Neuropathology Exam:
A) Hypoxia and Stroke:
1. If circulation ceases, the energy supplies stored in brain cells are enough to last:
a. 1-2 minutes
b. 3-5 minutes
c. 6-8 minutes
d. 10 minutes
2. The most common cause of HIE in a 60 year old patient is:
a. Respiratory arrest
b. Cardiac arrest
c. Hypoglycemia
d. Seizures
3. Neurons damaged by hypoxia or trauma discharge:
a. NO
b. Free radicals
c. Glutamate
d. GABA
4. Free radicals are generated in the:
a. Lysosomes
b. Cytosol
c. Mitochondria
d. Golgi apparatus
5. Cerebral edema in HIE is caused by:
a. Arachidonic acid.
b. Lactic acid.
c. Both.
d. Neither
6. Which of the following is most vulnerable in HIE?
a. The thalamus
b. The caudate nucleus
c. The substantia nigra
d. The inferior olive
7. The respirator brain is caused by:
a. A direct action of the respirator
b. Hypoxia
c. Autolysis
d. Inflammation
8. Most deaths following MCA occlusion in older patients occur:
a. During the 1st day
b. During the 3rd to 4th days
c. Between the end of the 1st week and 10 days
d. Mortality is the same in all periods
9. Restoring circulation to the ischemic penumbra can limit brain damage in an ischemic
infarct. The window of opportunity for rescuing the penumbra is:
a. 1 to 2 hours
b. 3 to 4 hours
c. 5 to 6 hours
d. 12 hours
*10. Most fusiform aneurysms of the basilar artery cause:
a. Thrombosis with ischemic infarction of the pons
b. Rupture with subarachnoid hemorrhage
c. Both
d. Neither. They are usually asymptomatic
11. Amnesia involving recent and old memory may result from bilateral lesions of:
a. The hippocampus and amygdala
b. The thalamus
c. Both
d. Neither
12. The intracellular process that triggers cell injury in HIE is:
a. Release of lysosomal enzymes into the cytosol
b. Intracellular edema
c. Increased intracellular potassium
d. Increased intracellular calcium.
13. The persistent vegetative state may result from extensive damage of:
a. The hippocampus and amygdala
b. The cerebral cortex and thalamus
c. The nucleus basalis
d. The reticular activating substance of the brainstem
14. Intracranial arterial aneurysms can cause all of the following except:
a. Mass-like lesions
b. Pontine hemorrhage
c. Hydrocepalus
d. Cranial nerve deficits
*15. The pathological process illustrated below is associated with all of the following
except:
a. Peripheral neuropathy
b. Fatal intracerebral hemorrhage
c. Subcortical demyelination
d. Lacunar infarcts in the basal ganglia
*16. Risk factors for cerebral arterial occlusion and ischemic infarction iclude:
a. Elevated homocysteine
b. Factor V Leiden
c. Both
d. Neither
17. The lesion illustrated below can cause:
a. Recurrent headaches
b. Homonymous hemianopsia
c. Focal seizures
d. All of the above
*18. The lesion illustrated in the slide below may be caused by occlusion of:
a. Basilar artery
b. Vertebral artery
c. Both
d. Neither
19. The lesion illustrated below is due primarily to vascular occlusion
a. True
b. False
20. The lesion illustrated below can cause severe neurologic deficits but is no threat to life
a. True
b. False
21-24. Match the numbered pathological pictures, 21, 22, 23, and 24, with the lettered
clinical deficits
21a, 21b, 21c, 21d
22a, 22b, 22c, 22d
23a, 23b, 23c, 23d
24a, 24b, 24c, 24d
a. Hemiparesis involving more severely the leg.
b. Visual deficit (cortical blindness).
c. Hemiparesis involving the face and arm.
d. Memory deficit
25-28. Match the numbered pathological pictures, 25, 26, 27, and 28, with the lettered
arteries
25a, 25b, 25c, 25d
26a, 26b, 26c, 26d
27a, 27b, 27c, 27d
28a, 28b, 28c, 28d
a. ACA
b. MCA
c. PCA
d. PICA, basilar, or vertebral artery
*29. The lesion illustrated below is caused by:
a. Gunshot wound
b. Contrecoup contusion
c. A ruptured AVM
d. Ruptured intracranial aneurysm
30. A 72 year old woman with a one year history of declining memory developed sudden
headache and decreased consciousness and collapsed while washing dishes.
Neuropathological examination revealed the lesion illustrated below. The most likely cause
is:
a. Hypertension
b. Cerebral amyloid angiopathy
c. Ruptured AVM
d. Trauma (the lesion developed when she fell)
*31. A 15 year old male with SLE had decreased consciousness, bilateral lower extremity
weakness, and the MRI changes illustrated below. Symptoms improved somewhat but a
follow-up MRI showed residual encephalomalacia. The lesions are most likely caused by:
a. Lupus vasculitis
b. Embolism from non-bacterial endocarditis
c. Bilateral border zone ischemia
d. Superior sagittal sinus occlusion due to antiphospholipid syndrome
32. The MRI images shown below were obtained one month apart (the left first). The
illustrated pathology can cause:
a. Seizures
b. Loss of the ability to learn new things
c. Both
d. Neither.
