Santos Ernestina, Scolding Neil J
Neurology Department, Hospital Geral Santo, António Porto, Portugal.
J Med Case Rep. 2010 Jan 12;4:5. doi: 10.1186/1752-1947-4-5.
Both neurosarcoidosis and central nervous system lymphoma can be very difficult to diagnose. We describe the case of a patient in whom neurosarcoidosis was strongly suspected, but who was eventually found to have lymphoma. We believe the case to be of interest and practical value to neurologists, oncologists and internists with an interest in inflammatory diseases.
A diagnosis of neurosarcoidosis was considered in a 49-year-old Caucasian man on the basis of the following symptoms and indications: a cough, bilateral hilar lymphadenopathy confirmed by thoracic computed tomography, the development of an S1 radiculopathy, cerebrospinal fluid abnormalities (raised protein level), bilateral lung hilar and lachrymal gland uptake on a gallium scan, and erythema nodosum confirmed with skin biopsy. These were followed by the development of multiple cranial neuropathies, including seventh nerve palsy. Exhaustive further investigations yielded no evidence for an alternative diagnosis. Treatments with steroids, cyclophosphamide, intravenous immunoglobulin and finally infliximab were of no benefit. He eventually developed cutaneous nodules, a biopsy of which revealed lymphoma that proved resistant to therapy.
Constant diagnostic vigilance is required in disorders such as neurosarcoidosis.
神经结节病和中枢神经系统淋巴瘤都可能很难诊断。我们描述了一例患者,最初强烈怀疑其患有神经结节病,但最终发现患有淋巴瘤。我们认为该病例对于关注炎症性疾病的神经科医生、肿瘤内科医生和内科医生具有一定的趣味性和实用价值。
一名49岁的白人男性因以下症状和体征被考虑诊断为神经结节病:咳嗽、胸部计算机断层扫描证实双侧肺门淋巴结肿大、出现S1神经根病、脑脊液异常(蛋白水平升高)、镓扫描显示双侧肺门和泪腺摄取增加、皮肤活检证实有结节性红斑。随后出现多种颅神经病变,包括第七神经麻痹。详尽的进一步检查未发现其他诊断依据。使用类固醇、环磷酰胺、静脉注射免疫球蛋白,最后使用英夫利昔单抗治疗均无效。他最终出现皮肤结节,活检显示为淋巴瘤,且对治疗耐药。
对于神经结节病等疾病,需要始终保持诊断警惕。