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61 Cards in this Set
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What does NOT cause leukocoria?
a. Coats disease b. Retinoblastoma c. Congenital cataracts d. Optic glioma e. PHPV |
optic glioma
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What do you test for in nasal discharge to determine a CSF leak?
a. Protein content b. Oligoclonal bands c. Glucose d. Beta 2 transferrin |
B2 Transferrin
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Only found in CSF and perilymph
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Child with unilatereal cerebral hemisphere atrophy, ipsilateral small sinuses, thick skull, T2 dark pial flow voids, pial/meningeal enhancement in atrophied hemisphere, hypertrophy of choroid plexus, and T2 hypointense subcortical nodules with hemosiderin deposits. Diagnosis?
A. Sturge-Weber syndrome B. Dyke Davidoff syndrome C. Osler-Weber-Rendu D. Tuberous sclerosis E. Gorlin's syndrome F. Von Hippal Lindau |
Sturge Webber Syndrome
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Sturge-Weber = capillary venous angiomas of face (port wine stain) with ipsilateral cortical atrophy, enlarged adjacent subarachnoid space, enlarged choroid plexus. Leptomeningeal venous angiomas demonstrate hight T2; hemiplegia; seizures.
Dyke-Davidoff syndrome: = hemiatrophy of one hemisphere, thickened ipsilateral calvarium, attributed to early MCA event. Gorlin syndrome = basal cell nevus syndrome; multiple basal cell nevi, odontogenic jaw cysts, prognathism, cleft palate, ACC, hydrocephalus, mult tumors, congenital blindness. Tuberous sclerosis = subependymal/ cortical hamartomas, cortical tubers (increased T2), subependymal giant cell astrocytoma with hydrocephalus, WM lesions in radial bands from 4th vent to cerebellar hemispheres. Von Hippal Lindau = cerebellar or retinal hemangioblastomas multiple, enhancing subpial nodules Osler Weber Rendu = cerebrospinal AVMs, mucocutaneous telangiectasias, visceral AVMs |
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Most common cause of hemifacial spasm:
a. CN 7 schwannoma b. Bell's palsy c. Vertebrobasilar dolichoectasia d. Ramsay-Hunt e. Parotid malignancy f. MS |
Vertebrobasilar dolichoectasia
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Unusual seizures in 10 year old with isotense, nonenhancing brain lesion posterior to infundibulum but anterior to mammillary bodies:
a. Tuber cinereum hamartoma b. Glioma c. Craniopharyngioma d. Germinoma |
Answer: Hamartoma of tuber cinereum
a. Hypothalamic hamartomas: rare congenital malformations located in tuber cinereum-mamillary body; a cause of precocious puberty and laughing seizures in children; boys are affected more often than girls. Hypothalamic hamartomas also may be found incidentally in adults, where they may mimic low-grade gliomas. MR shows a sessile or polypoid mass in the tuber cinemeum region of the hypothalamus that is isointense on T1 and isointense or mildly hyperintense on T2. The lesions are stable in size over time and typically do not show contrast enhancement. b. Hypothalamic gliomas frequently invade the optic chiasm and vice versa; hence, the primary site of origin may be difficult to determine. Children are affected more than adults. The diencephalic syndrome (emaciation, alertness, hyperactivity, and pallor) is seen in up to 20% of children younger than 3 years. Neurofibromatosis type I is present in 20-50% of these patients. Usually a low-grade astrocytoma, frequently juvenile pilocytic. MR shows a suprasellar mass involving the optic chiasm and hypothalamus that is hypointense on T1, is hyperintense on T2, and enhances inhomogeneously with contrast administration. c. Craniopharyngioma is a slow-growing, extra-axial, epithelial-squamous, calcified cystic tumor arising from remnants of the craniopharyngeal duct and/or Rathke cleft and occupying the (supra)sellar region. Presents as a single large cyst or multiple cysts filled with a turbid, proteinaceous material of brownish-yellow color that sparkles because of a high content of floating cholesterol crystals compared to machinery oil. It most frequently arises in the pituitary stalk and projects into the hypothalamus. d. Germinomas are most commonly located in the pineal region; their next most frequent site is the hypothalamus, can be affected without pineal involvement. Hypothalamic germinomas affect males and females equally, unlike pineal region germinomas, which show a strong male predominance. Tumor spread may occur via CSF pathways. Germinomas are low-grade malignant tumors that are radiosensitive. On MR, usually mildly hypointense on T1 and mildly hyperintense on T2, but may be isointense on both pulse sequence. They generally show homogeneous enhancement with administration of contrast material. |
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Plot of serial fetal ventricle diameter measurements rising linearly from 10 (15) mm at 18 weeks (at the atria) initially to 16 (25) mm in late third trimester. No significant change in skull size. Cortical mantle remains normal. Diagnosis?
a. Holoprosencephaly b. Chiari II malformation c. Normal variant d. Hydrancephaly e. Migration defect |
Answer: Chiari II malformation
Between 14-38 weeks, transverse atrial measurement is constant 7.6±0.6mm. Measurements > 10 mm suggest ventriculomegaly. Important causes of neonatal hydrocephalus: aqueductal stenosis (43%) > communicating hydrocephalus (38%) > Dandy-Walker (13%) > All others (6%). Chiari II malformations are associated with obstructive hydrocephalus in 90% of cases. Chiari II is secondary to the PF being too small. As a result the tonsils and vermis herniate thru foramen magnum. Holprosencephaly is lack of cleavage of the forebrain laterally, transversely, or horizontally as a consequence of arrested lateral ventricular growth, results in large monoventricular. Failure of the primitive brain to cleave into the right and left hemispheres. Diagnosis of semilobar type: absence of the falx and fusion of the thalami. Hydrancephaly is liquefaction necrosis of the cerebral cortex, not compatible with prolonged extrauterine life. Hydranancephaly will have absence of brain mantle. Agenesis of corpus callosum is a migrational defect: 0.7% of all births; Chiari II—0.02% of all births. Agenesis of the CC can cause isolated dilatation of the occipital horns (colpocephaly), but probably not as much dilatation of the atrium. |
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What is FALSE regarding juvenile nasopharyngeal angiofibroma?
