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Keywords:

  • Whipple's disease;
  • Ophthalmoplegia;
  • Myorhythmia

Abstract

Whipple's disease (WD) is a rare disorder that is more common in males than females. Progressive supranuclear ophthalmoplegia (SNO) in conjunction with oculomasticatory myorhythmia (OMM) or oculofacioskeletal myorhythmia are characteristic movement abnormalities when WD involves the nervous system. Limb myorhythmia without facial or ocular myorhythmia has not been reported in WD. We report such a case who had SNO and leg myorhythmia but no facial or ocular myorhythmia. She had onset of WD at age 28 and 16 years later developed SNO and leg myorhythmia. The neurological manifestations did not respond to antimicrobial agents or to the drugs used for parkinsonism or essential tremor. Valproate produced a remarkable improvement in leg myorhythmia, but the efficacy declined after 3 months. Because WD may manifest as a neurological disorder without gastrointestinal symptoms, all SNO cases, with or without OMM, and those with skeletal myorhythmia should be suspected of WD. These patients should be treated vigorously and followed carefully since neurological involvement is the most disabling feature and it has a propensity to relapse.