Multicystic demyelinating myelopathy: widening spectrum of pediatric aquaporin-4 autoimmunity.
case_report · Level V
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- Record sourced from PubMed, PMID 24616194.
- Also identified by DOI 10.1212/WNL.0000000000000192 and PMC identifier 3959749.
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Abstract
A 10-year-old girl presented with subacute lower limb weakness and gait ataxia. MRI revealed a large multicystic spinal cord lesion with patchy enhancement (figure 1, A–B) and 3 small (<6 mm) periventricular and deep white matter brain lesions. The presence of serum anti-aquaporin-4 immunoglobulin G (AQP4) (ELISA assay) and compatible neuropathologic features from neurosurgical specimens<sup>1</sup> (figure 2) suggested the diagnosis of a neuromyelitis optica spectrum disorder.<sup>2</sup> Targeted immunotherapy was started with partial lesion resolution (figure 1C).
Medical subject headings
- Aquaporin 4
- Autoimmunity
- Brain
- Demyelinating Diseases
- Neuromyelitis Optica
- Spinal Cord