Neuroradiology Flashcards
Cerebral cavernous malformations are most commonly associated with which of the following?
Answers:
A. Developmental venous anomaly
B. Intracranial aneurysm
C. Dural arteriovenous fistula
D. Capillary telangiectasia
E. Early draining vein
Developmental venous anomaly
Discussion:
Cerebral cavernous malformations (also revered to as cavernomas or cavernous hemangiomas)
are one of the most common intracranial vascular malformations with an estimated prevalence of
0.4-0.8% and account for 10-25% of all intracranial vascular malformations. Most cavernous
malformations are asymptomatic and found incidentally. The annual risk of hemorrhage is 1-3%,
with the most common presenting symptom being seizure. Radiographically, cavernous
malformations have a “popcorn-like” appearance on MRI with a rim of hemosiderin that is evident
on susceptibility weighted imaging (SWI), and they are frequently associated with developmental
venous anomalies (DVA). Angiographically, cavernous malformations are occult lesions; however,
an associated DVA will appear as a collection of dilated veins converging on an enlarged cortical
vein (“Caput medusae”) that fill in phase with the remainder of the venous system.
Cavernous malformations are not associated with a higher incidence of intracranial aneurysms,
dural AV fistulas, or capillary telangiectasias.
As DVAs fill in phase with the remainder of the venous system, there are no early draining veins.
This is in contrast to cerebral arteriovenous malformations and dural AV fistulas.
Figure 1. MRI, T1 weighted image with contrast demonstrating a DVA
*Image obtained with permission via Creative Commons Attribution 3.0 (Original source:
https://commons.wikimedia.org
/wiki/File:Developmental_Venous_Anomaly_MRT_T1KM_axial_04.jpg)
References:
Reference (1)
Awad IA, Polster SP. Cavernous angiomas: deconstructing a neurosurgical disease. J Neurosurg.
2019 Jul 1;131(1):1-13. doi: 10.3171/2019.3.JNS181724. PMID: 31261134; PMCID: PMC6778695.
Pubmed Web link
https://pubmed.ncbi.nlm.nih.gov/31261134/
Reference (2)
Harrigan MR, Devakis JP. Handbook of Cerebrovascular Disease and Neurointerventional
Technique. 2nd Ed. New York, NY: Springer-Verlag New York, LLC; 2012.
Reference (3)
Abdulrauf SI, Kaynar MY, Awad IA. A comparison of the clinical profile of cavernous malformations
with and without associated venous malformations. Neurosurgery. 1999 Jan;44(1):41-6; discussion
46-7. doi: 10.1097/00006123-199901000-00020. PMID: 9894962.
Pubmed Web link
https://pubmed.ncbi.nlm.nih.gov/9894962
The lesion indicated by the arrowheads on the gadolinium-enhanced T1 (left) and T2 (right) MR images is most likely a(n)
Answers:
A. Mega cisterna magna
B. Epidermoid cyst
C. Neuroenteric cyst
D. Dermoid cyst
E. Trigeminal schwannoma
Epidermoid cyst
Discussion:
The MRI image demonstrates an epidermoid cyst, which is an extra-axial cystic lesion that is
hyperintense on T2-weighted imaging, hypointense on T1-weighted imaging, and without
significant contrast enhancement. Notably, the lesion results in mass-effect on the brainstem.
Although not pictured, epidermoid cysts demonstrate diffusion restriction (bright) on diffusion
weighted imaging (DWI), which differentiates them from arachnoid cysts.
Mega cisterna magna is an anatomic variant in which the CSF space in the posterior and inferior
aspect of the posterior fossa is enlarged. The location of the lesion indicated by the arrow is not
consistent with a mega cisterna magna.
Dermoid cysts are developmental abnormalities lined with stratified squamous epithelium as well
as epidermal appendages (e.g. hair follicles, sweat glands). As opposed to epidermoid cysts,
dermoid cysts tend to occur along the midline. Radiographically, on MRI dermoid cysts tend to be
hyperintense on T1-weighted images without contrast enhancement and have a variable signal on
T2-weighted images.
Trigeminal schwannomas are rare nerve sheath tumors involving the trigeminal nerve (CN V).
Radiographically, they appear as a mass lesion on MRI that are isointense on T1-weighted images
with prominent enhancement and are hyperintense on T2-weighted images.
Neuroenteric cysts are rare benign congenital lesions more common in the spine than the
intracranial space. Radiographically, neuroenteric cysts tend to be hyperintense on T1- and T2-
weighted imaging.
