Section 4 Flashcards
Pathology and Radiology
The anterior neuropore closes at which gestational age?
A. 13 days
B. 17 days
C. 22 days
D. 24 days
E. 28 days
D. 24 days
The neural tube closure starts in the middle at 22 days, then the anterior neuropore at 24 days forming the lamina terminalis, and then the posterior neuropore at 28 days.
The following histologic finding is characteristic of
A. Glioblastoma multiformes
B. Toxoplasmosis
C. Subependymal giant cell astrocytDma
D. Bacterial brain abscess
B. Cysticercosis
B. Toxoplasmosis
Bradyzoites are bound in a cyst (bag) while tachyzoites are loose. They are both characteristic of toxoplasmosis. The infection risk is higher in AIDS patients. The lesions are ring enhancing and appear as a target sign on CT or MRI.
The mechanism that best describes the following CT finding is
A. Axial load
B. Extension injury
C. Flexion injury
D. Distraction injury
E. Flexion and distraction injury
E. Flexion and distraction injury
The typical mechanism for a Chance fracture is a seat-belt injury with Hexion distraction.
Which disease is X-linked recessive, characterized by ceramide accumulation in the tissues, and manifests as corneal opacities, cerebrovascular occlusions, peripheral neuropathy, and skin
angiokeratomas?
A. Fabry’s disease
B. Niemann-Pick’s disease
C. Tay-Sachs disease
D. Hurler’s disease
E. Gaucher’s disease
A. Fabry’s disease
Fabry’s disease is a lysosomal storage disease, X-linked recessive, characterized by a-galactosidase A deficiency. It causes reramide (sphingosine + long-chain fatty acid) accumulation in tissues, corneal opacities, rerebrovascular occlusions, CAD, kidney insufficiency, skin angiokeratomas, and weakness from peripheral neuropathy.
The abnormality depicted in the foJlowing MRI is caused by
A. Primacy neurulation defect
B. Abnormal disjunction
C. Abnormal ventral induction
D. Abnormal migration
D. Abnormal migration
Cavum vergae and polymicrogyria are both caused by abnormal migration and occur at 2-5 months gestation
All of the following are characteristics of Wilson’s disease, except
A. Kayser-Fleischer ring of the cornea
B. liver cirrhosis
C. Spongiform red degeneration of the lentifonn nucleus
D. Opalski cells
E. Increase serum copper (Cu)
E. Increase serum copper (Cu)
In Wilson’s disease. there is decreased serum Cu and ceruloplasmin, increased urinary Cu.
The following tumor was removed from the frontal lobe of a 35-year-old male with seizures. What is the diagnosis?
A. Central neurocytoma
B. Fibrillary astrocytoma
C. Oligodendroglioma
D. Ganglioglioma
E. Dysembcyoplastic neuroepithelial. tumor (DNET)
C. Oligodendroglioma
Oligodendrogliomas are characterized by small round nuclei and clear cytoplasm (perinuclear halo, artifact) giving it fried egg-yolk or bee-hive appearance. They also have chicken-wire vascular pattern. The presence of 1p19q deletion is a good prognostic factor. It favors response to chemotherapy: PCV (Procarbazine, CCNU [lomustine], Vincristine) or temozolomide. In the absence of increased cellularity, mitotic figures, cellular pleomorphism, and endothelial proliferation, the tumor shown is WHO grade II. Patients with WHO
grade III (anaplastic oligodendroglioma) should get radiotherapy as well.
The following neural tube defect occurs at which gestational age?
A. 3-4 weeks
B. 4-5 weeks
C. 5-10 weeks
D. 2-5 months
E. 6-7 months
A. 3-4 weeks
Myelomeningocele is a disorder of primary neurulation that occurs at 3-4 weeks gestation. Surgery (closure) should be performed within 48 hours of birth to avoid meningitis and in some specialized centers intrauterine (prenatal, fetal) surgery is done. The brain should be screened for hydrocephalus. With fetal surgery, there is less need for shunting and better functional outcome, but more premature delivery and uterine dehiscence.
Which disease is aumsomal recessive characterized by Zebra bodies, gargoyle face, thick meninges, and hepatosplenomegaly?
A. Morquio’s syndrome
B. Krabbe’s disease
C. Maroteaux-Lamy syndrome
D. Hurler’s disease
E. Sly’s syndrome
D. Hurler’s disease
These are features of Hurler’s disease, which is a mucopolysaccharidose (MPS IH). It is caused by alpha-L-iduronidase deficiency and urine shows increased dermatan sulfate.
Refsum’s disease is characterized by all of the following, except
A. Autosomal recessive
B. Cu/Zn superoxide dismutase deficiency
C. Peripheral neuropathy
D. Retinitis pigmentosum
E. Cardiomyopathy
B. Cu/Zn superoxide dismutase deficiency
Refsum’s disease is caused by phytanic acid oxidase deficiency. Cu/Zn superoxide dismutase deficiency is a feature of ALS.
