Vascular Imaging of the Head and Neck – Pathology

This lecture is the third part of a capstone course we have for our 4th year medical students. In the first lecture, we discussed general concepts about how to approach vascular imaging of the head and neck, including angiography, CT angiography, MR angiography, and ultrasound. The second part of the lecture covers a general search pattern for vascular imaging of the head and neck on a CT angiogram. To see more about this course, check out the full vascular capstone page. It contains interactive cases that you can scroll on your own as well as some additional videos explaining them.

In this lecture, we welcome back our special guest, Dr. Cynthia Wu, who is going to go over some of the common pathologies you might encounter on vascular imaging of the head and neck.

Common pathologies

There are a few common pathologies you might be looking for on vascular imaging of the head and neck, including aneurysm, thrombosis, dissection, and vascular malformations. Read on to learn more about each one.

Aneurysms

Aneurysms are abnormal outpouchings of the vessels. Sometimes they contain all the layers of the vessel wall (true aneurysms) or may be contained ruptures of one or more of the walls (false aneurysms, or pseudoaneurysms). Most intracranial aneurysms are true aneurysms while aneurysms in the neck are pseudoaneurysms.

Thrombosis

Thrombosis is occlusion of a vessel secondary to a blood clot. This is most commonly seen or suspected in the setting of a stroke, and can arise from rupture of an atherosclerotic plaque or from transmission of a thrombus more proximal in the circulation, such as from a cardiac valve or in the internal carotid artery. Most of the time, a thrombus will appear as abrupt severe narrowing or truncation of a vessel.

Thrombosis can also occur in veins, such as the dural venous sinuses. Venous thrombus will more commonly appear as a central filling defect.

Dissection

Dissection is a tear or injury to the lining of the artery, or intima. In this case, then blood goes into the space between the layers of the vessel wall, an area known as the false lumen. When the vessel is large enough, this vessel may fill with contrast on the injection, but in smaller vessels this may thrombose and be seen as a smooth tapering or narrowing of the vessel that can even result in occlusion. You can also sometimes get irregular enlargement of the vessel past it, or pseudoaneurysm.

Vascular malformations

Arteriovenous malformations, or AVMs, are abnormal clusters of vessels which have abnormal connections between the terminal arteries and veins. This results in a shunt, or passage of blood from an artery to a vein without a terminal capillary bed. Arteries and veins then are enlarged and at higher risk of rupture. The Spetzler Martin grading system is a grading system used to rate the risk of surgical mortality on resection which can help guide management.

What’s next?

Once you’ve finished this video, I recommend moving on to the next section of the vascular capstone, where you can review individual browseable cases with explanations. The capstone overview is here, if you’d like to see all the cases and videos.

Or, see all of the vascular capstone videos in the vascular imaging capstone playlist.

Vascular imaging of the head and neck course

This is a short course in learning about vascular imaging of the head and neck. The first videos have an overview of how to approach these studies, while the additional videos show you specific cases.

The cases are accessible on the vascular capstone page to view them yourself.

Introduction to Vascular Imaging of the Head and Neck

This lecture is the first part of a capstone course we have for our 4th year medical students about how to approach vascular imaging of the head and neck, including angiography, CT angiography, MR angiography, and ultrasound. To see more about this course, check out the full vascular capstone page. It contains interactive cases that you can scroll on your own as well as some additional videos explaining them.

In this lecture, we have a special guest, Dr. Cynthia Wu, who is going to explain to us some of the general concepts behind vascular imaging of the head and neck, different techniques you can do to perform vascular imaging, and when you might order each type of study.

Basic concepts

In general, to see vessels better on imaging, we need to use some sort of contrast to differentiate the vessels from the surrounding tissues. For techniques like CT, we can use an injected contrast agent to see the vessels. For other techniques such as MR angiography and ultrasound, we can use intrinsic properties of flowing blood to emphasize the vessels.

This section covers the key concepts behind how we see the vessels for these major techniques.

When do I order these tests?

Once you know about the different possible techniques, then you have to figure out when you would possibly order them. Each technique (CTA, MRA, and US) have different advantages and disadvantages that make them more or less suited to different scenarios.

What’s next?

Once you’ve finished this video, I recommend moving on to the next section of the vascular capstone, where you can learn a general strategy for interpreting a CT angiogram of the head and neck.

The capstone overview is here, if you’d like to see all the browseable cases and videos.

