Section 2 Flashcards
Pathology, Radiology, and Critical Care
The following section is characteristic of which brain tumor?
A. Medulloblastoma
B. Ependymoma
C. Pineoblastoma
D. Retinoblastoma
E. Hemangiopericytoma
B. Ependymoma
Ependymomas are characterized by pseudorosettes (built around blood vessels). Flexner-Wintersteiner rosettes have a central canal and may occur in ependymoma, retinoblastoma, or pineoblastoma. Homer-Wright rosettes have neither blood vessels nor central canal in the center and are seen in medulloblastoma or PNET. Pineocytomas have very large rosettes.
Which of the following is not a feature of Duchenne’s muscular dystrophy?
A. Autosomal recessive inheritance
B. Increased serum creatine kinase
C. Positive Gower’s test
D. Muscle fiber necrosis and regeneration
E. Congestive heart failure
A. Autosomal recessive inheritance
Duchenne’s muscular dystrophy is X-linked recessive, with decreased dystrophin, atrophy shoulder and pelvic girdles, calf pseudohypertrophy, increased CK, and a positive Gower’s test. Treatment: prednisone.
Ryanodine receptor mutation occurs in
A. Mitochondrial myopathies
B. Keams-Sayre syndrome
C. Familial periodic paralysis
D. Malignant hyperthermia
E. Acute intermittent porphyria
D. Malignant hyperthermia
Ryanodine receptor mutation occurs in malignant hyperthermia. Acute intermittent porphyria is caused by defective porphobilinogen deaminase, familial periodic paralysis by a genetic defect coding for ca in musde fiber membranes, Kearns-Sayre syndrome by mitochondrial DNA, non-maternally inherited, and mitochondrial myopathies by maternal mitochondria defect.
Phytanic add oxidase deficiency causes
A. Phenylketonuria
B. Sandhoff’s disease
C. Refsum’s disease
D. Fabry’s disease
E. Hurler’s disease
C. Refsum’s disease
Refsum’s disease is caused by phytanic acid oxidase deficiency. It is inherited as autosomal recessive and causes peripheral neuropathy, retinitis pigmentosum, deafness, and cardiomyopathy.
A 64-year-old female presents with paraparesis, decreased sensation in the lower extremities, and no pain, the most likely diagnosis is:
A. Nerve sheath tumor
B. Drop metastasis
C. Hemangiopericytoma
D. Myxopapillary ependymoma
E. Meningioma
E. Meningioma
Intradural extramedullary enhancing lesions in postmenopausal females are most likely meningiomas, especially in the thoracic region. Schwannomas are more common in the lumbar spine and cause radicular pain.
The optic n fibers have their cell of origin from
A. The ganglion cells
B. Amacrine cells
C. Bipolar cells
D. Horizontal cells
E. Photoreceptor cells (rods and cones)
A. The ganglion cells
The rods and cones transmit the visual signal to the bipolar cells, the latter relay to the ganglion cells, which are the cells of origin of the optic n fibers. Excitation of the photoreceptors results in activation of protein G, S’GMP, Na+ channel closure, hyperpolarization, and an electric graded conduction.
The best treatment for torsade de pointes is
A. 3% saline infusion
B. 1,8% saline infusion
C. Normal saline
D. Magnesium sulfate infusion
E. Potassium chloride infusion
D. Magnesium sulfate infusion
Torsade de pointes is a serious cardiac dysrhythmia that can be complicated by ventricular fibrillation. It should be promptly treated. Mg is the treatment of choice. An infusion at 3-10 mg/min can be given even if the serum Mg level is normal. Mg decreases Ca influx and suppresses early after depolarizations. Other treatments indude mexiletine, temporary transvenous ventricular pacing, stopping any offending agents, and correcting electrolyte imbalance. K can be helpful but Mg remains of choice.
All of the following are characteristics of tuberous sclerosis, except
A. Autosomal dominant inheritance on chromosome 17
B. Subependymal giant cell astrocytoma (SEGA)
C. Cardiac rhabdomyoma
D. Renal angiolipoma
E. Liver cysts
A. Autosomal dominant inheritance on chromosome 17
Tuberous sclerosis is inherited as autosomal dominant on chromosome 9 (hamartin) or 16 (tuberin). Features include SEGA, facial angiofibroma (adenorna sebaceum), subungual fibroma (Koenen tumor), ash leaf spots, cardiac rhabdomyoma, renal angiolipoma, and liver cysts.
