Section 7 Flashcards
Pathology, Radiology, and Neurology
When the following tumor is associated with meningiomas and basal cell nevus syndrome, the most likely abnormality occurs on which chromosome?
A. 3
B. 5
C. 9
D. 11
E. 22
C. 9
Medulloblastoma, here characterized by large blue nuclei with little cytoplasm molded to each other and Homer-Wright rosettes. When part of Gorlin’s syndrome (chromosome 9), it can be associated with meningioma and benign cell nevus
syndrome. In medulloblastomas, WNT has the best prognosis and group 3 has the worst. Other types, including sonic hedgehog (SHH) and group 4, have intermediate prognosis.
While preparing for surgery for the following posterior fossa tumor, the surgeon and anesthesiologist should be aware of
A. Risk of hypertension
B. Risk of hematuria
C. Pressure points due to associated cutaneous neurofibromas
D. Difficult intubation due to sphenoid dysplasia
E. Risk of seizures
A. Risk of hypertension
Hemangioblastoma is the most common primary intra-axial tumor in the posterior fossa. Radiologically, it typically appears cystic with an enhancing mural nodule. Histologically, it is characterized by hemorrhage and dear cells (not to be confused with metastatic renal cell carcinoma, which would be positive for EMA). It stains positive for reticulin and vimentin. In 20% of cases, it is associated with van Hippel-Lindau (VHL) disease (chromosome 3 ). Pheochromocytoma occurs in 10% of cases ofVHL and can cause severe hypertension during surgery. Patients should be screened by plasma metanephrine, urinary catecholamines, metanephrine, and VMA.
The following applies to malignant hyperthermia, except
A. Autosomal dominant inheritance
B. Caused by a defect in porphobilinogen deaminase
C. Elevated creatine kinase (CK)
D. Hyperlcalemia
E. Treated with dantrolene
B. Caused by a defect in porphobilinogen deaminase
Malignant hyperthermia is autosomal dominant, on chromosome 19q, is caused by ryanodine receptor mutation, and causes increased Ca++ release from sarcoplasmic reticulum, increased CK, hyperkalemia, and myoglobinuria. It occurs with halothane and succinylcholine, and is characterized by fever, rigidity, hyperventilation, tachycardia, dysrhythmia, hypertension, and hypotension. Treatment includes dantrolene. Defect in porphobilinogen deaminase is associated with acute intermittent porphyria, not malignant hyperthermia.
The following tumor was removed from the lateral ventricle of an adult male and was positive for synaptophysin. What is the diagnosis?
A. Oligodendroglioma
B. Medulloblastoma
C. Choroid plexus papilloma
D. Subependymoma
E. Central neurocytoma
E. Central neurocytoma
Central neurocytomas are intraventricular tumors of young adults. They occur in the region of the foramen of Monro, typically attach to the septum pellucidum, and enhance with contrast (moderate and heterogeneous). Histologically, they are small cells with halos and ca.n be mistaken for oligodendrogliomas. Immunohistochemistry is positive for synaptophysin, and electron microscopy reveals dense-core synaptic vesicles. Subependymomas are common in the fourth ventricle and don’t enhance. Histologically, they have a spongy appearance with microcysts.
A unilateral headache in a young adult male associated with rhinorrhea, lacrimation, and conjunctival injection is characteristic of
A. Classic migraine
B. Common migraine
C. Migraine variant
D. Cluster headache
E. Temporal arteritis
D. Cluster headache
Cluster headache is a “hypersecretion” headche due to parasympathetic discharge. dassic migraine has aura while common migraine has no aura. Migraine variant has less headache and more neurological deficits. Temporal arteritis is characterized by headache, jaw pain, shoulder pain, loss
of vision, thick tender scalp vessels, high ESR, and giant cells on temporal a biopsy. Treat with corticosteroids until the ESR is normal.
A 74-year-old female presents with a 2-week history of speech impainnent and right upper
extremity weakness. She subsequently worsened neurologically and died. Autopsy findings are below. The most likely diagnosis is
A. Left MCA aneurysm
B. Pericallosal artery aneurysm
C. Hypertensive bleed
D. Amyloid angiopathy
E. Arterio-venous malformation
Cerebral amylold angiopathy characterized by amyloid deposition in the vessel wall replacing the media. The patient is elderly with multifocal intracerebral hemorrhage.
