Abstract
Here we report a case with positive serum anti-aquaporin 4 (AQP4) antibody who presented
with hypersomnolence, symmetrical hypothalamic lesions and a reduced CSF orexin (hypocretin)
level without optic nerve and spinal cord lesions on MRI. All of the symptoms, MRI
finding and CSF orexin level improved simultaneously after steroid therapy. AQP4 is
a member of the AQP superfamily which is strongly expressed in the hypothalamus where
orexin (hypocretin)-containing neurons are primarily concentrated. Although there
have been only a few reports similar to our case, the present case suggests a close
relationship between the positive serum anti-AQP4 antibody and symmetrical hypothalamic
lesions with hypersomnolence and without optic /spinal lesion, which is improved by
steroid treatment.
Abbreviations:
AQP4 (aquaporin 4), CSF (cerebrospinal fluid), ESS (Epworth sleepiness score), eZIS (easy Z-score Imaging System), ECD-SPECT (99mTc-ethylcysteinate dimmer single photon emission computed tomography), FLAIR (a fast fluid-attenuated inversionrecovery), MMSE (Mini-Mental State Examination), MRI (magnetic resonance imaging), NMO (nueromyelitis optica)Keywords
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Article info
Publication history
Published online: September 14, 2011
Accepted:
August 22,
2011
Received in revised form:
August 18,
2011
Received:
July 24,
2011
Identification
Copyright
© 2011 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