The following lesion was removed from the temporal lobe of a 10-year-old boy with seizures. What is the diagnosis?
A. Ganglioglioma
B. Dysembryoplastic neuroepithelial tumor (DNET)
C. Oligodendroglioma
D. Central neurocytoma
E. Glioblastoma multiforme
B. Dysembryoplastic neuroepithelial tumor (DNET)
Large neurons floating in mudn, a chicken-wire vascular pattern, small oligodendrocyte-like cells are seen in DNET. Both DNET and ganglioglioma occur in the temporal lobe in children and cause seizures.
A 64-year-old man undergoes an angiogram for embolization of a posterior fossa mass prior to surgical resection. What anatomical variation (arrow) is observed in the following ECA angiogram?
A. Fetal PCOM a
B. Persistent primitive trigeminal a
C. Persistent primitive otic a
D. Persistent primitive hypoglossal a
E. Persistent primitive proatlantal a
E. Persistent primitive proatlantal a
Persistent primitive proatlantal a type 2 (arrow), corresponds to a segmental artery that connects the external carotid artery to the vertebral a.
Based on the previous angiogram, which vascular structure is identified by the arrow?
A. PICA
B. AlCA
C. PCA
D. Superior cerebellar a
E. Vertebral a
A. PICA
The vertebral a ends in PICA (arrow).
The metabolic disorder inherited as autosomal recessive, characterized by a L-iduronidase deficiency, mental retardation, gargoyle face, thick meninges, spinal cord compression, and zebra bodies is called
A. Hurler’s disease
B. Hunter’s syndrome
C. Gaucher’s disease
D. Tay-Sachs disease
E. Morquio’s syndrome
A. Hurler’s disease
Hurler’s disease (MPS IH) features increased urinary dermatan sulfate, zebra bodies, gargoyle face, hepatosplenomegaly, corneal opacities, deafness, mental retardation, and spinal cord compression.
Which of the following is not an inclusion criterion for neurofibromatosis type 2 (NF2)?
A. Bilateral vestibular schwannomas
B. Positive family history
C. Sphenoid dysplasia
D. Postcapsular cataract
E. Meningioma
C. Sphenoid dysplasia
Sphenoid dysplasia is a feature of NF1.
Gum hyperplasia, hirsutism, ataxia, thrombocytopenia, and Stevens-Johnson syndrome can all occur with which anti-epileptic drug?
A. Ethosuximide
B. Valproic acid
C. Carbamazepine
D. Phenytoin
E. Phenobarbital
D. Phenytoin
Phenytoin (Dilantin) is a Na channel blocker. It is used for generalized tonic-donic seizures and status epilepticus. Side effects include rash, Stevens-Johnson syndrome, cerebellar degeneration, diplopia, stupor, peripheral neuropathy, thrombocytopenia, megaloblastic anemia, lymphadenopathy, gum hyperplasia, hirsutism, hepatitis, gastrointestinal irritation, osteopenia, teratogenicity (congenital fetal hydantoin syndrome).
The lesion at T9-T10 depicted in the following figure could be safely removed through all of the following approaches, except
A. Thoracotomy
B. Thoracoscopy
C. Costotransversectomy
D. Transpedicular
E. Laminectomy
E. Laminectomy
Laminectomy for thoracic disc herniation carries a high risk of paraplegia.
The nuclear bag intrafusal fibers have which of the following feature
A. Eccentric in position
B. Flower-spray endings
C. Type Ia fibers conducting at 70-120 m/s
D. Are smaller than the nuclear chain fibers
E. Have a static response
C. Type Ia fibers conducting at 70-120 m/s
Nuclear bag fibers transmit signals through type 1a fibers, while nuclear chain transmit through type II. A. B, D, and E are also features of nuclear chain.
Which drug could be complicated by cyanide toxicity?