Or, see all of the vascular capstone videos in the vascular imaging capstone playlist.

Multiple sclerosis – white spots and red flags – part 2

Mimics and Variants

Demyelinating disease is a common situation we encounter in neuroradiology, and properly diagnosing and tracking it using MRI is a key skill for neuroradiologists. In this second part of the lecture, Dr. Michael Hoch gives us some tips about other causes of white matter lesion, and information we can use to make our imaging diagnosis of multiple sclerosis more specific.

Clinical history has an important role in determining how specific imaging findings are for multiple sclerosis. Some features may suggest that a patient does not have multiple sclerosis, such as if they are the wrong age (< 20 or > 50 years old), if they have abrupt swift progression, if they have systemic symptoms such as fever or weight loss, and if they have uncommon CNS symptoms such as a movement disorder or meningitis signs. MS lesions also usually occur in some specific locations, such as in the corpus callosum, temporal lobe, periventricular white matter, and juxtacortical white matter.

Mimics of Multiple sclerosis

So, what are some of the common mimics of MS?

Migraine – migraine is the most common cause of non-specific white matter abnormalities in young patients, occurring in more than 50% of patients with migraine

Chronic small vessel ischemia – more common with increasing age, and worsening with risk factors such as diabetes, hypertension, and smoking

CNS vasculitis – an inflammatory syndrome of the intracranial vessels. Be on the lookout if someone has a history of TIAs or thunderclap headache, or systemic symptoms.

Behcet’s disease – a vasculitis most common in young males, characterized by brainstem involvement and oral ulcers

Susac syndrome – an autoimmune microangiopathy overlapping MS in age distribution. However, patients more often have a triad of encephalopathy, hearing loss, and visual changes. Corpus callosum involvement is more likely to be central. 

CADASIL – an autosomal dominant syndrome characterized by frequent infarcts. Look out for the characteristic locations in the temporal poles, external capsules, and paramedian superior frontla lobes. It is also usually quite symmetric.

Other rarer mimics are Neuro-Sweets disease and Lyme disease, which can cause white matter abnormalities.

Key take home points of this lecture include:

  • Multiple sclerosis is a clinical diagnosis, not an MRI diagnosis
  • White spot lesion location matters
  • Juxtacortical lesions must touch the cortex
  • Aggressively window the spine to look for cord lesions
  • Leptomeningeal enhancement is possible in multiple sclerosis

Variants of demyelinating disease

There are several common variants that you should know about across the demyelinating spectrum:

ADEM – acute disseminated encephalomyelitis – an autoimmune mediated and often self limited fulminant demyelinating process. May be related to a viral illness or vaccination.

Marburg disease – a clinically fulminant demyelinating disease usually affecting younger patients with a febrile prodrome.

Balo concentric sclerosis – a rare and monophasic demyelinating disease characterized by large lesions with alternating zones of demyelination/myelination

Tumefactive demyelinating lesions (TDL) – large and often fulminant demyelinating lesions that have mass effect and can mimic tumors. Perfusion imaging with low blood volumes can help differentiate from masses.

Neuromyelitis optica (NMO) – a demyelinating syndrome characterized by post-chiasmatic optic neuritis and long segment spine lesions. This is mediated by an aquaporin-4 antibody.

Progressive multifocal leukoencepalopathy (PML) – a JC virus mediated demyelinating lesion that occurs in immune suppressed patients. Usually has little or no enhancement and favors a subcortical location.

Summary

In summary, there are a couple of key things to keep in mind when evaluating potential demyelinating lesions:

  • Read the chart for clinical red flags
  • Look at the MRI for imaging red flags, like strokes, hemorrhages, cysts, findings that are too symmetric, subcortical, or normal
  • Remember that white matter lesions from migraine and microvascular disease are far more common that multiple sclerosis
  • NMO has differentiating features
  • PML is a rare complication of immune suppressing medications in MS patients

 

The level of this lecture is appropriate for radiology residents, radiology fellows, and trainees in other specialties who have an interest in imaging or treating patients with potential demyelinating diseases.

This video is part of a two part series on multiple sclerosis presented by Dr. Hoch.

If you haven’t seen it already, go back and check out part 1, in which Dr. Hoch discusses the key findings of demyelinating lesions.