References:
Reference (1)
Osborn AG, Preece MT. Intracranial cysts: radiologic-pathologic correlation and imaging approach.
Radiology. 2006 Jun;239(3):650-64. doi: 10.1148/radiol.2393050823. PMID: 16714456.
Pubmed Web link
https://pubmed.ncbi.nlm.nih.gov/16714456/
Reference (2)
Taillibert S, Le Rhun E, Chamberlain MC. Intracranial cystic lesions: a review. Curr Neurol
Neurosci Rep. 2014 Sep;14(9):481. doi: 10.1007/s11910-014-0481-5. PMID: 25106500.
Pubmed Web link
https://pubmed.ncbi.nlm.nih.gov/25106500/
A 25-year-old woman is evaluated for a transient episode of left-sided weakness. Posterioranterior and lateral angiograms obtained from a right common carotid injection are shown. Based on these findings, which of the following is the most likely diagnosis?
Answers:
A. dural arteriovenous fistula (AVF)
B. Moyamoya Disease
C. arteriovenous malformation
D. thromboembolic occlusion of the right internal carotid artery (ICA)
E. cavernous carotid fistula
Moyamoya Disease
Discussion:
This angiogram demonstrates occlusion of the terminal ICA and its proximal branches and
development of a basal collateral network. Suzuki (1965) first described this entity as Moyamoya
Disease because the appearance of these collateral channels resembled “something hazy like a
puff of cigarette smoke drifting in the air.”
Note that there is no evidence of early venous drainage to suggest arteriovenous malformation,
cavernous carotid fistula, or dural AVF. Occlusion of the ICA terminus by an embolus would not
show filling of this hypertrophied vascular network.
References:
Suzuki J, Takaku A. Cerebrovascular “Moyamoya” Disease: Disease Showing Abnormal Net-Like
Vessels in Base of Brain. Arch Neurol. 1969;20(3):288–299.
doi:10.1001/archneur.1969.00480090076012
Fujimura M, Bang OY, Kim JS. Moyamoya Disease. Front Neurol Neurosci. 2016;40:204-220. doi:
10.1159/000448314. Epub 2016 Dec 2. PMID: 27960175
Patients with herpes simplex, type 1 encephalitis typically show abnormalities on FLAIR imaging involving the temporal lobe, insula, and which of the following regions?
Answers:
A. Occipital lobe
B. Cerebellar hemispheres
C. Midbrain
D. Parietal lobe
E. Frontal lobe
Frontal lobe
Discussion:
Herpes simplex virus encephalitis (HSVE) has an incidence of 1-4/1 million cases annually. In the
United States, HSV can be attributed to 50-75% of sporadic encephalitis cases, and 90% of these
are caused by HSV-1. Mortality for treated HSVE is about 20% in 6 months. Clinical confirmation
includes PCR testing of CSF. In a study of 40 patients with HSVE who had an MRI, 95% of
patients demonstrated FLAIR/T2 changes with the majority of patients demonstrating radiographic
findings in the temporal lobe (87%) and insula (70%). In addition, 67.5% of patients had
radiographic changes in the frontal lobe.
References:
Singh TD, Fugate JE, Hocker S, Wijdicks EFM, Aksamit AJ Jr, Rabinstein AA. Predictors of
outcome in HSV encephalitis. J Neurol. 2016 Feb;263(2):277-289. doi:
10.1007/s00415-015-7960-8. Epub 2015 Nov 14. PMID: 26568560.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/26568560
Tyler KL. Acute Viral Encephalitis. N Engl J Med. 2018 Aug 9;379(6):557-566. doi:
10.1056/NEJMra1708714. PMID: 30089069.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/30089069
Jayaraman K, Rangasami R, Chandrasekharan A. Magnetic Resonance Imaging Findings in Viral
Encephalitis: A Pictorial Essay. J Neurosci Rural Pract. 2018 Oct-Dec;9(4):556-560. doi:
10.4103/jnrp.jnrp_120_18. PMID: 30271050; PMCID: PMC6126294.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/30271050/
The most likely cause of the findings in the CT scan shown is injury or occlusion of which of the following cerebral blood vessels?
