背景:急性播散性脑脊髓炎(ADEM)是一种罕见的,获得性脱髓鞘综合征,导致认知障碍和局灶性神经功能缺损,可能是致命的。潜在的可逆性疾病主要影响儿童,通常在接种疫苗或病毒感染后,但在成年人中很少见。
方法:一名50岁女性出现左眼视力丧失。磁共振成像(MRI)显示鞍内和鞍上肿块,已成功删除。术后第1天,MRI显示病灶完全切除,无手术相关并发症。术后第2天,患者出现进行性左侧偏瘫,半封闭,认知能力下降。MRI显示两个半球白质水肿。脑脊液分析显示混合细胞增多症(355/µL),没有进一步的感染迹象。在调查结果的概要中,ADEM被诊断并用静脉免疫球蛋白治疗。此后不久,病人康复了,在随访检查中未发现感觉运动缺陷。
结论:垂体病理通常通过经蝶入路手术治疗,只有轻微的并发症风险。颅咽管瘤切除术后的ADEM病例以前尚未发表,应在进行性神经系统恶化伴多个白质病变的情况下考虑。
BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is a rare, acquired demyelination syndrome that causes cognitive impairment and focal neurological deficits and may be fatal. The potentially reversible disease mainly affects children, often after vaccination or viral infection, but may be seen rarely in adults.
METHODS: A 50-year-old woman presented with loss of visual acuity of the left eye. Magnetic resonance imaging (MRI) revealed an intra- and suprasellar mass, which was removed successfully. On postoperative day 1, MRI showed gross total resection of the lesion and no surgery-related complications. On postoperative day 2, the patient presented with a progressive left-sided hemiparesis, hemineglect, and decline of cognitive performance. MRI showed white matter edema in both hemispheres. Cerebrospinal fluid analysis revealed mixed pleocytosis (355/µL) without further evidence of infection. In synopsis of the findings, ADEM was diagnosed and treated with intravenous immunoglobulins. Shortly thereafter, the patient recovered, and no sensorimotor deficits were detected in the follow-up examination.
CONCLUSIONS: Pituitary gland pathologies are commonly treated by transsphenoidal surgery, with only minor risks for complications. A case of ADEM after craniopharyngioma resection has not been published before and should be considered in case of progressive neurological deterioration with multiple white matter lesions.