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93 Cards in this Set

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What CNS lesion has a "popcorn" appearance on MRI FLAIR?
Cavernous hemangioma
What is a way to tell the difference between cavernous hemangioma and arteriovenous malformation by histology?
Cavernous hemangioma has no intervening brain parenchyma between vessels while AVM has intervening brain parenchyma.
Most common locations for berry aneurysms?
Anterior communicating > posterior communicating > middle cerebral
What is the first 3 most common causes of death?
1. Heart disease
2. Cancer
3. Stroke
What percentage of strokes are occlusive vs. hemorrhagic? Which is more lethal?
Occlusive = 80%
Hemorrhagic = 20%

Hemorrhagic is more lethal
Which cells are most sensitive to hypoxia? (4)
1. Hippocampal pyramidal cells of C1
2. Pyramidal neocortical neurons (layers 3, 5, 6)
3. Purkinje cells
4. Striatal neurons
What regions of the brain are most sensitive to hypoxia? (5)
Most to least sensitive to hypoxia:
1. Cerebral cortex
2. Corpus striatum
3. Thalamus
4. Brainstem
5. Spinal cord
What are the locations for lacunar infarct? (4)
Basal ganglia
Thalamus
Gray-white matter junction
Brainstem
Timeline of a cerebral infarct:
Eosinophilic neurons, shrunken nuclei, neuronal dropout.
6-12 hours
Timeline of a cerebral infarct:
Neutrophils, vacuolation of white matter.
1-2 days
Timeline of a cerebral infarct:
Beginning of macrophage infiltrate.
3-5 days
Timeline of a cerebral infarct:
Maximum macrophage infiltrate gliosis.
1-3 weeks
Timeline of a cerebral infarct:
Dissolution of tissue, cavitation (macrophages may persist)
weeks to years
This hemorrhage is due to tear of the meningeal arteries, most often due to trauma.
Epidural
This hemorrhage is due to tear of the bridging veins, most often due to trauma.
Subdural
This hemorrhage is due to bursting of aneurysm or AVM.
Subarachnoid
This hemorrhage is due to hypertension or amyloid angiopathy.
Intraparenchymal
This hemorrhage occurs in permature babies secondary to perinatal hypoxic injury involves bleeding of the germinal matrix.
Intraventricular
What type of amyloid is found in Alzheimer's disease?
beta-amyloid
Familial forms of Alzheimer's disease has to do with mutations of what gene on what chromosome?
Amyloid precursor protein (APP) gene on chromosome 21
Meningitis in what population?
Haemophilus influenza
Haemophilus influenza: all ages
Meningitis in what population?
Streptococcus pneumoniae
Streptococcus pneumoniae: adults
Meningitis in what population?
Neisseria meningitidis
Neisseria meningitidis: children/young adults
Meningitis in what population?
Group B streptococci
Group B streptococci: neonates
Meningitis in what population?
Escherichia coli
Escherichia coli: neonates
Meningitis in what population?
Listeria monocytogenes
Listeria monocytogenes: neonates, adults
What is the most common pediatric brain tumor? Where is the most common location of this tumor?
Pilocytic astrocytoma, most commonly in the cerebellum
What grade are pleomorphic xanthoastrocytoma?
WHO Grade 2
What syndrome is associated with subependymal giant cell astrocytoma?
Tuberous sclerosis
Most common locations for ependymoma?
Cerebellum = 4th ventricle or vermis
What WHO Grade are ependymoma and myxopapillary ependymoma?
Ependymoma = WHO Grade 2
Myxopapillary ependymom = WHO Grade 1

NOTE: Anaplastic ependymom is WHO Grade 3 and there is no WHO Grade 4.
What is the location for myxopapillary ependymoma?
lower spinal cord
What is the location for subependymoma?
Anywhere in the intraventricular system and spinal cord
This lesion most commonly involves the temporal lobe and is associated with seizures. It is circumscribed and shows both glial and ganglionic elements.
Ganglioglioma
This lesion is cortically based with radiology showing bone erosion. This tumor is composed of well-differentiated neuroepithelial cells arranged along vascular structures and "floating" neurons. The histological architecture is typically nodular within cortical or subcortical regions. It is associated with seizures wand often coexists with cortical dysplasia.
Dysembryoplastic neuroepithelial tumor
Location for medulloblastoma.
Cerebellum/posterior fossa, vermis
Most common genetic abnormality in medulloblastoma? The presence of what gene amplification carries a poor prognosis?
Most common abnormality: isochromosome 17q

