TY - JOUR
T1 - A patient with anti-aquaporin 4 antibody presenting hypersomnolence as the initial symptom and symmetrical hypothalamic lesions
AU - Deguchi, Kentaro
AU - Kono, Syoichiro
AU - Deguchi, Shoko
AU - Morimoto, Nobutoshi
AU - Ikeda, Masami
AU - Kurata, Tomoko
AU - Ikeda, Yoshio
AU - Matsuura, Tohru
AU - Kanbayashi, Takashi
AU - Takahashi, Toshiyuki
AU - Abe, Koji
N1 - Funding Information:
This work was partly supported by a Grant-in-Aid for Scientific Research (B) 21390267 and the Ministry of Education, Science, Culture and Sports of Japan , and by Grants-in-Aid from the Research Committee of CNS Degenerative Diseases (Nakano I), and grants (Itoyama Y, Imai T, Sobue G) from the Ministry of Health, Labour and Welfare of Japan .
PY - 2012/1/15
Y1 - 2012/1/15
N2 - 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.
AB - 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.
KW - Aquaporin 4
KW - Hypersomnolence
KW - Neuromyelitis optica
KW - Symmetrical hypothalamic lesions
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U2 - 10.1016/j.jns.2011.08.031
DO - 10.1016/j.jns.2011.08.031
M3 - Article
C2 - 21917270
AN - SCOPUS:84155167263
SN - 0022-510X
VL - 312
SP - 18
EP - 20
JO - Journal of the neurological sciences
JF - Journal of the neurological sciences
IS - 1-2
ER -