Conus medullaris syndrome in Vogt-Koyanagi-Harada disease: an unusual presentation

Síndrome de cone medular na doença de Vogt-Koyanagi-Harada: uma apresentação não usual

A 25-year-old woman presented over one week with subacute flaccid paraparesis, saddle anesthesia and sphincter dysfunction, compatible with conus medullaris syndrome. Lumbar magnetic resonance imaging (MRI) (►Figure 1) showed spinal cord edema and intense leptomeningeal enhancement. Six months after, she presented with aseptic meningitis and bilateral vision loss. Optical coherence tomography (OCT) showed bilateral exudative retinal detachments (►Figure 2). Incomplete Vogt-Koyanagi-Harada disease (VKHD) was diagnosed based on the combination of bilateral granulomatous panuveitis and aseptic meningitis.¹ Spinal cord involvement is a frequent manifestation of neurological inflammatory diseases, and VKHD should be considered when typical eye, ear, and skin symptoms are present.²,³

Figure 1
Axial T2-weighted (A) and sagittal (C) short-TI inversion recovery (STIR) magnetic resonance imaging with central hyperintense signal associated with lumbosacral intumescence and conus medullaris edema. Axial (B) and sagittal (D) contrast-enhanced T1-weighted magnetic resonance imaging with leptomeningeal enhancement.

Figure 2
Right (A) and left (C) eyes retinography depicting hyperemic optic discs and areas of retinal detachment (white arrows). Macular horizontal B-scans reveal bilateral serous retinal detachment, located in the foveal and parafoveal regions in the right eye (B) and in the nasal retina in the left eye (D) (white arrows).

References

• ¹
  Read RW, Holland GN, Rao NA, et al. Revised diagnostic criteria for Vogt-Koyanagi-Harada disease: report of an international committee on nomenclature. Am J Ophthalmol 2001;131(05):647-652. Doi: 10.1016/S0002-9394(01)00925-4
  » https://doi.org/10.1016/S0002-9394(01)00925-4
• ²
  Triplett JD, Buzzard KA, Lubomski M, et al. Immune-mediated conditions affecting the brain, eye and ear (BEE syndromes). J Neurol Neurosurg Psychiatry 2019;90(08):882-894. Doi: 10.1136/jnnp-2018-319002
  » https://doi.org/10.1136/jnnp-2018-319002
• ³
  Gu S, Liu Y, Song Z, Zi X, Deng H. Acute myelitis in a patient with vogt-koyanagi-harada disease: case report and review of the literature. J Clin Neurol 2013;9(01):61-64. Doi: 10.3988/jcn.2013.9.1.61
  » https://doi.org/10.3988/jcn.2013.9.1.61

Publication Dates

• Publication in this collection
  21 Nov 2022
• Date of issue
  Aug 2022

History

• Received
  26 Jan 2022
• Accepted
  11 Feb 2022