Schwartz M A, Selhorst J B, Ochs A L, Beck R W, Campbell W W, Harris J K, Waters B, Velasco M E
Ann Neurol. 1986 Dec;20(6):677-83. doi: 10.1002/ana.410200605.
We describe two patients and a previously reported patient who acquired unique pendular vergence oscillations of the eyes and concurrent contractions of the masticatory muscles, i.e., oculomasticatory myorhythmia (OMM). The smooth disjunctive eye movements cycled with a frequency of 0.8 to 1.2 Hz. An analysis of peak velocities (15 to 200 degrees/sec) with respect to peak amplitudes (5 to 25 degrees) revealed dynamics characteristic of normal vergence movements. The pathological alterations resulting in pendular vergence oscillations implicate a separately functioning, physiologically normal vergence system within the brainstem. In addition to paralysis of vertical gaze, each patient also experienced progressive somnolence and intellectual deterioration. An intestinal biopsy in 1 patient established a diagnosis of Whipple's disease, which led to appropriate treatment and amelioration of the OMM. A pathological diagnosis of Whipple's disease of the central nervous system was made in the other 2 patients; results of an intestinal biopsy in one of these patients were normal. No patient had palatal myoclonus, and olivary pseudohypertrophy was not found in two autopsy examinations. Thus, OMM is a distinct movement disorder and has been recognized only in Whipple's disease. We conclude that patients with OMM should be treated presumptively for Whipple's disease of the central nervous system, even if a jejunal biopsy is normal.
我们描述了两名患者以及一名先前报道过的患者，他们出现了独特的眼球钟摆样聚散振荡以及咀嚼肌的同步收缩，即动眼咀嚼肌节律失调（OMM）。平滑的分离性眼球运动以0.8至1.2赫兹的频率循环。对相对于峰值幅度（5至25度）的峰值速度（15至200度/秒）进行分析，揭示了正常聚散运动的动力学特征。导致钟摆样聚散振荡的病理改变表明脑干内存在一个独立运作、生理功能正常的聚散系统。除了垂直凝视麻痹外，每位患者还出现了进行性嗜睡和智力衰退。对1例患者进行的肠道活检确诊为惠普尔病，这导致了对OMM的适当治疗并使其症状改善。另外2例患者被诊断为中枢神经系统惠普尔病；其中1例患者的肠道活检结果正常。没有患者出现腭肌阵挛，在两次尸检中均未发现橄榄体假性肥大。因此，OMM是一种独特的运动障碍，仅在惠普尔病中被认识到。我们得出结论，即使空肠活检正常，对于患有OMM的患者也应假定为中枢神经系统惠普尔病进行治疗。
