, have frequent calcifications, may be cystic and lobulated, and tend to be well demarcated

231

What percent of tuberous scleorsis patients have SEGA?

15%

232

What are the pathology findings with SEGA?

large multinucleated cells and rare mitoses

233

What is the most common type of diffuse astrocytoma?

fibrilary

234

Which type of diffuse astrocytoma has the worse prognosis?

gemistocytic

235

What percent of astrocytomas are grade II?

15%

236

What percent of grade II astrocytomas increase in grade over time?

50%

237

What is the median survival rate of grade II astrocytomas?

5-year survival rate with total resection and radiation is 70% and with subtotal resection and radiation is 38%. Median survival rate is 8.2 years

238

What percent of astrocytomas are grade III?

30%

239

What is the median survival rate for grade III astrocytomas?

2-3 years

240

What percent of astrocytomas are GBMs? What percent of all brain tumors are GBMs?

50% 

 

20%

241

What is seen on pathology for GBM?

heterogeneous with cysts, degeneration, necrosis, hemorrhages, edema, marked hypercellularity, nuclear atypia, frequent mitoses, pseudopalisading, and endothelial hyperplasia with glomeruloid structures

242

What percent of GBMs are multicentric?

3-6%

243

Epithelial growth factor receptor is on Ch ____ and mutations are associated with what tumor?

7

 

GBM

244

IDH1 mutation patients have (better/worse) outcomes. 

better

245

What is the MGMT gene? What is its significance in GBMs?

Epigenetic silencing (DNA methylation) of the MGMT gene, which encodes a DNA repair protein and represents an important mechanism of chemotherapy resistance, is an independent predictor of improved survival as well as survival benefit from temozolomide

246

What is the difference between adult and juvenile pilocytic astrocytoma?

adult version is not well circumscribed and has worse prognosis

247

What percent of GBMs are gliosarcoma?

2%

248

What stains are useful in the evaluation of gliosarcome?

Silver stains the reticulin in the sarcoma
component and GFAP stains the GBM component

249

Malignant optic gliomas are associated with what disease?

NF-1

250

What is the treatment paradigm for optic gliomas?

(1) distal to the chiasm, remove optic nerve and attached globe


(2) If the chiasm is involved, resect up to the chiasm preserving vision in the better eye and consider radiation if tumor progression is noted

251

What percent of intracranial tumors in children are brainstem gliomas?

about 20%

252

What mutation is associated with brainstem gliomas?

H3 K27M

253

What percent of gliomas are oligoendrogliomas?

10%

254

Immunohistochemistry in oligodendrogliomas is postivie for ______. 

GFAP and S100

255

What is seen on pathology for oligodendrogliomas?

round nuclei with scant cytoplasm, a chicken-wire vascular pattern with thin vessels

 

fried egg yolk-appearing cells and nucleus are caused by an artifact from cytoplasmic retraction seen in permanent, but not in frozen sections

256

What's the survival rate for oligodendrogliomas?

low grade tumors have a 5-year survival rate of 74% and 10-year survival rate of 46%

 

high-grade tumors have a 5-year
survival rate of 41% and a 10-year survival rate of 20%.

257

What percent of oligodendrogliomas have calcifications?

85%

258

What chemotherapy is used for oligodendorgliomas?

Procarbazine, carmustine, and vincristine (PCV) or temozolomide chemotherapy

259

What is the most common genetic mutation with oligodendrogliomas? What does it entail for prognosis?

Combined loss of heterozygosity of chromosomes 1p and 19q (the most common genetic alteration in oligodendrogliomas) is associated with a better response to chemotherapy and improved progression-free and overall survival in patients with anaplastic oligodendrogliomas

260

Ependymomas account for _____ of intramedullary spinal tumors and usually occur at the _____. 

60%

 

filum

261

Multiple spinal cord ependymomas is associated with ______. 

NF-2

262

What percent of ependymomas have calcifications?

50%

263

What are the four types of ependymomas?

- cellular

- papillary

- myxopapillary

- clear cell

264

Which type of ependymoma histologically resembles oligodendrogliomas?

clear cell

265

What is seen on pathology with cellular ependymomas?

heetlike growth of polygonal cells with true rosettes (around a central canal), pseudorosettes (around a blood vessel), and blepharoplasts (ciliary basal bodies in the apical cytoplasm)

266

Ependymoma immunohistochemistry is positive for ____. 