Which vasculopathy is characterized by orogenital ulcers, uveitis, ulcerative colitis, and meningo-encephalitis?
A. Behcet’s disease
B. Takayasu’s arteritis
C. Kawasaki’s disease
D. Wegenets granulomatosis
E. Polyarreritis nodosa
A. Behcet’s disease
Behcet’s disease is characterized by orogenital ulcers, uveitis, ulcerative colitis, meningoencephalitis, brain stem edema, confusion, erythema nodosum, and polyarthritis. Treatment includes corticosteroids.
GorJin’s syndrome, which includes medulloblastoma, meningioma, and basal cell nevus syndrome is caused by an abnormality of which chromosome?
A. 3
B. 5
C. 9
D. 10
E. 17
C. 9
Gorlin’s syndrome is inherited as autosomal dominant on chromosome 9.
The following brain tumor is seen in which disease?
A. Von Hippel-Lindau disease
B. Tuberous sclerosis
C. Neurofibromatosis 1
D. Neurofibromatosis 2
E. Li-Fraumeni syndrome
B. Tuberous sclerosis
Large astrocytes with large eccentric nuclei are characteristics of SEGA. SEGA occurs in 15% of patients with tuberous sclerosis. Tuberous sclerosis is inherited as autosomal dominant on chromosome 9 or 16.
Which of the following is not a mitnchondrial myopathy?
A. MELAS (myopathy, encephalopathy, lactic acidosis, and stroke-like episodes)
B. MERRF (myoclonic epilepsy with ragged red fibers)
C. MNGIE (mitochondrial neurogastrointestinal encephalopathy)
D. Kearns-Sayre syndrome
E. Familial periodic paralysis
E. Familial periodic paralysis
Mitochondrial myopathies inherited through maternal mitochondria include MELAS, MERRF, MNGIE, Luft’s, and Leigh’s diseases. Keams-Sayre is a defect in mitochondrial DNA not maternally inherited. Familial periodic paralysis is caused by a genetic defect coding for Ca in muscle fiber membranes.
Neurofibrillary tangles and neuritic plaques are found in which disease?
A. Alzheimer’s disease
B. Pick’s disease
C. Huntington’s chorea
D. Parkinson’s disease
E. Shy-Drager disease
A. Alzheimer’s disease
Alzheimer’s disease is characterized by neurofibrillary tangles (tau protein), neuritic plaques (beta amyloid), Hirano bodies, and lack of cholinergic output from the amygdala to the cerebral cortex. Treatment includes donepezil (Aricept) which is an acetylcholinesterase inhibitor.
The following tissue is from a temporal lobe lesion of a child with seizures. What is the diagnosis?
A. Ganglioglioma
B. Dysembryoplastic neuroepithelial tumor (DNET)
C. Chondrosarcoma
D. Oligodendroglioma
E. Central neurocytoma
B. Dysembryoplastic neuroepithelial tumor (DNET)
In DNET neurons are floating in mucinous material, small cells resemble oligodendrocytes, and vessels have a chicken-wire pattern.
The congenital abnormality in the following MRI is caused by
A. Primary neurulation defect
B. Abnormal disjunction
C. Abnormal ventral induction
D. Abnormal migration
C. Abnormal ventral induction
Alobar holoprosencephaly occurs at 5-10 weeks gestation and is caused by abnormal ventral induction.
Which type of headache is unilateral, more common in males, associated with rhinorrhea, lacrimation, and conjunctival injection?
A. Classic migraine
B. Commonmigraine
C. Cluster headache
D. Trigeminal neuralgia
E. Ramsay Hunt syndrome
C. Cluster headache
Cluster headache is characterized by increased parasympathetic discharge causing hypersecretions. Classic migraine has aura while common migraine has no aura. Tolosa-Hunt is painful ophthalmoplegia and Ramsay Hunt is herpes zoster of the geniculate ganglion.
The tumor depicted below was recovered from the cerebellum of a patient with abnormality of which chromosome?
A. 3
B. 5
C. 9
D. 17
E. 22
A. 3
Clear cells (lipid content) occur in hemangioblastoma. They stain positive for vimentin while renal cell carcinoma stain positive for EMA and cytokeratin. When part of von Hippel-Lindau disease, it is inherited as autosomal dominant on chromosome 3.
Which toxicity is characterized by encephalitis, peripheral neuropathy, and basophilic stippling of red blood corpuscles?
A. Mercury
B. Methotrexate
C. Arsenic
D. Manganese
E. Lead
E. Lead
Basophilic stippling of RBCs is found in cases of lead poisoning, Mees’ lines (white on finger nails) in arsenic cases, cerebellar tremors and renal tubular necrosis in mercury poisoning, parkinsonism in manganese toxicity, and subacute necrotizing leukoencephalitis (SNLE) with methotrexate.