Answers:
A. Recurrent artery of Heubner
B. Medial posterior choroidal artery
C. Anterior cerebral artery
D. Lateral lenticulostriate arteries
E. Anterior choroidal artery
Recurrent artery of Heubner
Discussion:
The CT image depicts a hypodensity that includes the head of the caudate nucleus and the
anterior limb of the internal capsule, which is the classic appearance for a recurrent artery of
Heubner infarct. The recurrent artery of Heubner is the largest lenticulostriate artery arising from
the anterior cerebral artery, most commonly arising from the proximal A2 segment. It supplies
several structures, including the head of the caudate nucleus, medial globus pallidus, anterior limb
of the internal capsule, and the anterior hypothalamus. A recurrent artery of Heubner stroke
classically presents as dysarthria and contralateral weakness of the arm and face. Clinically this is
important, as it is a commonly described complication after microvascular clipping of anterior
communicating artery aneurysms.
The anterior choroidal artery arises from the distal internal carotid artery and supplies the posterior
limb of the internal capsule, lateral thalamus, optic tract, lateral cerebral peduncle, globus pallidus
internus, and tail of the caudate. Anterior choroidal artery infarcts are a commonly described
complication after microvascular clipping of posterior communicating artery aneurysms and
present with contralateral hemiparesis, hemianesthesia, and hemianopia.
The medial posterior choroidal artery arises from the P2 segment of the posterior cerebral artery
and supplies the tegmentum, midbrain, posterior thalamus, and pineal gland, and typically
presents with abnormal eye movements and diplopia.
The anterior cerebral artery (ACA) arises as one of the two terminal branches of the internal
carotid artery. The ACA has several branches that supply frontal lobe and medial cerebral
hemisphere. ACA strokes typically present with predominant contralateral lower extremity
weakness and sensory changes.
The lateral lenticulostriate arteries arise from the horizontal segment of the middle cerebral artery
and supply the lateral putamen and external capsule, and present with contralateral hemiparesis
with largely preserved sensation.
References:
Reference (1)
Munakomi S, M Das J. Neuroanatomy, Recurrent Artery of Heubner. 2021 Sep 3. In: StatPearls
[Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan–. PMID: 31424806.
Pubmed Web link
https://pubmed.ncbi.nlm.nih.gov/31424806/
Reference (2)
Osborn AG. Diagnostic Neuroradiology. St. Louis: Mosby; 1994: 133-134.
On the lateral view internal carotid artery angiogram shown, the arrow is pointing to which of the following arteries?
Answers:
A. Inferior parietal artery
B. Callosomarginal
C. Internal frontal
D. Frontopolar
E. Pericallossal
Pericallossal
Discussion:
The anterior cerebral artery distal to the anterior communicating artery is referred to as the
pericallosal artery (arrow). The frontopolar artery and the inferior parietal arteries are typically
branches of the pericallossal artery. The callosomarginal artery is a branch of the pericallossal
artery. It runs parallel to the pericallossal artery in the sulcus above the cingulate gyrus
(Perlemutter 1978). Approximately 15% of the time it is absent. When it is present, it supplies the
internal frontal and paracentral arteries. Note that there is considerable variability of the ACA
branching pattern between individual patients.
References:
Perlmutter D, Rhoton AL Jr. Microsurgical anatomy of the distal anterior cerebral artery. J
Neurosurg. 1978 Aug;49(2):204-28. doi: 10.3171/jns.1978.49.2.0204. PMID: 671075.
Cilliers K, Page BJ. Review of the Anatomy of the Distal Anterior Cerebral Artery and Its
Anomalies. Turk Neurosurg. 2016;26(5):653-61. doi: 10.5137/1019-5149.JTN.14294-15.1. PMID:
27337235
During diagnostic cerebral angiography, while navigating the great vessels of the arch, the operator finds that the diagnostic catheter repeatedly falls into the left subclavian artery when a move from the brachiocephalic to the left common carotid artery (LCCA) is attempted. The most likely explanation for this is which of the following?
Answers:
A. aberrant subclavian artery origin
B. situs invertus
C. arch is bovine
D. LCCA origin occlusion
E. subclavian steal
arch is bovine
Discussion:
There are typically (3) vessels exiting the aortic arch: brachiocephalic, left common carotid, and left
subclavian artery. In about 15% of cases the left common carotid artery does not come directly off
the arch but rather comes off as a branch of the brachiocephalic artery. This is often called a
bovine aortic arch. When this is the case, pulling the catheter out of the brachiocephalic artery into
the arch in the usual fashion will result in catheterization of the left subclavian artery rather than
the left common carotid artery. Of note, it has been pointed out that this common nomenclature is
a misnomer. The typical arch configuration in cows is comprised of a single brachiocephalic trunk
giving rise to all of the cerebral vessels (Layton 2006).