A. Nitroglycerin
B. Sodium nitroprusside
C. Norepinephrine
D. Dopamine
E. Dobutamine
B. Sodium nitroprusside
Sodium nitroprusside can be used in hypertensive emergencies. It may cause cyanide toxicity. Treatment: O2, nitrites, and thiosulfate (Cyanide Antidote Kit).
At what gestational date does the posterior neuropore dose?
A. 16 days
B. 18 days
C. 22 days
D. 24 days
E. 28 days
E. 28 days
During primary neurulation, the neural tube closure starts in the middle at 22 days, then the anterior neuropore at 24 days (lamina terminalis), and then the posterior neuropore at 28 days.
Which receptor transmits vibration sensation?
A. Pacinian corpuscle
B. Hair end organ
C. Ruffini end organ
D. Merkel’s disc
E. Free nerve endings
A. Pacinian corpuscle
Pacinian and Meissner’s corpuscles transmit vibration sense. Touch and pressure are sensed by Ruffini end organs and Merkel’s discs. Touch is also sensed by hair end organs and Meissner’s corpuscles. Pain is transmitted by free nerve endings.
The abnormality depicted in this MRI occurs at which gestational age?
A. 3-4 weeks
B. 4-5 weeks
C. 5-10 weeks
D. 2-5 months
E. 5-9 months
D. 2-5 months
Agenesis of the corpus callosum and colpocephaly are caused by a defective migration and occur between 2 and 5 months.
Warfarin (Coumadin) level is increased by all of the following drugs, except
A. Phenobarbital
B. Salicylates
C. Bactrim
D. Cimetidine
E. Benzodiazepines
A. Phenobarbital
Phenobarbital stimulates hepatic microsomal enzymes and increases degradation of Coumadin. Bactrim, cimetidine, and benzodiazepines inhibit
liver enzymes and promote bleeding on Coumadin. Salicylates increase Coumadin level by displacing it from plasma proteins.
Orogenital ulcers, uveitis, ulcerative colitis, erythema nodosum, polyarthritis, and confusion could all occur in
A. Takayasu’s arteritis
B. Wegener’s granulomatosis
C. Buerger’s disease
D. Behcet’s disease
E. Cogan’s syndrome
D. Behcet’s disease
Behcet’s disease affects small vessels and causes meningo-encephalitis, brain stem edema, confusion, orogenital ulcers, uveitis, ulcerative colitis, erythema nodosum, polyarthritis. Treatment: corticosteroids
Superoxide dismutase mutation causes
A. McArdle’s disease
B. Amyotrophic lateral sclerosis (ALS)
C. Duchenne’s muscular dystrophy
D. Werdnig-Hoffmann disease
E. Charcot-Marie-Tooth disease
B. Amyotrophic lateral sclerosis (ALS)
Cu/Zn superoxide dismutase mutation is seen in ALS, myophosphorylase deficiency in McArdle’s disease, and decreased dystrophin in Duchenne’s muscular dystrophy.
The lesion depicted in the following figure is from an AIDS patient. It was biopsied and the histology is shown. The most likely diagnosis is
A. Bacterial brain abscess
B. Fungal brain abscess
C. Toxoplasmosis
D. Glioblastoma
E. Multiple sclerosis (MS)
C. Toxoplasmosis
Sagittal MRI with contrast shows the target sign. Histology reveals bradyzoites (bound in a bag). These are typical for toxoplasmosis, an opportunistic infection in AIDS patients. Treatment: pyrimethamine + sulfadiazine + leucovorin. Treatment can be started empirically based on clinical suspicion and no biopsy.
The Golgi tendon organ sends signals through which of these fibers?
A. Ia
B. lb
C. II
D. III
E. IV
B. lb
Golgi tendon organ sends impulses through lb (A alpha) fibers at 120 m/s. It stimulates Renshaw cells to inhibit a motor neurons. Renshaw cells utilize the inhibitory neurotransmitter glycine that causes Cl- influx into the neurons. Renshaw cells are inhibited by tetanus and strychnine, causing tetanic contraction.
Fibers in the corpus callosum originate in which layer of the cerebral cortex?
