Masson Catherine, Colombani J M
Service de neurologie, Hôpital Beaujon, 100, bd du Général Leclerc, 92110 Clichy.
Presse Med. 2002 Nov 23;31(37 Pt 1):1739-45.
The relationship between acute disseminated encephalomyelitis (ADEM), a monophasic disease, and multiple sclerosis (MS), a potentially progressive disease with periods of recurrence or remission, remains debatable. In a large number of patients, the diagnosis initially retained is that of ADEM, whilst the progression is subsequently towards a defined MS. This has incited some authors to consider that there is a continuum between these two diseases and to underline the frailty of the criteria retained for the diagnosis of ADEM. Further interest has presently been paid to this subject and the question of whether a basic treatment of MS should be started after the first episode has been raised, although such a treatment is obviously not justified if the diagnosis retained is an ADEM.
Two cases observed in adults, followed-up for 6 years, contribute to the validation of a number of clinical, biological and radiological arguments which are all in favor of the diagnosis of ADEM versus that of an initial episode of MS.
These are the absence of any neurological past history, the existence of a multi-focal neurological symptomatology right from the start and, particularly in children, the presence of a meningeal syndrome.
Presence in the cephalo-spinal liquid, of an increase in cells and cervicospinal proteins to levels not usually found in MS.
MRI reveals multi-focal, extensive and bilateral lesions, beneath the cortex rather than peri-ventricular, which may extensively involve the thalamus and the bone marrow; these lesions are of the same age and often regress.
单相疾病急性播散性脑脊髓炎(ADEM)与具有复发或缓解期的潜在进展性疾病多发性硬化症(MS)之间的关系仍存在争议。在大量患者中,最初诊断为ADEM,但随后病情进展为明确的MS。这促使一些作者认为这两种疾病之间存在连续性,并强调用于诊断ADEM的标准的脆弱性。目前人们对这个问题给予了更多关注,并且提出了在首次发作后是否应开始MS基础治疗的问题,尽管如果诊断为ADEM,这种治疗显然是不合理的。
观察了两例成年患者,随访6年,有助于验证一些临床、生物学和放射学依据,这些依据均支持诊断为ADEM而非MS的初始发作。
无任何既往神经病史,从一开始就存在多灶性神经症状,特别是在儿童中,存在脑膜综合征。
脑脊液中细胞和颈脊髓蛋白增加至MS通常未发现的水平。
磁共振成像(MRI)显示皮质下而非脑室周围的多灶性、广泛性和双侧性病变,这些病变可能广泛累及丘脑和骨髓;这些病变年龄相同且常消退。