Multiple sclerosis – white spots and red flags

Demyelinating disease is a common situation we encounter in neuroradiology, and properly diagnosing and tracking it using MRI is a key skill for neuroradiologists. In this two part lecture, Dr. Michael Hoch instructs us on how to approach white matter abnormalities in the brain and use them towards making a diagnosis of multiple sclerosis. The first part is focused on key tips on making a diagnosis of demyelinating disease while the second is focused on potential pitfalls.

Be sure to watch them both to get the complete overview of imaging findings of common autoimmune and inflammatory conditions.

Multiple sclerosis – white spots and red flags – part 1

Making the diagnosis

Demyelinating disease is a common situation we encounter in neuroradiology, and properly diagnosing and tracking it using MRI is a key skill for neuroradiologists. Today, Dr. Michael Hoch gives the first part of a two part lecture on how to approach white matter abnormalties in the brain and use them towards making a diagnosis of multiple sclerosis.

Multiple sclerosis is a clinical diagnosis that depends on several possible presenting signs (such as depression, fatigue, vertigo, numbness or other neurological symptoms, bladder dysfunction, visual changes, or other phenomena including L’Hermitte’s sign or Uhthoff’s phenomenon) and other clinical sign (including tremor, decreased perception, hyperreflexia, and ataxia).

The imaging diagnosis of multiple sclerosis is based on the McDonald criteria, most recently revised in 2017. This requires dissemination in space, dissemination in time, and lack of an alternate explanation. You should evaluate different spaces for white matter abnormality, including the cortex, juxtacortical, subcortical and deep white matter, corpus callosum, and deep white matter, periventricular white matter. 

The locations of the lesions can provide a clue as to whether white matter lesions are more likely to be caused by demyelinating disease or other nonspecific insults, such as chronic microvascular ischemia. For instance, central lesions in the pons or lesions in the deep white matter are more nonspecific, while cortical/juxtacortical, periventricular, and anterior temporal lesions are more specific for multiple sclerosis.

The enhancement pattern is also a clue to whether a lesion might be demyelinating. Demyelinating lesions typically have an incomplete rim of enhancement, where the post-contrast enhancement has a broken circle type of appearance. Leptomeningeal enhancement can often be seen in patients with MS, although it is an alarm bell if patients don’t have a known diagnosis, as it can represent other diseases such as leptomeningeal carcinomatosis.

Key take home points of this lecture include:

  • Multiple sclerosis is a clinical diagnosis, not an MRI diagnosis
  • White spot lesion location matters
  • Juxtacortical lesions must touch the cortex
  • Aggressively window the spine to look for cord lesions
  • Leptomeningeal enhancement is possible in multiple sclerosis

 

The level of this lecture is appropriate for radiology residents, radiology fellows, and trainees in other specialties who have an interest in imaging or treating patients with potential demyelinating diseases.

This video is part of a two part series on multiple sclerosis presented by Dr. Hoch.

For the next part of the lecture, check out part 2, in which Dr. Hoch discusses potential mimics and pitfalls when assessing for demyelinating disease.

How to read a CT angiogram (CTA) of the Head and Neck

With increasing ability to image vascular phenomena, such as stroke and vascular malformations, through the use of very efficient and high speed computed tomography scanner (CT), we now have the ability to perform angiographic imaging of large segments of the body at one time. This has led to an explosion of vascular imaging of the head and neck to look for a variety of pathologies, including stroke, vascular malformations, and other vascular abnormalities. Every starting radiology resident needs a firm foundation in how to interpret these images.

Overview

Because there are a ton of images and a ton of structures that you need to look at, you really need a regimented approach, or search pattern, to use as you look through the images. In this video, I teach you how to look at a CT angiogram of the head and neck from start to finish so you can learn how to do it yourself. There are interactive examples that you can follow along on the vascular capstone page.

In this video, we go through a normal example.

I divide the study into the CTA of the neck, which I review first, and the CTA of the head, which I review second. My pattern goes as follows:

Nonvascular structures

I first like to look at all the nonvascular structures to make sure I’m not missing anything. I look at the lungs, the thyroid, the soft tissues of the neck (particularly to look for lymph nodes or mucosal masses), and the brain. I use a soft tissue window to look at these. Then I go back through with a bone window to look at the bones.

Neck Vessels

For the vessels of the neck, I use an approach that first does anterior vessels and then posterior vessels, moving from right to left. I follow the right common carotid artery through the bifurcation, and then follow the internal carotid to the skull base. I then repeat this pattern for the left carotid system. Then, I start from the right vertebral origin and follow it up to the skull base. Then the left vertebral artery. Once I’m finished, I restart at the skull base.