A. I
B. II
C. III
D. V
E. VI
C. III
Commissural fibers originate in layer III of the cerebral cortex (external pyramidal layer). lpsilateral cortical connections arise in layers I-III. Layer IV is the main afferent; layer V (internal pyramidal layer) is the main efferent and contains the large pyramidal (Betz) cells. Layer VI sends efferents to the thalamus.
Which of the following drugs is a specific alpha-1 receptor blocker?
A. Prazosin
B. Clonidine
C. Guanethidine
D. Reserpine
E. Labetalol
A. Prazosin
Prazosin, phentolamine, and phenoxybenzamine are alpha-1 receptor blockers; clonidine alpha-2 receptor stimulant, thus decreasing NE release; labetalol alpha and beta blocker. Reserpine inhibits synthesis and storage of NE, while guanethidine inhibits NE release.
Basophilic stippling of red blood corpuscles is caused by which toxicity?
A. Methotrexate
B. Cisplatin
C. Arsenic
D. Mercury
E. Lead
E. Lead
Basophilic stippling of RBCs occurs in Pb poisoning, cerebellar dysfunction and renal tubular necrosis with Hg. Mees’ lines in As, peripheral neuropathy and deafness with cisplatin, and subacute necrotizing leukoencephalitis (SNLE) with methotrexate.
Which autosomal recessive disease is caused by N-acetyi-aspartoacylase deficiency and causes mental retardation, macrocephaly, and blindness?
A. Krabbe’s disease
B. Adrenoleukodystrophy
C. Pelizaeus-Merzbacher disease
D. Canavan’s disease
E. Cockayne’s syndrome
D. Canavan’s disease
Metabolic disorders causing macrocephaly include Alexander’s, Canavan’s, and Tay-Sachs diseases.
The following sections are from a temporal lobe lesion in a patient with seizures, what is the diagnosis?
A. Lymphoma
B. Oligodendroglioma
C. Ganglioglioma
D. Dysembryoplastic neuroepithelial tumor (DNET)
E. Giant cell astrocytoma
C. Ganglioglioma
Perivascular lymphocytes (center in a) and large multinucleated ganglion cells (b) characterize ganglioglioma. Due to the mixed nature, they are positive for both GFAP and neurofilament. Calcifications may be observed. They are frequently cystic. They commonly occur in the temporal lobe of children and cause seizures.
A 63-year-old female with left L5 radiculopathy, has the following MRI, the most likely diagnosis is:
A. Extruded left L4-L5 disc fragment
B. Synovial cyst
C. Cystic schwannoma
D. Arachnoid cyst
E. Osteoid osteoma
B. Synovial cyst
Synovial cysts are usually hyperintense on T2 MRI and are associated with the facet joints. When symptomatic, they need to be resected with or without fusion depending on associated instability.
Muscarinic acetylcholine receptors are found at the
A. Neuromusrular junction
B. Preganglionic endings of sympathetic fibers
C. Postganglionic endings of most sympathetic fibers
D. Preganglionic endings of parasympathetic fibers
E. Postganglionic endings of parasympathetic fibers
E. Postganglionic endings of parasympathetic fibers
Muscarinic acetylcholine receptors are found at the postganglionic endings of parasympathetic fibers as well as postganglionic sympathetic to sweat glands. Nicotinic receptors are in the sympathetic and parasympathetic ganglia, adrenal gland, and the neuromuscular junction.
Which drug has no beta-2 agonist effect?
A. Phenylephrine (Neosynephrine)
B. Epinephrine
C. Isoproterenol
D. Dobutamine
E. Dopamine
A. Phenylephrine (Neosynephrine)
Phenylephrine is a vasopressor (alpha-1 stimulation) with no beta agonist effects; is a good choice in shock with tachycardia or myocardial infarction. On the other hand, isoproterenol is mainly alpha beta agonist, and epinephrine stimulates both alpha and beta receptors. Dobutamine has very strong beta-1 inotropic effect and can be used in acute heart failure. Dopamine is also good in cardiogenic shock; it is a positive inotrope (beta-1 ), but may cause tachycardia; at low doses, dopamine is vasodilator (beta-2 and dopaminergic receptors); at high doses vasoconstrictor (alpha-1). Amrinone is a direct inotrope through cAMP.
