纵向广泛性横贯性脊髓炎,作为伴有硬结病的神经肉瘤

IF 0.4 Q4 CLINICAL NEUROLOGY Neurology and Clinical Neuroscience Pub Date : 2023-02-19 DOI:10.1111/ncn3.12705
Yasuyuki Takai, Y. Warabi, Y. Sunami, Natsuki Miyakoshi, Kazushi Takahashi
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引用次数: 0

摘要

一名61岁男性,30年前发生摩托车事故,双侧上肢慢慢瘫痪,亚急性痉挛性截瘫,胸下段麻木,膀胱-肠功能障碍。脊柱磁共振成像(MRI)显示,C2至L2椎骨水平的髓内病变伴有软脑膜钆(Gd)增强,腹侧椎管内积液伴有含铁血黄素沉积(图1A-H)。大脑MRI显示小脑和脑干周围的Gd增强和含铁血黄素沉积(图1K-N)。实验室测试显示没有胶原疾病、感染和恶性肿瘤的证据,抗quapolin4抗体和抗髓鞘少突胶质细胞糖蛋白抗体的结果为阴性。尽管病理结果尚未得到证实,但根据可溶性白细胞介素2受体和溶菌酶(正常血管紧张素转换酶水平)升高、CSF中有黄色色素沉着的淋巴细胞、镓闪烁扫描阳性的肝门淋巴结肿大,怀疑伴有硬结病的神经肉瘤(图1I,J),1皮质类固醇治疗有效,甲氨蝶呤维持缓解,但上肢瘫痪没有改善。该病例表现为纵向广泛性横贯性脊髓炎。我们已经报道了神经肉瘤有无症状的脑微出血,2但相反,外源性出血刺激含铁血黄素,如浅表含铁血,3可能导致更严重的神经肉瘤。
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Longitudinally extensive transverse myelitis as neurosarcoidosis accompanying duropathy
A 61year old male, who had had a motorbike accident 30 years ago and slowly progressed paralysis of bilateral upper extremities, subacutely developed spastic paraplegia, numbness under inferior thoracic level, and bladderintestinal dysfunction. Spinal magnetic resonance imaging (MRI) revealed intramedullary lesions from the C2 to the L2 vertebral level with leptomeningeal gadorium (Gd)enhancement, and ventral intraspinal fluid collection with hemosiderin deposition (Figure 1A– H). Brain MRI revealed Gdenhancement and hemosiderin deposition around the cerebellum and brainstem (Figure 1K– N). Laboratory tests showed no evidence of collagen disease, infection, and malignant tumor, and negative results for antiaquapolin4 antibody and antimyelin oligodendrocyte glycoprotein antibody. Although the pathological findings were not confirmed, neurosarcoidosis accompanying duropathy was suspected on the basis of elevated soluble interleukin2 receptor and lysozyme (normal angiotensinconverting enzyme level), lymphocytes with xanthochromia in CSF, enlarged hilar lymph nodes with positive gallium scintigraphy (Figure 1I, J), and elevated CD4/CD8 ratio in bronchoalveolar lavage.1 Corticosteroid treatment was effective, and remission was maintained with methotrexate, but the upper extremity paralysis did not improve. This case presented longitudinally extensive transverse myelitis. We have reported that neurosarcoidosis had asymptomatic cerebral microbleeds,2 but conversely, stimulation of hemosiderin by exogenous hemorrhages, such as superficial siderosis,3 may lead to more severe neurosarcoidosis.
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76
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