Multicystic demyelinating myelopathy: widening spectrum of pediatric aquaporin-4 autoimmunity.

Longoni, Giulia; Bigi, Sandra; Branson, Helen M; Hawkins, Cynthia; Rutka, James T; Filippi, Massimo; Yeh, E Ann · Neurology · 2014

case_report · Level V

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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).

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