The finding in the following MRI is suggestive of
A. Gerrninoma
B. Tectal glioma
C. Pineal teratoma
D. Vein of Galen malformation
E. Giant aneurysm of the posterior cerebral artery (PCA)
D. Vein of Galen malformation
A well-circumscribed mass in the pineal region with various MRI signal from blood products is consistent with a diagnosis of Vein of Galen malformation. In neonates, high cardiac output heart failure can be fatal and may warrant urgent treatment, preferably endovascular. In older children, it
can cause obstructive hydrocephalus.
Based on the following CT scan. what is the most important next step in the management of this patient?
A. Gardners-Wells traction
B. Halo-vest
C. Posterior C1-C2 fusion
D. Anterior odontoid screw
E. None of the above, this is a normal finding
B. Halo-vest
Atlanto-occipital dislocation is evidenced by increase in the basion-dens interval. CTA or MRA would be important to rule out associated vascular injuries. Halo-vest stabilization with some axial compression is an important next step. Definitive
treatment will require posterior occipito-cervical fusion. C1-C2 fusion will not fix the instability which is between C1 and the occiput. Traction would be detrimental. The odontoid is intact.
The following tissue was removed from a C2 vertebral body lesion. What is the diganosis?
A. Chondrosarcoma
B. Chordoma
C. Metastaticcarcinoma
D. Osteoid osteoma
E. Osseous meningioma
B. Chordoma
Chordomas are characterized by physaliphorous cells and a mucinous background. They are common in the sacrum and divus and have a bright T2 signal on MRI. The best cure is en-bloc resection. Proton-beam radiation could be helpful if there is residual tumor.
Which leukodystrophy is X-llnked recessive characterized by tigroid pattern on the MRI, mental retardation, ataxia, nystagmus, and spastidty?
A. Krabbe’s disease
B. Metachromatic Jeukodystrophy
C. Pelizaeus-Merzbacher disease
D. Canavan’s disease
E. Alexander’s disease
C. Pelizaeus-Merzbacher disease
Pelizaeus-Merzbacher disease is an X-linked recessive leukodystrophy caused by a defect in synthesis of proteoJipid lipoprotein. It causes mental retardation with cerebellar involvement, ataxia, nystagmus, spasticity, and tigroid pattern on MRI.
The following tissue was removed from the brain of a child with seizure.s, what is the most likely diagnosis?
A. Ganglioglioma
B. Dysembrycplasticneuroepithelial tumor (DNET)
C. Subpendymal giant cell astrocytoma (SEGA)
D. Oligodendroglioma
E. Central neurocytoma
A. Ganglioglioma
Large multinucleat:ed ganglion cells are present in ganglioglioma. Perivascular lymphocytes can also be observed. These tumors as well as DNET are common in children and cause seizures.
The finding on the following CTA is suggestive of
A. ACOM aneurysm
B. Right ophthalmic artery aneurysm
C. Left ophthalmic artery aneurysm
D. Right MCA aneurysm
E. Left MCA aneurysm
E. Left MCA aneurysm
Left MCA aneurysm (arrow) is observed on the following brain CTA.
The tumor depicted below typically has all of the following radiological characteristics, except
A. Cerebellopontine angle location
B. Hypodense on CT
C. Hypointense on Tl-weighted MRI
D. Hyperintense on T2-weightrd MRI
E. No restricted diffusion
E. No restricted diffusion
Epidermoid cyst is evident by stratified squamous epithelium and abundant keratin with no hair follicles. There is typically restricted diffusion on MRI. Dennoid cysts have hair follicles in addition.
The following histology represents a lesion
removed from the skull of a child What is the diagnosis?
A. Lymphoma
B. Metastatic carcinoma
C. Chordoma
D. Eosinophilic granuloma
E. Cysticercosis
D. Eosinophilic granuloma
Bilobed nuclei of eosinophils (black arrow head), foamy macrophages (white arrow), and large grooved nudei (black arrow) are seen in eosinophilic granuloma (Langerhans cell histiocytosis).
The following imaging finding is characteristic of
A. Glioma of the fornix
B. Subependymoma
C. Colloid cyst
D. Subependymal giant cell astrocytoma (SEGA)
E. Rathke’s cleft cyst
C. Colloid cyst
Colloid cysts of the third ventricle occur in the region of the for amen of Monro and cause obstructive hydrocephalus. They are typically hyperdense on CT. On MRI, they are hyperintense on T1, hypointense on T2, but the reverse could occur depending on the cyst content. Histologically they are lined by a single layer of columnar cells.
