Yamagata Toru, Takami Toshihiro, Yamamoto Naoki, Tanaka Sayaka, Wakasa Kenichi, Ohata Kenji
Department of Neurosurgery, Osaka City University Graduate School of Medicine, Osaka, Osaka 545-8585, Japan.
Neurol Med Chir (Tokyo). 2013;53(4):245-8. doi: 10.2176/nmc.53.245.
A 28-year-old male presented with a rare case of primary intramedullary spinal Langerhans cell histiocytosis (LCH) manifesting as the chief complaint of a 6-month history of gait disturbance and back pain, and difficulty with sphincter control. Serial T2-weighted magnetic resonance imaging of the thoracic spine revealed enlargement and intramedullary hyperintensity of the spinal cord at T2 to T4. Biopsy of the lesion was performed. Histological examination of the biopsy specimens verified vascular proliferation and remarkable infiltration of histiocytes that were positive for CD1a, suggesting a diagnosis compatible with LCH. The patient was treated successfully by steroid pulse therapy. LCH is a rare disease that occurs mainly in children and may cause a broad range of manifestations, from a single osseous lesion to multiple lesions involving more than one organ or system. The present case illustrates the unexpected occurrence and important differential diagnosis of primary intramedullary spinal LCH of the thoracic spine in adult patients presenting with progressive paraparesis and back pain.
一名28岁男性，患有罕见的原发性脊髓内朗格汉斯细胞组织细胞增多症（LCH），主要表现为步态障碍和背痛6个月病史，伴有括约肌控制困难。胸椎系列T2加权磁共振成像显示T2至T4脊髓增粗和髓内高信号。对病变进行了活检。活检标本的组织学检查证实有血管增生和CD1a阳性的组织细胞显著浸润，提示诊断符合LCH。该患者通过类固醇脉冲疗法成功治愈。LCH是一种罕见疾病，主要发生在儿童中，可引起广泛的表现，从单个骨病变到涉及多个器官或系统的多个病变。本病例说明了成年患者出现进行性截瘫和背痛时，胸椎原发性脊髓内LCH的意外发生及重要鉴别诊断。