*33. A 60 year old previously healthy patient developed hemiparesis and aphasia. MRI
showed an enhancing left hemispheric lesion. A brain biopsy was done (shown below)
The pathology is most compatible with:
a. Changes around the wall of an abscess
b. A gemistocytic astrocytoma
c. An two month old ischemic infarct
d. An old MS plaque
Perinatal Disorders:
1. Asphyxia means pulselessness
A. True
B. False
2. A 36 week gestation fetus is more susceptible to HIE than a 40 year old person
A. True
B. False
3. Porencephaly is a developmental malformation occurring in the second trimester
A. True
B. False
4. The pathology illustrated below is a developmental malformation occurring in the second
trimester
A. True
B. False
5. Complications of germinal matrix hemorrhage occurring at 22 weeks of gestation include
all of the following except:
A. Hydrocephalus due to blockage of the aqueduct
B. Psychomotor retardation from loss of neuronal and glial precursors
C. Porencephaly, due to disruption of the periventricular white matter
D. Fetal anemia from excessive blood loss
*6. A baby boy was born at 29 weeks of gestation and was discharged from the NICU at 34
weeks. At 7 months of age, spasticity of the lower extremities is apparent. The CT scan is
shown below. The most likely cause of the abnormality is:
A. X-linked hydrocephalus
B. Undetected prenatal infection
C. White matter damage due to ischemia
D. A metabolic disorder
*7. A 39 week pregnant woman detected decreased fetal movement. Biophysical profile
was poor and the baby was delivered by Cesarean section with Apgar scores of 1 and 3 at 1
and 5 minutes. The baby was hypotonic, had poor respiratory effort, and died at 4 four days
of gestation. Pathological examination revealed HIE. Which of the following would be least
severely affected?
A. The hippocampus
B. The cerebral cortex
C. The thalamus
D. The brainstem
8. The white matter is most frequently affected by HIE in:
A. Premature infants
B. Adults
C. Both
D. Neither
9. This baby was born at 36 weeks of gestation and developed E. Coli sepsis and meningitis.
He died two weeks later. The changes in the brain shown below are due
to:
A. HIE
B. Cerebritis due to E. Coli
C. Both
D. Neither
10. This child was born at term and had spasticity and psychomotor retardation. He lived in
an institution for retarded children and died at 12 years of age. The brain
lesion shown below is due to:
A. Congenital infection
B. Ischemia
C. A genetic disorder
D. Teratogen effect
11. This baby was born at term. He was hypotonic, had a large transilluminating head, and
died at 2 days of age. The brain at autopsy is shown below. Which of the following
statements about the brain pathology is not true:
A. The primary lesion is extreme hydrocephalus
B. The lesion is due to destruction of cortex and aqueductal atresia
C. There may be preservation of the temporal lobe, brainstem and cerebellum
D. The infant may appear normal in the first day of life
12. This infant was born at 30 weeks of gestation and died three weeks later. The
brain lesions shown below represent:
A. Congenital CMV
B. Congenital toxoplasmosis
C. A metabolic disorder
D. Ischemic pathology
13. This baby was born at 28 weeks of gestation. At 24 hours, he became hypotonic and his
hematocrit dropped to 23. He died three days later. The pathology illustrated below is due
to:
A. Maternal immune thrombocytopenia
B. Germinal matrix hemorrhage
C. Birth injury
D. Congenital infection
14. The pathology in the brain of this six day old girl shown below may be caused
by:
A. Deficiency of glucuronyl transferase
B. Congenital biliary atresia
C. Both
D. Neither
Trauma:
1. Ischemic lesions or HIE are a major component of the shaken baby syndrome
A.True
B. False
2. Which of the following identifies damaged axons in diffuse axonal injury?
A. Beta amyloid.
B. Beta amyloid precursor protein
3. Epidural hematomas do not occur without skull fractures
A. True
B. False
4. All subdural hematomas have a free interval between the trauma and onset of symptoms
A. True
B. False
5. Pure diffuse axonal injury is characterized by severe brain swelling
A. True
B. False
6. Transsection of the cervical spinal cord may occur in the shaken baby syndrome
A. True
B. False
7. Beta amyloid precursor protein is produced by neurons at the time of traumatic brain
injury
A. True
B. False
*8. A fibrous inner membrane encapsulating the subdural hematoma develops in:
A. 5 days
B. 10 days
C. 20 days
D. 4 to 6 weeks
9. Who is more susceptible to developing a subdural hematoma?
A. A 6 month old infant living with the mother and her boyfriend
B. A 16 year old skate boarder
C. A 40 year old race driver
D. A 72 year old patient with Alzheimer disease
10. The most common traumatic brain injury is:
A. Epidural hematoma
B. Subdural hematoma
C. Subarachnoid hemorrhage
D. Intracerebral hemorrhage
*11. Yellow (or orange) plaques ("Taches Jaunes") are:
A. Areas of organized subdural hematoma
B. Old subarachnoid hemorrhage
C. Old contusions
D. Old intracranial hemorrhages
12. Axonal swellings occur mainly in :
A. Ischemic infarcts
B. Diffuse axonal injury
C. Around intracerebral hematomas
D. All of the above
13. The mechanism by which axonal swellings occur in diffuse axonal injury involves all of
the following processes except:
A. Snapping of axons as a result of mechanical force
B. Deformation of axons at internodes
C. Compaction of neurofilaments
D. Influx of calcium into the axoplasm
*14. Among the following structures, petechiae, in diffuse axonal injury, are found most
commonly in:
A. The internal capsule
B. The corpus callosum
C. The subcortical white matter
D. The dentate nuclei of the cerebellum
*15. Petechiae, in diffuse axonal injury, are most commonly found in which of the
following?
