Encephalomyelitis disseminata in a child with severe outcome and atypical clinical findings – Neuromyelitis optica Devic or multiple sclerosis?

We report on a ten-year old girl with an inflammatory relapsing-remitting disease, involving brain, spinal cord and optic nerves. In a course of three and a half years there was a severe progression of the disease leading to tetraparesis and blindness, in spite of treatment with methylprednisolon, IVIG, mitoxantrone and IFN-beta. No abnormal laboratory findings were detected. The cerebral spinal fluid revealed normal protein level and cell count without oligoclonal bands. Multiple magnetic resonance imaging studies showed several white matter abnormalities of both hemispheres and corpus callosum, and changing inflammatory lesions mainly in the cervical spinal cord, but also visible in the thoracic spinal cord and the optic nerves. Some of the cerebral white matter lesions were associated with definite neurological symptoms. These cerebral lesions are rather typical findings in multiple sclerosis than in neuromyelitis optica Devic, while the clinical deteriation is more characteristically for neuromyelitis optica Devic.

We discuss in detail the differential diagnosis of multiple sclerosis and neuromyelitis optica Devic in childhood.