A 48-year-old male presents with stupor and right-sided hemiplegia. The patient’s grade is
A. Hunt and Hess Ill, Fisher 3
B. Hunt and Hess IV, Fisher 4
C. Hunt and Hess III, Fisher 4
D. Hunt and Hess IV, Fisher 3
E. Hunt and Hess V, Fisher 4
B. Hunt and Hess IV, Fisher 4
Because of hemiplegia, the patient is Hunt and Hess grade IV, and with intraventricular blood, the patient is Fisher grade 4
Neurofibrillary tangles and neuritic plaques are characteristic of which disease?
A. Alzheimer’s
B. Pick’s
C. Wilson’s
D. Parkinson’s
E. Hallervorden-Spatz
A. Alzheimer’s
Alzheimer’s disease is characterized by neurofibrillary tangles (tau protein), neuritic plaques (P/a protein amyloid), and Hirano bodies. Pick’s disease is characterized by Pick bodies, Parkinson’s disease by Lewy bodies, Wilson’s disease by Opalski cells, and Hallervorden-Spatz disease by iron deposition in SN and GP.
A cerebral angiogram is performed, revealing an aneurysm of
A. ACOM
B. MCA
C. Pericallosal artery
D. PCOM
E. Paraclinoid
A. ACOM
The angiogram reveals an ACOM aneurysm (arrow).
Which of the following vasculitides affects large and medium-sized vessels and causes interstitial
keratitis, Meniere’s symptoms. hearing loss, and
cavernous sinus thrombosis?
A. Cogan’s syndrome
B. Behcet’s disease
C. Buerger’s disease
D. Wegener’s granulomatosis
E. Takayasu’s arteritis
A. Cogan’s syndrome
Cogan’s syndrome can manifest by interstitial keratitis, Meniere’s symptoms, hearing loss, headaches, peripheral neuropathy, and cavernous sinus thrombosis. Behi;et’s disease is characterized by orogenital ulcers, arthritis, and uveitis. Buerger’s disease (or thromboangiitis obliterans) occurs with tobacco smoking. Wegener’s granulomatosis
affects respiratory, renal, and intracranial vessels and causes periphenl and cranial neuropathies. Takayasu’s arteritis affects the aorta and pulmonary arteries md can cause visual losss.
Which of the following does not apply to the tumor depicted below?
A. Male predominance
B. Mostly supratentoriat
C. High recurrence rate
D. Never metastasizes
E. Excessive bleeding in surgery
D. Never metastasizes
Staghorn blood vt5sels are characteristic of hemangiopericytoma. They also stain positive for reticulin. They arc common in middle-age, with slight male preponderance. They tend to recur and can metastasize.
Gargoyle face, thick meninges, deafness, and
hepatosplenomegaly are found in which mucopotysaccharidose?
A. Hunter’s syndrome
B. Hurler’s disease
C. Sanfilippo’s syndrome
D. Morquio’s syndrome
E. Maroteaux-Lamy syndrome
B. Hurler’s disease
Hurler’s disease (mucopolysaccharidoseMPS1H) is m autosomal recessive disease due to a-Liduronidase deficiency. Features include: Zebra bodies, increased urine heparin and dermatan sulfate, gargoyle face, mental retardation, and hepatosplenomegaly.
A cherry-red spot in the macula is found in all of the following lysosomal storage diseases, except
A. GMl gangliosidosis
B. Sandhoff’s disease
C. Tay-Sachs disease
D. Gaucher’s disease
E. Niemann-Pick’s disease
D. Gaucher’s disease
A cherry-red spot In the macula Is characteristic of GMl gangliosidosis, Sandhoffs disease, Tay-Sachs disease. and Niemann-Pick’s disease. Gaucher’s disease and Fabry’s diseaSe do not have a cherry-red spot in the macula.
The following depicted abnormality is caused by a disorder of
A. Primary neuruJation
B. Secondary neurulation
C. Ventral induction
D. Migration
C. Ventral induction
MRI reveals a lobar holoprosencephaly, which Is a disorder of ventral induction that occurs between 5 and 10 weeks.