A. The inferior frontal lobes
B. The caudate nuclei
C. The basis pontis
D. The dorsolateral brainstem
*16. The Bielschowsky stain shows axonal swellings in:
A. 2 to 3 hours
B. 8 hours
C. 15 hours
D. 24 hours
*17. The brain of a 62 year old former professional boxer who has dementia and
Parkinsonian manifestations shows:
A. Neurofibrillary tangles
B. Alzheimer plaques
C. Both
D. Neither (dementia results from multiple traumatic frontal lobe lesions)
18. A patient with a glioblastoma multiforme in the right frontoparietal area develops right
hemiparesis and a fixed dilated right pupil. The cause of these neurological findings is:
A. Extension of the tumor across the corpus callosum to the lefthemisphere
B. Extension of the tumor to the brainstem
C. Right temporal lobe herniation
D. Cerebellar tonsillar herniation
20. The pathology in the section across the midbrain shown below is the result of:
A. A 2 day old right MCA infarct
B. A left subdural hematoma
C. A right hemisphere GBM
D. An intrinsic midbrain glioma
21. The pathological lesions shown below are the result of:
A. Diffuse axonal injury
B. Anticoagulant treatment
C. Arteriovenous malformation
D. Hypertension
E. Increased intracranial pressure
22. The lesions in this 7 month old baby girl shown below are most likely caused by:
A. Coagulopathy
B. Resuscitation
C. Chest trauma
D. Traumatic brain injury
E. All of the above
*23. The most likely mechanism for the lesions shown below is:
A. Embolus from bacterial endocarditis
B. Anticoagulant therapy
C. Auto accident
D. A fall to the back
24. The brain lesions in the 57 year old man shown below are most likely due to:
A. A fall to the back
B. A viral encephalitis
C. A posterior cerebral artery embolic infarct
D. Thrombotic thrombocytopenic purpura
25. The lesions illustrated below are most likely to occur as a result of:
A. An auto accident
B. The shaken baby syndrome
C. A fall to the back
D. A long boxing career
*26. This image below shows:
A. An epidural hematoma
B. A subdural hematoma
C. Both
D. Neither
CNS Infections:
1. A natural epidural space exists around
a. The brain
b. The spinal cord
c. Both
d. Neither
2. The most common cause of subdural empyema is:
a. Open trauma
b. Meningitis
c. Osteomyelitis
d. Sinusitis
3. A 50 year old patient was admitted to the hospital with a hemorrhagic rash, a
temperature of 40C, and shock. He was treated with antibiotics but died in 3 hours. The
best way establish the diagnosis in this case is:
a. Bacterial culture
b. Postmortem PCR for bacteria
c. Latex studies
d. Gram stains
4.Which is more likely to develop one week after the onset of untreated bacterial
meningitis?
a. Ischemic infarcts
b. Subarachnoid hemorrhage
c. Deafness
d. Hydrocephalus
e. Hypoxic-ischemic encephalopathy
5. Which of the following is most common among the following complications of meningitis?
a. Cerebritis
b. Brain abscess
c. Ventriculitis
d. Subdural abscess
6. The most dangerous feature of an abscess is:
a. Sepsis
b. Loss of neurological function
c. Increased intracranial pressure
d. Development of meningitis
*7. CNS syphilis causes all of the following except:
a. Ischemic lesions
b. Suppurative meningitis
c. Radiculopathy and myelopathy
d. Mass-like lesions
8. A 3 year old boy presents to the ER in mid September with a history of diarrhea,
headache and obtundation. A spinal tap is done. The most appropriate studies are:
a. RT-PCR
b. PCR and RT-PCR
c. Bacterial cultures, PCR and RT-PCR
d. The patient can be safely observed for 24 hours
9. The most common cause of brain damage in HIV encephalitis is:
a. Opportunistic infections
b. Cerebral lymphoma
c. HIV infection of neurons
d. Cytokines and viral toxins.
10. Most CJD is:
a. Familial
b. Sporadic
c. Transmitted from animals
d. Iatrogenic
11. The most frequent source of iatrogenic CJD is:
a. Pituitary extracts
b. Dura transplants
c. Contaminated electrodes
d. Transfusions
*12. Are there prion protein gene mutations in sporadic CJD?
a. Yes
b. No
13. Familial prion diseases are:
a. Autosomal dominant
b. Autosomal recessive
c. Both
d. Sporadic
14. Meningitis usually crosses the pial barrier and involves the brain
a. True
b. False
*15. A 15 year old boy was admitted to the hospital with a hemorrhagic rash, temperature
of 40C and shock. He was treated with antibiotics but died 5 hours later. The autopsy
showed:
a. Meningitis
b. Inflammation of thechoroid plexus
c. Microthrombi in pulmonary vessels and hemorrhagic adrenals
d. All of the above
*16. A 36 year old AIDS patient had fever, headaches, and neurological deficits. MRI
showed multiple enhancing brain lesions. A stereotactic biopsy of one lesion was done. The
findings are consistent with:
a. A DNA virus infection
b. A protozoan infection
c. A fungal infection
d. Primary HIV pathology
*17. Which of the statements about spinal cord pathology in HIV infection is not true?
a. Spinal cord lesions in AIDS may be caused by HSV2 myelitis.
b. Spongy myelinopathy in the spinal cord is causedby HIV.
c. The lesions have some similarity to tropical myeloneuropathy.
d. The pathology resembles motor neuron disease.