GFAP and PTAH

267

What is the survivial rate for ependymomas?

45% at 5 years

268

What is the histology of the ependyma?

single layer of cuboidal/columnar cells that are ciliated have microvilli; dual epithelial–glial nature and lie over the subependymal glia

269

Where are subependymomas most commonly located?

lateral ventricle or inferior fourth ventricle

270

What is seen on histology with subependymomas?

nests of cells separated by glial fibers

271

______ are the only intraventricular tumors that do not enhance. 

subependymomas

272

What is the most common location for choroid plexus paplilomas in children? In adults?

left atrium of lateral ventricle

 

fourth ventricle

273

What is seen on pathology for choroid plexus paplilomas?

cauliflower papillary shape with cuboidal and columnar cells and no cilia (except in children)

274

What does immunohistochemistry show for choroid plexus papilomas?

positive for transthyretin, vimentin, keratin, S100, and GFAP

275

What is radiographically seen with ganglioglioma?

temporal lobe, well circumscribed, cystic, firm, and often has a calcified nodule

276

What is seen on pathology for ganglioglioma?

perivascular inflammatory cells, reticulin, glia, and BINUCLEATE neurons

277

What does immunohisotchemistry show for ganglioglioma?

positive for neurofilament, synaptophysin, neurosecretory granules, and GFAP

278

What is seen radiographically demoplastic infantile ganglioglioma?

massive frontal cystic lesion that's adherent to the dura

279

What is the immunohistochemistry for desmoplastic infantile ganglioglioma?

GFAP +

 

EMA -

280

What cerebral structural abnormality is associated with dysembryoplastic neuroepithelial tumor?

cortical dysplasia

281

Histologically what separates DNET from ganglioglioma?

DNET: cortical with normal neurons and abnormal oligo and astrocytomas

 

Ganglioglioma: white matter with abnormal neurons 

282

Where do central neurocytomas originate from?

septum pellucidum

283

What is seen on pathology in central neurocytoma?

demonstrates monotonous hypercellularity similar to oligodendrogliomas with rare mitoses, frequent cysts, and occasional hemorrhages

284

What is seen on immunohisotchemistry in central neurocytoma?

positive for synaptophysin

285

What are medulloblast cells? Where do they originate?

bipotential cells capable of differentiating into glia or neurons

 

postulated that they are derived from the external granular layer of the cerebellum or from dysplastic cell rests in the anterior and posterior medullary velum

286

Medulloblastomas are WHO grade _____. 

iV

287

  What is the most common genetic abnormality in medulloblastoma?

isochromosome 17q

288

What syndrome is associated with medulloblastoma? What is the genetic cause of this syndrome?

Gorlin syndrome (basal nevus syndrome)

 

mutation of PTCH on Ch 9q

289

What is the most common extracranial malignant solid tumor in children?

retinoblastoma

290

Retinoblastoma derives from _____. 

neural crest precursor of the sympathetic ganglia

291

What are the key pathology features for retinoblastoma?

Flexner-Wintersteiner and Homer-Wright rosettes

292

ATRTs are WHO grade _____. 

IV

293

Pineoblastoma are WHO grade ____. 

IV

294

What is seen on pathology for ATRTs?

densely cellular blue cell tumors mixed with rhabdoid cells. Rhabdoid cells have eosinophilic rounded, rhabdoid cytoplasmic inclusions

 

 

295

What is the genetic associated of ATRT?

deletions of chromosome 22 containing the INI1/hSNF5 gene

296

What is seen on pathology for medulloblastoma?

Closely packed undifferentiated cells with no discernable cytoplasm (small blue cells).

297

How does central neuroblastoma appear on imaging?

usually supratentorial, hemispheric, and circumscribed

298

What is the survival rate of central neurocytoma?

30% at 5 years

299

Explain the different between pseudorosettes, homer wright rosettes, and flexner wintersteiner rosottes?

Pseudo: around blood vessel

 

Homer Wright: around central granular fibrilar substance

 

Flexner Wintersteiner: columnar cells with a small lumen seen with retinoblastomas and also pineoblastomas

300

What percent of primary intracranial tumors are meningiomas?

15%

301

What genetic abnormality is common seen in meningiomas?

72% of tumors have monosomy 21

302

True ependymal rosettes are most commonly seen in _____.

ependymoma

303

Meningiomas originate from _______.

