Relevance of Medullary Vein Sign in Neurosarcoidosis

Liberio, Richard N.; Kramer, Emily; Memon, Anza B; Reinbeau, Ryan; Feizi, Parissa; Joseph, Joe; Wu, Janet P.; Sriwastava, Shitiz

doi:10.3390/neurolint14030052

Cited by 3 publications

(6 citation statements)

References 25 publications

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“…Spinal involvement was observed in 23% of our sample, mostly as leptomeningeal enhancement 24 , 25 . Notably, the deep medullary vein sign on MRI was absent in all our patients with histological features of neurosarcoidosis (n = 14), despite a reported sensitivity of 71.4% and a specificity of 92.3% for the diagnosis of neurosarcoidosis 26 , 27 .…”

Section: Discussionmentioning

confidence: 60%

A comprehensive diagnostic approach in suspected neurosarcoidosis

Berntsson

Elmgren

Gudjonsson

et al. 2023

Sci Rep

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Neurosarcoidosis presents a diagnostic challenge in clinical settings, as it has no pathognomonic symptoms or signs and a wide range of differential diagnoses. The aim of this report is to present the pathological features of our group of patients, obtained through a systematic diagnostic approach. This retrospective cohort study enrolled all adult patients primarily diagnosed with neurosarcoidosis at the neurology department of a tertiary center in Sweden over a period of 30 years, from 1990 to 2021. We identified 90 patients, 54 with possible neurosarcoidosis and 36 with probable neurosarcoidosis. CNS biopsy revealed an alternative diagnosis for 24 patients, who were then excluded. The collected data from medical records included demographic and clinical characteristics, systemic and/or neurological isolated involvement, various laboratory tests, including cerebrospinal fluid (CSF), serum analysis, imaging studies (MRI, FDG-PET/CT, and HRCT), nerve conduction studies, electromyography, and pathology reports of central nervous system (CNS), and extra-neural tissue biopsies. Sixty-six patients were included in our cohort. The median age at onset of symptoms was 49 years, with a similar sex distribution. Cranial neuropathies (38%), motor deficit (32%), headache (16%), and pituitary dysfunction (12%) were the most common presenting features. CSF studies were abnormal in 77% of the patients, who showed lymphocytosis (57%), elevated protein (44%), oligoclonal bands (40%), elevated ACE (28%), and raised T lymphocyte CD4+/CD8+ ratios (13%). Strikingly, MRI showed that 17% of the patients presented with isolated pituitary gland lesions. FDG-PET/CT was performed in 22 patients (33%) and confirmed systemic sarcoidosis in 11. Despite our extensive workup, the final classification for our patients only allowed for a definite diagnosis in 14 patients; the remainder were classified as probable (32) or possible (20) neurosarcoidosis. Since 2007, the employment of a structured laboratory and imaging approach and the increasing number of CNS biopsies have facilitated and improved the process of correct attribution in patients with presumptive neurosarcoidosis, especially in patients with isolated neurological lesions. We highlight a higher frequency of pituitary lesions due to neurosarcoidosis than has been classically described. A detailed laboratory diagnostic workup is included.

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Section: Discussionmentioning

confidence: 60%

A comprehensive diagnostic approach in suspected neurosarcoidosis

Berntsson

Elmgren

Gudjonsson

et al. 2023

Sci Rep

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“…Most importantly, in the Liebermeister voxelwise analysis, lesion patterns of subcortical lesions did not differ between neurosarcoidosis and multiple sclerosis patients. Therefore, although more specific imaging parameters in neurosarcoidosis would be helpful [ 17 ], it does not seem possible to draw any conclusions out of the distribution of parenchymal, subcortical lesions. Because subcortical white matter lesions are common in patients without sarcoidosis as well, it is not clear that the lesions are always related to the sarcoidosis [ 15 ].…”

