Department of Neurology, University of Texas Southwestern, Austin, TX, USA.
JAMA Neurol. 2013 Jan;70(1):120-4. doi: 10.1001/2013.jamaneurol.469.
A 62-year-old man with no significant medical history experienced fatigue, night sweats, hoarseness of voice, and dry cough, which were followed by vision disturbances in his left eye. He lost about 4.5 kg (10 lb) in just over a month. Three weeks later, he had difficulty recollecting his e-mail password and trouble with word finding. The next day, he experienced numbness in his left arm. He also developed a maculopapular and erythematous rash in the groin, genitalia, and buttocks. The results of an initial neurological examination were normal, including his higher mental functions. An initial blood workup revealed normocytic normochromic anemia. The results of cerebrospinal fluid studies were unremarkable. Magnetic resonance imaging of his brain at hospital admission revealed multifocal increased T2 signals in the subcortical white matter. A conventional cerebral angiogram was unremarkable. A biopsy specimen from the right frontal lobe revealed demyelination and perivascular lymphocytic infiltration. A provisional diagnosis of acute disseminated encephalomyelitis was made. In spite of steroid treatment and plasmapheresis, his clinical status deteriorated rapidly. The approach to the diagnosis of a rapidly progressive multifocal brain disorder is discussed.
一位 62 岁的男性,既往无重大病史,出现疲劳、夜间盗汗、声音嘶哑和干咳,随后左眼视力障碍。在短短一个多月的时间里,他体重下降了约 4.5 公斤(10 磅)。三周后,他开始难以回忆自己的电子邮件密码,并且出现了找词困难。第二天,他感到左臂麻木。他还在腹股沟、生殖器和臀部出现了斑丘疹和红斑皮疹。最初的神经学检查结果正常,包括他的高级精神功能。初步血液检查显示正细胞正色素性贫血。脑脊液研究结果无明显异常。入院时的脑部磁共振成像显示皮质下白质内多发 T2 信号增强。常规脑血管造影无明显异常。右额叶活检显示脱髓鞘和血管周围淋巴细胞浸润。初步诊断为急性播散性脑脊髓炎。尽管接受了类固醇治疗和血浆置换,但他的临床状况迅速恶化。讨论了快速进展性多灶性脑疾病的诊断方法。