18. Animal prion diseases occur naturally in the US
a. True
b. False
*19. A 6 day old baby developed sepsis, hypotonia, respiratory insufficiency, and died. The
autopsy revealed the brain stem (left) and spinal cord (right) lesions shown below. The
most likely diagnosis is:
a. Congenital CMV infection
b. Congenital HSV infection
c. Congenital Coxsackie infection
d. Congenital toxoplasmosis
20. The lesion illustrated below may be caused by:
a. A DNA virus
b. A retrovirus
c. A mycobacterium
d. CJD
21. The pathology shown below most likely represents:
a. Cryptococcal infection
b. HIV encephalitis
c. A prion infection
d. Clostridial infection
*22. The CSF in a patient with the lesion illustrated below shows:
a. No organisms, few lymphocytes, normal protein, and normal glucose
b. Fungal hyphae and yeasts, acute unflammation,increased protein, decreased glucose
c. Acid fast organisms, lymphocytes, increased protein, decreased glucose
d. Gram positive organisms, neutrophils, increased protein, decreased glucose
*23. The lesions illustrated below are caused by:
a. Fungi with yeasts and hyphae
b. Fungi with yeasts
c. Fungi with branching septate hyphae
d. Postmortem bacterial overgrowth
24. The lesion illustrated below is caused by:
a. DNA virus
b. Retrovirus
c. Enterovirus
d. All of the above
25. The lesion illustrated below is caused by:
a. Trauma
b. Ruptured anterior communicating aneurysm
c. A DNA virus
d. Cerebral malaria
*26. A 60 year old patient presented with fever, headaches, seizures and hemorrhagic CSF.
MRI showed a left frontotemporal lesion with edema. The biopsy of the hemorrhagic lesion
is shown below. The diagnosis can be best obtained by:
a. Bacterial culture
b. Viral PCR
c. Fluorescent antibody studies
d. ELISA test
27. The lesions below are caused by:
a. Congenital toxoplasmosis.
b Congenital HSV.
c. Congenital CMV.
d Congenital varicella zoster virus
28. The most likely cause of the pathology in the 59 year old patient illustrated below is:
a. Meningococcus
b. Pneumococcus
c. Haemophilus influenzae
d. E. Coli
Demyelinating Diseases:
1. Multiple sclerosis plaques involve spinal roots
A. True
B. False
2. All MS follows a relapsing and exacerbating course
A. True
B. False
3. Is remyelination in the central nervous system possible?
A. Yes
B. No
4. Axons are not damaged in multiple sclerosis
A. True
B. False
5. MS is more common in northern countries
A. True
B. False
6. Which of the following is useful for treating MS?
A. INF Beta
B. INF Gamma
*7. A 32 year old woman developed acute neurologic deficits and contrast enhancing
periventricular lesions. A stereotactic biopsy revealed changes shown below.
Immunohistochemistry of this lesion will show:
A. A vast majority of B cells.
B. T cells.
C. A mixture of T and B cells
8. Conduction velocity in the peroneal nerve of a patient with MS and paraparesis is:
A. Increased
B. Decreased
C. Normal
D. Decreased only if the leg is totally paralyzed
9. The risk of MS in the identical twin of a patient with MS is:
A. 5%
B. 15%
C. 25%
D. 50%
10. Which of the following is not seen in old MS lesions?
A. Gliosis
B. Macrophages
C. Axonal loss
D. Dense perivascular mononuclear cells
11. Devic's disease is characterized by involvement of:
A. The optic nerve only
B. The optic radiation
C. The brainstem
D. The spinal cord only
E. None of the above
12. Which of the following is most commonly affected in MS?
A. Cerebellum
B. Brainstem
C. Periventricular white matter
D. The subcortical white matter
13. A 37 year old man had progressive neurologic deficits including hemiparesis and ataxia
for nine months. CSF shows 37 lymphocytes, protein 54, glucose 50, and no oligoclonal
bands. The most likely diagnosis is:
A. AIDS
B. Lyme disease
C. MS
D. Tuberculous meningitis
14. A 12 year old white male had progressive psychomotor decline for three years and the
MRI findings shown below
The CSF shows 15 lymphocytes, glucose 67, protein 43, and normal CSF IgG/albumin ratio.
The most likely diagnosis is:
A. MS
B. X-linked adrenoleukodystrophy
C. Mucopolysaccharidosis
D. Metachromatic leukodystrophy
*15. A 37 year old man with AIDS developed ataxia and paralysis. The MRI shows
cerebellar and brainstem nonenhancing lesions. The cerebellar biopsy includes mostly
normal cerebellar cortex and a small amount of white matter which shows a few
mononuclear cells, macrophages and reactive astrocytes. The best use of the material in
order to obtain a diagnosis is:
A. Reprocessing the material for EM
B. Using the material for viral PCR
C. Doing immunohistochemistry for papova viruses
D. Recommending a spinal tap with culture and PCR
16. Which of the following structures is most commonly affected in MS?
A. The internal capsule
B. The optic radiation
C. The optic chiasm
D. The brainstem
17. A 26 year old woman developed severe dizziness, hoarseness, extension of the neck,
and right arm weakness in the course of 8 days. The MRI shows enhancing lesions in the
lower brainstem and upper spinal cord. A stereotactic biopsy is shown below.
The most likely diagnosis is:
A. Cerebral lymphoma
B. Herpes simplex encephalitis
C. Acute disseminated encephalomyelitis
D. Multiple sclerosis
*18. A 32 year old hemophiliac patient developed neurologic deficits and enhancing
cerebral and cerebellar white matter lesions over a period of four months. A stereotactic
biopsy of the cerebellum is shown below.
