Tyrakowska Zuzanna, Jakubowicz-Lachowska Dominika, Kułakowska Alina, Galińska-Skok Beata, Drozdowski Wiesław, Tarasów Eugeniusz
Department of Radiology, Medical University of Białystok, Białystok, Poland.
Department of Neurology, Medical University of Białystok, Białystok, Poland.
Pol J Radiol. 2016 Dec 28;81:622-628. doi: 10.12659/PJR.898647. eCollection 2016.
Bickerstaff's brainstem encephalitis (BBE) is a very rare disease of the central nervous system. Aetiology of the disease is auto-immunological. However, it is not entirely understood. Clinically BBE manifests in progressive ophthalmoplegia, ataxia and consciousness disturbances. Clinical symptoms are usually preceded by an unidentified infection of the upper respiratory tract. Usually, the disease has one phase, but individual relapses have also been described. Despite quite severe clinical symptoms, the prognosis is usually good.
The article presents a case of a patient with relapsing-remitting severe BBE. The case is presented due to the relapsing-remitting clinical course of the disease that resulted in patient's death, rarely described in the literature. We also present the results of subsequent MR scans in the course of the disease, so far described only in individual reports. It is also the first report in the world's literature presenting the results of series of MR spectroscopy (MRS) examinations in the course of BBE.
MR examination is an important component in BBE diagnostics, allowing to differentiate atypical cases and place them under special supervision due to the possibility of the severe clinical course. MR also facilitates differentiation between Miller-Fisher Syndrome (MFS) and BBE in cases of diagnostic doubts. Adding MRS and MRI to the protocol allows us to define the nature of morphological changes more accurately in patients with suspected or diagnosed BBE.
比克斯特费尔德脑干脑炎（BBE）是一种非常罕见的中枢神经系统疾病。该疾病的病因是自身免疫性的。然而，其病因尚未完全明确。临床上，BBE表现为进行性眼肌麻痹、共济失调和意识障碍。临床症状通常先于未明确的上呼吸道感染出现。通常，该病有一个阶段，但也有个别复发的报道。尽管临床症状相当严重，但预后通常良好。
本文介绍了一例复发缓解型严重BBE患者的病例。之所以介绍该病例，是因为该病的复发缓解型临床病程导致患者死亡，这在文献中很少有描述。我们还展示了疾病过程中后续磁共振成像（MR）扫描的结果，迄今为止这些结果仅在个别报告中有描述。这也是世界文献中第一篇展示BBE病程中一系列磁共振波谱（MRS）检查结果的报告。
MR检查是BBE诊断的重要组成部分，由于存在严重临床病程的可能性，它能够鉴别非典型病例并对其进行特殊监测。在诊断存疑的情况下，MR还有助于区分米勒-费希尔综合征（MFS）和BBE。在检查方案中增加MRS和MRI能够让我们更准确地确定疑似或确诊BBE患者形态学改变的性质。
