Shaikh Aasef G, Ghasia Fatema F
Daroff-Dell'Osso Ocular Motility Laboratory, Cleveland VA Medical Center, Cleveland, VA, United States.
Department of Neurology, Case Western Reserve University, Cleveland, OH, United States.
Front Neurol. 2017 Jul 11;8:321. doi: 10.3389/fneur.2017.00321. eCollection 2017.
Whipple's disease, a rare systemic infectious disorder, is complicated by the involvement of the central nervous system in about 5% of cases. Oscillations of the eyes and the jaw, called oculo-masticatory myorhythmia, are pathognomonic of the central nervous system involvement but are often absent. Typical manifestations of the central nervous system Whipple's disease are cognitive impairment, parkinsonism mimicking progressive supranuclear palsy with vertical saccade slowing, and up-gaze range limitation. We describe a unique patient with the central nervous system Whipple's disease who had typical features, including parkinsonism, cognitive impairment, and up-gaze limitation; but also had diplopia, esotropia with mild horizontal (abduction more than adduction) limitation, and vertigo. The patient also had gaze-evoked nystagmus and staircase horizontal saccades. Latter were thought to be due to mal-programmed small saccades followed by a series of corrective saccades. The saccades were disconjugate due to the concurrent strabismus. Also, we noted disconjugacy in the slow phase of gaze-evoked nystagmus. The disconjugacy of the slow phase of gaze-evoked nystagmus was larger during monocular viewing condition. We propose that interaction of the strabismic drifts of the covered eyes and the nystagmus drift, putatively at the final common pathway might lead to such disconjugacy.
惠普尔病是一种罕见的全身性感染性疾病,约5%的病例会并发中枢神经系统受累。眼睛和下颌的摆动,即眼嚼肌阵挛,是中枢神经系统受累的特征性表现,但通常并不出现。中枢神经系统惠普尔病的典型表现包括认知障碍、类似进行性核上性麻痹的帕金森综合征(伴有垂直扫视减慢)以及上视范围受限。我们描述了一位患有中枢神经系统惠普尔病的独特患者,他具有典型特征,包括帕金森综合征、认知障碍和上视受限;但也有复视、内斜视伴轻度水平(外展大于内收)受限以及眩晕。该患者还存在凝视诱发性眼球震颤和阶梯状水平扫视。后者被认为是由于小扫视编程错误,随后出现一系列矫正性扫视所致。由于同时存在斜视,扫视是不共轭的。此外,我们注意到凝视诱发性眼球震颤慢相存在不共轭。在单眼观察条件下,凝视诱发性眼球震颤慢相的不共轭更大。我们提出,被遮盖眼睛的斜视性漂移与眼球震颤漂移之间的相互作用,可能在最终的共同通路上导致这种不共轭。