Cerebral Manifestations of Whipple's Disease

Abstract

The review of Whipple's disease by Fleming and associates in the June 1988 issue of the Proceedings (pages 539 to 551) is complete and helpful for clinicians attempting to identify and treat patients with Whipple's disease. For your readership, we wish to add that, although rare, a virtually pathognomonic movement disorder has recently been described in patients with Whipple's disease. In 1963, van Bogaert and associates1Van Bogaert L Lafon R Pages P Labauge R Sur une encéphalite subaiguë non classable, principalement carac-térisée par des myorythmies oculo-facio-cervicales.Rev Neurol (Paris). 1963; 109: 443-453Google Scholar described a patient with rhythmic vergence movements of the eyes and cocontractions of the jaw. The neuropathologic features of Whipple's disease, however, were not recognized until the brain was reexamined by De Jonghe and colleagues2De Jonghe P Martin JJ Budka H Ceuterick C Cerebral manifestations of Whipple's disease.Acta Neurol Belg. 1979; 79: 305-313PubMed Google Scholar in 1979. In a report of two more patients in 1986, Schwartz and co-workers3Schwartz MA Selhorst JB Ochs AL Beck RW Campbell WW Harris JK Waters B Velasco ME Oculomasticatory myorhythmia: a unique movement disorder occurring in Whipple's disease.Ann Neurol. 1986; 20: 677-683Crossref PubMed Scopus (130) Google Scholar termed this unique movement disorder “oculomasticatory myorhythmia.” Electromyography showed that contractions of the jaw and tongue coincided with convergence of the eyes. The continuous movements ranged from 0.5 to 1.2 Hz and persisted during sleep. In one patient, the pendular vergence oscillations of the eyes preceded the spontaneous movements of the jaw. Recently, a fourth patient with identical movements of the eyes and jaw has been identified in Houston.4Grotta JC Pettigrew LC Schmidt WA Friedman G Snatic S Oculomasticatory myorhythmia (letter to the editor).Ann Neurol. 1987; 22: 395-396Crossref PubMed Scopus (19) Google Scholar All these patients also had a vertical gaze paralysis and eventually hyper-somnolence, but none had the usual gastrointestinal symptoms of Whipple's disease. The importance of clinical recognition of these features is underscored by the finding of normal magnetic resonance images early in the course of the disorder and a favorable response to antibiotic therapy in two of the treated patients. This movement disorder is pertinent not only to neurologists and ophthalmologists but also to all clinicians with a respect for the power of a clinical diagnosis, especially gastroenterologists who may be asked to obtain an intestinal biopsy in otherwise well patients. Cerebral Manifestations of Whipple's Disease: Dr. Wiesner repliesMayo Clinic ProceedingsVol. 63Issue 10PreviewMy colleagues and I appreciate the comments of Drs. Selhorst and Schwartz concerning their experience with the cerebral manifestations of Whipple's disease. Because our study was retrospective, minor details of manifestations of Whipple's disease may have been overlooked, inasmuch as oculomasticatory myorhythmia has only recently been described (in 1986) and most of our cases date back to the 1960s and 1970s. We thank Drs. Selhorst and Schwartz for their additional information, and we concur that these cerebral manifestations may be an important clinical sign that may contribute to the diagnosis of Whipple's disease. Full-Text PDF