References:
Layton KF, Kallmes DF, Cloft HJ, Lindell EP, Cox VS. Bovine aortic arch variant in humans:
clarification of a common misnomer. AJNR Am J Neuroradiol. 2006 Aug;27(7):1541-2. PMID:
16908576; PMCID: PMC7977516.
Lazaridis N, Piagkou M, Loukas M, Piperaki ET, Totlis T, Noussios G, Natsis K. A systematic
classification of the vertebral artery variable origin: clinical and surgical implications. Surg Radiol
Anat. 2018 Jul;40(7):779-797. doi: 10.1007/s00276-018-1987-3. Epub 2018 Feb 19. PMID:
29459992
An 11-year-old boy is evaluated for left-sided lower extremity weakness and paresthesias. A vascular lesion is noted at the T10 spinal level. Which of the following vessels is indicated by the arrows in the spinal angiogram shown?
Answers:
A. Artery of Adamkiewicz
B. anterior spinal artery
C. posterior radiculomedullary artery
D. posterior spinal artery
E. anterior radiculomedullary artery
anterior spinal artery
Discussion:
The correct answer is the anterior spinal artery. In this angiogram of a spinal arteriovenous
malformation (AVM), the proximal portion of the anterior spinal artery (black arrows) is normal, but
the descending loop is enlarged because it is feeding the AVM. In the thoracic spine, the anterior
spinal artery receives contributory flow from multiple anterior radiculomedullary arteries, the largest
of which is called the Artery of Adamkiewicz. It makes a classic “hairpin loop” upon entry to the
anterior spinal artery. There are generally no communication or anastomoses between the anterior
and posterior spinal arteries, thus making the artery of Adamkiewicz similar to an end artery. This
vascular anatomy can often lead to anterior spinal cord syndrome within the region of the lumbar
spinal cord, leading to loss of motor function and sensory function carried by the anterior columns
including pain and temperature (sparing proprioception within the dorsal column, which is supplied
by the posterior spinal artery). The posterior spinal arteries are more lateral relative to the spinal
cord.
References:
Yoshioka K, Niinuma H, Ehara S, Nakajima T, Nakamura M, Kawazoe K. MR angiography and CT
angiography of the artery of Adamkiewicz: state of the art. Radiographics. 2006 Oct;26 Suppl
1:S63-73. doi: 10.1148/rg.26si065506. PMID: 17050520.
Gofur EM, Singh P. Anatomy, Back, Vertebral Canal Blood Supply. [Updated 2021 Jul 26]. In:
StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Available from:
https://www.ncbi.nlm.nih.gov/books/NBK541083
Which of the following helps reduce the radiation exposure to operating personnel during surgery?
Answers:
A. Attempt to bring the image intensifier as far away from the patient and the x-ray source as close to the patient as possible
B. Wear proper lead or lead-equivalent shielding at all times during fluoroscopy
C. Attempt to use rapid continuous fluoroscopy over spaced out pulsed fluoroscopy
D. Attempt to limit use of collimation whenever possible
E. Attempt to stand on the side of the patient with the x-ray source instead of the image intensifier
Wear proper lead or lead-equivalent shielding at all times during fluoroscopy
Discussion:
When utilizing intra-operative radiation, it is imperative that all unnecessary personnel leave the
room while fluoroscopy is performed. Personnel who must remain in the operating room should
wear proper lead or lead-equivalent shielding at all times during fluoroscopy. It is also important to
know that the distance from the radiation source has an inverse-squared relationship to the
radiation dose. It has been shown that a distance 2-3 feet from the x-ray source will reduce total
radiation exposure by 8 times.
It is preferred to use pulsed fluoroscopy over continuous fluoroscopy to decreased total radiation
exposure.
Collimation of the x-ray source will decrease the radiation dose to the patient as well as reduce the
scatter experienced by the operating room team, and therefore should be used whenever possible.
X-ray scatter is greater on the side of the x-ray source compared to the side of the image
intensifier; therefore, it is preferable to stand on the side of the patient with the image intensifier.
Positioning a C-arm with the image intensifier as close to the patient as possible will decrease the
radiation scatter experienced by the surgeon.