A defect in hypoxanthine guanine phospho-ribosyl transferase (HGPRT) causes
A. Lesch-Nyhan disease
B. Menke’s kinky hair disease
C. Lowe’s syndrome
D. Zellweger’s syndrome
E. Leigh’s disease
A. Lesch-Nyhan disease
Collet-Sicard syndrome involves all of the following cranial nerves, except
A. VII
B. IX
C. X
D. XI
E. XII
A. VII
Collet-Sicard syndrome is a unilateral lower cranial nerve syndrome. It affects cranial nerves IX-XII. It is caused by skull base lesions dose to the jugular foramen, e.g.. glomus jugulare tumor or occipital condyle fractmes.
The tumor depicted in the following figure can be associated with all of the following, except
A. Seizures
B. Posterior fossa location
C. Subungal fibroma (Koenen tumor)
D. Adenoma sebaceum (facial angiofibroma)
E. Cardiac rhabclomyoma
B. Posterior fossa location
Subependymal giant cell astrocytoma (SEGA) can be associated with tuberous sdcerosis. It is inherited on chromosome 9 or 16 as autosomal dominant. SEGA occurs in the region of the foramen of Monro and is characterized by large cells with large eccentric nuclei.
The lesion depicted in the following Tl-weighted MRI without contrast is most likely a(n)
A. Subependymoma
B. Choroid plexus papilloma
C. Colloid cyst
D. Ependymoma
E. Subependymal giant cell astrocytoma (SEGA)
C. Colloid cyst
Colloid cysts occur in the region of foramen of Monro and are typically hyperdense on a non-contrast head CT, hyperintense on Tl MRI, and hypointense on T2 MRI.
The abnormality depicted in this MRI is caused by a congenital anomaly that occurs at which gestational age?
A. 3-4 weeks
B. 4-5 weeks
C. 5-10 weeks
D. 2-5 months
E. After 5 months
B. 4-5 weeks
MRI reveals diastematomyelia, or split spinal cord, which occurs due to abnormal disjunction (secondary neurulation) at 4-5 weeks gestation.
The following tumor was most likely removed from
A. The temporal lobe of a child with seizures
B. The cerebellum of a child
C. The fourth ventricle In an adult
D. The lateral ventricle at the foramen of Monro
E. The frontal lobe of an adult with seizures
A. The temporal lobe of a child with seizures
Dysembryoplastic neuroepithelial tumor (DNET) typically occurs in the temporal lobe in children and causes seizures. It is characterized by neurons floating in mucinous material, chicken wire vessels, and small oligodendrocyte-like cells.
The following are the histological findings when this posterior fossa mass was removed. What is the diagnosis?
A. Pilocytic astrocytoma
B. Hemangioblastoma
C. Blastomycosis
D. Ependymoma
E. Medulloblastoma
C. Blastomycosis
Blastomycosis is characterized by budding yeast.
The following is an axial CT scan at T10 performed on a 13-year-old boy with back pain that responds well to Aspirin. The most likely diagnosis is
A. Osteoblastoma
B. Osteosarcoma
C. Chondrosarcoma
D. Paget’s disease
B. Osteoid osteoma
B. Osteoid osteoma
Osteoid osteoma occurs in children, has a target sign on CT, and responds well to NSAIDs.
Hearing loss, visual loss, peripheral neuropathy, and impaired microtubule function are observed with which of the following toxicities?
A. Arsenic
B. Lead
C. Mercury
D. Cisplatin
E. Methotrexate
D. Cisplatin
Cisplatincausesperipheralneuropathy, impaired hearing, and vision. Cisplatin attacks tubulin which affects microtubule function. Vincristine and vinblastine impair microtubule function, resulting in axonal peripheral neuropathy. Mercury affects the rough endoplasmic reticulum, thus inhibiting translation and causes peripheral neuropathy.
Lisch nodules are a feature of which phacomatosis?
A. Neurofibromatosis type 1
B. Neurofibromatosis type 2
C. Tuberous sclerosis
D. Von Hippel-Lindau disease
E. Stmge-Weber syndrome
A. Neurofibromatosis type 1
Lisch nodules are a major diagnostic criterion for NFl, which is inherited as autosomal dominant on chromosome 17. NF2 is characterized by bilateral vestibular schwannomas, tuberous sclerosis by SEGA, facial angiofibroma and ash leaf spots, and von Hippel-Lindau by hemangioblastomas. Sturge-Weber features tram-track calcification of blood vessels (on CT) and port-wine stain of the skin of the face.