Which disease causes cherry-red spot in the macula, hepatosplenomegaly, and mental retardation and is caused by sphingomyelinase deficiency?
A. Gaucher’s disease
B. Fabry’s disease
C. Niemann-Pick’s disease
D. Maple syrup urine disease
B. Hunter’s disease
C. Niemann-Pick’s disease
Lysosomal storage diseases causing a cherry-red spot in the macula are GMl, Sandhofl’s, Tay-Sachs (also causes macrocephaly), and Niemann-Pick diseases.
For a 58-year-old male with bilateral lower extremity pain has the following MRI. What is the most likely diagnosis?
A. Hemangioblastoma
B. Drop metastasis
C. Meningioma
D. Schwannoma
E. Neurofibroma
D. Schwannoma
Cystic tumors with enhancing wall in the lumbar spine in males with radicular pain are likely to be cystic schwannomas. The other diagnoses are possible but less common. Meningiomas are more common in the thoracic region in postmenopausal
females.
The following tumor was removed from the cerebellopontine angle of a patient. What is the diagnosis?
A. Meningioma
B. Vestibular schwannoma
C. Dermoid cyst
D. Epidermoid cyst
E. Metastasis
D. Epidermoid cyst
Diffuse keratin is typical for epidermoid and dermoid cysts. The absence of hair follicles goes against dermoid. Epidermoids are common in the cerebellopontine angle and show restricted diffusion on MRI.
High anion gap acidosis can be caused by all of the following, except
A. Renal tubular acidosis
B. Diabetic ketoacidosis
C. Lactic acidosis
D. Methanol
E. Salicylate toxicity
A. Renal tubular acidosis
A metabolic acidosis with high anion gap (AG) is usually caused by organic acid accumulation (unmeasured anions~ Normal AG is 12 mEq/L (more recently 3-10 mEq/L). Low AG can be observed in hypoproteinemia. High AG is seen in: MUD PILES: methanol, uremia, diabetic ketoacidosis, paracetamol, isoniazid, lactic acidosis (including starvation), ethylene glycol, and salicylates. Renal tubular acidosis is caused by a defect in distal tubule H+ secretion and/or proximal tubule HCO3-absorption causing normal AG metabolic acidosis.
Spike-dome waves at 3 Hz on an EEG are characteristic: of
A. Stage 3 of sleep
B. Absence seizures
C. Generalized seizures
D. West syndrome
E. REM sleep
B. Absence seizures
Spike-dome waves at 1-2 Hz are seen in Lennox-Gastaut syndrome, 3 Hz in absence seizures, and 4-6 Hz in juvenile myoclonic epilepsy. West syndrome (infantile spasms) is characterized by hypsarrhythmias.
What is a DNA repair disorder characterized by CNS and peripheral nerve demyelination, cataract, and dwarfism?
A. Maroteaux-Lamy syndrome
B. Sandhoff’s disease
C. Alexander’s disease
D. Lowe’s syndrome
E. Cockayne’s syndrome
E. Cockayne’s syndrome
Coclcayne’s syndrome is a leulcodystrophy caused by defective DNA repair.
Increased urine delta-aminolevulinic acid (d-ALA) occurs in
A. Keams-Sayre syndrome
B. Familial periodic paralysis
C. Acute intermittent porphyria
D. Malignant hyperthermia
E. Central core disease
C. Acute intermittent porphyria
Acute intermittent porphyria is autosomal dominant caused by a defect in porphobilinogen deaminase. The urine has increased delta-ALA and porphobilinogen. It turns dark as it oxidizes. Patients have severe peripheral neuropathy, seizures, psychiatric problems, and abdominal pain. Treatment: hematin.
What type of tumor is depicted below?
A. Schwannoma
B. Glioblastoma
C. Meningioma
D. Metastasis
E. Chordoma
C. Meningioma
Calcified psammoma body (center) and whorls
are characteristics of meningiomas. Meningiomas
are positive for EMA and vimentin. They can occur
with NF2 (chromosome 22).