References:
Reference (1)
Srinivasan D, Than KD, Wang AC, La Marca F, Wang PI, Schermerhorn TC, Park P. Radiation
safety and spine surgery: systematic review of exposure limits and methods to minimize radiation
exposure. World Neurosurg. 2014 Dec;82(6):1337-43. doi: 10.1016/j.wneu.2014.07.041. Epub
2014 Aug 1. PMID: 25088230.
Pubmed Web link
https://pubmed.ncbi.nlm.nih.gov/25088230/
Reference (2)
Kim HJ, Park ES, Lee SH, Park CH, Chung SW. Reduction of Radiation Exposure by Modifying
Imaging Manner and Fluoroscopic Settings during Percutaneous Pedicle Screw Insertion. J
Korean Neurosurg Soc. 2021 Nov;64(6):933-943. doi: 10.3340/jkns.2020.0338. Epub 2021 Aug
24. PMID: 34420290; PMCID: PMC8590905.
Pubmed Web link
https://pubmed.ncbi.nlm.nih.gov/34420290/
In the patient whose CT scan (A) and angiogram (B) are shown, the most likely cause of hemorrhage is rupture of a(n)
Answers:
A. Dural Arteriovenous Fistula
B. Spinal arteriovenous malformation
C. Posterior Inferior Cerebellar Artery (PICA) aneurysm
D. Venous varix
E. Cerebellar arteriovenous malformation (AVM)
Posterior Inferior Cerebellar Artery (PICA) aneurysm
Discussion:
This vertebral angiogram demonstrates an aneurysm in the telovelotonsillar segment of PICA.
Distally, the PICA feeds a small cerebellar arteriovenous malformation (AVM). The CT shows
hemorrhage in the 4th ventricle and inferior vermis. There is also a fair amount of subarachnoid
hemorrhage (SAH). The telovelotonsillar segment of PICA is in close proximity to the 4th ventricle
and inferior vermis, and this pattern of hemorrhage is typical of distal PICA aneurysms. Bleeding
from the AVM would have been more localized to the cerebellar parenchyma and would not likely
have caused so much subarachnoid or intraventricular blood. A detailed review of this anatomy
was published by Hudgins and Rhoton (1983).
References:
Hudgins RJ, Day AL, Quisling RG, Rhoton AL Jr, Sypert GW, Garcia-Bengochea F. Aneurysms of
the posterior inferior cerebellar artery. A clinical and anatomical analysis. J Neurosurg. 1983
Mar;58(3):381-7. doi: 10.3171/jns.1983.58.3.0381. PMID: 6827323.
Orakcioglu B, Schuknecht B, Otani N, Khan N, Imhof HG, Yonekawa Y. Distal posterior inferior
cerebellar artery aneurysms: clinical characteristics and surgical management. Acta Neurochir
(Wien). 2005 Nov;147(11):1131-9; discussion 1139. doi: 10.1007/s00701-005-0599-y. Epub 2005
Aug 1. PMID: 16052289
Based on the radiograph of the spine shown, which of the following is the most likely diagnosis?
Answers:
A. Ankylosing spondylitis
B. Diffuse idiopathic skeletal hyperostosis
C. Rheumatoid arthritis
D. Spondylolysis
E. Degenerative spondylosis
Diffuse idiopathic skeletal hyperostosis
Discussion:
The lateral lumbar radiograph demonstrates diffuse idiopathic skeletal hyperostosis (DISH). DISH
has three diagnostic criteria:
1. “Flowing” ossification of at least four contiguous vertebral levels, most commonly involving
the anterior longitudinal ligament
2. Relative preservation of disk height and lack of significant degenerative changes
3. The absence of key features of ankylosing spondylitis, including ankylosis posteriorly at the
facet joint interface (“bamboo spine”) and sacroiliac joint erosion
Ankylosing spondylitis (AS) is a seronegative spondyloarthropathy that may appear similar to
DISH. However, AS is strongly associated with HLA-B27 and tends to present in younger patients.
Radiographically, AS tends not to have the “flowing” pattern of ossification and includes ankylosis
of the posterior spinal elements (Figure 1.)
Rheumatoid arthritis (RA) is a chronic autoimmune inflammatory arthropathy, classically affecting
the fingers and hands. When RA involves the spine, it typical results in erosion of the dens and
development of atlantoaxial instability.
Spondylolysis is defined as a defect in the pars interarticularis, which is not demonstrated in the
radiograph.
While patients with DISH may also develop degenerative spondylosis, the classic degenerative
findings of loss of disk space height, osteophyte formation, and endplate sclerosis are not seen in
DISH.
