Elsevier

Pediatric Neurology

Volume 35, Issue 5, November 2006, Pages 370-372
Pediatric Neurology

Case report
Neuromyelitis Optica Immunoglobulin G in a Child

https://doi.org/10.1016/j.pediatrneurol.2006.06.008Get rights and content

Neuromyelitis optica or Devic’s syndrome is an uncommon demyelinating disorder that preferentially attacks the spinal cord and optic nerves. Although it is well described in adults, childhood neuromyelitis optica has rarely been reported in the literature and is frequently misdiagnosed as severe multiple sclerosis. Recently, a serum immunoglobulin G test for neuromyelitis optica has become available which may clarify and accelerate the diagnosis. This report describes a child with recurrent myelitis and an elongated spinal cord lesion who was found to have positive neuromyelitis optica autoantibody. We believe that neuromyelitis optica autoantibody testing should be performed in cases of pediatric transverse myelitis with multiple vertical segments or recurrence.

Introduction

Neuromyelitis optica, or Devic’s syndrome, is a rare and aggressive demyelinating disease of the spinal cord and optic nerves that usually spares the brain. Recently, a serum autoantibody immunoglobulin G marker for neuromyelitis optica with a sensitivity of 73% and specificity of 91% in patients with clinically defined neuromyelitis optica has become available [1]. This autoantibody allows early detection of neuromyelitis optica before fulfillment of formal diagnostic criteria, and may allow earlier institution of immunosuppressive therapy. Compared with multiple sclerosis, neuromyelitis optica usually has a later age onset and even greater preponderance of female cases [2], but rare pediatric cases have been described. Neuromyelitis optica antibody has not been previously reported in children. This report presents the case of a child with recurrent transverse myelitis and a large cervical spinal cord lesion responsive to intravenous methylprednisolone with a positive neuromyelitis optica immunoglobulin G.

Section snippets

Case Report

An 8-year-old female subacutely developed bilateral upper extremity paresthesias. Over the next 5 days, she manifested weakness and clumsiness of all four limbs with gait instability. She denied visual, sensory, or sphincter involvement. She had no recent infectious illnesses. Gestational, birth, past medical and family history were unremarkable. On examination, she was alert and oriented with normal cranial nerve function. She had weakness to resistance in all four extremities, more on the

Discussion

Transverse myelitis is well recognized in children, with nearly 400 cases annually in the United States [3]. Prognosis in children is variable, but Knebusch estimated 44% had good recovery, 33% had persistent deficits but could walk unassisted, and 23% manifested severe neurological impairment in gait, micturition, and defecation [4]. Our patient had a fairly typical episode consistent with transverse myelitis, but with a lesion greater than three vertebral segments long. The second episode was

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