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Published online before print May 28, 2008, doi:10.1212/01.wnl.0000314832.24682.c6)
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Received November 26, 2007
Accepted March 13, 2008

CNS aquaporin-4 autoimmunity in children

A. McKeon MB, MRCPI, V. A. Lennon MD, PhD, T. Lotze MD, S. Tenenbaum MD, J. M. Ness MD, PhD, M. Rensel MD, N. L. Kuntz MD, J. P. Fryer MS, H. Homburger MD, J. Hunter MD, B. G. Weinshenker MD, K. Krecke MD, C. F. Lucchinetti MD, and S. J. Pittock MD*

From the Departments of Neurology (A.M., V.A.L., N.L.K., B.G.W., C.F.L., S.J.P.), Laboratory Medicine and Pathology (V.A.L., J.P.F., H.H., S.J.P.), Immunology (V.A.L.), and Radiology (K.K.), Mayo Clinic College of Medicine, Rochester, MN; Divisions of Child Neurology (T.L.), and Radiology (J.H.), Texas Children's Hospital, Baylor College of Medicine, Houston; Department of Pediatric Neurology, National Pediatric Hospital Dr. J. P. Garrahan (S.T.), Buenos Aires, Argentina; Department of Pediatrics, University of Alabama at Birmingham and Children's Hospital (J.M.N.); and Division of Child Neurology, The Cleveland Clinic Foundation (M.R.), OH.


* To whom correspondence should be addressed. E-mail: Pittock.Sean{at}mayo.edu.

Background: In adult patients, autoantibodies targeting the water channel aquaporin-4 (AQP4) are a biomarker for a spectrum of CNS inflammatory demyelinating disorders with predilection for optic nerves and spinal cord (neuromyelitis optica [NMO]). Here we describe the neurologic, serologic, and radiographic findings associated with CNS AQP4 autoimmunity in childhood.

Methods: A total of 88 consecutive seropositive children were identified through service evaluation for NMO-IgG. Sera of 75 were tested for coexisting autoantibodies. Clinical information was available for 58.

Results: Forty-two patients (73%) were non-Caucasian, and 20 (34%) had African ethnicity. Median age at symptom onset was 12 years (range 4–18). Fifty-seven (98%) had attacks of either optic neuritis (n = 48; 83%) or transverse myelitis (n = 45; 78%), or both. Twenty-six (45%) had episodic cerebral symptoms (encephalopathy, ophthalmoparesis, ataxia, seizures, intractable vomiting, or hiccups). Thirty-eight (68%) had brain MRI abnormalities, predominantly involving periventricular areas (in descending order of frequency): the medulla, supratentorial and infratentorial white matter, midbrain, cerebellum, thalamus, and hypothalamus. Additional autoantibodies were detected in 57 of 75 patients (76%), and 16 of 38 (42%) had a coexisting autoimmune disorder recorded (systemic lupus erythematosus, Sjögren syndrome, juvenile rheumatoid arthritis, Graves disease). Attacks were recurrent in 54 patients (93%; median follow-up, 12 months). Forty-three of 48 patients (90%) had residual disability: 26 (54%) visual impairment and 21 (44%) motor deficits (median Expanded Disability Status Scale 4.0 at 12 months).

Conclusions: Aquaporin-4 autoimmunity is a distinctive recurrent and widespread inflammatory CNS disease in children.




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