a. Preoperative embolization may be helpful b. Erodes, remodels posterior wall of maxillary sinus c. Commonly involves sphenoid sinus at presentation c. More common in adolescent boys d. Arises from pterygopalantine fossa e. Frequently invades the orbit f. Commonly associated with epistaxis |
Answer: Arises from pterygopalatine fossa (FALSE)
Most common benign tumor of nasopharynx, adolescent males, presents with recurrent epistaxis. Grow through pterygopalatine foramina and fossa (widens the foramina). Although commonly SEEN in the pterygopalatine fossa, they originate from sphenopalatine foramen in the lateral nasopharyngeal wall. Bowing of the posterior wall of the maxillary antrum is a common finding. These tumors are VERY vascular. It is a highly vascular mass which only enhances on CT scan immediately after bolus injection and is supplied primarly by the internal maxillary artery. MR: intermediate signal intensity on T1 w/discrete punctate areas of hypointensity (vascular stroma). Biopsy is contraindicated. The ultimate danger of unchecked growth by JNA is intracranial extension. |
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What does NOT cause leukocoria?
a. Coats disease b. Retinoblastoma c. Congenital cataracts d. Optic glioma e. PHPV |
Answer: Optic glioma (does NOT cause leukocoria)
Leukocoria, in which the normal red reflex of the retina is replaced by a yellowish or grayish white color. Retinoblastoma is the most common cause of leukocoria. Other causes of leukocoria include PHPV (persistent hyperplastic vitreous which also causes a white light reflex) (19%–28%), retrolental fibroplasia (5%–13%), posterior cataract (13.5%), coloboma, a congenital posterior choroidal defect (11.5%), larval endophthalmitis (6.5%–16%), and Coats disease (4%–16%) Coat's is vascular malformation with serum and lipids leaking under the retina. Optic nerve glioma is the most common tumor of the optic nerve in children and is frequently associated with neurofibromatosis type 1. These gliomas are usually pilocytic astrocytomas and cause fusiform enlargement of the nerve. |
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Newborn with unilateral microopthalmos, leukokoria, increased density of globe. Diagnosis?
A. Retinoblastoma B. Coat's disease C. Juvenile macular degneration D. Persistent Hyperplastic Primary Vitreous (PHPV) E. Phthisis bulbi F. Retrolental fibroplasia |
Answer: Persistent hyperplastic primary vitreous (PHPV)
Persistent hyperplastic primary vitreous (second most common cause of leukokoria): caused by vascularized membrane behind the lens. The eye is small, no calcification. Vitreous is hyperdense from previous hemorrhage. If not treated, result in vision loss. Coats disease: an exudative vasculopathy of the eye which includes, retinal detachment, aneurysms, telangectactic vessels, venous dilation, fusiform capillary dilatation. NO focal mass or calcification. CT - unilateral (90%) dense vitreous with a normal sized globe; however, slight microphthalmia may be present. Juvenile macular degeneration: only rods and cones in macula die; only have okay peripheral vision; normal size eye. Retinoblastoma, most common cause of leukokoria (58%). Normal size eye. Calcification almost always present. Retrolental fibroplasia is retinopathy of prematurity. Bilateral postnatal fibrovascular organization of vitreous humor, which usually leads to retinal detatchment. Makes up 3-5% of all childhood leukokoria cases but results in bilateral microopthlamia. |
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What do you test for in nasal discharge to determine a CSF leak?
a. Protein content b. Oligoclonal bands c. Glucose d. Beta 2 transferrin |
Answer: Beta-2 transferrin
Historically, the laboratory test of choice was a glucose oxidase test. However, beta transferrin, a protein produced by neuraminidase activity in the brain, is uniquely found in CSF and perilymph fluid. If rhinorrhoea contains glucose, the specificity of the test for CSF can be improved by excluding other factors that increase the glucose concentration of nasal discharge. If the nasal discharge is not blood stained, the blood glucose (measured at the same time as CSF glucose) is <6 mmol/L, and there are no other symptoms of upper respiratory tract infection, such as sneezing, nasal blockage, cough, sore throat, sputum, or purulent nasal discharge, then this increases the likelihood that the discharge contains CSF. CSF glucose concentrations may be low in people with bacterial meningitis, subarachnoid hemorrhage, neoplasia, and neurological complications of connective tissue disease. |
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Wernicke's encephalopathy is associated with abnormalities/atrophy of which structure?
a. Middle cerebellar peduncles b. Periaqueductal gray c. Subcortical white matter d. Caudate nuclei e. Medulla |
Answer: Periaqueductal gray (or mammillary bodies)
About 50% of patients have symmetrical, greyish discolouration, congestion, and fresh pinpoint haemorrhages, mainly in the periaqueductal grey matter, the mamillary bodies, and medial thalamus. Typical histopathological changes affect specific areas of the brain, such as the medial dorsal thalamic nucleus bilaterally, in 100% of patients. The superior vermis of the cerebellum is involved in a third of cases. Other areas sometimes affected include the periaqueductal region, the pontine tegmentum, the reticular formation of the midbrain, the posterior corpora quadrigemina, and the cerebral cortex. On histological examination, acute lesions caused by extreme rapidity of thiamine deficiency show a symmetrical distribution of multiple, small, new haemorrhages in the brainstem and the thalamus, with some spongiosis between the haemorrhages without interstitial infiltration of macrophages and without relevant capillary proliferation. Chronic lesions show swelling of astrocytes, minimal loss of neurons, oedema, a decrease in myelinated fibres, activated microglia, reactive astrogliosis, and prominent vessels as a result of swelling and hyperplasia. |
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A parenchymal brain lesion measures 60-70 HU on precontrast CT and demonstrates well-defined rim enhancement post-contrast. Most likely represents:
A hematoma B lymphoma C aggressive glioma D ependymoma E MS |
Answer: Hematoma
Acute blood typically measures 56-76 HU. A hematoma should initially be 50-70 HU decreasing 1-2 HU/day, and may have rim enhancement at inner border secondary to BBB breakdown at 7-14 days. An abscess would have lower density and has a thick enhancing rim. Multiple sclerosis is of normal to lower density than white matter and shows very faint central enhancement in the acute. GBM is low-density with strong contrast. Metastases also often enhance, but may be denser than brain secondary to hemorrhage, calcification, or dense cell packing (e.g. germ cell). Lymphoma is slightly denser than normal brain and would show peripheral rim enhancement. |
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All of the following can mimic subarachnoid hemorrhage on FLAIR sequence EXCEPT:
a. Meningitis b. Hyperoxygenation during general anesthesia c. Propofol anesthesia d. Diffuse cerebral edema e. Leptomeningeal carcinomatosis |
Answer: Diffuse cerebral edema (vs propofol anesthesia)
Lymphangitic carcinomatosis and meningitis obviously mimic SAH. Disruption of the BBB may result in leakage of protein or contrast material chelates or both into the subarachnoid space. Contrast material leaking into the CSF has been observed to occur in other patients undergoing high dose contrast-enhanced MRA in patients who are in renal failure and in patients with active seizures. Examples in which high protein may be the source of the high intensity on FLAIR MR images include cases of sinus thromboses and case of neurosarcoidosis. Other false-positives occur when oxygen tension in CSF is elevated from the use of inspired oxygen fractions >0.60 during general anesthesia. Although propofol administration was initially implicated, the overriding impact of the paramagnetic influences of dissolved oxygen in the CSF leading to T1 shortening have been subsequently validated. FLAIR images of children anesthetized with propofol have artifactual hyperintense signal in the basal cisterns and subarachnoid spaces, and this artifact mimics disease of the subarachnoid space. Cerebral edema mimics SAH on CT. |
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A posterior deep cervical lymph node is at what level?
