J Korean Med Sci.  2005 Dec;20(6):1085-1088. 10.3346/jkms.2005.20.6.1085.

A Catastrophic-Onset Longitudinal Myelitis Accompanied by Bilateral Internuclear Ophthalmoplegia in a Patient with Systemic Lupus Erythematosus

• Rheu CW
• Lee SI
• Yoo WH

• KMID: 2157770
• DOI: http://doi.org/10.3346/jkms.2005.20.6.1085

Abstract

Transverse myelitis (TM) extending from midbrain to the entire spinal cord accompanied by internuclear ophthalmoplegia is extremely rare but cause serious central nervous system complications in patients with systemic lupus erythematosus. We report a case of a 28-yr-old woman with TM extending from the midbrain to the conus medullaris longitudinally and internuclear ophthalmoplegia associated with systemic lupus erythematosus. Her neurological symptoms had an abrupt catastrophic onset and rapidly progressed to respiratory failure within 24 hr. Bilateral internuclear ophthalmoplegia was also followed by TM. Brain MR images showed definite brainstem lesions, which were deeply associated with internuclear ophthalmoplegia, and diffuse signal changes in the whole spinal cord, medulla, pons and midbrain. Clinical improvement of her ophthalmoplegia and of neurological dysfunction of the upper extremities was noted after prompt and aggressive treatment with intravenous pulsed methylprednisolone and cyclophosphamide. However, the neurological dysfunction of the lower limbs and bladder and colon paralysis were almost unchanged until six months passed.

Keyword

Lupus Erythematosus, Systemic; Myelitis, Transverse; Internuclear Ophthalmoplegia; Ocular Motility Disorders

MeSH Terms

Adult
Cyclophosphamide/therapeutic use
Female
Humans
Lupus Erythematosus, Systemic/*complications
Magnetic Resonance Imaging
Myelitis, Transverse/diagnosis/drug therapy/*etiology
Ocular Motility Disorders/diagnosis/drug therapy/*etiology
Prednisolone/therapeutic use

Figure

• 

  Fig. 1 (A) Left lateral gaze and (B) Right lateral gaze. This patient shows marked bilateral limitation of adduction not crossing midline (arrows), but no limitation of abduction, elevation and depression in eyeball movement.

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  Fig. 2 (A) Sagittal T2-weighted MR image of the brainstem and the spinal cord before treatment. This image shows generalized multifocal high signal intensities involving midbrain, pons, medulla and whole spinal cord longitudinally. (B) This T2-weighted MR image after one month shows decrease in the previous high signal intensities in the midbrain, pons, medulla, and cervical spinal cord. However, more increased high signal intensities and distorted conus medullaris are seen in the thoracic and lumbar spinal cord.

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  Fig. 3 (A) T2-weighted brain MR image before treatment. This image shows nodular high signal intensity in periaqueductal region of the midbrain (arrow). (B) This follow up image after treatment shows decrease in the previous high signal intensities in periaqueductal region of the midbrain (arrow).

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