Conomy J P, Michel B, Ferguson J, Graham R, Kellermeyer R
Neurol Neurocir Psiquiatr. 1977;18(2-3 Suppl):421-6.
Involvement of the brain, spinal cord, peripheral nerves, meninges, and cerebrospinal pathways in mycosis fungoides manifests itself, clinically, as an obscure neurologic disturbance, occurring in patients with cutaneous manifestations of that disorder. Neurologic expressions of this particular cutaneous lymphoma are infrequently reported, in spite of the fact that many patients dying in the course of mycosis fungoides have pathologically demonstrable central nervous system, peripheral nervous system, and meningeal involvement. We report here a patient who incurred devastating neurologic illness as one of the initial features of the course of systemic dissemination of mycosis fungoides. Death occurred nine years after the onset of cutaneous illness. In this instance, cerebrospinal fluid cytologic studies established the diagnosis of nervous system involvement. Mycosis fungoides cells shed into the cerebrospinal fluid were studied by special techniques of light and electron microscopy. Brain scan abnormalities were present, which were clinically correlative with foci of brain involvement. The initial response of patient's nervous system illness to therapy was gratifying but unsustained, and she went on to fatal brain and multiple organ involvement with disseminated mycosis fungoides. The techniques of spinal fluid cytologic diagnosis, confirmatory of the etiology of disease in this case, may have practical appications in the diagnosis of otherwise obscure neurologic disorders.
蕈样肉芽肿累及脑、脊髓、周围神经、脑膜和脑脊液通路，在临床上表现为一种隐匿的神经功能障碍，发生于患有该疾病皮肤表现的患者中。尽管许多死于蕈样肉芽肿病程的患者在病理上可证实有中枢神经系统、周围神经系统和脑膜受累，但这种特殊皮肤淋巴瘤的神经表现却鲜有报道。我们在此报告一名患者，其在蕈样肉芽肿全身播散病程的初始阶段出现了严重的神经疾病。皮肤疾病发病九年后患者死亡。在这个病例中，脑脊液细胞学研究确立了神经系统受累的诊断。通过光镜和电镜的特殊技术对脱落到脑脊液中的蕈样肉芽肿细胞进行了研究。脑扫描显示有异常，在临床上与脑受累灶相关。患者神经系统疾病对治疗的初始反应令人满意，但未能持续，随后她因播散性蕈样肉芽肿导致脑和多器官受累而死亡。脑脊液细胞学诊断技术在本病例中证实了疾病的病因，可能在诊断其他隐匿性神经疾病方面有实际应用价值。
