Vascular Involvement in Neurosarcoidosis

Abstract

Background and ObjectivesCerebrovascular manifestations in neurosarcoidosis (NS) were previously considered rare but are being increasingly recognized. We report our preliminary experience in patients with NS who underwent high-resolution vessel wall imaging (VWI).MethodsA total of 13 consecutive patients with NS underwent VWI. Images were analyzed by 2 neuroradiologists in consensus. The assessment included segment-wise evaluation of larger- and medium-sized vessels (internal carotid artery, M1-M3 middle cerebral artery; A1-A3 anterior cerebral artery; V4 segments of vertebral arteries; basilar artery; and P1-P3 posterior cerebral artery), lenticulostriate perforator vessels, and medullary and deep cerebral veins. Cortical veins were not assessed due to flow-related artifacts. Brain biopsy findings were available in 6 cases and were also reviewed.ResultsMean patient age was 54.9 years (33–71 years) with an M:F of 8:5. Mean duration between initial diagnosis and VWI study was 18 months. Overall, 9/13 (69%) patients had vascular abnormalities. Circumferential large vessel enhancement was seen in 3/13 (23%) patients, whereas perforator vessel involvement was seen in 6/13 (46%) patients. Medullary and deep vein involvement was also seen in 6/13 patients. In addition, 7/13 (54%) patients had microhemorrhages in susceptibility-weighted imaging, and 4/13 (31%) had chronic infarcts. On biopsy, 5/6 cases showed perivascular granulomas with vessel wall involvement in all 5 cases.DiscussionOur preliminary findings suggest that involvement of intracranial vascular structures may be a common finding in patients with NS and should be routinely looked for. These findings appear concordant with previously reported autopsy literature and need to be validated on a larger scale.

Relevance of Medullary Vein Sign in Neurosarcoidosis

“…Many patients with neurosarcoidosis have already initiated treatment for sarcoidosis before they present with neurologic symptoms, which renders MRI less sensitive, as the classic imaging findings of neurosarcoidosis may be resolving or resolved. SWI provides a unique utility for the efficient diagnosis of NS [ 1 , 5 , 22 ]. This study demonstrates that SWI is a useful tool for early diagnosis of neurosarcoidosis and expedited treatment.…”

“…Neurosarcoidosis affects 3–10% of individuals with sarcoidosis, and 75% of this subset will develop neurological symptoms within the first two years of being diagnosed with sarcoidosis. However, up to 80% of patients with neurosarcoidosis will present with neurological symptoms prior to the diagnosis of sarcoidosis [ 4 , 5 , 6 ]. Neurosarcoidosis can affect any part of the central nervous system; however, the most common presentation is cranial neuropathy.…”

“…The most common imaging finding in neurosarcoidosis is multiple non-enhancing periventricular white matter lesions. The most specific is the “trident sign,” in the dorsal cord region, which is thought to relate to perivascular spread of leptomeningeal granulomatous inflammation [ 5 , 8 , 9 ]. Cerebrospinal fluid (CSF) analysis is also non-specific, although elevated levels of angiotensin converting enzyme (ACE) in the CSF is common in neurosarcoidosis.…”

Insights into neurosarcoidosis: an imaging perspective

“…Concomitant involvement of caudate veins is also seen in these patients. When pre-sent, dural sinus thrombosis can also cause engorgement of medullary veins [ 30 ]. The incidence of DMVE ranges from 33 to 38% [ 30 , 31 ].…”

Neurosarcoidosis, manifestaciones clinicas, abordaje diagnóstico y opciones terapéuticas

“…Los pacientes con neurosarcoidosis suelen tener una buena respuesta a los glucocorticoides en dosis altas. Sin embargo, algunos pacientes no toleran los efectos adversos asociados con tratamiento inicial de dosis alta o ameritan tratamiento prolongado con dosis bajas, por lo que se utilizan como tratamiento de segunda línea el metrotexate, la ciclofosfamida, micofenolato de mofetilo, la azatioprina y la talidomida, cada uno con estudios variables que demuestran la eficacia en cuanto a uso sobre esta entidad patológica (16). En muchos casos se utiliza tratamiento combinado con glucocorticoides, pero es aún un tratamiento controvertido por los numerosos efectos secundarios (6).…”