Cerebrovascular Manifestations of Neurosarcoidosis: An Underrecognized Aspect of the Imaging Spectrum

Abstract

Involvement of the central nervous system by sarcoidosis, also referred to as neurosarcoidosis, is seen clinically in about 5% of patients with systemic disease. CNS involvement most frequently affects the leptomeninges and cranial nerves, though the ventricular system, brain parenchyma, and pachymeninges may also be involved. Even though the involvement of the intracranial vascular structures is well-known on postmortem studies, there is scant literature on imaging manifestations secondary to the vessel wall involvement, being confined mostly to isolated case reports and small series. The authors present a review of various cerebrovascular manifestations of neurosarcoidosis, along with a brief synopsis of the existing literature.

Engorgement of Deep Medullary Veins in Neurosarcoidosis: A Common-Yet-Underrecognized Cerebrovascular Finding on SWI

“…13 In this study, we found that DMVE is a common finding in neurosarcoidosis, though not as yet recognized as a distinct cerebrovascular manifestation. 5 It was significantly more common in men, consistent with cerebrovascular manifestations of sarcoidosis being more frequent in this group. 5 DMVE came to our attention owing to its conspicuity on SWI, a sequence that is now widely available and was originally developed as a means for MR venography.…”

“…5 It was significantly more common in men, consistent with cerebrovascular manifestations of sarcoidosis being more frequent in this group. 5 DMVE came to our attention owing to its conspicuity on SWI, a sequence that is now widely available and was originally developed as a means for MR venography. 14 A recent review showed 1 case with mild periatrial DMVE in a typical radial configuration on SWI; however, the presence of engorgement itself was not described.…”

“…14 A recent review showed 1 case with mild periatrial DMVE in a typical radial configuration on SWI; however, the presence of engorgement itself was not described. 5 The pathophysiology of engorgement is uncertain but does not appear to be directly caused by downstream venous occlusion because dural sinuses and deep cerebral veins were patent in almost all our patients. Although there was no evident cortical thrombosis, we did notice a relative paucity of cortical veins along the cerebral hemispheres in some patients from both groups, which raised the question of whether abnormalities in the cortical veins could explain the appearance of DMVE.…”

“…6 Intracranial hemorrhage is also associated with a poor prognosis and has been reported as a possible sequela of vascular disease. 5,7,8 We have noted that some patients with neurosarcoidosis show a characteristic pattern of deep medullary vein engorgement (DMVE) on SWI, which, to our knowledge, has not been previously described. Our purpose was to report the prevalence of this finding and to establish whether there may be an association with an increased frequency of intracranial hemorrhage, hydrocephalus, or worse neurologic outcomes.…”

“…2,3 Neurosarcoidosis has a predilection for perivascular dissemination but can also lead to parenchymal, leptomeningeal, or pachymeningeal disease and may result in a granulomatous angiitis. 4,5 CNS complications can be diverse and are a function of the nature and extent of tissues involved. Patients can have cranial neuropathies, neuroendocrine dysfunction due to involvement of the pituitary-hypothalamic axis, aseptic meningitis, and myelopathy, among other symptoms.…”