Arachnoid cap cells

304

What is seen on pathology for meningioma?

basophilic psammoma bodies and whorls

305

What is seen with meningioma on angiography?

sunburst pattern of dural feeders

306

What does immunohistochemistry show for meningiomas?

positive for EMA and vimentin

307

What percent of meningiomas are grade I?

92%

308

What is the survival rate for grade II meningiomas? What percent recur?

30% in 5 year survival rate

50% recur in 1.5 years

309

What is Foster-Kennedy Syndrome?

optic atrophy in one eye and papilledema in the other with anosmia; seen occasionally with olfactory groove meningiomas

310

What are the survival rates for hemangiopericytoma?

5, 10, and 15-year survival rates are 63, 37, and 21%, respectively

311

What is the recurrence rate with hemangiopericytoma?

70%

312

What's the pathology in hemangiopericytoma?

dense cellularity with frequent mitoses, increased reticulin, lobules around “staghorn” vascular channels, and the absence of whorls or psammoma bodies

 

 

313

What is the immunohistochemistry of hemangiopericytoma?

vimentin and CD 34 (no EMA which is meningioma)

314

What is seen radiographically with hemangioblastoma?

Sixty percent are cystic with an enhancing mural nodule abutting the pia and 40% are solid

 

 

315

What is seen on pathology for hemangioblastoma?

capillaries with hyperplastic endothelial cells and pericytes surrounded by stromal cells with vacuoles and lipids

316

What can be secrteted by hemangioblastoma?

EPO causing polycythemia vera

317

What is seen on immunohistochemistry in hemangioblastoma?

postive for vimentin (neg for EMA)

318

What is the recurrence rate with hemangioblastoma?

25%

319

What origin do craniopharyngiomas derive from?

squamous cells from Rathke's cleft cysts

320

What is unique about the cystic component in craniopharyngioma?

filled with “machine oil” fluid and cholesterol crystals that can elicit a granulomatous reaction

321

What percent of craniopharyngiomas are calcified?

100% in children, 50% in adults

322

What is seen on pathology in craniopharyngioma?

adamantinomatous pattern with rests of epithelial cells surrounded by a layer of columnar basal cells separated by a myxoid stroma of loose stellate cells, whorls of cells, and keratinized nodules of wet keratin

323

What are the two types of craniopharyngioma? What is more common overall? What is more common in adults?

adamantous and papillary

 

adamantous

 

papillary

324

Pinealocytes are derived from _____. 

APUD cells

325

What stimulates melatonin secretion from pineal gland?

sympathetic input from superior cervical ganglion

326

What is the most frequent subset of pineal tumors?

germ cell tumors

327

What is seen on pathology with pinealcytoma?

medium-sized round cells, and Homer Wright rosettes with central fibrillar material

328

Where do pineoblastomas metastasize to?

bone, lung, and lymph node

329

Germ cell tumors originate from ______. 

yolk sac endoderm

330

What is the most common pineal tumor?

germinoma

331

What's the most common germ cell tumor?

germinoma

332

What is seen on pathology for germinomas?

soft with large polygonal cells with clear cytoplasm and lacks necrosis or hemorrhage. There are interspersed lymphocytic infiltrates present

333

What is seen on tumor staining for germinoma?

placental alkaline phosphatase

334

What is the treatment for germinoma?

radiation to the entire neuroaxis

335

What serum markers are elevated in germinoma?

15% have increased bHCG

336

What serum markers are elevated in embryonal carcinoma?

bHCG and AFP

337

What is the key feature on yolk sac tumor pathology?

Schiller-Duval bodies

338

What serum markers are elevated with yolk sac tumors?

AFP

339

What serum markers are elevated in choriocarcinoma?

bHCG

340

What is the second most common germ cell tumor?

teratoma

341

What serum marker is elevated in teratoma?

CEA

342

Which pituitary tumors have a female predominance and which have a male predominance?

Female: prolactin and ACTH

 

Male: growth hormone

343

What percent of pituitary tumors do not secrete hormones?

25%

344

Pituitary tumors are associated with tumor syndrome?

MEN type 1

345

How are pituitary's stained and how is the breakdown?

H&E staining reveals acidophils (40%; PRL, GH, and follicle-stimulating hormone [FSH]/luteinizing hormone [LH]), basophils (10%; ACTH, and thyrotroph-stimulating hormone [TSH]), and null cells (50%) 

346

What percentage of pituitary tumors are associated with an intracerebral aneurysm?

4-7%

347

What is the most common pituitary tumor?