a. II b. III c. IV d. V e. VI |
Answer: Level V
Level 1: Submandibular, submental Level 2: Internal jugular from skull base to carotid bifurcation Level 3: Internal jugular below carotid bifurcation to omohyoid Level 4: Internal jugular below omohyoid Level 5: Posterior triangle Level 6: Adjacent to thyroid Level 7: Tracheal esophageal groove and superior mediastinum |
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Most common parotid tumor VS most common parotid malignancy:
a. Pleomorphic adenoma b. Warthins c. Mucoepidermoid carcinoma d. Adenoid cystic tumor |
Answer: Pleomorphic adenoma is most common tumor, Mucoepidermoid carcinoma is most common malignancy in adults
80% parotid tumors benign with most common being pleomorphic adenoma followed distantly by Warthrin tumor. The most common intraparotid mass is the benign lymph node. The most common benign tumor in children is the hemangioma. Except for Warthin tumors, benign tumors of the parotid gland are more likely to occur in women than in men. The most common malignant parotid tumors in adults are the following: (1) mucoepidermoid carcinoma, (2) adenoid cystic carcinoma, and (3) malignant mixed tumor. The most common malignant parotid tumors in children are the following: (1) mucoepidermoid carcinoma, (2) acinic cell carcinoma, and (3) undifferentiated carcinoma. |
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TRUE statement regarding parotid neoplasms?
a. Warthin’s tumor commonly has perineural spread b. Warthin’s is most common parotid gland neoplasm c. Pleomorphic adenomas are medial to facial nerve d. Pleomorphic adenomas are more common in women |
Answer: Pleomorphic adenomas are more common in women
Warthin’s tumor, or cystadenoma lymphomatosum, are the most common bilateral and multiple tumors of the parotid and are completely benign without the perineural spread characteristic of adenoid cystic carcinoma. With Warthin’s, men are affected more than women (5:1). Warthin’s tumor is classically painless. Pleomorphic adenomas are the most common benign tumor of the parotid, and 80% are in the superficial lobe and therefore, by definition, lateral to the facial nerve, and are usually seen in middle-aged women. |
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Which C-spine injury is most associated with neurologic deficit in children:
a. Jefferson fracture b. UID c. Hangman’s fracture d. C5 anterior teardrop |
C5 anterior teardrop
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40 year old female with several small subcortical hemorrhages in both parietal lobes, near the vertex. Most likely cause:
a) septic emboli b) amyloid angiopathy c) sagital sinus thrombosis d) post trauma shear (DAI) |
Answer: Sagittal sinus thrombosis
Intracerebral hemorrhage and hemorrhagic infarction are complications of sinus and cortical vein thrombosis. Sinus thrombosis can present with bilateral subcortical hemorrhage, gyriform hemorrhage, or hemorrhagic infarcts not in an arterial distribution. Hemorrhage from venous thrombosis may be small or large. Locations of hemorrhages are variable, depending on the site of thrombosis. Hemorrhages are generally proximal to the draining sinus. MRI/MRV: most sensitive exam for demonstrating thrombus and occluded dural sinus or vein. In about one-third of cases, CT demonstrates direct signs of CVT: 1) Dense triangle sign seen on noncon head CT as hyperdensity with a triangular or round shape in posterior superior sagittal sinus caused by venous thrombus. 2) Empty delta sign (also called empty triangle or negative delta sign), seen on head CT with contrast as a triangular pattern of enhancement surrounding central region lacking contrast enhancement in posterior part of superior sagittal sinus. 3) Cord sign, seen on head CT with contrast as curvilinear or linear hyperdensity over cerebral cortex caused by thrombosed cortical vein. |
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What is NOT an imaging characteristic of intracranial/cerebral hypotension?
a. hydrocephalus b. (peripheral meningeal) dural enhancement (or increased enhancement of dural veins) c. increase subdural space d. downward herniated (low-lying) cerebellar tonsils e. draping optic chiasm f. protrusion of cerebellar tonsils g. effaced basal cisterns h. crowding of the foramen magnum |
Answer: Hydrocephalus (NOT characteristic)
CSF pressures, by definition, are quite low. Intracranial hypotension almost invariably results from a spontaneous CSF leak. Only very infrequently is this leak at the skull base (cribriform plate). In the overwhelming majority of patients, the leak is at the level of the spine, particularly T-spine and cervicothoracic junction. CSF pressures may be within limits of normal. MRI: diffuse pachymeningeal gadolinium enhancement, often with imaging evidence of sinking of the brain, and less frequently with subdural fluid collections, engorged cerebral venous sinuses, enlarged pituitary gland, or decreased size of ventricles. Epidural blood patch is probably the treatment of choice in patients who have failed an initial trial of conservative management. When adequate trials of epidural blood patches fail, surgery can offer encouraging results in selected cases in which the site of the leak has been identified. Some of the spontaneous CSF leaks are related to weakness of the meningeal sac, likely in connection with a connective tissue abnormality. |
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Most common cause of cerebral (brain) abscess:
a. CCAM b. Pulmonary sequestration c. Hereditary hemorrhagic telangiectasia d. Carney's syndrome |
Answer: Hereditary hemorrhagic telangiectasia
Most common cause overall: secondary infection from primary sources elsewhere in the body, such as extension from paranasal sinuses (41%) or generalized septicemia (32%). Concerning HHT: Stroke and brain abscess are more common in patients with Osler-Weber-Rendu syndrome compared to the healthy population. This is due to loss of the normal filtering function of the pulmonary vasculature in patients with pulmonary AVMs. These AVMs allow thrombotic and septic emboli to travel to the brain. Untreated patients have a 2% risk of stroke and a 1% risk of brain abscess per year. Carney Triad: GIST, pulmonary chondroma, and extra-adrenal paraganglioma. |
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The tectorial membrane is a superior extension of what?
