Grisold W, Jellinger K, Lutz D
L. Boltzmann Institute of Clinical Neurobiology, Vienna, Austria.
Clin Neuropathol. 1990 Sep-Oct;9(5):224-30.
A 62-year-old woman with chronic lymphatic leukemia (CLL) (RAI stage IV) with multiple organ involvement and diabetes mellitus, three months prior to death presented with a symmetrical sensory neuropathy of the upper extremities with little motor impairment and, two months later, sensory atactic neuropathy of the lower limbs. No cranial nerve or CNS impairment was noted. Clinical diagnosis was predominantly sensory neuropathy, but nerve conduction velocities were normal on upper limbs and moderately abnormal on lower limbs, the latter attributing to long lasting diabetes mellitus. The women died from acute subarachnoid hemorrhage. Autopsy revealed CLL of B-cell type with generalized organ involvement and acute craniospinal subarachnoid hemorrhage from ruptured cerebral aneurysm. There was selective neoplastic infiltration of the dorsal root ganglia and peripheral nerves, particularly the median nerve. Although selective infiltration of peripheral nerves by B-cell lymphoma cells was not associated with myelo-axonal degeneration, the relationship of this case to human neurolymphomatosis is discussed.
一名62岁患有慢性淋巴细胞白血病(CLL)( Rai分期IV期)且有多器官受累及糖尿病的女性,在死亡前三个月出现了上肢对称性感觉神经病变,运动障碍轻微,两个月后出现下肢感觉共济失调性神经病变。未发现颅神经或中枢神经系统损害。临床诊断主要为感觉神经病变,但上肢神经传导速度正常,下肢中度异常,后者归因于长期糖尿病。该女性死于急性蛛网膜下腔出血。尸检显示为B细胞型CLL,伴有全身器官受累,以及脑动脉瘤破裂导致的急性颅脊髓蛛网膜下腔出血。背根神经节和周围神经,特别是正中神经有选择性肿瘤浸润。尽管B细胞淋巴瘤细胞对周围神经的选择性浸润与髓鞘-轴突变性无关,但仍对该病例与人类神经淋巴瘤病的关系进行了讨论。