PRL-secreting

348

What is the PRL serum cut off for prolactinoma?

> 150

349

What is the treatment for prolactinomas?

dopamine agonists (bromocriptine, pergolide, cabergoline)

350

What medical treatment is there for growth hormone secreting pituitary tumors?

octreotide (somatostatin analog)

351

What is Nelson's syndrome? What is seen on physical exam?

occurs when there is pituitary enlargement after adrenalectomy (that was performed for hypercortisolism thought to be peripherally mediated or as treatment for known Cushing’s disease

 

patients are hyperpigmented because of excess α-melanocyte-stimulating hormone production

352

What is seen on pathology in ACTH secreting tumors?

characterized by Crooke’s hyaline change in the pituitary gland (accumulation of intermediate filaments in the nontumoral corticotrophs in the presence of elevated steroid levels)

353

What causes primary empty sella syndrome?

incomplete development of the diaphragma sella; arachnoid bulges into the sella and may compress the pituitary gland

354

What are secondary causes of empty sella syndrome?

radiation, surgery, stroke, or intrapartum shock with ischemic necrosis of the anterior pituitary gland (Sheehan’s syndrome)

355

Where do rathke's cleft cysts originate from?

remnant of the craniopharyngeal duct that develops when the proximal part closes early and the distal cleft remains open between the pars distalis and pars nervosa

356

What is the most common location for epidermoid cysts?

CPA (50%)

357

What are the key characteristics of epidermoids on MRI?

similar to CSF except on FLAIR and DWI where it is hyperintense compared to CSF

358

What is seen on pathology for epidermoids?

stratified squamous epithelium around thin "dry" keratin

359

What is Mollaret meningitis?

recurrent aseptic meningitis with large cells in the CSF; it occurs in some patients with epidermoid tumors

360

Where are dermoids generally located?

midline: parasellar, fourth ventricular, or interhemispheric 

361

How do dermoids appear on MRI?

similar to fat

362

How do dermoids causes meningitis?

filled with oily fluid and cholesterol that causes chemical meningitis when it leaks, and this may lead to vasospasm and death

363

Where do lipomas derive from?

meninx primitiva, a mesenchyme derivative of the neural crest with both ectodermal and mesodermal tissue that forms the dura, arachnoid, and arachnoid cisterns

364

Where are chordomas located?

60% clivus and 40% sacrum

365

Chordomas are derived from _____. 

notochord remnants

366

What percent of chordomas metastasize?

25-40%

367

What is seen on pathology for chordomas?

lobulated, gray, soft, with sheets or cords of large vacuolated cells (physaliphorous or bubble-bearing cells) surrounded by mucin

368

What is seen on immunohistochemistry for chordomas?

positive for cytokeratin and EMA (epithelial) and S100 (mesenchymal, neural crest)

369

What is a chondroid chordoma?

variant that contains cartilage and has a better prognosis. Low-grade chondrosarcoma is negative for cytokeratin and EMA, but positive for S100

370

What is the survival for chordomas?

5-7 years

371

What percent of carotid body tumors are bilateral?

5%

372

Where do esthesioneuroblastoma arise from?

high nasal cavity from neurosecretory receptor cells or basal cells

373

What are the most common metastatic tumors to the skull?

breast, lung, prostate, multiple myeloma

374

What are the most common hemorrhagic mets to the brain?

melanoma, RCC, and choriocarcinoma

375

What are the most common mets to the brain?

Lung, breast, kidney, melanoma, and GI

376

What is seen on pathology with lymphoma?

diffuse perivascular infiltrate with small blue cells

377

What percent of lymphoma are B cell derived vs T cell derived?

B cell 98%, T cell 2%

378

What is the characteristic finding on pathology in Hodgkin's lymphoma?

Reed Sternburg binucleated cells

379

What is seen on pathology for plasmocytoma/multiple myeloma?

demonstrates mixed small and large cells of intermediate or high grade. There is concentric reticulin and Russell bodies (eosinophilic intracytoplasmic inclusions filled with immunoglobulins)

380

What is seen on pathology in Langerhans cell histiocytosis?

multinucleated giant cells

381

Birbeck bodies are seen on electron microscopy in _______ and look like ______. 

langerhans histiocytosis

 

tennis rackets

382

What is Letterer-Siwe disease?

