Yamamoto T, Imai T, Yamasaki M
Department of Neurology, Kitano Hospital, Osaka, Japan.
J Neurol Sci. 1989 Feb;89(2-3):313-24. doi: 10.1016/0022-510x(89)90032-4.
Life-threatening acute ventilatory failure has been considered to be a very rare manifestation of multiple sclerosis (MS) and only a few cases have been investigated clinicopathologically. We examined 4 patients with MS who developed acute ventilatory failure, accompanied by a constellation of neurological symptoms and signs that were consistent with midline ventral medullary lesions. All but one of them survived the episode with artificial ventilation over a period of 9-11 days. One of the remaining 3 patients died a year later from subsequently developed anoxic encephalopathy. Neuropathological examination of the 2 deceased cases revealed the suspected lesions in the medulla as well as widespread chronic and active MS foci, which were characterized by largely necrotic changes rather than by pure demyelination. MRI in another case revealed a lesion in the caudal medulla. The apparently not so rare occurrence of ventilatory failure among Japanese MS patients might be related to the known differences in pathological features between Japanese and Western MS patients. Since there is a good chance of recovery, early recognition of the symptom complex appears important in the assessment and treatment of such patients.
危及生命的急性呼吸衰竭被认为是多发性硬化症(MS)非常罕见的一种表现,仅有少数病例进行过临床病理研究。我们检查了4例出现急性呼吸衰竭的MS患者,他们伴有一系列与延髓腹侧中线病变相符的神经症状和体征。除1例患者外,其余患者均在人工通气9至11天的期间内度过了这一阶段。其余3例患者中有1例在一年后死于随后发生的缺氧性脑病。对2例死亡病例进行的神经病理学检查发现,延髓存在疑似病变以及广泛的慢性和活动性MS病灶,这些病灶的特征主要是坏死性改变而非单纯的脱髓鞘。另一例患者的MRI显示延髓尾部有病变。日本MS患者中呼吸衰竭的发生率显然并非如此罕见,这可能与已知的日本和西方MS患者病理特征差异有关。由于恢复的可能性很大,因此早期识别这种症状复合体对于此类患者的评估和治疗似乎很重要。