The following is a picture of a newborn. This disorder is caused by failure of
A. Primary neurulation
B. Secondary neurulation
C. Disjunction
D. Ventral induction
E. Migration
A. Primary neurulation
Myelomeningocele is caused by failure of closure of the neural tube (caudal end normally doses at 28 days). It is a disorder of primary neurulation.
Which disease is autosomal recessive, caused by decreased liver peroxisomes, with long-chain fatty acid accumulation, and a cerebro-hepato-renal
syndrome?
A. Lowe’s syndrome
B. Zellweger’s syndrome
C. Leigh’s disease
D. Lesch-Nyhan disease
E. Alexander’s disease
B. Zellweger’s syndrome
Zellweger’s syndrome is an autosomal recessive disorder, caused by decreased liver peroxisomes, with long-chain fatty acid accumulation, and manifests as a cerebro-hepato-renal syndrome
A 38-year-old female presented with headache and vertigo and was found to have the following abnormality on head CTA. What is the arrow pointing at
A. TrigeminaJ schwannoma
B. PCOM
C. Anterior choroidal a
D. Tentorial a of Bernasconi and Cassinari
E. Persistent trigeminal a
E. Persistent trigeminal a
Persistent trigeminal a connects the ICA (precavernous or proximal cavernous) to the basilar
a. It occurs in 0.2% (0.1-0.5%) of angiograms and
courses along the Meckel’s cave.
The following extra-axial tumor was very hemorrhagic during resection. What is the most likely diagnosis?
A. Chordoma
B. Meningioma
C. Hemangioblastoma
D. Hemangiopericytoma
E. Chondrosarcoma
D. Hemangiopericytoma
The large staghorn blood vessels are seen in hemangiopericytoma. The tumor is positive for
reticulin and vimentin but unlike meningiomas
negative for EMA. These tumors tend to be very
hemorrhagic and have a high recurrence rate.
The following tumor is observed in which syndrome?
A. Von Hippel-Lindau syndrome
B. Tuberous sclerosis
C. Neurofibromatosis type 1
D. Neurofibromatosis type 2
E. Godin’s syndrome
A. Von Hippel-Lindau syndrome
Hemangioblastomas are rich in blood vessels and clear cells (lipid content). They are positive for vimentin. Unlike renal cell carcinoma, they are
negative for EMA and cytokeratin. They are common in the posterior fossa and spinal cord. They can secrete erythropoietin and cause polycythemia. They can be sporadic or part of von Hippel-Lindau syndrome (chromosome 3).
Which of the following is an autosomal dominant disorder caused by a genetic defect in coding for calcium in muscle fiber membranes and results in paralysis when exposed to cold weather or
exercise?
A. Mitochondrial myopathy
B. Kearns-Sayre syndrome
C. Familial periodic paralysis
D. Malignant hyperthermia
E. Central core disease
C. Familial periodic paralysis
Familial periodic paralysis is autosomal dominant disorder caused by genetic defect in coding for calcium in muscle fiber membranes: there is
decreased level of serum K. Treatment: KCl, carbonic anhydrase inhibitors (dichlorphenamide), low NaCl, and avoid carbohydrates.
The medial forebrain bundle connects
A. Septal nuclei to hippocampus
B. Subiculum to mammillary nuclei
C. Mammillary nuclei to midbrain
D. Lateral hypothalamic nuclei to amygdala
E. Lateral hypothalamic nuclei to septal nuclei
E. Lateral hypothalamic nuclei to septal nuclei
The medial forebrain bundle connects the
septal nuclei to the lateral h
The following histology is seen in which intracranial tumor?
A. Oligodendroglioma
B. Chordoma
C. Meningioma
D. Hemangiopericytoma
E. Hemangioblastoma
B. Chordoma
Chrodomas are characterized by physaliphorous cells and a mucinous background. Normal bone is observed on the left. They are common in the sacrum and diws. The goal of surgery is en bloc resection. Proton beam therapy can be helpful.
Which artery is causing the blush in the following angiogram?