*Image used with permission via CC BY-SA 4.0 (Original source: https://commons.wikimedia.org
/wiki/File:AnkSponTLateral2016.png)
References:
Reference (1)
Luo TD, Varacallo M. Diffuse Idiopathic Skeletal Hyperostosis. 2021 Aug 23. In: StatPearls
[Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan–. PMID: 30855792.
Pubmed Web link
https://www.ncbi.nlm.nih.gov/books/NBK538204/
Reference (2)
Mario Cammisa, Antonio De Serio, Giuseppe Guglielmi. Diffuse idiopathic skeletal hyperostosis.
European Journal of Radiology. Volume 27, Supplement 1, 1998, Pages S7-S11. ISSN 0720-048X,
https://doi.org/10.1016/S0720-048X(98)00036-9.
Reference (3)
Mader R, Baraliakos X, Eshed I, et al. Imaging of diffuse idiopathic skeletal hyperostosis (DISH).
RMD Open 2020;6:e001151. doi: 10.1136/rmdopen-2019-001151
A 22-year-old woman is brought to the emergency department 90 minutes after a high-speed rollover motor vehicle collision. Physical examination shows proptosis and erythema of the left eye. Lateral (Figure A) and anteroposterior (Figure B) angiograms of the left internal carotid artery are
shown. Which of the following structures is indicated by the arrows in the angiograms?
Answers:
A. pterygoid plexus
B. cavernous sinus
C. superior ophthalmic artery
D. inferior ophthalmic vein
E. contrast extravasation
pterygoid plexus
Discussion:
The angiogram shows a left cavernous carotid fistula (CCF). Venous drainage is predominantly
anterior through a dilated superior ophthalmic vein and the pterygoid plexus (arrow). The
pterygoid plexus (via the maxillary vein) can be used as a route to the cavernous sinus for
transvenous embolization of a CCF if the inferior petrosal sinus cannot be accessed (Chun, 2002).
References:
Chun GF, Tomsick TA. Transvenous embolization of a direct carotid cavernous fistula through the
pterygoid plexus. AJNR Am J Neuroradiol. 2002 Aug;23(7):1156-9. PMID: 12169474; PMCID:
PMC8185731.
Gemmete JJ, Ansari SA, Gandhi DM. Endovascular techniques for treatment of carotid-cavernous
fistula. J Neuroophthalmol. 2009 Mar;29(1):62-71. doi: 10.1097/WNO.0b013e3181989fc0. PMID:
19458580.
A male newborn is evaluated because of the lesion shown in the MR images and angiograms. Which of the following additional findings is most likely in this patient?
Answers:
A. Enlarged straight sinus
B. High output cardiac failure
C. Enlarged Vein of Galen
D. Slit ventricles
E. agenesis of choroidal arteries
High output cardiac failure
Discussion:
The MRI and angiogram demonstrate a Vein of Galen Malformation (VOGM) and associated
hydrocephalus in a neonate. The VOGM is a high flow arteriovenous shunt between the choroidal
arteries and the embryonic precursor to the Vein of Galen, the Median Porencephalic Vein of
Markowski (MPVM). Therefore, VOGM is a actually a misnomer. In neonates, the VOGM causes
so much arterial shunting that it often leads to high output cardiac failure. Other common
associated findings include hypertrophy of the choroidal vessels, hydrocephalus, and dilated scalp
veins. Note that it will often appear that the straight sinus is dilated, but drainage from the MPVM
to the torcula is via an abnormally enlarged falcine sinus. The true sagittal sinus is typically small
or even absent (Gailloud 2005).
References:
Gailloud P, O’Riordan DP, Burger I, Levrier O, Jallo G, Tamargo RJ, Murphy KJ, Lehmann CU.
Diagnosis and management of vein of galen aneurysmal malformations. J Perinatol. 2005
Aug;25(8):542-51. doi: 10.1038/sj.jp.7211349. PMID: 16015373.
Hoang S, Choudhri O, Edwards M, Guzman R. Vein of Galen malformation. Neurosurg Focus.
2009 Nov;27(5):E8. doi: 10.3171/2009.8.FOCUS09168. PMID: 19877798
The unenhanced (left) and gadolinium-enhanced (right) T1-weighted MR images shown are most consistent with a diagnosis of
Answers:
A. Epidermoid cyst
B. Abscess
C. Schwannoma
D. Arachnoid cyst
E. Hemorrhage
Epidermoid cyst
Discussion:
Intracranial epidermoids are epithelial cell tumors that commonly occur in the cerebellopontine
angle, where they can compress the brainstem and cranial nerves. They appear as lobulated
lesions that fill and expand CSF spaces and often encase adjacent nerves and
vessels. Epidermoids are often indistinguishable from CSF signal (such as in arachnoid cysts,
though these typically have smooth borders) on many MRI sequences, except that they commonly
restrict diffusion. Schwannomas and abscesses most frequently enhance with contrast, and the
appearance of hemorrhage varies based on time course but can be identified acutely on
susceptibility-weighted images.
