Lin C-H, Jeng J-S, Hsieh S-T, Yip P-K, Wu R-M
Department of Neurology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan.
J Neurol Neurosurg Psychiatry. 2007 Feb;78(2):162-7. doi: 10.1136/jnnp.2005.084194. Epub 2006 Oct 6.
Acute-disseminated encephalomyelitis (ADEM) is a demyelinating disorder of the central nervous system, whose epidemiology, clinical presentations and functional outcome are incompletely understood in Asian populations.
To assess the clinical presentations, predisposing factors and functional outcome of ADEM in Taiwan.
50 patients initially diagnosed with ADEM (male, 19; female, 31) were enrolled from 1991 to 2005. Diagnosis of ADEM or multiple sclerosis was established during a follow-up period of 2-120 months. 8 adult patients were noted to have taken the immunomodulatory drug, levamisole, within 3 months before onset of symptoms. The remaining 42 patients (male, 17; female, 25) were categorised by age as children (<16 years, n = 12), young adults (16-49 years, n = 21) and elderly adults (> or =50 years, n = 9). The clinical manifestations, predisposing factors and radiological findings were compared between different age groups and adult patients with or without levamisole use. Functional outcome was compared by a log-rank test.
Preceding upper respiratory tract infection was evident in 21 (50%) patients and only one young-adult patient had received Rubella vaccine immunisation. The frequency of fever was higher in children (p = 0.04) and psychiatric symptoms were more prevalent in elderly patients (p = 0.03). Functional recovery was faster in children than in adults (p = 0.002). Initial Expanded Disability Status Scale score (odds ratio (OR) 1.9, p = 0.03) and no fever (OR 0.04, p = 0.06) were associated with poor outcome (modified Rankin scale > or =2). After a mean (SD) follow-up of 31.8 (9.9) months, 4 (9.5%) patients developed multiple sclerosis (3 (25%) children, 1 (4.7%) young adult, p = 0.03). The neurological disability, radiological and cerebrospinal fluid findings did not differ between patients with and without levamisole use. One elderly adult patient previously receiving levamisole developed multiple sclerosis of relapse-remitting type after a mean follow-up period of 36.9 months.
The clinical presentations, functional outcome and risk of developing multiple sclerosis differed between different age groups. Functional recovery was faster in children than in adults. Poor functional outcome was related to initial high Expanded Disability Status Scale score and absence of fever.
急性播散性脑脊髓炎(ADEM)是一种中枢神经系统脱髓鞘疾病,在亚洲人群中,其流行病学、临床表现及功能转归尚未完全明确。
评估台湾地区ADEM的临床表现、诱发因素及功能转归。
选取1991年至2005年期间最初诊断为ADEM的50例患者(男19例,女31例)。在2至120个月的随访期内确诊为ADEM或多发性硬化症。8例成年患者在症状发作前3个月内服用过免疫调节药物左旋咪唑。其余42例患者(男17例,女25例)按年龄分为儿童(<16岁,n = 12)、青年成人(16 - 49岁,n = 21)和老年成人(≥50岁,n = 9)。比较不同年龄组以及使用和未使用左旋咪唑的成年患者的临床表现、诱发因素及影像学检查结果。采用对数秩检验比较功能转归。
21例(50%)患者发病前有上呼吸道感染,仅1例青年成人患者接种过风疹疫苗。儿童发热频率较高(p = 0.04),老年患者精神症状更为常见(p = 0.03)。儿童功能恢复较成人快(p = 0.002)。初始扩展残疾状态量表评分(优势比(OR)1.9,p = 0.03)及无发热(OR 0.04,p = 0.06)与预后不良(改良Rankin量表≥2)相关。平均(标准差)随访31.8(9.9)个月后,4例(9.5%)患者发展为多发性硬化症(3例(25%)儿童,1例(4.7%)青年成人,p = 0.03)。使用和未使用左旋咪唑的患者神经功能障碍、影像学及脑脊液检查结果无差异。1例曾服用左旋咪唑的老年成人患者在平均随访36.9个月后发展为复发缓解型多发性硬化症。
不同年龄组的临床表现、功能转归及发生多发性硬化症的风险存在差异。儿童功能恢复较成人快。功能预后不良与初始扩展残疾状态量表评分高及无发热有关。