A. Artery of Bernasconi and Cassinari
B. Persistent trigeminal a
C. Vidian a
D. Inferior hypophyseal a
E. McConnell’s capsular a
A. Artery of Bernasconi and Cassinari
The tentorial a of Bernasconi and Cassinari causes blush in cases of tentorial meningiomas. It is a branch of the meningohypophyseal trunk (from the cavernous ICA), which also gives the dorsal meningeal and inferior hypophyseal aa. The cavernous ICA also gives rise to the McConnell’s capsular a and the inferolateral trunk. The vidian and caroticotympanic aa are branches of the petrous ICA. Persistent trigeminal a is very rare and connects ICA to basilar a.
Which of the listed eye signs correspond to the NF2 disease?
A. Kayser-Fleischer ring in the cornea
B. Glaucoma
C. Lisch nodules in the iris
D. Postcapsular cataract
E. AVM of the retina and optic nerve
D. Postcapsular cataract
Postcapsular cataract is a feature of NF2.
Which of the listed eye signs correspond to Wilson’s disease?
A. Kayser-Fleischer ring in the cornea
B. Glaucoma
C. Lisch nodules in the iris
D. Postcapsular cataract
E. AVM of the retina and optic nerve
A. Kayser-Fleischer ring in the cornea
Kayser-Fleischer ring occurs in Wilson’s disease.
Which of the listed eye signs correspond to Wyburn-Mason disease?
A. Kayser-Fleischer ring in the cornea
B. Glaucoma
C. Lisch nodules in the iris
D. Postcapsular cataract
E. AVM of the retina and optic nerve
E. AVM of the retina and optic nerve
Wyburn-Mason disease is characterized by AVM of the retina and optic nerve.
Which of the listed eye signs correspond to the NF1disease?
A. Kayser-Fleischer ring in the cornea
B. Glaucoma
C. Lisch nodules in the iris
D. Postcapsular cataract
E. AVM of the retina and optic nerve
C. Lisch nodules in the iris
Lisch nodules occur in NF1.
Which of the listed eye signs correspond to the Sturge-Weber syndrome?
A. Kayser-Fleischer ring in the cornea
B. Glaucoma
C. Lisch nodules in the iris
D. Postcapsular cataract
E. AVM of the retina and optic nerve
B. Glaucoma
Glaucoma is a feature of Sturge-Weber syndrome.
Which of the following syndromes correspond to polyostotic fibrous dysplasia?
A. Fahr’s disease
B. McCune-Albright syndrome
C. Hallervorden-Spatz disease
D. Von Hippel-Lindau disease
E. Sturge-Weber syndrome
B. McCune-Albright syndrome
Polyostotic fibrous dysplasia occurs in McCune-Albright syndrome.
Which of the following syndromes correspond to bilateral basal ganglia hypodensities?
A. Fahr’s disease
B. McCune-Albright syndrome
C. Hallervorden-Spatz disease
D. Von Hippel-Lindau disease
E. Sturge-Weber syndrome
C. Hallervorden-Spatz disease
Hallervorden-Spatz disease features bilateral basal ganglia hypodensities.
Which of the following syndromes correspond to tram track appearance on CT?A. Fahr’s disease
B. McCune-Albright syndrome
C. Hallervorden-Spatz disease
D. Von Hippel-Lindau disease
E. Sturge-Weber syndrome
E. Sturge-Weber syndrome
Tram track appearance on CT is seen in Sturge-Weber syndrome due to vascular calcification.
Which of the following syndromes correspond to bilateral basal ganglia calcifications?
A. Fahr’s disease
B. McCune-Albright syndrome
C. Hallervorden-Spatz disease
D. Von Hippel-Lindau disease
E. Sturge-Weber syndrome
A. Fahr’s disease
Fahr’s disease is characterized by bilateral basal ganglia calcifications.
Which of the following syndromes correspond to cystic lesion with a mural enhancing nodule?
A. Fahr’s disease
B. McCune-Albright syndrome
C. Hallervorden-Spatz disease
D. Von Hippel-Lindau disease
E. Sturge-Weber syndrome
D. Von Hippel-Lindau disease
Cystic lesion with a mural enhancing nodule describes either hemangioblastoma or pilocytic astrocytoma. Neither of these are choices, but the former can occur with von Hippel-Lindau disease.
