Neuropediatrics 2014; 45 - p025
DOI: 10.1055/s-0034-1390597

Spinal Cord Infarction: A Rare Case of Neurological Sequeale of Lupus Erythematodes

U. Gaiser 1, I. Krägeloh-Mann 1, T. Nägele 2, M. Schöning 1, M. Alber 1, M. Moll 3
  • 1Universitätsklinikum Tübingen, Neuropädiatrie, Entwicklungsneurologie, Sozialpädiatrie, Tübingen, Germany
  • 2Universitätsklinik Tübingen, Neuroradiologie, Tübingen, Germany
  • 3Universitätkinderklinik Tübingen, Rheumatologie, Tübingen, Germany

Background: Infarction of the spinal cord is rare compared with cerebral infarction. Spinal cord infarction is most frequently caused by surgical procedures, pathologies affecting the aorta or trauma. Other causes of spinal cord infarction are diverse (infectious, neoplastic, vascular, autoimmune, and hereditary degenerative disorders).

Case Report: A 16-year-old otherwise healthy girl presented with acute paraplegia; onset was at night and development rapid (< 2 hours). Trauma was not reported.

The initial magnetic resonance imaging (MRI) showed focal hyperintensity in rostral myelin regions (cervical vertebra C5/C6). CSF showed both elevated IgG and cell count. Under treatment with high dose prednisolone paraparesis remained unchanged.

MRI at follow-up demonstrated a T2-signal change consistent with cord ischemia. In the following days, the girl developed vasculitis spots on both hands. Laboratory results showed leukopenia, elevated antinuclear antibodies, as well as antiphospholipid-antibodies. The skin biopsy showed lupus band in immunofluorescence. Whole body MRI showed several spots of myositis.

As ACR criteria for lupus erythematodes were fulfilled, we started treatment with immunoglobulin and plasmapheresis and cyclophosphamide.

Long-term cortisone and hydroxychloroquine treatment was started.

Unfortunately, several weeks’ later severe pain developed in the lower extremities. Whole body MRI control showed increase of myositis changes. Muscle biopsy showed myopathic changes without signs of inflammation consistent with to cortisone myopathy. Cortisone was discontinued. The pain and MRI changes resolved over time.

The patient remained fully paraplegic for nearly 4 months, and then motor function of the lower extremities slightly improved.

She continued with intensive physiotherapy and hydroxychloroquine medication for 4 years, and showed continuous improvement during the past 3 years. She is now able to stand and walk a few steps with assistance.

Conclusion: Spinal cord infarction is a rare condition caused by a wide range of pathologic conditions. Lupus erythematodes, especially in younger patients, is a rare cause. The severity can vary. Permanent and disabling neurologic deficits remain in most patients. Our patient demonstrates that though improvement is possible over a long period of time.