a. posterior longitudinal ligament b. alar ligament c. cruciate ligament d. ligamentum flavum e. transverse ligament |
Answer: Posterior longitudinal ligament
The tectorial membrane is the superior extension of the posterior longitudinal ligament and attaches to the anterolateral aspect of the foramen magnum. Its main role is to limit extension at the occipitoatlantal joints. The alar ligaments connect the lateral aspect of the dens and the medial inferior aspect of the occipital condyles. The main function of the alar ligaments is to limit rotation to the contralateral side. Rotational instability may result from this type of injury. The transverse ligament extends from the tubercle on the inner aspect of one side of the atlas to the tubercle on the other side. The anterior atlantoaxial ligament extends from the anterior midportion of the dens to the inferior aspect of the anterior arch of C1. The apical ligament lies between superior longitudinal fasciculus of cruciform ligament and anterior atlanto-occipital membrane. |
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Would have increased density on non-con head CT:
a. lymphoma b. hemangioma c. glioblastoma d. ependymoma |
Answer: Lymphoma
Lesions with increased attenuation on non-contrast CT: Hypercellular lesions: lymphoma, medulloblastoma, ependymoma, germinoma, meningioma, other PNET Hemorrhagic tumors: GBM, mets (Choriocarcinoma, Thyroid, Melanoma, RCC). Calcified tumors: mucinous mets, osteogenic tumors |
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OMU consists of following sinuses (obstruction of OMU leads to what pattern of sinus opacification):
a. frontal, maxillary, anterior and middle ethmoid b. mastoid c. bilateral frontal d. maxillary e. posterior ethmoid and sphenoid |
Answer: Frontal, anterior and middle ethmoid, and maxillary sinuses
OMU refers to the maxillary sinus ostium, the infundibulum, and the middle meatus; represents the common drainage pathways of the frontal, maxillary , and ethmoid air cells. Recurring patterns of inflammatory sinonasal disease: infundibular, OMU, sphenoethmoidal recess, sinonasal polyposis, sporadic. Infundibular (26%): focal obstruction in maxillary sinus ostium & ethmoid infundibulum => maxillary sinus disease. OMU (25%): ipsilateral maxillary, frontal, anterior ethmoid disease from obstruction of middle meatus. Sphenoethmoidal (6%): sphenoid or posterior ethmoid sinus disease from sphenoethmoidal recess obstruction. Sinonasal polyposis (10%): diffuse nasal and paransal sinus polyps. |
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Central cord syndrome is most commonly due to the following:
a. Hyperflexion injury b. Hyperextension injury c. Axial loading d. Hyperextension and rotation e. Hyperflexion and rotation |
Answer: Hyperextension injury
Central spinal cord syndrome: this syndrome is caused by damage to the corticospinal tract. It is characterized by weakness, greater in the upper extremities than the lower extremities and more pronounced in the distal aspect of extremity. The syndrome usually is associated with a hyperextension injury in patients with spondylosis or congenital stenosis of the cervical canal. Extension of the cervical spine, causing buckling of the ligamentum flavum into the spinal cord, is believed to cause central spinal cord syndrome. Anterior spinal cord syndrome: involves complete motor paralysis and loss of temperature and pain perception distal to the lesion. Since posterior columns are spared, light touch, vibration, and proprioceptive input are preserved. This syndrome is caused by compression of the anterior spinal artery, which results in anterior cord ischemia or direct compression of the anterior cord. It is associated with burst fractures of spinal column with fragment retropulsion caused by axial compression. (e.g., flexion teardrop). |
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40 year old man presents to the ER with slowly progressing lower extremity weakness. MRI of the thoracic spine demonstrates a focal area of increased T2 in the cord (enhancing conus) with low intensity linear T2 signal posterior to the distal thoracic cord (T2 signal flow voids in subarachnoid space at dorsal aspect of cord).
A Spinal/Dural AVF B Hemangioma C Contusion D Hemorrhage E AVM F metastases G mucopapillary ependymoma |
Answer: Spinal/Dural AVF
Classic description middle aged man with progressive LE weakness and gradual atrophy due to venous hypertension rather than arterial ischemic steal. AKA Foix-Alajouanine. Need to diagnose because this is treatable with embolization. Affects the distal thoracic cord from one transdural feeder in the watershed area. The anterior spinal artery with it’s hairpin turn feeds 70% of the cord. Need contrast enhancement to visualize on MR. Findings of a dural AVM include increased T2 signal within the cord without expansion. Posterior to the cord are multiple abnormal blood vessels likely representing abnormal draining veins. AVM is the most common vascular anomaly in the spine and accounts for 3-11% of space occupying lesions within the spine. Dural AVF, the most common type of spinal AVMs occurs in the dorsal aspect of the thoracic spine and at the conus medullaris. Most often seen in men in their 5th-8th decades and present with progressive neurological deterioration secondary to chronic venous hypertension. Trauma accounts for 40% of dural avf’s while the other 60% are thought to be spontaneous. • |
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Which is TRUE regarding enlarged vestibular aqueduct syndrome?
a. Occurs in females more than males b. Uncommonly associated with cochlear dysplasia c. Larger size of aqueduct correlates with severity d. Infants born with deafness e. Usually bilateral f. congenital versus acquired |
Answer: Usually bilateral (disease is congenital but hearing loss is acquired)
Large vestibular aqueduct syndrome, or enlarged endolymphatic duct (EED) and sac (EES) syndrome, is an inner ear malformation that manifests as progressive sensorineural hearing loss starting in infancy or childhood. a. Occurs in females slightly more than males. However, other sources say the reverse is true. b. Cochlear anomalies are present in most ears with LEDS and appear as a spectrum, ranging from subtle modiolar deficiency to gross dysplasia. The degree of modiolar deficiency did not consistently correspond to the severity of hearing loss. d. The deformity of the EED and EES is congenital; however, hearing loss in this syndrome is acquired. CT imaging demonstrates a vestibular aqueduct that is greater than 1.5 mm at the midway between the common crus and the external aperture. e. Bilateral involvement is twice as common as unilateral involvement. It is usually bilateral sensorineural hearing loss. |
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Skier has a bad fall and continues to ski. Next day, he has hemicranial headache and drooping lid with left Horner’s (ptosis, miosis, anhydrosis). Where is the injury?
