Baloh R W, DeRossett S E, Cloughesy T F, Kuncl R W, Miller N R, Merrill J, Posner J B
Department of Neurology, UCLA Medical Center.
Neurology. 1993 Dec;43(12):2591-6. doi: 10.1212/wnl.43.12.2591.
Two patients successfully treated for prostatic cancer developed a progressive neurologic syndrome beginning with loss of voluntary horizontal eye movements followed by severe, persistent muscle spasms of the face, jaw, and pharynx. Both had mild gait unsteadiness, and one exhibited facial and abdominal myoclonus. Extensive diagnostic studies, including MRIs of the brainstem (with and without contrast), were normal. CSF examination showed mild pleocytosis and elevated IgG. Quantitative eye movement recordings documented selective involvement of voluntary horizontal saccades with sparing of horizontal slow eye movements. Neither patient had antineuronal antibodies in the blood. Postmortem examination revealed perivascular chronic inflammatory cells and microglial infiltration of the pons and medulla. One patient also had perivascular infiltrates in both mesial temporal lobes. Neuronal loss was localized to the pontine tegmentum, the medullary sensory nuclei, and the cerebellum. Brainstem motor nuclei were preserved. The clinical and pathologic findings suggest an autoimmune process (probably paraneoplastic) with selective damage to a subpopulation of brainstem neurons critical for horizontal eye movements and recurrent inhibition of bulbar nuclei.
两名成功接受前列腺癌治疗的患者出现了一种进行性神经综合征，起初是自主水平眼球运动丧失，随后是面部、下颌和咽部严重且持续的肌肉痉挛。两人均有轻度步态不稳，其中一人表现出面部和腹部肌阵挛。包括脑干磁共振成像（增强和未增强）在内的广泛诊断性检查均正常。脑脊液检查显示轻度细胞增多和免疫球蛋白G升高。定量眼球运动记录表明自主水平扫视选择性受累，而水平慢眼球运动未受影响。两名患者血液中均无抗神经元抗体。尸检显示脑桥和延髓有血管周围慢性炎症细胞和小胶质细胞浸润。一名患者双侧颞叶内侧也有血管周围浸润。神经元丢失局限于脑桥被盖、延髓感觉核和小脑。脑干运动核得以保留。临床和病理结果提示一种自身免疫过程（可能为副肿瘤性），对脑干中对水平眼球运动和延髓核团反复抑制至关重要的神经元亚群有选择性损害。