Acute fulminant disseminated histiocytosis. Occurs in children of ages 2–4 years. Death usually ensues within 2 years. It involves multiple organs

383

What is eosinophilic granuloma?

unifocal langerhans histiocytosis

384

Diabetes insipidus can be a symptom of what systemic oncologic disease?

histiocytosis X (langerhans histiocytosis)

385

What is the triad of Hand-Schuller-Chrisitian disease?

lytic bone lesions, exophthalmos, and diabetes insipidus 

386

What do coloid cyts derive from?

endoderm, vesitigial third ventricular structure (paraphysis)

387

Coloid cysts are ______ on T1 and _____ on T2. 

hyperintense

 

hypointense 

388

Arachnoid cysts increase the risk of ______ due to _____. 

SDH 

 

tearing of bridging veins that transverse the cyst

389

What is the difference between the following: 

Cavum Septum Pellucidum

Cavum Vergae

Cavum velum interpositum 

Cavum spetum pellucidum: CSF between septum pellucidum 

 

Cavum vergae: posterior extension of the above

 

cavum velum interpositum: in the third ventricle because of failure of fusion of the telae choroidea

390

Multiple schwannomas is associated with ____. 

NF-2

391

Where is the most common intracranial location for a schwannoma?

superior vestibular nerve at the root entry zone

392

Trigeminal schwannomas occur most frequentyl in what cranial compartment?

middle fossa

393

What is seen on pathology for schwannomas?

biphasic pattern

 

Verocay bodies, anuclear material with palisading
cells, compact Antoni A (fusiform cells, reticulin, and collagen)

 loose Antoni B (stellate round cells in stroma)

394

What is the immunohistochemistry for Schwannoma?

S100 positive 

395

What's seen on pathology with neurofibromas?

loose wavy nuclei in a matrix with axons (detected by silver stain)

396

What is the immunohistochemistry on neurofibromas?

positive for vimentin, Leu7, S100, and occasionally GFAP

397

Which neurofibromas is pathognomonic for NF-1?

plexiform

398

What percent of germinomas of the pineal region have concomitant pituitary germ cell tumor?

5%

399

What is the classic presentation of a foramen magnum mass?

progressive weakness of the ipsilateral upper limb followed in order by the ipsilateral lower limb, contralateral lower limb, and then the contralateral upper limb

400

Hypothalamic/Chiasm gliomas are associated with ______.

NF-1

401

What is juvenile angiofibroma?

vascular, invasive, originates near the sphenopalatine foramen of adolescent males, most common benign nasopharyngeal tumor, and spreads along the foramen into the pterygopalatine fossa, orbit, sinus, etc

402

Visual loss in fibrous dysplasia occurs from what?

narrowing of the optic foramen

403

What is McCune-Albright syndrome?

polyostotic fibrous dysplasia, pigmented skin lesions, and endocrine abnormalities such as precocious puberty or GH-secreting pituitary tumors

404

Woven bone is characteristic of _____.

fibrous dysplasia

405

Pagets disease causes _____ early in the course and ______ late in the course.

destruction of bone sclerosis

406

What drug can cause general calvarial thickening?

phenytoin

407

What metastatic tumors cause calvarial thickening?

breast and prostate

408

What is the clinical significance of parietal foramina? Is it unilateral or bilateral?

no clinical significance bilateral

409

What skull defects can be present with NF-1?

absent sphenoid wing and lamboid suture defects

410

What is the key radiographic word for skull hemangiomas on imaging?

sunburst pattern

411

What is seen on MRI imaging with carbon monoxide poisening? What is seen on gross specimen?

B/l T1 hypointensity in the GP

 

b/l hemorrhagic necrosis of the GP

412

What is the most common orbital tumor in adults? Children?

melanoma

 

retinoblastoma 

413

What is the most common orbital lesion in adults?