Poor prognosis: myc amplificaiton
Which subtypes of meningioma are WHO Grade 2? What defines an atypical meningioma?
Subtypes:
Clear cell
Chordoid
Atypical

Def Atypical:
> 4 mites/10 HPF
Brain parenchymal invasion
3 or more of the following:
- small cell morphology
- sheeting
- high N/C ratio
- geographic necrosis
- prominent nucleoli
Which subtypes of meningioma are WHO Grade 3? What defines anaplastic meningioma?
Subtypes:
Rhabdoid
Papillary
Anaplastic

Def Anaplastic:
- >20 mites/10 HPF
- appearance of high grade sarcoma
Hemangiopericytoma IHC:
CD34
BCL-2
EMA
S100
GFA
CD99
Hemangiopericytoma IHC:
CD34 +
BCL-2 +
EMA -
S100 -
GFA -
CD99 +
Location of Rathke cleft cyst?
Intrasellar
Location of epidermoid cyst?
Base of skull or cerebellar
Location of dermoid cyst?
Midline
Metastatic tumors to brain from most common to least common?
1. Pulmonary
2. Breast
3. Melanoma
4. Unknown
5. GI
6. GU
Metastatic tumors to spinal cord from most to least common?
1. Breast
2. Pulmonary
3. Prostate
4. Lymphoma
Which CNS tumor is associated with VHL?
Hemangioblastoma
Which CNS tumors are associated with Turcot syndrome? What are other features of Turcot syndrome?
Turcot Syndrome:
- colonic polyps and/or carcinoma
- cranofacial exostosis
- Medulloblastoma (APC 5q21-22)
- Glioblastoma (MLH1 3p21; PMS2 7q)
What CNS tumors are associated with NF2?
Meningioma, acoustic neuroma (Schwannomas), ependymomas, astrocytomas
What CNS tumor is associated with Gorlin syndrome?
Medulloblastoma

Gorlin Syndrome = Nevoid basal cell carcinoma syndrome:
- BCCs
- OKC
- Medulloblastoma
- Germline mutation of hte PTCH gene 9q22.3
What gene is mutated in NF1 and NF2.
NF1: neurofibromin 17q11.2
NF2: merlin 22q12
What is the mechanism of central pontine myelinolysis?
Rapid correction of serum hyponatremia
What is a cephalocele? Meningocele? Encephalocele?
Cephalocele: Defect in the dura and cranium with herniation.
Meningocele: type of cephalocele with herniation of meninges only
Encephalocele: type of cephalocele with herniation of brain and meninges
In the setting of anencephaly, AFP is ____ and there is ____hydramnios.
In the setting of anencephaly, AFP is HIGH and there is POLYhydramnios.
What is holoprosencephaly?
Failure to separate into hemispheres
What is the Dandy Walker malformation?
Defective development of the roof of the fourth ventricle.
What is schizencephaly?
Gray matter extension from the ventricle to the cortex with direct communication of the ependymal surface with cortical leptomeninges.
What is lissencephaly?
Smooth brain