Epidermoid cysts are extra-axial lesions commonly found in the posterior fossa, with the
cerebellopontine angle cistern being the most common location. On imaging, they classically
demonstrate hypodensity on CT scan, hypointensity on T1WI, and hyperintensity on T2-weighted
images (T2WI), almost similar to cerebrospinal fluid (CSF), with characteristic diffusion restriction
on diffusion-weighted imaging (DWI).
References:
Jamjoom, D.Z., Alamer, A. & Tampieri, D. Correlation of radiological features of white epidermoid
cysts with histopathological findings. Sci Rep 12, 2314 (2022).
Gao, P. Y., Osborn, A. G., Smirniotopoulos, J. G. & Harris, C. P. Radiologic-pathologic correlation.
Epidermoid tumor of the cerebellopontine angle. AJNR Am. J. Neuroradiol. 13, 863–872 (1992)
A 40-year-old man is evaluated for fever, rash, headache, stiff neck, and lethargy two weeks after a camping trip. A lumbar puncture and CSF Western blot analysis confirm Lyme disease. On neurological examination, a cranial neuropathy is noted. The most likely cranial nerve palsy caused by this infection is which of the following?
Answers:
A. CN IV
B. CN V
C. CN VI
D. CN VII
E. CN III
CN VII
Discussion:
Lyme disease, also known as borreliosis, is a disease acquired through infection by Borrelia
burgdorferi spirochetes with an incidence of about 30K cases/year in the United States. Clinically,
patients first present with the typical erythema migrans rash with antibodies developing over the
next 8 weeks. Neurologic symptoms related to Lyme disease may include Bannwarth’s syndrome,
often demonstrating a triad of radiculitis, peripheral motor deficits, and lymphocytic CSF. In less
than 5% of cases, patients may develop vasculitis, myelitis, or encephalitis. The most common
cranial neuropathy is a facial nerve palsy (>90% of cases), often occurring bilaterally, and is the
most common deficit in pediatric cases. Oculomotor nerve palsies and abducens nerve palsies
may also occur, however they occur in only 5% of cases.
References:
Ross Russell AL, Dryden MS, Pinto AA, Lovett JK. Lyme disease: diagnosis and management.
Pract Neurol. 2018 Dec;18(6):455-464. doi: 10.1136/practneurol-2018-001998. Epub 2018 Oct 3.
PMID: 30282764.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/30282764
Lindland ES, Solheim AM, Dareez MN, Eikeland R, Ljøstad U, Mygland Å, Reiso H, Lorentzen ÅR,
Harbo HF, Beyer MK. Enhancement of cranial nerves in Lyme neuroborreliosis: incidence and
correlation with clinical symptoms and prognosis. Neuroradiology. 2022 May 24. doi:
10.1007/s00234-022-02957-2. Epub ahead of print. PMID: 35608630.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/35608630
Which of the following MR scanning sequences will most effectively distinguish between abscess and tumor?
Answers:
A. T1
B. DWI/ADC
C. T1 + contrast
D. GRE
E. T2
DWI/ADC
Discussion:
A cerebral abscess is an infected pocket in the brain, typically with bacteria or fungus. It can be
difficult to diagnosis on imaging when comparing the lesion to a tumor. Both abscesses and
malignant brain tumors typically demonstrate a rim-enhancing pattern. Intracranial abscesses
frequently restrict diffusion, whereas contrast-enhancing malignant neoplasms rarely do. T1- and
T2-weighted sequences vary with both entities, and there is rarely GRE blooming artifact in the
absence of hemorrhage or calcification in either.
Multimodal MRI improves the differential diagnosis between brain abscesses and tumors, as the
capsule of pyogenic abscess appears hyperintense in T1-weighted sequence and hypointense in
T2-weighted sequence, with regular enhancement after gadolinium injection. The necrotic center
of pyogenic abscess is hypointense in T1-weighted sequence, hyperintense in T2-weighted
sequence, and typically hyperintense in diffusion weighted imaging (DWI) with restricted apparent
diffusion coefficient (ADC).