a. Left (ipsilateral) ICA dissection b. Left (ipsilateral) ICA thrombosis c. Right (contralateral) ICA dissection d. Left (ipsilateral) vertebral art dissection e. Left (ipsilateral) MCA thrombosis |
Answer: Ipsilateral ICA dissection
Clinical presentation of ICA dissection is neck pain, Horner’s syndrome (drooping eyelid and constricted pupils), TIA or stroke. Of extracranial dissections, carotid dissection 3-5x more common than vertebral dissection. Both present with mean age of 40. The 3rd order sympathetic neurons travel via the ICA to the orbit. Etiologies: lesion of primary neuron; brainstem stroke or tumor or syrinx of the preganglionic neuron; trauma to the brachial plexus; tumors (eg, Pancoast) or infection of the lung apex; a lesion of the postganglionic neuron; dissecting carotid aneurysm; carotid artery ischemia; migraine; or middle cranial fossa neoplasm. Cervical internal artery dissection can cause Raeder Syndrome with ptosis, miosis and headache with preservation of ipsilateral facial swelling (Requisites p133). |
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A 37 year-old man was painting when he acutely developed left tongue deviation, left facial numbness (loss of pain and temperature sensation within left face), ataxia and right body weakness (loss of sensation on right side of body). The most likely diagnosis is:
a. Carotid artery dissection b. Vertebral artery dissection (Wallenberg syndrome secondary to vertebral artery dissection) c. Middle cerebral artery infarct d. Multiple cerebral infarctions e. Systemic lupus erythematosus |
Answer: Vertebral artery dissection
Vertebral artery dissection- more likely to have brainstem symptoms, Wallenburgs (lateral medullary syndrome). History: The typical presentation of VAD (vertebral artery dissection) is a young person with severe occipital headache and posterior nuchal pain following a recent, relatively minor, head or neck injury. The trauma is generally from a trivial mechanism but is associated with some degree of cervical distortion. Cause of stroke in patients younger than 45 yrs. Typically after trivial trauma. Associated with ceiling painting, judo, yoga, chiropractic manipulation, and even nose blowing, among other things. The medial medullary syndrome is less common than the lateral medullary syndrome. It may be due to occlusion of the vertebral artery, a branch of the vertebral artery, or the lower basilar artery. The findings with this syndrome include an ipsilateral lower motor neuron paralysis of the tongue and contralateral paralysis of the arm and leg. In addition, there is contralateral hemibody loss of tactile, vibratory, and position sense. Ataxia is seen with either syndrome. Lateral Medullary Syndrome (Wallenberg's Syndrome) is due to occlusion of the PICA, vertebral, or medullary arteries. This syndrome produces an ipsilateral Horner syndrome; loss of pain and temperature sensation of the face; weakness of the palate, pharynx, and vocal cords; and cerebellar ataxia. Contralateral hemibody loss of pain and temperature sensation. |
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Luckenshadel (lacunar skull) is associated with which of the following:
a. Myelomeningocele b. Dandy-Walker c. Craniosynostosis d. Prematurity e. Increased ICP |
Answer: Myelomeningocele
Luckenshadel are clusters of deep calvarial pits with steep edges and interspersed bony trabecular ridges that involve the inner table and diploe and less frequently the outer table. They are present at birth, are unrelated to increased intracranial pressure and spontaneously disappear by 4-6 months old. They are almost always associated with meningomyelocele and commonly seen in Chiari II malformations. |
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Myelination occurs in what direction?
a. Rostral to caudal b. Peripheral to central c. Dorsal to ventral d. Sensory to motor |
Answer: Dorsal to ventral, caudal to rostral, central to peripheral, motor to sensory
Myelination occurs in the following directions: a. Caudal to rostral. Myelination progresses cephalad from the brain stem at 29 weeks to reach the centrum semiovale by 42 weeks. b. Central to peripheral. c. Dorsal to ventral. d. Motor to sensory. T1 weighted images are useful in 1st 8 months of life and myelin follows fat on T1-weighted images given its high lipid content and hydrophobia. T2-weighted images are more useful than T1-weighted images after 8 months because the infant brain has a high water content so heavy T2-weighting is needed to distinguish between many tissues with long T2 relaxation times. In evaluating myelination, hyperintensity on T1 precedes hypointensity on T2. Hyperintensity on T1 can be thought of as indicating active myelination whereas hypointensity on T2 indicates completed myelination. Dates in this chart are times of active myelination (T1 bright) unless otherwise specified. |
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The spinal cord most commonly ends at normal level (has normal conus level) in the following:
a. Sacrococcygeal teratoma b. Imperforate anus c. Diastematomyelia d. Myelomeningocele e. Chiari II with lipomyelomeningocele |
Answer: Sacrococcygeal teratoma
a. Sacrococcygeal teratomas usually show normal level of conus. However, sacrococcygeal teratomas can be associated with the Currarino triad (Anorectal malformation, Sacrococcygeal osseous defect, and Presacral mass), incidence of tethered cord i20%. b. Imperforate anus as part as VACTERL is associated with tethered cord. c. Diastematomyelia associated with tethered cord in 75%. d and e. Chiari II: small posterior fossa, tonsil herniation, 4th ventricle compressed and elongated, beaked tectum, enlarged foramen magnum, low torcula, associated lumbosacral meningocele or meningomyelocele that tethers the cord is nearly always present. |
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Flat tire sign in orbit is indicative of what:
a. Ruptured globe b. Vitreal hemorrhage c. Dislocated lens d. Post septal hematoma |
Answer: Ruptured globe
Posterior collapse of the sclera causes flattening of the posterior contour of the globe, the "flat tire" sign. Other associated findings that are suggestive of scleral rupture are intraocular foreign body or gas, thickening of the sclera posteriorly, and a blood-vitreous fluid-fluid level. |
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Amorphous calcification just below anterior arch of C1. Likely diagnosis?
a. HADD - hydroxyappetite crystal deposition disease b. CPPD c. DISH d. Gout e. RA |
Answer: HADD (Hydroxyappetite crystal deposition disease)
Calcific retropharyngeal tendonitis is an under-recognized cause of acute cervical pain produced by inflammation of longus colli muscle. Amorphous calcification anterior to C1-2 with associated asymmetric soft tissue swelling is pathognomonic for calcium hydroxyapatite deposition disease. HADD is most commonly seen about the shoulder. However, it may also occur within the longus colli muscle, which is the principal flexor of the cervical spine. Calcification most significantly in superolateral group of the longus colli. The typical radiographic findings of this disorder consist of prevertebral soft tissue swelling in upper cervical region, as well as amorphous calcification, usually anterior to C-2, and just below the anterior arch of C-1. Resorption of this calcification and soft tissue swelling is common, and it may disappear completely in 1 to 2 weeks. CPPD could also produce calcification in this region, but it would also involve other adjacent C1-C2 ligaments. |
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What is TRUE regarding metastasis to the brain?