NOTE: Acquired from CMV invection or inherited
What is the defective protein in Canavan's disease? What gene? DX?
Canavan's Disease:
Protein: aspartoacylase deficiency
Gene: ASPA 17pter-p13
DX: hihg N-acetyl aspartic acid in urine
What is the defective protein in Krabbe's disease? What gene? DX?
Krabbe's disease:
Protein: galactocerebrosidase (GALC) deficiency
Gene: GALC 14q31
DX: Deficient enzyme in WBC or skin fibroblasts
What is porencephaly?
Big cyst in the brain lined by gliotic what matter due to neonatal infarct or trauma.
Pick's disease affects what part of the brain? When familial, what gene is involved?
Picks: frontal and temporal lobes
Gene: tau protein (17q)
Huntingtons disease:
Pattern of inheritance
Age of onset
Gross findings
Gene
Huntingtons disease:
Pattern of inheritance: AD
Age of onset: 4th decade
Gross findings: basal ganglia (caudate, putamen) and coritcal atrophy
Gene: HD gene 4p with CAG expansion
Parkinson disease is associated with what neurotransmitter at what pathway? Gross finding?
Parkinson:
NT: dopamine
Pathway: Nigrostriatal dopamine pathway
Gross: depigmentation of substantia nigra and locus ceruleus
Niemann-Pick disease is due to deficiency of what enzyme?
Sphingomyelinase
What type of fracture?
Raccoon eyes
Raccoon eyes: Anterior fossa
What type of fracture?
CSF rhinorrhea
CSF rhinorrhea: Anterior fossa
What type of fracture?
Battle sign (blood over mastoid)
Battle sign (blood over mastoid): middle fossa

NOTE: also often involves CN 3, 4, and 6
What type of fracture?
Results from fall from height
Fall from height: ring fracture
This infection can be associated with acute hemorrhagic encephalitis involving one or both temporal lobes.
HSV
Negri bodies in Purkinje cells and hippocampal neurons may be seen in this infection.
Rabies
This virus which causes progressive multifocal leukoencephalopathy mostly in AIDS patients attacks what cells?
JC virus attacks oligodendroglia causing demyelination.
This infection causes basilar meningitis or intraparenchymal granulomas.
Histoplasma
Changes seen in this disease include spongy change, vascular hyperplasia and hemorrhage in the mammillary bodies, dorsal thalamus, hypothalamus and periaqueductal gray matter.
Wernicke's encephalopathy
IHC of JC virus?
SV40 T antigen (will cross-react with papovavirus) highlight viral inclusions in oligodendorcyte nuclei.
Common mutations:
Medulloblastoma
1. Isochromosome 17q (most common)
2. PTCH (pathced gene) 9q22.3 found in Gorlin syndrome
3. MYC amplification (poor prognosis)
Common mutations:
Glioblastoma
EGFR amplification
Major protein in Lewy bodies?
alpha-synuclein
Organism that causes neurocysticercosis?
Taenia solium

NOTE: Neurocysticercosis comes from ingestion of eggs which become larvae in the gut, penetrate walls and go disperse through the body. Ingestion of cysts result in tapeworm in gut but no disseminated disease.
Duret hemorrhages are what?
Duret hemorrhages: hemorrhage in pons due to uncal herniation
What axons are lost in tertiary syphilis?
Dorsal columns
In Alzheimer disease, nueorfibrillary tangles are made of what protein? What about plaques?
Tangles: tau
Plaques: beta-amyloid
These enlarged astrocytes with lucent nuclei can be seen in association with many liver diseases including posthepatic and alcoholic cirrhosis, Rey's syndrome, and urea cycle enzyme deficiencies. These cells are never associated with Alzheimer disease.
Alzheimer type 2 astrocytes
What brain abnormality is seen in Wilson's disease?
Degeneration of basal ganglia
What brian abnormality is seen in Huntington's disease?
Loss of neurons in the striatum = caudate and putamen.
What CNS abnormality is seen in B12 deificiency?
Myelinopathy of the spinal cord
What histology is seen in toxoplasmosis?
Tissue cysts containing tachyzoites
What cells are involved?
CMV infection
Ependymal cells
What cells are involved?
HIV
cerebral microglial/monocyte/macrophage
What cells are involved?
JC
Oligodendorytes, some astrocytes
What cells are involved?
HSV1
Cortical neurons
What cells are involved?
Rabies
Neurons of hippocampus pyramidal cells, cerebral cortex, and Purkinje cells
What blood test finding for adrenoleukodystrophy?
increased long chain fatty acid levels

NOTE: can be found in tissue or serum
What blood test finding for metachromatic leukodystrophy?
low sulfatase levels