The most likely diagnosis is:
A. Glioblastoma multiforme
B. AIDS encephalitis
C. An opportunistic viral infection
D. Lhermitte-Duclos syndrome
19. The pathology illustrated in this myelin stained section of the pons is likely to occur in
all of the following conditions except:
A. Severe burns
B. Advanced alcoholic cirrhosis
C. Severe bacterial infections
D. Vitamin B1 deficiency
E. Chronic renal failure
20. Stereotactic brain biopsy of a ring enhancing lesion of the left frontal lobe in a
previously healthy 37 year old woman who had neurological symptoms for 2 months. Work
up showed no fever, sinusitis or other findings. The most likely diagnosis is:
A. MS
B. Cerebral abscess
C. A viral infection
D. Cerebral lymphoma
22. The pathology in a 17 year old male shown below represents:
A. An inflammatory demyelinative disease
B. A metabolic disorder of peroxisomes
C. A metabolic disorder of lysosomes
D. Post anoxic demyelination
Brain Tumors:
1. Which are most common overall?
A. Primary brain tumors
B. Metastatic
2. A child with craniospinal irradiation for ALL has a higher risk for developing an
astrocytoma compared to other children
A. True
B. False
3. Radiation to the head and neck is a risk factor for subsequent development of
meningioma
A. True
B. False
*4. Most primary cerebral lymphomas are:
A. T-cell.
B. B-cell.
C. They are evenly split
5. Homer-Wright rosettes are relatively uncommon in medulloblastoma
A. True
B. False
6. Low grade tumors have as many but different chromosomal abnormalities as high grade
tumors do
A. True
B. False
*7. A 5 year old boy had multifocal brain lesions one of which is shown below. The most
likely underlying factor is:
A. Genetic immunodeficiency
B. Previous cranial irradiation for ALL
C. EBV infection
D. None - this is probably a random event
8. Which of the following are the most ubiquitous neurocarcinogens?
A. Ambient tobacco smoke
B. Industrial solvents
C. Nitroso compounds
D. Pesticides
9. Which of the following is not a feature of pilocytic astrocytoma?
A. Granular eosinophilic droplets
B. Brisk mitotic activity
C. Drop metastases
D. Location in the spinal cord
E. Hydrocephalus
10. What finding distinguishes glioblastoma multiforme from lower grade astrocytomas?
A. Mitoses
B. Cellular density
C. Necrosis
D. Marked anaplasia (giant cells)
*11. Which of the following lesions extends across the corpus callosum?
A. Glioblastoma
B. Cerebral lymphoma
C. Schilder's disease
D. All of the above
12. Which of the following may present as a ring enhancing lesion?
A. Acute multiple sclerosis plaques
B. Metastatic tumors
C. Glioblastoma
D. All of the above
13. A 3 year old boy with a history of headaches and morning vomiting for 10 days presents
to the ER after a bad episode of vomiting. He is afebrile. The diagnostic studies might
include all of the following except:
A. Fundoscopic examination
B. CSF for enterovirus PCR
C. GI endoscopy
D. MRI
*14. The most common intracranial site of germ cell tumors is:
A. The pineal gland
B. The pituitary gland
C. The hypothalamus
D. The midline of the cerebellum
*15. Which of the following statements about the posterior fossa lesion shown below is not
true
A. It is classified as WHO Grade I
B. It evolves to malignant over many years
C. Most such lesions arise in the cerebellum
D. It shows vascular endothelial proliferation
E. It enhances on MRI imaging
*17. Which of the following statements about the lesion illustrated below
is not true?
A. Most such lesions are WHO grade 2
B. It has the highest incidence of CSF seeding
C. It may be exophytic
D. It may contain areas indistinguishable from low grade astrocytoma
E. It is not highly radiosensitive
18. Which of the following statements about the brain tumor in a 3 year old boy shown
below is not true?
A. Most such tumors are supratentorial
B. It can be familial
C. It can cause hydrocephalus
D. It may present with blurred vision
E. Ataxia is relatively uncommon as a presenting sign
19. A 4 year old boy with a history of headaches and on-and-off vomiting for 2 weeks had
the MRI findings shown below. The most likely diagnosis is:
A. Medulloblastoma
B. Glioblastoma of the cerebellum
C. Ependymoma of the 4th ventricle
D. Aqueductal stenosis
20. Which of the following statements about the lesion shown below is not true?
A. There are over ten histological subtypes
B. The tumor is attached to the dura
C. Deletion of chromosome 22 is frequent
D. Brain invasion is frequent
E. It is more common in women
21. Which of the statements about the lesion shown below is not true?
A. It may invade bone and extend into the extracranial soft tissues
B. Recurrence indicates malignancy
C. It shows folding processes and dense junctions
D. It is EMA positive
*22. Which of the following statements about the illustrated lesion is not true?
A. It is an extra-axial lesion
B. It is often seen in neurofibromatosis 1
C. It is benign
D. There may be multiple tumors
E. It extends along the vertebral foramina
*23. Which of the following statements about the lesion shown below is not true?
