Department of Neurology, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nürnberg (FAU), Schwabachanlage 6, 91054, Erlangen, Germany.
Department of Neuroradiology, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nürnberg (FAU), Schwabachanlage 6, 91054, Erlangen, Germany.
J Neurol. 2023 Nov;270(11):5392-5397. doi: 10.1007/s00415-023-11863-3. Epub 2023 Jul 12.
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).
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.
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.
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.
结节病是一种病因不明的肉芽肿性疾病，约 15%的患者会累及中枢神经系统。由于其临床表现的异质性，神经结节病的诊断极具挑战性。本研究旨在通过基于体素的病变症状映射（VLSM）评估神经结节病患者脑病变部位的分布情况以及潜在的特定病变簇的存在。
回顾性地确定了 2011 年至 2022 年间的神经结节病患者，并进行了纳入。使用非参数置换检验，将脑病变部位与神经结节病的存在与否进行了逐体素相关分析。多发性硬化症患者被用作 VLSM 分析的对照。
共确定了 34 例患者（平均年龄 52±15 岁），其中 13 例被诊断为可能的神经结节病，19 例为可能的神经结节病，2 例为确诊的神经结节病。神经结节病患者的病变重叠显示出所有脑区的白质病变分布，具有与多发性硬化症相似的脑室周围倾向。与多发性硬化症对照组相比，没有观察到胼胝体附近病变的倾向。神经结节病患者的病变较小，病变体积也较低。VLSM 分析显示，神经结节病与双侧额基底皮质受损的体素之间存在轻微的关联。
VLSM 分析在双侧额叶皮层产生了显著的关联，这表明脑膜炎症性疾病伴随后续的皮质受累是神经结节病的一个相当特异的特征。神经结节病的病变负荷低于多发性硬化症。然而，神经结节病并未显示出特定的皮质下白质病变模式。
