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

Masslike inflammatory disease

This is the third 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 masslike inflammatory diseases which can occur in the orbit and brain. These don’t really fit well into other categories with the exception that they are more likely to have a mass and adjacent expansion.

Orbital inflammatory disease

Orbital inflammatory disease is an infiltrative disease most often seen in the orbit and anterior skull base. It is characterized by masslike replacement of the orbital contents, sometimes with extension in the orbits. It is often T2 intermediate to dark and has avid enhancement. Many cases are associated with IgG4 abnormalities, while other cases are purely idiopathic. This disease was previously called orbital pseudotumor, but it is more exact now to call it IgG4 orbital disease or idiopathic orbital inflammation. The differential diagnosis for this appearance includes metastatic disease, lymphoma, and sarcoidosis.

Sarcoidosis

Sarcoid can also affect the brain, orbits, and spine, and when it does is referred to as neurosarcoidosis. The most common manifestation is leptomeningeal enhancement, but it can be accompanied by parenchymal nodules. It preferentially affects the basal cisterns and skull base. When you have a basilar meningitis, the differential includes metastatic disease (leptomeningeal carcinomatosis), unusual infections (such as fungi, tuberculosis, and other atypical organisms), and sarcoidosis, as was the case here.

Summary and Conclusion

In summary, when you have nodular masslike enhancement centered in the skull base or orbits, there is a pretty limited differential diagnosis. The inflammatory differential includes sarcoidosis and orbital inflammatory disease. Other things such as metastatic disease and atypical infections are also considerations.

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

Encephalitis

This is the second 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 second lecture covers causes of encephalitis, including inflammatory and autoimmune etiology. This section also covers infectious encephalitis, which is the most common mimic.

Limbic encephalitis

Limbic encephalitis is an immune mediated encephalitis often associated with neoplasm and a variety of circulating antibodies. It most commonly is manifested as bilateral (asymmetric or symmetric) temporal lobe FLAIR/T2 abnormalities. Enhancement is variable but less common than seen in herpes encephalitis.

Chronic limbic encephalitis

Over time, chronic limbic encephalitis can result in temporal lobe sclerosis and atrophy. Volumetric analysis of the brain and temporal lobes may be useful in highlighting the differences over time.

Lupus encephalitis

Lupus is a systemic autoimmune condition which can be associated with intracranial findings, including encephalitis, vasculitis, and posterior reversible encephalopathy syndrome (PRES). Vessel imaging may be normal, as it tends to affect the small vessels of the brain.

Viral encephalitis

Viral encephalitis has a nonspecific imaging appearance with patchy white matter FLAIR/T2 abnormalities, often involving the temporal lobes. There are a number of causes of viral encephalitis, including herpes, west nile virus, St. Louis encephalitis, and others.

Herpes encephalitis

Herpes encephalitis is the most dreaded of the encephalitis causes because it has high morbidity and mortality. Compared to nonspecific viral and inflammatory encephalitis, it tends to have more enhancement and more DWI abnormality. Patients suspected of having herpes encephalitis should be started empirically on treatment immediately to improve the prognosis.

Summary and Conclusion

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