a. Uncommon in posterior fossa in adults b. Papillary thyroid carcinoma commonly present with (hemorrhagic) metastasis c. Squamous cell mets solitary < 5% of the time d. Cystic metastases suggest (are seen with) mucinous adenocarcinoma e. Most commonly occur in the ventricles |
Answer: Cystic metastases suggest mucinous adenocarcinoma
a. About 80% of metastatic lesions are in the cerebrum, 15% are in the cerebellum, and 5% are in the brainstem. Of note, renal, GI, and pelvic cancers tend to metastasize to the cerebellum, whereas breast cancer most commonly affects the posterior pituitary. Although metastases tend to go supratentorial, they are the most common infratentorial mass in adults. b. Papillary thyroid cancer is the most common malignancy of gland, accounting for 60 - 80% of thyroid neoplasms. It has a tendency to metastasise early to local lymph nodes (50% at presentation c.f. 10% for follicular thyroid cancer). In approximately 15% of patients, lymph node metastases are the first presentation. Distal hematogenous dissemination is less common than with follicular cancer, only in 5 - 10% of patients at presentation. c. The primary tumors that spread to the brain are lung, breast, melanoma, renal and GI tumors. Among these, presentation with a solitary metastatic lesion is noted in 30-50% of cases with lung and breast primaries accounting for largest share. d. Cystic mets: astrocytoma/GBM, hemangioblastoma, squamous cell CA metastasis, and lung adenocarcinoma metastasis. |
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Left occipital infarct. What visual field deficit?
a. A contralateral (right) homonymous hemianopsia b. Ipsilateral (left) homonymous hemianopsia c. Bitemporal hemianopsia d. Scotoma |
Answer: Contralateral (right) homonymous hemianopsia
Interruption of the optic tract as well as injury to the lateral geniculate nucleus result in homonymous hemianopsia. Thus an injury beyond the chiasm, anywhere from optic tract, lateral geniculate, optic radiation or calcarine cortex, can result in homonymous hemianopsia. Most common cause is CVA in PCA territory. (Others: tumor, demyelinating dz) Bitemporal hemianopia: injury to middle part of optic chiasm: pituitary tumor or craniopharyngioma. Injury to the optic nerve causes ipsilateral monocular blindiness. Injury to the medial geniculate nucleus may cause deafness, but fibers from each auditory nerve pass through both nuclei. Damage to the lateral edge of the chiasm will cause ipsilateral nasal hemianopsia. Injury to the occipital lobe will also cause contralateral homonymous hemianopsia. While occipital cortical strokes do affect vision, they typically spare macular vision (vision at the center of the visual field). |
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All of the following are associated with Chiari II EXCEPT:
a. Large posterior fossa b. Large mass intermedia c. Tectal beaking d. Myelomeningocele |
Answer: Large posterior fossa (NOT associated with Chiari II)
Chiari II malformation is associated with the following: small posterior fossa, 4th ventricle compressed in vertical slit, beaked tectum, enlarged foramen magnum, low torcula, associated lumbosacral meningocele or meningomyelocele that tethers the cord (nearly always present), hydromelia usually seen in lower T or L spine region, hydrocephalus, enlarged massa intermedia (gray matter connecting thalami), fenestration of the falx cerebri with herniation of individual gyri across midline, dysplastic posterior cingulate gyrus, upward, anterior, and downward cerebellar herniation, kinking at the cervicomedullary junction, and syrinx. Chiari I malformation is generally clinically occult and consists of the cerebellar tonsils herniating by >5 mm below the foramen magnum. CSF flow dynamic alterations can give rise to cervical syrinx. |
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65 year-old man, normotensive, presents to ER with symptoms of CVA. Non-contrast CT demonstrates large irregular shaped hemorrhage in right frontal lobe. Most likely cause?
a. amyloid angiopathy b. hypertensive hemorrhage c. hemorrhagic metastasis d. dural venous sinus thrombosis |
Answer: Amyloid angiopathy
Amyloid angiopathy hemorrhages are characteristically lobar and spare the basal ganglia, brain stem, and cerebellum. Hemorrhage with a superficial location, cortical involvement, +/- extension to subarachnoid space and ventricles. Frontal and parietal regions most common, involving cortex and subcortical WM. Deposition of B-amyloid in the media of small/mid size arteries (less freq veins) of cerebral cortex and meninges. NOT associated with systemic amyloidosis. Present with intracranial hemorrhage, dementia or TIA. 25-40% of patients that hemorrhage will recur, highest in first year. Hypertensive bleed: basal ganglia, pons, cerebellum, and deep white matter of the brain. |
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16 week-old fetus with cystic lesion in lateral neck most likely has which chromosomal abnormality:
a. Trisomy 13 b. Trisomy 18 c. Trisomy 21 d. Turner’s syndrome |
Answer: Turner's syndrome
Prenatal cystic neck masses include cystic hygroma, cephalocele, cervical teratoma and cervical meningocele. Cystic hygromas are lymphatic malformations which may be obstructed and associated with more diffuse signs of hydrops. They are most strongly associated with Turner's syndrome (XO) but also with trisomy 18 and 21. |
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What is TRUE regarding normal pituitary gland?
a. larger in males than females b. enhances like normal brain parenchyma c. enhances rapidly and prominently d. enlarges during menopause e. has a flat or concave superior border f. normal pituitary is hypoenhancing relative to adenoma |
Answer: Enhances rapidly and prominently, has a flat or concave superior border, larger in females (during cycle and pregnancy), normal pituitary is hyperintense compared to adenoma
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Which is FALSE regarding Thornwaldt cysts?
A associated with (midline) skull base (and C1) defect B notochord remnant C MRI T1 signal varies with protein content D located in posterior nasopharyngeal space E usually asymptomatic F midline abnormality |
Answer: Associated with skull base defect (FALSE)
Thorwaldt’s cysts are midline congenital pouch/cysts lined by ectoderm within the posterior wall of the nasopharyngeal mucosal space, asymptomatic incidental finding. May present with middle ear symptoms (from eustachian tube obstruction), halitosis (leaking sinus tract), foul taste in mouth, and, rarely, occipital headaches. Hypointense or hyperintense on T1 depending on the protein content. NO bone erosion. There is no sex preference and peak occurrence is 15-30 years old. It is related to the embryogenesis of the notochord. The contents of a cyst are generally high in protein and anaerobic bacteria. |
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In neurofibromatosis (NF-1), what is the cause of pulsatile exophthalmos?
a. sphenoid wing dysplasia b. enlargement of optic/carotid canal c. carotid cavernous fistula d. bupthalmos e. choroidal hamartomas |
Answer: Sphenoid wing dysplasia
NF1 distinctive osseous lesions: partial absence of greater and lesser sphenoid wing, hypoplasia and elevation of lesser wing of sphenoid, defect in sphenoid bone, +/- extension of middle cranial fossa structures into orbit. Pulsatile exophthalmos is caused by herniation of subarachnoid space and temporal lobe into the orbit. Criteria for diagnosing NF-1 met with 2 or more of the following: 1. Six or more cafe au lait spots (5 mm greatest diameter prepubertal or 15 mm postpubertal). 2. Two or more neurofibromas or one plexiform neurofibroma. 3. Freckling in the axillary or inguinal regions. 4. Optic glioma. 5. Two or more Lisch nodules. 6. Distinctive osseous lesions such as sphenoid dysplasia, anterior tibial bowing or pseudoarthrosis. 7. First-degree relative with NF-1 by the above criteria |
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MR imaging in a 12 year old reveals an enlarged optic chiasm (optic nerve lesion) and a focus of increased T2 signal within the basal ganglia and cerebellum. Most likely represents?