cavernous hemangioma

414
What is a phakoma?
tumor-like retinal lesion
415
Most phakomatoses have what inheritance pattern?
autosomal dominant (sturge weber is an exception)
416
What does the NF1 gene code for?
neurofibromin
417
What percent of NF1 patients develop malignant peripheral nerve sheath tumors?
5%
418
What is the inclusion criteria for NF-1?
Must have two of the following: - Six café au lait spots - two neurofibromas - one plexiform neurofibroma - axillary or inguinal freckling - an osseous lesion (sphenoid dysplasia or thinning of long bones or cortex) - an optic glioma - two or more Lisch nodules (iris hamartomas, only seen with NF1) - a relative with NF1
419
Where do neurofibromas develop in NF-1?
posterior nerve roots
420
What is the gene in NF-2?
Merlin
421
In neurofibromatosis, spinal ependymomas are more common in _____ and spinal astrocytomas are more common in _____.
NF-2 NF-1
422
What is the inclusion criteria for NF-2?
Bilateral vestibular schwannoma or a relative with NF2 and one vestibular schwannoma or two of the following: - neurofibroma - meningioma - glioma - schwannoma - post capsular cataract at a young age
423
What percent of people with vestibular schwannoma have NF-2?
2-10%
424
What is the transmission for NF-1?
AD
425
What is the transmission for NF-2?
AD
426
What is the transmission for Tuberous sclerosis?
AD, usually sporadic
427
What is the genetic cause of tuberous sclerosis?
mutations on Ch 9 (TSC 1, Hamartin) and Ch 16 (TSC 2, tuberin)
428
What is the classic triad in tuberous sclerosis?
- mental retardation - seizures - adenoma sebaceum (angiofibroma)
429
What percent of tuberous sclerosis have SEGA?
15%
430
What are common skin manifestations of tuberous sclerosis?
- angiofibromas - ash-leaf hypopigmented macules - shagreen patches (subepidermal orange peel fibrosis of the lower trunk)
431
What are "salaam" spasms? How are they treated?
flexion myoclonus seen in tuberous sclerosis ACTH
432
What is the inheritance pattern for VHL?
AD
433
What is the genetic association of VHL?
mutation in VHL gene, tumor suppressor, on Ch 3
434
What percent of hemangioblastomas are in VHL patients?
20%
435
What is a Lindau tumor?
cerebellar hemangioblastoma
436
What is Lindau disease?
cerebellar hemangioblastoma with an extra CNS lesion
437
What is a von Hippel tumor?
retinal hemangioblastoma
438
Polycythemia vera is associated with which of the phakomatoses?
VHL
439
What percent of VHL patients have hemangioblastomas?Where are the most common locations?
60% cerebellum (65%), brainstem (20%), spine (15%)
440
What is the diagnostic criteria for VHL?
multiple CNS hemangioblastomas or one CNS hemangioblastoma and one visceral lesion with a first-order relative with VHL
441
What is the transmission for Sturge weber?
sporadic
442
What is sturge weber disease?
phakomatoses characterized by a port-wine stain (facialnevus flammeus, often in the distribution of the first division of the trigeminal nerve) and ipsilateral venousmalformation of the leptomeninges (enhances), choroid of the eye, or choroid plexus
443
What is a characteristic finding of Sturge weber on imaging?
ipsilateral cortical (parietooccipital usually) tram track calcifications
444
What is another name for Osler Weber Rendu syndrome?
hereditary hemorrhagic telengectasia
445
What is the transmission for HHT?
AD
446
What mutations lead to HHT?
two genes involved in tumor growth factor beta (TGF-β) signal transduction pathways, HHT1 (endoglin, Ch 9) and HHT2 (ALK1, Ch 12)
447
What is HHT?
phakomatoses characterized by multiple mucocutaneous telangiectasias (in the skin, GI, and GU tracts), visceral vascular malformations (AVMs of the liver, lung, brain, and spinal cord), and rarely aneurysms
448
______ percent of patients with multiple AVMs have ________.
33% Osler Weber Randu or Wyburn Mason syndrome
449
What are racemose angiomas?
retinal AVMs
450
What is wyburn mason syndrome?
Unilateral cutaneous vascular nevi of the face and trunk with retinal, optic nerve, visual pathway, and midbrain AVMs
451
What is the transmission of ataxia telengectasia?
AR
452
What is the defect in ataxia telengectasia?
defect in DNA repair
453
What characterizes ataxia telengectasia?
Oculocutaneous telangiectasias and cerebellar ataxia (caused by anterior vermian atrophy), increased risk of cancers and infection
454
Death in ataxia telengectasia usually occurs by ____.
20 years
455
What is Klippel–Trenaunay–Weber syndrome?
Angio-osteohypertrophy (overgrowth of vessels and bones). One limb is usually enlarged. It is associated with leptomeningeal AVMs (some spinal) and dermatomal cutaneous hemangiomas
456