The following axial T2-weighted MRI at L4-L5 from a patient presenting with left L5 radiculopathy is mostly consistent with
A. Cystic schwannoma
B. Meningioma
C. Tarlov cyst
D. Synovial cyst
E. Metastasis
D. Synovial cyst
Synovial cysts are common at L4-L5 level, usually unilateral, associated with the facet joints, and hyperintense on T2 due to synovial fluid content. Flexion/extension X-rays may demonstrate instability. Tarlov cysts are described in the sacrum. Schwannomas and meningiomas are typically intradural. Metastases are usually associated with the pedicle or vertebral body, not the facet joints.
The structure shown below (arrow) is typically seen in which tumor?
A. Pineocytoma
B. Pineoblastoma
C. Medulloblastoma
D. Ependymoma
E. Neuroblastoma
D. Ependymoma
Ependymomas are characterized by pseudorosettes that occur around blood vessels. Medulloblastomas have Homer-Wright rosettes (no central vessel or Inman). Pineoblastomas, retinoblastomas, and ependymomas have Flexner-Wintersteiner rosettes (columnar cells with central lumen), while pineocytomas have very large rosettes.
A 38-year-old female is being worked up for acute onset of headaches and vertigo. The vessel shown by the arrow on her DSA is
A. PCOM
B. Anterior choroidal a
C. Persistent trigeminal a
D. Hypoglossal a
E. Proatlantal a
C. Persistent trigeminal a
Persistent trigeminal a occurs in 0.1-0.5% of cerebral angiograms. It connects the precavernow or proximal cavernous ICA to the basilar a. Note that the vessel feeds the basilar bifurcation into PCAs. It usually oourses in Meckel’s cave. Hypoglossal a connects cervical ICA to basilar a while proatlantal a connects ECA or cervical ICA to vertebral a. Otic (acoustic) a is rare and coMects petrous ICA to basilar a.
The main finding on the CT scan shown below is
A. C1-C2 subluxation
B. Atlanto-occipital dislocation (AOD)
C. Type II dens fracture
D. Jefferson fracture
E. Rupture of the transverse ligament of atlas
B. Atlanto-occipital dislocation (AOD)
AOD is observed on CT, note the increase in the basion-dens interval on the sagittal view (normally <12 mm on X-ray) and the splaying of the joint between the occipital condyle and the lateral mass of C1, especially on the left side.
The following pathological findings from a clival tumor are consistent with
A. Meningioma
B. Osteosarcoma
C. Chordoma
D. Chondrosarcoma
E. Metastasis
C. Chordoma
Chordomas are characterized by physaliphorow (large vacuolated) cells and a mucinous background They are common in the sacrum and clivus and are bright on T2 MRI. Surgery is the best cure. En-bloc resection should be performed whenever possible, proton beam therapy could be applied to residual tumor.
The following T1-weighted MRI imagoes with contrast and diffusion are consistent with the diagnosis of
A. Dennoid tumor
B. Epidermoid tumor
C. Arachnoid cyst
D. Vestibular schwannoma
E. Meningioma
B. Epidermoid tumor
Epidermoid cyst, brain abscess, and infarction are bright on diffusion (restricted). Also, lymphoma, co. prion disease, and ADEM show restricted diffusion.
The following MRl findings are characteristic for
A. Germinoma
B. Teratoma
C. Dermoid cyst
D. Giant thrombosed basilar tip aneurysm
E. Vein of Galen malformation
E. Vein of Galen malformation
Vein of Galen malformation: well-circumscribed pineal region mass with heterogeneous signal due to blood content Note compression of the aqueduct and hydrocephalus. They can present with heart failure in neonates or hydrocephalus later in life.