Head Vessels

For the vessels of the head, I follow a similar approach. I first follow the right ICA through the carotid terminus and then follow the right middle cerebral artery (MCA) and anterior cerebral artery(ACA). Once finished, I return to the skull base and repeat this pattern on the left. I then move to the posterior circulation, where I first follow the right vertebral artery, the basilar artery, and the right posterior cerebral artery (PCA). Then I repeat it on the left. My final search is to quickly look at the deep venous sinuses.

Summary

While others may have a different strategy, the most important part is to have a strategy and stick to it. This pattern of dividing the study into discrete sections will help you see all the findings and describe them accurately when reading cases on your own.

See all of the search pattern videos on the Search Pattern Playlist.

Imaging CNS autoimmune and inflammatory disease

Vascular Processes

This is the sixth and final lecture in a case based review of imaging of the brain and spine for autoimmune and inflammatory conditions. We will cover the MRI findings of some of the common conditions and some potential pitfalls and mimics.

This lecture covers intracranial vasculopathies, including central nervous system (CNS) vasculitis, Moya Moya disease, CADASIL, and MELAS. These vasculopathies can have a similar imaging appearance.

CNS vasculitis

CNS vasculitis is a process affecting the vessels which supply the brain with blood/oxygen. This can be a primary CNS process or can be associated with other systemic vasculopathies, such as lupus. The imaging manifestations are unexpected infarcts that are out of proportion for age and other causes of infarct. On vascular imaging, you will see areas of narrowing followed by areas of dilation or normal caliber, almost like beads on a string. While this can be caused by atherosclerotic disease, usually it will be out of proportion for age.

Vessel wall imaging

Vessel wall imaging is a specialized technique to look for inflammation in the walls of intracranial vessels. This consists of specialized sequences that suppress both fat and flow so you can see enhancement in the walls. On vessel wall imaging, vasculitis tends to enhance circumferentially while atherosclerotic disease enhancement is more eccentric. This is a useful advanced technique.

Moya Moya disease

Moya moya disease is a primary obliterative angiopathy which primarily occurs in young Asian women. In this disease, the large vessels of the circle of Willis, namely the middle cerebral artery, are obliterated and replaced with a number of abnormal collateral vessesl. This leads to a characteristic “puff of smoke” appearance on angiography. Patients may present with multiple strokes or hemorrhage and can be treated with ECA/ICA bypass. There are other causes of a moya moya like appearance, as it can happen with anything that causes chronic obliterative angiopathy, like sickle cell disease. However, although the terms are used interchangeably, this is really a moya moya syndrome, not the primary disease..

CADASIL

CADASIL, or cerebral autosomal dominant arteriopathy with subcortical infarcts and leukencephalopathy, is a genetic condition associated with the NOTCH3 gene abnormality. These patients have small and middle vessel abnormalities which manifest as a number of subcortical strokes and white matter abnormality in the bilateral temporal lobes. If you see abnormal subcortical temporal white matter, think about CADASIL.

MELAS

MELAS, or mitochondrial encephalopathy with lactic acidosis and stroke-like symptoms, is a similar syndrome where you get stroke-like episodes often preferentially involving the temporal and parietal lobes. They can often improve with time, but it can mimic other vasculopathies and strokes.

Summary and Conclusion

There are a number of vasculopathies which can affect the brain, with the most common being CNS vasculitis and moya moya disease or syndrome. CADASIL and MELAS can also have a similar appearance. This is the last lecture in this series. Please be sure to check out the other lectures in this series.

The level of this lecture is appropriate for radiology residents, radiology fellows, and trainees in other specialties, such as neurology, who have an interest in neuroradiology or may see patients with CNS demyelinating or inflammatory conditions.

Other videos on the autoimmune and demyelinating playlist are found here

Imaging CNS autoimmune and inflammatory disease

Amyloid related disease

This is the fifth lecture in a case based review of imaging of the brain and spine for autoimmune and inflammatory conditions. We will cover the MRI findings of some of the common conditions and some potential pitfalls and mimics.

This lecture covers three central nervous system (CNS) manifestations of amyloid disease: cerebral amyloid angiopathy (CAA), inflammatory amyloidosis, and amyloidomas. While the pathology is similar, the imaging manifestations are markedly different.