References:
Osborn AG. Diagnostic Neuroradiology
Feraco P, Donner D, Gagliardo C, Leonardi I, Piccinini S, Del Poggio A, Franciosi R, Petralia B,
van den Hauwe L. Cerebral abscesses imaging: A practical approach. J Popul Ther Clin
Pharmacol. 2020 Jul 11;27(3):e11-e24. doi: 10.15586/jptcp.v27i3.688. PMID: 32757543.
Carmine Franco Muccio, Ferdinando Caranci, Felice D’Arco, Alfonso Cerase, Luca De Lipsis,
Gennaro Esposito, Enrico Tedeschi, Cosma Andreula. Magnetic resonance features of pyogenic
brain abscesses and differential diagnosis using morphological and functional imaging studies: A
pictorial essay. Journal of Neuroradiology, Volume 41, Issue 3, 2014, Pages 153-167, ISSN
0150-9861
A 65-year-old woman has had generalized seizures and episodes of confusion and memory loss over the past ten weeks. On examination, the patient is alert but has profound short-term memory loss; neurologic function is otherwise intact. A T1-weighted MR image shows no contrast
enhancement. A T2-weighted MR image shows brightness within the mesial temporal lobes bilaterally. Which of the following is the most likely diagnosis?
Answers:
A. Coccidiodomycosis
B. Paraneoplastic limbic encephalitis
C. Abscess
D. Low-grade glioma
E. Mesial temporal sclerosis
Paraneoplastic limbic encephalitis
Discussion:
Paraneoplastic limbic encephalitis (PLE) is a rare neurological syndrome associated with cancer
and selectively affects limbic system structures, including the hippocampus, hypothalamus, and
amygdala. Imaging most frequently manifests with cortical thickening and increased T2/FLAIR
signal intensity of these regions. Mesial temporal sclerosis and low-grade glioma, although both T2
intense pathologies, are most commonly unilateral. Mesial temporal sclerosis is a vital etiology
underlying some forms of epilepsy. Multi-focal infections typically contrast enhance and are rarely
symmetric.
A study conducted by Gultekin et al in 2000 proposed the first diagnostic criteria for paraneoplastic
limbic encephalitis, which included the following: 1) short-term memory loss, seizures, or
psychiatric symptoms; 2) <4 years between symptom onset and cancer diagnosis; 3) exclusion of
metastases, infection, metabolic, or other causes; and 4) one of the following: inflammatory CSF
findings, temporal lobe T2 or FLAIR hyperintensity on MR imaging, or electroencephalogram
abnormality in the temporal lobes. Tüzün and Dalmau subsequently modified these criteria in 2007
to account for the growing subset of nonparaneoplastic forms of autoimmune encephalitis, which
also demonstrated prominent limbic involvement.
References:
Shen, K., Xu, Y., Guan, H. et al. Paraneoplastic limbic encephalitis associated with lung cancer. Sci
Rep 8, 6792 (2018).
Gultekin SH, Rosenfeld MR, Voltz R, et al. Paraneoplastic limbic encephalitis: neurological
symptoms, immunological findings and tumor association in 50 patients. Brain 2000;123(pt
7):1481–94 doi:10.1093/brain/123.7.1481 pmid:10869059
üzün E, Dalmau J.Limbic encephalitis and variants: classification, diagnosis and treatment. Neurologist 2007;13:261–71 doi:10.1097/NRL.0b013e31813e34a5 pmid:17848866
Which of the following structures is indicated by the arrow in the angiogram shown?
Answers:
A. ophthalmic artery
B. ethmoidal artery
C. superior ophthalmic vein
D. cavernous sinus
E. facial vein
superior ophthalmic vein
Discussion:
The common carotid artery angiogram shows a patient with a cavernous carotid fistula draining
anteriorly through a pathologically dilated superior ophthalmic vein (arrow) and, distally, the
anterior facial vein (Debrun 1988).
References:
Debrun GM, Viñuela F, Fox AJ, et al. Indications for treatment and classification of 132 carotidcavernous fistulas. Neurosurgery. 1988 Feb;22(2):285-9.2.
Barrow DL, Spector RH, Braun IF, Landman JA, Tindall SC, Tindall GT. Classification and
treatment of spontaneous carotid-cavernous sinus fistulas. J Neurosurg. 1985 Feb;62(2):248-56.
doi: 10.3171/jns.1985.62.2.0248. PMID: 3968564