Editors' note: IgLON5-mediated neurodegeneration is a differential diagnosis of CNS Whipple disease

Abstract

In “IgLON5-mediated neurodegeneration is a differential diagnosis of CNS Whipple disease,” Morales-Briceno et al. described a case of anti-IgLON5 disease presenting as cold intolerance, myorhythmia, myoclonus, dysarthria, hypersalivation, dysphagia, sleep apnea, and mildly impaired cognition mimicking CNS Whipple disease. Drs. Bally and Lang report that the presentation in this case was similar to cases of Whipple disease in the literature; however, palatal involvement of the myorhythmia—which was present in the case reported by Morales-Briceno et al.—is rare in Whipple disease, and the cranial myorhythmia in Whipple disease is associated with a vertical supranuclear gaze palsy, which was not present in the case reported by Morales-Briceno et al. Morales-Briceno et al. respond that while the absence of vertical gaze palsy made Whipple disease less likely, such gaze palsies can occur in a certain percentage of patients with IgLON5-mediated neurodegeneration. Both Morales-Briceno et al. and Drs. Bally and Lang agree that the distinction between IgLON5-mediated neurodegeneration and Whipple disease can be challenging. In “IgLON5-mediated neurodegeneration is a differential diagnosis of CNS Whipple disease,” Morales-Briceno et al. described a case of anti-IgLON5 disease presenting as cold intolerance, myorhythmia, myoclonus, dysarthria, hypersalivation, dysphagia, sleep apnea, and mildly impaired cognition mimicking CNS Whipple disease. Drs. Bally and Lang report that the presentation in this case was similar to cases of Whipple disease in the literature; however, palatal involvement of the myorhythmia—which was present in the case reported by Morales-Briceno et al.—is rare in Whipple disease, and the cranial myorhythmia in Whipple disease is associated with a vertical supranuclear gaze palsy, which was not present in the case reported by Morales-Briceno et al. Morales-Briceno et al. respond that while the absence of vertical gaze palsy made Whipple disease less likely, such gaze palsies can occur in a certain percentage of patients with IgLON5-mediated neurodegeneration. Both Morales-Briceno et al. and Drs. Bally and Lang agree that the distinction between IgLON5-mediated neurodegeneration and Whipple disease can be challenging.