Cerebral amyloid angiopathy

Cerebral amyloid angiopathy is a small vessel vasculitis caused by deposition of amyloid beta in the walls of the vessels. The result is a vasculitis manifested by repeat hemorrhages, often in the periphery of the brain or at gray/white junctions, with relative sparing of areas often affected by hypertensive hemorrhage (such as the basal ganglia, thalamus, and pons). Patients are usually over 70 and may have progressive mental decline out of proportion for age. Imaging findings are most visible on gradient or susceptibility weighted T2 images, where you can see the sequelae of chronic hemorrhage. CAA is more common in patients with Alzheimer’s disease and Down’s syndrome, which are also amyloid related diseases.

Inflammatory amyloidosis

Inflammatory amyloidosis is another intracranial manifestation of amyloid disease that may affect slightly younger patients. Imaging findings are similar in that small areas of microhemorrhage can be seen, but the major difference is in the amount of cerebral edema as best characterized on FLAIR and T2 weighted images. Patients with inflammatory amyloid may present with headache, altered mental status, or focal neurologic symptoms. It can be challenging to diagnose, particularly if areas of hemorrhage are not clearly seen, and biopsy may be required.

Amyloidoma

Amyloidomas are tumor-like conditions in which the patients often present with focal neurologic symptoms and an intracranial mass. It often has surrounding edema, but a key feature is its internal T2 hypointensity. On post-contrast imaging, it can mimic a high grade glioma or metastasis because it can enhance avidly. Often resection or biopsy is required because they look so tumor-like.

Summary and Conclusion

Amyloid related disease in the CNS is uncommon, with the most common manifestation a vasculopathy from amyloid deposition in vessels. If accompanied by edema and inflammation, it is called inflammatory amyloid. Masses of amyloid deposition within the brain are uncommon but in rare cases can mimic tumors. All of these disease can be hard to diagnose and can frequently require a biopsy.

The level of this lecture is appropriate for radiology residents, radiology fellows, and trainees in other specialties, such as neurology, who have an interest in neuroradiology or may see patients with CNS demyelinating or inflammatory conditions.

Other videos on the autoimmune and demyelinating playlist are found here

Imaging CNS autoimmune and inflammatory disease

Spine Inflammatory Disease

This is the fourth lecture in a case based review of imaging of the brain and spine for autoimmune and inflammatory conditions. We will cover the MRI findings of some of the common conditions and some potential pitfalls and mimics.

This lecture covers two inflammatory diseases which can occur in the spinal cord: transverse myelitis and sarcoidosis. Multiple sclerosis can also cause an inflammatory myelitis, but it usually is associated with intracranial MS and has shorter segment lesions.

Transverse myelitis 

Transverse myelitis is a common infectious or inflammatory cause of myelopathy in the cervical or thoracic spine. This is most commonly manifested with long segment T2 abnormality (more than one vertebral body in length). It is commonly central and may have enhancement, particularly in the acute phase. It is often associated with a recent viral illness and can be caused by direct viral infection or as inflammatory post-viral syndrome. Treatment is largely limited to symptomatic control and immune suppression similar to treatment of multiple sclerosis.

Sarcoidosis

Sarcoid can affect the spine although it is less common that involvement of the brain. The most common manifestation in the spine is long segment T2 hyperintensity with associated enhancement. The enhancement pattern is often nodular and peripheral, which can help differentiate it from other causes of abnormal cord signal. As with sarcoidosis in the brain, you may also see nodular areas of enhancement or sarcoidomas. The diagnostic workup is similar. You should start by imaging the brain and chest to look for other potential areas of sarcoid involvement. ACE levels or IL-2 surface antigen can also be useful when available.  

Summary and Conclusion

In summary, when you have longer segment T2 hyperintense lesions in the spine, you should think about transverse myelitis or sarcoidosis. There is a broader differential which includes multiple sclerosis, lymphoma, and other tumors (such as astrocytoma or ependymoma). You may not be able to tell without other diagnostic clues, which makes imaging of the brain and correlation with the clinical scenario important.

The level of this lecture is appropriate for radiology residents, radiology fellows, and trainees in other specialties, such as neurology, who have an interest in neuroradiology or may see patients with CNS demyelinating or inflammatory conditions.

Other videos on the autoimmune and demyelinating playlist are found here