Section: Discussionmentioning

confidence: 99%

“…The deep medullary vein sign, an engorgement of the deep medullary veins in brain imaging, has been proposed as a specific sign for neurosarcoidosis, However, its sensitivity is rather low (about 70%) and its role is still under debate. [ 17 ]. It is worth to note that there is the “trident sign” in cases with spinal involvement that has a significant diagnostic yield to distinguish neurosarcoidosis from other causes of myelitis [ 28 ].…”

Section: Discussionmentioning

confidence: 99%

“…To our knowledge, previous imaging studies in neurosarcoidosis only comprised descriptive case series [ 7 , 10 , 13 , 14 , 17 – 20 ]. Moreover, statistical imaging analysis like the voxel-wise lesion symptom mapping (VLSM) allows investigating voxel-by-voxel associations between cerebral lesion location and an outcome without having any a priori hypothesis.…”

Section: Introductionmentioning

confidence: 99%

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Cerebral lesions sites in neurosarcoidosis: a lesion mapping study

et al. 2023

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Background and purpose Sarcoidosis is a granulomatous disease of unknown etiology affecting the central nervous system in up to 15% of the patients. Diagnosis of neurosarcoidosis is very challenging due to the heterogeneity of its clinical manifestation. This study intended to evaluate the distribution of cerebral lesion sites and the potential presence of specific lesion clusters in neurosarcoidosis patients using voxel-based lesion symptom mapping (VLSM). Methods Patients with neurosarcoidosis were retrospectively identified and included between 2011 and 2022. Cerebral lesion sites were correlated voxel-wise with presence and absence of neurosarcoidosis using non-parametric permutation test. Multiple sclerosis patients served as controls for the VLSM-analysis. Results Thirty-four patients (mean age 52 ± 15 years) of whom 13 were diagnosed with possible, 19 with probable and 2 with confirmed neurosarcoidosis were identified. Lesion overlap of neurosarcoidosis patients demonstrated a distribution of white matter lesions in all brain areas, with a periventricular predilection similar to multiple sclerosis. In contrast to multiple sclerosis controls, no propensity for lesions in proximity of the corpus callosum was observed. Neurosarcoidosis lesions appeared smaller and lesion volume was lower in the neurosarcoidosis cohort. The VLSM analysis showed minor associations between neurosarcoidosis and damaged voxels in the bilateral frontobasal cortex. Conclusions The VLSM analysis yielded significant associations in the bilateral frontal cortex, suggesting that leptomeningeal inflammatory disease with following cortical involvement is a quite specific feature in neurosarcoidosis. Lesion load was lower in neurosarcoidosis than in multiple sclerosis. However, no specific pattern of subcortical white matter lesions in neurosarcoidosis was revealed.

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“…Imaging findings are frequently nonspecific, making diagnosis challenging, especially in the absence of systemic sarcoidosis. Concurrent brain involvement can be seen in up to 70% of cases [ 32 , 33 ].…”

Section: Imaging Findingsmentioning

confidence: 99%

Insights into neurosarcoidosis: an imaging perspective

Cicilet,

Reddy K,

Kancharla

2023

Pol J Radiol

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Neurosarcoidosis is a complex and multifaceted inflammatory disorder affecting the nervous system. The disease, characterized by non-caseating granulomas, primarily involves the central and peripheral nervous systems. Neurological manifestations vary widely and can include cranial nerve palsies, meningeal involvement, parenchymal lesions, hydrocephalus, and more. Diagnosis remains challenging due to the lack of specific imaging features, necessitating a combination of clinical evaluation, cerebrospinal fluid analysis, imaging studies, and sometimes tissue biopsy. This review article underscores the importance of the identification of various imaging features to mitigate the mortality and morbidity associated with neurosarcoidosis.

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Relevance of Medullary Vein Sign in Neurosarcoidosis

Cited by 3 publications

References 25 publications

A comprehensive diagnostic approach in suspected neurosarcoidosis

A comprehensive diagnostic approach in suspected neurosarcoidosis

Cerebral lesions sites in neurosarcoidosis: a lesion mapping study

Insights into neurosarcoidosis: an imaging perspective

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