A Neurofibromatosis 1 B Neurofibromatosis 2 C Tuberous Sclerosis D Sturge-Weber |
Answer: Neurofibromatosis I
NF 1 typically has nonneoplastic hamartomas (80-90%) that are hyperintense on T2, not visible on T1. They tend to occur in the basal ganglia, white matter, and dentate nuclei. Optic nerve gliomas occur in 15%. NF2 is typified by bilateral acoustic schwannomas, schwannomas involving other cranial nerves, and meningiomas. (NF2: Multiple Inherited Schwannomas, Meningiomas, and Ependymomas—MISME.) Neurofibromatosis-2: 1. Bilateral acoustic neuromas. 2. First-degree relative with NF-2 and either a unilateral acoustic neuroma or two of the following: neurofibroma, meningioma, glioma, schwannoma, or juvenile posterior subcapsular lenticular opacity. |
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Cerebellar lesion with T2 hyperintensity in the cerebral peduncles and optic nerves. Diagnosis?
a. Neurofibromatosis II b. Neurofibromatosis I c. Tuberous sclerosis |
Answer: Neurofibromatosis I
NF 1 typically has nonneoplastic hamartomas (80-90%) that are hyperintense on T2, not visible on T1. They tend to occur in the basal ganglia, white matter, and dentate nuclei. Optic nerve gliomas occur in 15%. NF2 is typified by bilateral acoustic schwannomas, schwannomas involving other cranial nerves, and meningiomas. (NF2: Multiple Inherited Schwannomas, Meningiomas, and Ependymomas—MISME.) Neurofibromatosis-2: 1. Bilateral acoustic neuromas. 2. First-degree relative with NF-2 and either a unilateral acoustic neuroma or two of the following: neurofibroma, meningioma, glioma, schwannoma, or juvenile posterior subcapsular lenticular opacity. |
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Cerebellar lesion with T2 hyperintensity in the cerebral peduncles and optic nerves. Diagnosis?
a. Neurofibromatosis II b. Neurofibromatosis I c. Tuberous sclerosis |
Answer: Neurofibromatosis I
NF1 has optic nerve gliomas in 15% that demonstrate variable enhancement. In 80-90% nonneoplastic hamartomas are present that are hyperintense on T2 and not visible on T1. Tuberous sclerosis has multiple hamartomas and tubers, both of which are generally cortically based. Optic nerve rarely involved. |
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60 year old male with small cell lung cancer presents with asymmetric increased bilateral T2 signal in the mesotemporal regions. Diagnosis?
a. Limbic encephalitis b. Herpes encephalitis |
Answer: Limbic encephalitis
Paraneoplastic encephalomyelitis (PEM) is a multifocal inflammatory disorder of the CNS associated with remote neoplasia. Frequently, the disorder is accompanied by subacute sensory neuronopathy (SSN) due to involvement of the dorsal root ganglia. Although various malignancies have been reported, 80% of cases are associated with bronchial cancer, typically small-cell lung carcinoma. Neurologic manifestations commonly precede the diagnosis of cancer, although variable presentations have been reported. Symptoms usually progress over the course of weeks to months, reaching a plateau of neurologic disability. Neurologic impairment may be more debilitating than associated cancer. MR usually is unremarkable, although T2 hyperintensity may be noted in mesial temporal lobes and associated limbic structures. Posterior thalamic T2 hyperintensity, or the "pulvinar sign," may be present. Contrast enhancement may be demonstrated with subsequent atrophy and gliosis. |
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CN between PCA and superior cerebellar artery?
a. oculomotor nerve b. abducens nerve |
Oculomotor
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What is FALSE about hemangiomas of the orbit:
a. Prominent arterial supply b. Slowly enlarges c. Retrobulbar |
Answer: Prominent artery supply (FALSE)
Cavernous hemangiomas are the most common intraorbital tumors found in adults. These benign, vascular lesions are slow growing and can manifest as a painless, progressively proptotic eye. Most of these tumefactions are exceedingly unilateral. Histology: engorged vascular channels, tightly knit, separated by fibrous septae. Lined by a single layer of endothelial cells. Orbital cavernous angiomas can increase intraorbital volume with resultant mass effect. Can encroach on intraorbital or adjacent structures. Visual acuity or field compromise, diplopia, and extraocular muscle or pupillary dysfunction can result. |
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All have T1 hyperintense basal ganglia EXCEPT:
a. Neurofibromatosis b. Lehigh disease c. TPN (parenteral nutrition) d. Liver disease (hepatocellular degeneration) e. Calcifications |
Answer: Lehigh disease (NOT associated)
The following are associated with increased T1W signal in the basal ganglia: Ca2+, NF, hepatic encephalopathy, manganese toxicity (long-term TPN). High signal intensity in the basal ganglia on T1WI has been described in patients with hepatic encephalopathy, portosystemic shunting without hepatic encephalopathy, disorders of calcium-phosphate regulation, and in those patients receiving parenteral nutrition therapy. |
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In a patient with hepatocerebral degeneration (hepatocellular encephalopathy), the basal ganglia demonstrate what signal pattern on an MRI?
a. Low intensity of basal ganglia on T1 b. Low intensity of basal ganglia on T2 c. High intensity of basal ganglia on T1 d. High intensity of basal ganglia on T2 |
Answer: High T1 signal in the basal ganglia
Acquired hepatocerebral degeneration is an irreversible neurodegenerative syndrome that occurs with chronic liver disease, Pathologically, the brain has laminar or pseudolaminar necrosis with microcavitary changes at the gray-white matter junction, in the corpus striatum, and in the cerebellar white matter. Imaging changes correlate with plasma ammonia levels. The typical finding is bilateral basal ganglia hyperintensities on T1, seen in 50-75% of patients with advanced chronic liver disease. Other areas of increased signal include the pituitary, caudate, subthalamic region, and the mesencephalon around the red nucleus. |
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What is associated with myelomeningocele on prenatal US?
