Völker Hans-Ullrich, Kraft Klaus, Arnold Eva, Steinhoff Silke, Kolios Georgios, Sommer Stephanie
Institute of Pathology, Military Hospital Ulm, Germany.
Clin Neurol Neurosurg. 2007 Sep;109(7):624-30. doi: 10.1016/j.clineuro.2007.05.009. Epub 2007 Jun 29.
Coincidence of pulmonal sarcoidosis and progressive multifocal leukoencephalopathy (PML) rarely occurs. So far an entire course has been recorded in only very few cases. We demonstrate the case of a 49-year-old male developing an infratentorial localized PML in the setting of advanced pulmonal sarcoidosis. PML was not included in the diagnostic considerations in the first instance. Regarding the diagnosis of pulmonal sarcoidosis proved by lung biopsy, the neurological impairment was first thought to be due to a neurosarcoidosis. But magnetic resonance tomography (MRI) clearly showed a demyelination process in the cerebellum. Because of the inconsistency of the radiological findings with a neurosarcoidosis the diagnosis of an acute disseminated encephalomyelitis (ADEM) was favoured. Therefore, the patient was initially treated with corticosteroids. Because of increasing deterioration further diagnostic testings were performed. In the cerebrospinal fluid (CSF) as well as in the paraffin-embedded tissue of a stereotactical brain biopsy JCV-DNA was successfully demonstrated by PCR. Cidofovir was administered. The progression of the disease could not be influenced. The patient died 5 months after the first neurological symptoms. This report stresses the diagnostic difficulties considering patients with sarcoidosis and neurological symptoms.
肺结节病与进行性多灶性白质脑病（PML）同时发生的情况极为罕见。迄今为止，仅有极少数病例记录了完整病程。我们报告一例49岁男性，在晚期肺结节病背景下发生幕下局限性PML。最初诊断时未考虑PML。鉴于肺活检证实为肺结节病，神经功能障碍最初被认为是神经结节病所致。但磁共振断层扫描（MRI）清楚显示小脑存在脱髓鞘过程。由于影像学表现与神经结节病不符，故倾向诊断为急性播散性脑脊髓炎（ADEM）。因此，患者最初接受了皮质类固醇治疗。由于病情不断恶化，遂进行了进一步诊断检查。通过聚合酶链反应（PCR）在脑脊液（CSF）以及立体定向脑活检的石蜡包埋组织中成功检测到JC病毒DNA（JCV-DNA）。给予了西多福韦治疗。但疾病进展未得到控制。患者在出现首次神经症状后5个月死亡。本报告强调了对于患有结节病且有神经症状患者进行诊断的困难。