A. It may be an intra-spinal tumor
B. Malignant degeneration is more frequent after irradiation
C. The optic nerve is a common intracranial location
D. It may occur in an autosomal dominant pattern
24. Which of the following is not a feature of the illustrated lesion?
A. Infrasellar location
B. Calcification
C. Gross cysts
D. Papillary pattern
E. Rosenthal fibers in surrounding tissue
25. Which of the following statements about the lesion shown below is not true?
A. The lesion is benign but may recur
B. It resembles some tumors arising in the jaws
C. It is usually part of a benign teratoma
D. It may be intraventricular
26. A 43 year old patient has had headaches and declining mental function for 5 weeks. MRI
shows mild hydrocephalus with periventricular enhancing lesions. A stereotactic biopsy is
obtained (shown below). The most likely diagnosis is:
A. HIV encephalitis
B. Progressive multifocal leukoencephalopathy
C. Cerebral lymphoma
D. HSV encephalitis
E. Cereral abscess
*27. A 42 year old woman had right-sided weakness progressing to hemiparesis in 3 to 4
weeks. MRI shows an enhancing lesion in the left centrum semiovale adjacent to the corpus
callosum. A stereotactic biopsy is obtained (shown below).
The most likely diagnosis is:
A. An inflammatory demyelinative disease
B. Cerebral lymphoma
C. CNS vasculitis with ischemic infarction
D. Progressive multifocal leukoencephalopathy
E. Glioblastoma
*28. A 57 year old patient had fever, seizures and obtundation for 4 days. MRI shows a
necrotic hemorrhagic lesion of the right temporal lobe with surrounding edema. A
stereotactic biopsy is shown below.
The most likely diagnosis is:
A. Cerebral abscess
B. Hemorrhagic infarct
C. Cerebral lymphoma
D. HSV encephalitis
*29. A 6 year old girl with diabetes insipidus, visual disturbances, and headaches for 3
weeks had an MRI scan showing a 2 cm suprasellar mass and mild hydrocephalus. A
stereotactic biopsy is shown below.
The most likely diagnosis is:
A. Craniopharyngioma
B. Germ cell tumor
C. Langerhans histiocytosis
D. Pituitary adenoma
*30. A 27 year old woman with headaches and vomiting had a cystic cerebellar mass with a
protruding nodule. Stereotactic biopsy is shown below.
The most likely diagnosis is:
A. Metastatic clear cell carcinoma
B. Hemangioblastoma
C. Oligodendroglioma
D. Old infarct with macrophage reaction
*31. The illustrated structures may be present in:
A. Pilocytic astrocytoma
B. Oligodendroglioma
C. Both
D.They are non-specific and may be present in diverse neoplastic and non-neoplastic conditions
32. A previously healthy 53 year old man had an insidious onset of headaches, obtundation,
and facial paralysis. CSF shows 33 lymphocytes, glucose 36, and protein 72. The most likely
diagnosis is:
A. Meningeal carcinomatosis
B. Tuberculous meningitis
C. Subacute encephalitis
D. Multiple sclerosis
*33. The most common tumors of the pineal gland are:
A. Pineal parenchymal tumors (pinealoma and pineoblastoma)
B. Germ cell tumors
*34. You are called to the OR to do a frozen section of a suprasellar mass in a 5 year old
boy. Which of the following would be most likely?
A. Pituitary adenoma
B. Craniopharyngioma
C. Pilocytic astrocytoma
D. Endodermal sinus tumor
35-40. Match the following tumors with the underlying conditions
A. Neurofibromatosis 1 (VRNF)
B. Neurofibromatosis 2 (BANF)
C. von Hippel-Lindau disease
D. Tuberous sclerosis
35. Spinal schwannoma: 35A, 35B, 35C, 35D
36. Meningioma: 36A, 36B , 36C, 36D
37. Optic nerve astrocytoma: 37A, 37B, 37C, 37D
38. Subependymal giant cell astrocytoma: 38A, 38B, 38C, 38D
39. Pheochromocytoma: 39A, 39B, 39C, 39D
40. UBO-type lesions on MRI: 40A, 40B, 40C, 40D
41-45. Match the numbered tumors with the lettered locations
A.Intra-axial
B.Extra-axial
41. Meningioma: 41A, 41B
42. Ependymoma of the 4th ventricle: 42A, 42B
43. Eight nerve schwannoma: 43A, 43B
44. Medulloblastoma: 44A, 44B
45. Pituitary adenoma: 45A, 45B
*46. A three year old boy had a cerebellopontine angle tumor with the ultrastructural
features illustrated below.
