Pizzuti P, Pertuiset E, Chaumonnot F, Chesneau A, Mikol J, Leblond-Missenard V, Fermand J P
Département de médecine interne, hôpital René-Dubos, Pontoise, France.
Rev Med Interne. 1997;18(8):646-51. doi: 10.1016/s0248-8663(97)82467-8.
Neurologic manifestations are not unusual in multiple myeloma. Conversely meningeal and cerebral involvement have been very rarely reported. We report here on three patients with multiple myeloma and meningeal or cerebral involvement (two of them with autopsy study): one case of cerebellar involvement associated with secondary plasma cell leukemia and two cases of meningeal involvement. We reviewed the characteristics of 20 cases of meningeal involvement with demonstration of plasma cells at cerebrospinal fluid analysis (18 previously reported cases and our two patients). Meningeal involvement occurs in patients with initially stage III multiple myeloma in 85% of cases and is associated with the occurrence of plasma cell leukemia in 20% of cases. The most frequent neurologic signs are: confusion (60%), altered consciousness (25%), gait disorder (25%), cranial nerve palsy (25%). Meningismus is rarely present. Diagnosis is based on cerebrospinal fluid analysis after lumbar puncture which should be made after cranial magnetic resonance imaging. The diagnosis of intra-cranial haemorrhage and infectious meningitis have to be cautiously ruled out. Despite treatments (systemic and/or intrathecal chemotherapy, radiation therapy), prognosis is very poor: mean time of survival after the occurrence of neurologic signs is about 2 months.
神经系统表现在多发性骨髓瘤中并不罕见。相反,脑膜和脑受累的报道却极为罕见。我们在此报告3例伴有脑膜或脑受累的多发性骨髓瘤患者(其中2例进行了尸检研究):1例小脑受累伴继发性浆细胞白血病,2例脑膜受累。我们回顾了20例经脑脊液分析证实有浆细胞的脑膜受累病例的特征(18例先前报道的病例及我们的2例患者)。脑膜受累在初诊为III期多发性骨髓瘤的患者中占85%,在20% 的病例中与浆细胞白血病的发生有关。最常见的神经系统体征为:意识模糊(60%)、意识改变(25%)、步态障碍(25%)、颅神经麻痹(25%)。很少出现颈项强直。诊断基于腰椎穿刺后的脑脊液分析,腰椎穿刺应在头颅磁共振成像后进行。必须谨慎排除颅内出血和感染性脑膜炎的诊断。尽管进行了治疗(全身和/或鞘内化疗、放射治疗),但预后很差:出现神经系统体征后的平均生存时间约为2个月。