What is epidermal nevus syndrome?
Ipsilateral nevus and bone thickening associated with mental retardation, seizures, hemiparesis, and gyral malformations
457
CO poisoning causes necrosis of what structure?
medial GP b/l
458
Cyanide binds ______ causing _____.
cytochrome oxidase halt in cellular respiration
459
What is the world's leading cause of mental retardation and birth defects?
fetal alcohol syndrome
460
What are the CNS effects of methanol consumption?
necrosis of lateral putamen and claustrum, optic nerve swelling and eventual blindness
461
Consumption of what alcohol DOES NOT cause acidosis?
isopropyl alcohol
462
What causes congenital fetal hydantoin syndrome?
dilantin use
463
Blockage of what receptors causes parkinson like side effects? What class of medications can cause it? How is it treated?
D1 antipsychotics anticholinergics
464
What is Marchiafava-Bignami disease?
rare; demyelination and necrosis of the genu and body of corpus collosum; can be seen in alcoholics
465
What is the mechanism of MAO inhibitors?
increase serotonin, NE, and Epi levels/release
466
What is the mechanism for TCAs?
decrease uptake of amines
467
What are some side effects of lithium?
nephrogenic DI and asterixsis
468
What is the MOA of methotrexate?
folic acid antagonist
469
Subacute necrotizing leukoencephalitis is a side effect of what medication? What is seen on pathology?
methotrexate when used with radiation coagulation necrosis, lipid-laden macrophages, absence of inflammatory cells, mineralizing angiopathy of the gray matter, and mainly affects astrocytes
470
What are some side effects of cisplatin?
hearing loss, visual loss, leukoencephalopathy, neuropathy
471
What chemotherapy agent can cause decreased ADH secretion?
vincristine
472
What is the MOA of vincristine?
impairs microtubule formation
473
Vincristine is generally used to treat ______.
lymphoma and leukemia
474
How is nitrosourea used in neurooncology treatment?
directly implanted in the tumor bed to treat GBM (serious side effects if given arterially)
475
What are two side effects of temozolomide that require regular monitoring?
neutropenia and thrombocytopenia
476
What is the treatment for arsenic poisoning?
2,3-dimercaptopropanolol (BAL)
477
Hyperpigmentation and hyperkeratosis of the palms and soles is seen in what toxicity?
arsenic
478
What are Mees lines?
transverse white lines on the fingernails seen with arsenic poisoning
479
What is the treatment for lead poisoning?
EDTA, BAL, penicillamine
480
What is the presentation for lead poisoning?
severe encephalitis in kidspure motor demyelinating peripheral neuropathy (especially WRIST DROP), anemai, gingival lead lines
481
What is the treatment for mercury poisoning?
penicillamine
482
Which metal toxicity causes parkinsonian symptoms?
manganese
483
What ocular findings are seen in Wernicke's encephalopathy?
conjugate and lateral rectus palsies, nystagmus
484
Kosacoff psychosis is caused by a lesion in the _____.
dorsomedial thalamus
485
Berberi is due to a deficiency in ______. What is the clinical presentaiton?
thiamine (common in rice eaters)axonal degeneration/demyelination, autonomic dysfunction (orthostatic), rarely heart disease
486
What is Pellegra? What causes it?
triad: dermatitis, diarrhea, dementianiacin deficiency (common in corn eaters)
487
Subacute combined degeneration is caused by _______ and presents with _______.
B12 deficiencylower cervical and upper thoracic posterior column and lateral column spongiform degeneration
488
Alzheimer type II cells are associated with what disease?
hepatic encephalopathy
489
What is Reye syndrome?
hepatic encephalopathy in kids with flu or varicella who received ASA
490
Myxedema in adults can be caused by ______.
hypothyroidism
491
What is the mechanism of tetanus toxin?
cleaves synaptobrevin preventing glycine release from renshaw cells
492
What is the mechanism for botulin toxin?
cleaves SNAPs and SNARES, decreased ACh release at NMJ
493
What is the mechanism of black widow spider venom?
depletes ACh stores at NMJ causing cramps/spasms followed by weakness
494
What is the treatment for black widow venom?
Ca gluconate and MgSO4
495
What is the most common aminoacidopathy?
phenyketonuria
496
What is the inheritance of phenylketonuria?
AR
497
What are hallmark exam findings in PKU? How is it treated?
fair skinned blue eyed kid with musty odorlimit phenylalanine consumption
498
The aminoacidopathies are generally inherited in _____ fashion.
AR
499
What is the inheritance of homocystinuria?
AR
500
What is the deficient enzyme in PKU?
phenylalanine hydroxylase

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