The diagnosis is:
A. Meningioma
B. Ependymoma
C. Schwannoma
D. Exophytic pilocytic astrocytoma
Degenerative Diseases:
1. Abnormal Tau deposits are found in:
A. Senile plaques
B. Neuropil threads
C. Both
D. Neither
2. The main component of neurofibrillary tangles is:
A. Tau protein
B. Ubiquitin
C. Synuclein
D. Beta amyloid
*3. Neurofibrillary tangles occur also in:
A. Friedreich ataxia
B. Huntington's disease
C. Creutzfeldt-Jacob disease
D. Progressive supranuclear palsy
4. Dementia can be caused by all of the following except:
A. Creutzfeldt-Jacob disease
B. MS
C. A large MCA infarct
D. Diffuse axonal injury
5. Most cases of Alzheimer's disease are:
A. Autosomal dominant
B. Autosomal recessive
C. Multifactorial
D. Environmental
6. The amount of beta amyloid made by cells of a patient with Down syndrome is:
A. The same as normal people
B. 1.5 times normal
C. 2 times normal
D. 3 times normal
*7. The earliest changes in Alzheimer's disease are usually found in:
A. Hippocampus
B. Entorhinal cortex
C. Amygdala
D. Association cortex
8. A patient with large ventricles, dementia, incontinence, and abnormal gait should:
A . Have a brain biopsy to rule out Alzheimer's disease
B . Have a shunt placed to relieve hydrocephalus
9. Which type of Alzheimer's disease plaque is more important clinically:
A. Diffuse plaque
B. Neuritic plaque
C. Both are equally important
10. The pathology shown in this Bielschowsky silver stain below occurs in:
A. In post-traumatic dementia
B. In some cases of Parkinsonism
C. Both
D. Neither, it only occurs in Alzheimer's disease
11. The pathology shown below occurs in:
A. Alzheimer's disease
B. Pick's disease
C. Creutzfeldt-Jacob disease
D. HIV encephalitis
12. The best match for the illustrated changes is:
A. An 83 year old person with memory loss and spacial disorientation
B. A 43 year old person with abnormal movements who committed suicide>
C. A 56 year old person with progressive paralysis
D. A 60 year old person with tremor, rigidity and dementia
13. The best match for the illustrated changes is:
A. A 76 year old person with memory loss and spacial disorientation
B. A 43 year old person with abnormal movements who committed suicide
C. A 56 year old person with progressive paralysis
D. A 60 year old person with tremor, rigidity and dementia
14. The best match for the illustrated changes is:
A. An 83 year old person with memory loss and spacial disorientation
B. A 43 year old person with abnormal movements who committed suicide
C. A 56 year old person with progressive paralysis
D. A 60 year old person with tremor, rigidity and dementia
*15. Axonal swellings in diffuse axonal injury contain:
A. Beta amyloid precursor protein
B. Beta amyloid
C. Both
*16. The CSF in Alzheimer's disease shows:
A. Elevated Tau and decreased beta amyloid
B. Decreased Tau and elevated beta amyloid
*17. Elevation of protein 14-3-3 in CSF occurs in:
A. CJD
B. Alzheimer's disease
C. Both
D. Neither
18. Which of the following APO E genotypes confers the lowest risk for dementia:
A. APO E2/2
B. APO E2/3
C. APO E3/3
D. APO E3/4
E. APO E4/4
19. Many patients with Alzheimer's disease also have cerebral amyloid angiopathy:
A. True
B. False
20. The pathology shown is associated with:
A. Dementia and severe memory impairment
B. Dementia, abnormal movements, and seizures
C. Dementia and tremor
D. Dementia with language dysfunction
21. Frontotemporal dementias may be accompanied by degeneration of all of the following
structures except:
A. The substantia nigra
B. The cerebellum
C. The anterior horns
D. The amygdala
22. Ubiquitin is important for degradation of:
A. Nucleic acids
B. Lipids
C. Carbohydrates
D. Proteins
23. Which of the following is least likely to be affected in Parkinson's disease:
A. The cerebral cortex
B. The nucleus basalis of Meynert
C. The hypothalamus
D. The caudate nucleus
*24. The pathology shown in this ubiquitin immunostain of the cerebral cortex is associated
with:
A. Dementia and hallucinations
B. Dementia and motor neuron disease
C. Dementia and choreoathetosis
D. Dementia and severe memory loss
25. MPTP damages:
A. Lysosomes
B. Mitochondria
C. Proteasomes
D. Nuclei
*26. Parkinsonian manifestations may develop following poisoning with:
A. Manganese
B. Organophosphates
C. Lead
D. Acrylamide
27. Parkinsonian manifestations may develop with all of the following except:
A. HIE
B. Heavy metal poisoning
C. MS
D. Prion disease
28. Which of the following is not a trinucleotide repeat disorder:
A. Parkinson•'s disease
B. Huntington's disease
C. Friedreich•'s ataxia
D. Myotonic dystrophy
29. The pathology shown below is least likely to be caused by:
A. The Guillain-Barre syndrome
B. Diabetic neuropathy
C. Spinal muscular atrophy
D. ALS
30. The pathology shown below causes:
A. Spasticity and exaggerated reflexes
B. Muscle atrophy
C. Both
D. Neither
*31. The muscle pathology (ATPase stain) shown in this severely hypotonic infant is caused
by:
A. GAA trinucleotide repeat
B. SMN1 deletion
C. PMP22 duplication
D. PMP22 deletion
32. The pathology shown is most likely to occur in:
A. A 79 year old demented patient
B. A 60 year old patient with pes cavus and arrhythmias
C. A 61 year old patient with Parkinsonian manifestations
D. A 59 year old patient withextensive intestinal resection
33. Frataxin is located in the:
A. Mitochondria
B. Lysosomes
C. Nucleus
D. Cytosol
*34. Which of the following is not a polyglutamine disorder:
A. Huntington's disease
B. Alzheimer's disease
C. DRPLA
D. Machado-Joseph disease
*35. Which of the following is a polyglutamine disorder:
A. Friedreich's ataxia
B. Autosomal dominant spinocerebellar ataxia
C. OPCA
D. The Fragile X syndrome
Congenital Malformations:
1. Exposure to a teratogen may cause CNS malformations if it occurs at 3 to 8 weeks of
gestation and have minimal or no effects if it occurs later
A. True
B. False
2. Congenital CMV infection at 9-12 weeks of gestation can cause:
A. Porencephaly
B. Polymicrogyria
C. Schizencephaly
D. All of the above
3. Congenital CMV infection starting at 37 weeks gestation can cause:
A. Polymicrogyria
B. Schizencephaly
C. Microcephaly
D. CMV encephalitis
4. To be effective in preventing neural tube defects, women of child-bearing age must take
0.8 mg folic acid daily:
A. From conception to 8 weeks of gestation
B. 4 weeks before to 4 weeks after conception
C. 4 weeks before to 8 weeks after conception
D. 8 weeks before and 8 weeks after conception
5. Which is not a neural tube defect
A. Hydranencephaly
B. Exencephaly
C. Anencephaly
D. Encephalocele
6. Mutation of which is associated with NTDs?
A. Factor V Leiden
B. Prothrombin 20210A
C. MTHFR
D. Factor XIII
*7. Which of the following mutations is associated with NTDs?