All of the following features are true about the tumor depicted below, except
A. Commonly occurs in children
B. Frequently in the posterior fossa
C. Rarely cystic
D. Complete surgical resection is curative
E. Prognosis is good
C. Rarely cystic
Pilocytic astrocytomas contain Rosenthal fibers (white arrow), eosinophilic granular bodies (black arrow), and biphaslc architecture with both cystic (C) and fascicular (F) patterns. Grossly, they are typically cystic with a mural nodule.
The aneurysm depicted in the following angiogram is located at
A. PCOM (posterior communicating artery)
B. ACOM (anterior communicating artery)
C. Cavernous carotid artery
D. Carotid terminus
E. MCA (middle cerebral artery)
A. PCOM (posterior communicating artery)
A PCOM aneurysm (arrow} is seen on this angiogram.
A 35-year-old female has decreased vision and the following flair MRI. What is the most likely diagnosis?
A. Pseudotumor cerebri
B. Multiple sclerosis (MS)
C. Brain abscesses
D. Lymphoma
E. Multifocal glioblastoma multiforme
B. Multiple sclerosis (MS)
Typical clinical picture and imaging for MS. The T2 flair signals (hyperintense, triangular, and perpendicular to the lateral ventricle) are called Dawson’s fingers. The patient needs a lumbar puncture to look for oligodonal bands. Treatment includes corticosteroids, plasmapheresis, and disease-modifying agents including interferon and ocrelizumab.
The following tumor is found in patients with abnormalities in which chromosome?
A. 3
B. 5
C. 10
D. 17
E. 22
E. 22
Schwannomas contain compact areas called Antoni A (A) and loose areas called Antoni B. Verocay bodies M have nudear
palisading around anudear areas. Some nuclei are arranged like a school of fish (S). They can occur in NF2, which is inherited as autosomal dominant on chromosome 22.
What is the abnormality in the following axial Tl-weighted MRI at L4-L5?
A. An extruded disc fragment
B. An arachnoid cyst
C. A synovial cyst
D. A schwannoma
E. A meningioma
C. A synovial cyst
Synovial cysts are hyperlntense on T2 MRI and are associated with the facet joints. In this case, it compresses the traversJng left L5 nerve root causing radiculopathy. Occasionally, they present with neurogenic daudication. Dynamic X-rays are
helpful to look fur instability. If patients fail conservative measures, surgica1 treatment includes decompression with or without fusion, depending on the presence or absence of instability, respectively.
The following histology is found in
A. Meningioma
B. Glioblastoma multiforme
C. Medulloblastoma
D. Ganglioglioma
E. Neurofibroma
A. Meningioma
A calcified psammoma body is seen In the center. Whorls are also present, confirming the diagnosis of meningioma. These tumors are positive for BMA and Vimentin and may occur in patients with NF2.
A 42-year-old female presents with quadriparesis and tingling. Based on the following MRI, what is the most likely diagnosis?
A. Ependymoma
B. Astrocytoma
c. Hemangioblastoma
D. Syrngomyelia
E. Transverse myelitis
A. Ependymoma
Cervical spinal cord expansion maximum at CS-C6 suggests an intramedullary tumor. Syringomyelia would have the same signal as CSF. Edema and hemoslderin deposition at both ends may show as a cap sign and are suggestive of ependymoma. The goal of surgery is gross total resection.
The histology of the tumor depicted below most likely will show all of the following, except
A. Antoni A
B. Antonie
C. School of fish
D. Keratin
E. Verocay bodies
D. Keratin
Schwannomas are S 100 positive. Diffuse keratin is found in epidermoid and dermoid cysts. Focal keratin is found in craniopharyngioma (sellar and suprasellar), adamantinomatous type.
The following CT shows
A. Os odontoideum
B. Type I dens fracture
C. Type II dens fracture
D. Type III dens fracture
B. Type IV dens fracture
C. Type II dens fracture
Os odontoideum is more circular with cortical bone all around, treatment is posterior Cl -C2 fusion. Type I dens fracture involves the tip of the dens (apical/alar ligament avulsion) and can be treated in a collar: type III involves the body of C2
and usually heals in a collar. Type II (the presented CT) is at the base of the dens, when non-displaced can have a trial of a cervical collar, when displaced surgery should be considered since the rate of non-union is high. They can be treated by an anterior odontoid screw ifthe anatomy is favorable, or posterior C1-C2 screws.
What structure is depicted in the following figure?
A. Pseudorosette
B. Pseudopalisading necrosis
c. Verocay body
D. Homer-Wright rosette
B. Flexner-Wintersteiner rosette
A. Pseudorosette
Pseudorosettes are around blood vessels and occur in ependymomas. Flexner-Wintersteiner rosette occurs around a central canal and are seen in ependymoma, pineoblastoma, and retinoblastoma. Homer-Wright rosette occurs in medulloblastoma or PNET and has no central canal or blood
vessel. Very large rosettes are seen in pineocytoma.
