Case reportNeuromyelitis Optica Immunoglobulin G in a Child
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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Cited by (7)
Visual Loss: Optic Neuropathies
2018, Liu, Volpe, and Galetta's Neuro-Ophthalmology: Diagnosis and ManagementNeuromyelitis Optica in a Young Child With Positive Serum Autoantibody
2008, Pediatric NeurologyCitation Excerpt :Long-term combination treatment with prednisone and azathioprine was reported to be beneficial in a small adult series [14]. In the pediatric literature, only several single case studies were reported, and these used different agents [10,11,15]. Among recent pediatric patients, Domingues et al. described a 10-year-old boy with neuromyelitis optica associated with extensive cerebral white-matter involvement who continued to relapse until he was maintained on a combination of prednisone, mitoxantrone, and glatiramer acetate [10].
Multiple sclerosis in children: clinical diagnosis, therapeutic strategies, and future directions
2007, Lancet NeurologyCitation Excerpt :Aquaporin 4 is an active astrocytic water channel implicated in cellular electrolyte influx, particularly at the blood–brain barrier.76 Serum NMO-IgG was seen in a case report of a child with clinical neuromyelitis optica.77 The prognostic role of NMO-IgG in children with demyelination is an area for further study.
Neuro-Ophthalmology: Diagnosis and Management, Second Edition
2010, Neuro-Ophthalmology: Diagnosis and Management, Second Edition