The vessel causing the blush from the tumor depicted below is the
A. A of Bernasconi and cassinari
B. McConnell’s capsular a
C. A of Percheron
D. Inferior hypophyseal a
E. Superior hypophyseal a
A. A of Bernasconi and cassinari
Presented is the typical location of the tentorial a of Bernasconi and cassinari. It causes a blush in tentorial meningiomas. It originates from the meningohypophyseal trunk that also gM5 the inferior hypophyseal a and the dorsal meningeal a. Other branches of the cavernous ICA are the McConnell’s capsular a and the inferior cavernous a. The superior hypophyseal a is a branch of the ophthalmic segment. The a of Percheron is a variable branch of Pl segment of the PCA and supplies both thalami.
The findings in the following picture are typical for
A. Plasmacytoma
B. Central neurocytoma
C. MedullobJastoma
D. Greminoma
E. Primary CNS lymphoma
E. Primary CNS lymphoma
Large B-cells with prominent nudeoli are characteristic of CNS lymphoma. Cells are CD 20+. They can be perivascular and/or intravascular. There is increased risk with AIDS. Lesions are periventricular, enhance, and show restricted diffusion.
A 32-year-old female presents with blurring of vision and left arm numbness. MRI findings are consistent with
A. Transient is chemic attacks (TIAs)
B. Brain abscesses
C. Hypertension
D. Multiple sclerosis (MS)
E. Gliomatosis cerebri
D. Multiple sclerosis (MS)
T2 hyperintensity on Flair MRI (frequently described as Dawson’s fingers), and mild contrast enhancement in a young female are typical for MS. Symptoms are usually remittent. The blurry vision probably correlates with optic neuritis. The diagnosis can be confirmed by oligodonal bands on lumbar puncture. Treatment includes cortioosteroids, interferon beta, plasmapheresis, dimethyl fumarate (Tecfidera), ocrelizumab (Ocrevus), daclizumab (Zinbryta), and glatiramer acetate (Copaxone).
The following MRI findings are more likely to be seen in
A. Sporadic meningioma
B. Neurofibromatosis type 1
C. Neurofibromatosis type 2
D. Tuberous sclerosis
E. Von Hippel-Lindau disease
C. Neurofibromatosis type 2
NF2 patients develop bilateral vestibularschwannomas and meningiomas. The disease is inherited as autosomal dominant on chromosome 22.
The following histological finding is typical for
A. Measles
B. Rabies
C. Parkinson’s disease
D. Alzheimer’s disease
B. Amyotrophic lateral sderosis
C. Parkinson’s disease
Lewy body (rounded pink with a halo) observed in a substantia nigra cell is typical for Parkinson’s disease. Its main component is a-synudein. Other components include ubiquitin, neurofilament, and alpha beta crystallin.
The following two MRI findings most lilcely show
A. Cranyopharyngoma
B. Rathke’s cleft cyst
C. Pituitary adenoma
D. Lymphocytic hypophysitis
E. Germinoma
B. Rathke’s cleft cyst
Rathke’s cleft cysts have a typical MRI appearance. They are well-circumscribed intra and/or suprasellar masses. have variable signal on T1, and are usually hyperintense on n. The pituitary gland is typically inferior to the tumor and enhances,
giving it the appearance of an egg in a cup (“un ceuf dans son coquetier”-French). The cysts probably arise from remnants of Rathke’s pouch. Biopsy and drainage of the cyst via a transsphenoidal approach are usually sufficient to establish the diagnosis and relieve the symptoms.
The lesion depicted by the following T1-(a) and T2-weighted (b) MRI pictures most likely
represents
A. Giant thrombosed aneurysm
B. Meningioma
C. Intraparenchymal hematoma
D. Glioblastoma multiforme (GBM)
E. Pteomorphic xanthoastrocytOma (PXA)
A. Giant thrombosed aneurysm
This well-circumscribed mass with variable signal intensity on T1 and n due to blood content is likely a giant thrombosed aneurysm. A vascular study (CTA, MRA, or DSA) is the next step. Stereotactic biopsy should not be offered and may cause fatal bleeding.
The following histologic findings are consistent with
A. Meningioma
B. Fibrillary astrocytoma
C. Glioblastoma multiforme
D. Pleomorphic xanthoastrocytoma (PXA)
E. Pilocytic astrocytoma
E. Pilocytic astrocytoma
Cystic areas and Rosenthal fibers (arrow) are characteristic of pilocytic astrocytoma.