A. MTHFR 677
B. MTHFR 1928
C. Both
D. Neither
8. Alobar holoprosencephaly (HPE) is incompatible with survival
A. True
B. False
9. HPE may be associated with all of the following except:
A. Fusion of the thalami
B. Hydrocephalus
C. Absence of the olfactory nerves
D. Abnormal cortical cytoarchitecture
10. HPE may be associated with all of the following except:
A. Fusion of the thalami
B. Fusion of the frontal lobes
C. Cerebellar hypoplasia
D. Absence of olfactory nerves
*11. A newborn baby with a single central incisor may have:
A. Schizencephaly
B. Agenesis of the corpus callosum
C. HPE
D. Cerebellar hypoplasia
12. Which of the following is implicated in the pathogenesis of HPE?
A. Folate deficiency
B. Fetal viral infection
C. Abnormal cytoskeletal assembly
D. Abnormal cholesterol metabolism
13. Agenesis of the corpus callosum may be confused in fetal ultrasound with alobar HPE
A. True
B. False
14. Patients with agenesis of the corpus callosum may have relatively normal intelligence
A. True
B. False
15. The most common chromosomal abnormality associated with HPE is:
A. Trisomy 13
B. Trisomy 18
C. Trisomy 21
D. Triploidy
16. Lissencephaly is most commonly associated with:
A. Fetal CMV infection
B. Mutations of sonic hedgehog
C. Mutations of L1CAM
D. Abnormal folate metabolism
E. Microdeletions of chromosome 17p
17. Which of the following is associated with abnormal cortical layering:
A. Peroxisomal defect
B. Folate deficiency
C. Sonic hedgehog mutation
D. Dystrophin mutation
*18. Abnormal neuronal migration may cause:
A. Schizencephaly
B. Lissencephaly
C. HPE<
D. Agenesis of the corpus callosum
*19. A smooth cortex and large ventricles on fetal ultrasound without other abnormalities is
most likely caused by:
A. An inherited metabolic disorder of peroxisomes
B. A genetic condition associated with congenital muscular dystrophy
C. Both
D. Neither
20. A microdeletion of 17p may be associated with:
A. Polymicrogyria
B. Lissencephaly
C. Schizencephaly
D. Agenesis of the corpus callosum
21. A thick cortex with abnormal neuronal layering is seen in:
A. Pachygyria
B. Lobar HPE
C. Dandy Walker Syndrome
D. All of the above
22. Lissencephaly may be associated with all of the following except:
A. X-linked inheritance
B. Mid-facial abnormalities
C. Seizures
D. Hypotonia in infancy
23. Hydrocephalus ex vacuo is seen in all of the following conditions except:
A. Huntington’s disease
B. Parkinson’s disease
C. Alzheimer’s disease
D. MS
24. The most common cause of obstructive hydrocephalus in children is:
A. X-linked aqueductal stenosis
B. Meningitis
C. Tuberous sclerosis
D. Posterior fossa tumors
25. The Chiari I malformation shows all of the following except:
A. Dilated ventricles in some cases
B. Syringomyelia
C. Myelomeningocele
D. Displacement of cerebellar tonsils into the spinal canal
26. The key feature of the Dandy-Walker Syndrome is:
A. Agenesis of the cerebellar vermis
B. Dilatation of the lateral ventricles
C. A large cisterna magna
D. Myelomeningocele
27. Patients with the Chiari II malformation may have all of the following except:
A. Seizures
B. Paralysis of the lower extremities
C. Bladder incontinence
D. Bacterial ventriculitis
28. The picture below shows the spinal cord in the center. A portion of a vertebral body is
seen anterior to it. The illustrated pathology may be associated with all of the following
except:
A. Occipital encephalocele
B. Hydrocephalus
C. Herniation of the cerebellar vermis
D. Small posterior fossa
*29. The picture below shows the cerebellum viewed from behind and above. The
illustrated pathology is:
A. Usually an inherited defect
B. Usually a sporadic lesion
30. The illustrated pathology is invariably associated with severe psychomotor
retardation
A. True
B. False
31. The illustrated pathology is associated
with:
A. Irreversible neurological damage
B. Reversible damage if shunted in time
32. The pathology in a newborn infant depicted below is associated
with:
A. Folate deficiency
B. Sonic hedgehog mutations
C. X-linked inheritance
D. Excess retinoic acid
33. The pathology depicted is associated with all of the following
except:
A. An autosomal trisomy
B. Absence of olfactory nerves
C. Myelomeningocele
D. Fusion of the thalami
*34. The illustrated lesion may be associated with all of the following
except:
A. Absence of olfactory nerves
B. Normal intelligence
C. Dilated posterior horns of the lateral ventricles
D. Abnormal inferior olives
35. Lobar HPE may be associated with relatively normal intelligence
A. True
B. False
End.