a. hydronephrosis b. gastroschisis c. obliterated cisterna magna |
Answer: Obliterated cisterna magna (Chiari II malformation)
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Which artery is associated with Wallenberg (lateral medullary) syndrome?
a) Ipsilateral PICA b) Contralateral PICA c) Superior cerebellar d) Branches off the basilar artery |
Answer: Ipsilateral PICA
Wallenberg syndrome is caused by a PICA infarct resulting in loss of pain and temperature sense of the contralateral body and ipsilateral face, ataxia, ipsilateral dysphagia and taste disorders, hoarseness, vertigo, nystagmus, and ipsilateral Horner’s syndrome. Basilar artery sends out many tiny branches to pons and medulla. AICA supplies the inner ear through labyrinthine branches, the anteroinferior cerebellum, and medulla and pons. PCA infarcts typically affect vision and may affect memory, smell, and emotion. |
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Woman is post partum, headache; labs show pan-hypopituitarism. MR: T1 hypointense pituitary mass with homogenous enhancement. Diagnosis:
a. Pituitary apoplexy b. Lymphocytic hypophysitis c. Rathke’s cleft cyst d. Craniopharyngioma |
Answer: Lymphocytic hypophysitis vs Pituitary apoplexy
Lymphocytic hypophysitis is an autoimmune inflammatory process involving mainly the anterior pituitary. The mass can push on the chiasm, involve the cavernous sinus, and can cause panhypopituitarism. Occurs more in women, brightly enhances. |
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A 55 year old male undergoes brain MR: temporal lobe lesion which is very dark centrally and bright peripherally on T2. The entire lesion is isointense on T1 weighted images. This most likely represents:
a) Cavernous angioma b) Hemorrhagic contusion c) Herpes d) Abcess |
hemorrhagic contusion
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Brain MR demonstrates area of bright signal on diffusion, low signal on ADC and normal T2 in insular cortex. Diagnosis?
a. Herpes b. Acute infarct (minutes to hours) c. Subacute infarct (days to weeks) d. Chronic infarct (months to years) |
Acute infarct (min-hr)
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The most medial CN in the cavernous sinus is:
a. II b. III c. IV d.VI |
Answer: CN VI Abducens
Lateral: III, IV, V1, and V2. Only CN VI runs medially next to the carotid artery. The ICA is even more medial than CN VI. |
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Which of the following conditions places a pediatric patient at high risk for latex sensitivity?
A. asthma B. myelomeningocele C. allergy to penicillin D. contrast allergy |
Answer: Myelomeningocele and bladder extrophy
This is thought to happen secondary to the repeated surgeries that these kids get, with subsequent sensitization to the latex in the gloves. Spina bifida patients have a documented latex allergy prevalence as high as 73% and have been considered the surgical population most at risk for a latex reaction. They have been shown to be at higher risk for intraoperative anaphylaxis. |
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Patient has bilateral (optic globe) retinoblastomas. Patient most likely has a third lesion located in:
a) the hypothalamus b) the anterior (or posterior) portion of the pituitary c) the pineal gland d) the suprasellar region |
Answer: Pineal gland
Trilateral retinoblastoma = bilateral retinoblastoma plus pineoblastoma. Pineoblastoma is in PNET group of highly malignant tumors of young children. It demonstrates lobular contour, local invasion and frequent calcifications. Intratumoral hemorrhage is rare. Iso-hyperdense on CT. MR: T1 isointense, hypointense to gray matter. T2, isointense to hyperintense to gray matter. It enhances intensely with contrast. FYI, quadrilateral retinoblastoma would be trilateral plus a lesion in the suprasellar cistern. |
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Common location of intraventricular meningioma:
a. atria of lateral ventricles b. 4th ventricle c. posterior 3rd ventricle d. frontal horns of the lateral ventricles |
Answer: Atria of lateral ventricles
Meningiomas are most commonly intraventricular in children (B&H Fundamentals 2nd ed p230). 2-5% of all meningiomas are intraventricular, and 80% of those are intraventricular (Dähnert Radiology Review 5th ed p 300), especially at the trigone of the left lateral ventricle (Neuro Requisties p. 70). |
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What is most characteristic of neurocystercercosis?
a. Seizures are a common complication b. Females are more often affected than males c. Associated with immunocompromised patients |
Seizures are a common complication
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40 year old male w/ hx of GSW to T4 undergoing a cystourethrogram when he experiences increased pulse pressure, rapid breathing, decreased heart rate, flushed face, and pale cool torso. Diagnosis?
a. vasovagal reaction b. autonomic dysreflexia c. allergic response to latex d. allergic response to contrast e. detrussor sphincter dysfunction |
Answer: Autonomic dysreflexia
The key here is headache, flushing, and diaphoresis. Bradycardia is less specific. Autonomic dysreflexia is a well known process observed in queadriplegics. It occurs with any type of irritation including skin irritants, decubitus ulcers, and catheterization. The hallmark is an acute onset of bradycardia, dangerously high hypertension and difficult to control, flushing, and diaphoresis. Spinal transection below T6: detrusor hyperreflexia, sphincter dyssynergia leading to incomplete emptying. Rx: anticholinergics Spinal transection above T6: sx as above but also autonomic dysreflexia: unopposed sympathetic outflow: sweating, HA, HTN, bradycardia. Rx: adrenergic blocker. Acutely: chlorpromazine. Prophlactically: terazosin or spinal anesthetic. |
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Patient who underwent chemotherapy and radiation therapy to brain. CT shows new white matter disease bilaterally. Most likely diagnosis?
a) ADEM b) PML c) Necrotizing leukoencephalopathy d) Adrenoleukodystrophy e) Subacute sclerosing panencephalitis f) Central pontine myelinosis |
Answer: Necrotizing leukoencephalopathy
Necrotizing leukoencephalopathy occurs primarily in patients undergoing chemotherapy (most commonly methotrexate); occurs with or without radiation. CT and MR show white matter lesions in the centrum semiovale. May be focal or diffuse. ADEM is an autoimmune disorder following measles, mumps, varicella, pertussis, rubella infection/vaccination. Seizures and focal neuro signs 7-14 days after clinical onset of viral infection. Multifocal subcortical white matter disease is typical, although the deep white matter, brainstem, and cerebellum can be affected. Steroids may result in dramatic improvement. PML is usually immunocompromised such as in AIDS. |
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