TY - JOUR
T1 - A novel paraneoplastic syndrome with acquired lipodystrophy and chronic inflammatory demyelinating polyneuropathy in an adolescent male with craniopharyngioma
AU - Lockemer, Hillary Elizabeth
AU - Sumpter, Kathryn Maria
AU - Cope-Yokoyama, Sandy
AU - Garg, Abhimanyu
N1 - Publisher Copyright:
© 2018 Walter de Gruyter GmbH, Berlin/Boston.
PY - 2018/3/28
Y1 - 2018/3/28
N2 - Acquired lipodystrophy, craniopharyngioma and chronic inflammatory demyelinating polyneuropathy (CIDP) are individually rare disorders, and have never before been reported in a single patient. A 15-year-7 month old Caucasian male presented with lower extremity weakness, frequent falls and abnormal fat distribution occurring over the previous 1 year. He was diagnosed with CIDP, craniopharyngioma and acquired lipodystrophy. The patient underwent tumor debulking and cranial irradiation for the craniopharyngioma, and received monthly intravenous immunoglobulin for the CIDP. The patient initially had some resolution of the lipodystrophy phenotype, but subsequently the abnormal fat distribution recurred and the patient developed additional systemic abnormalities, including mild pancytopenia and hepatic fibrosis. Our patient represents a novel association of acquired lipodystrophy, craniopharyngioma, and CIDP, possibly due to an as yet unidentified paraneoplastic autoantibody.
AB - Acquired lipodystrophy, craniopharyngioma and chronic inflammatory demyelinating polyneuropathy (CIDP) are individually rare disorders, and have never before been reported in a single patient. A 15-year-7 month old Caucasian male presented with lower extremity weakness, frequent falls and abnormal fat distribution occurring over the previous 1 year. He was diagnosed with CIDP, craniopharyngioma and acquired lipodystrophy. The patient underwent tumor debulking and cranial irradiation for the craniopharyngioma, and received monthly intravenous immunoglobulin for the CIDP. The patient initially had some resolution of the lipodystrophy phenotype, but subsequently the abnormal fat distribution recurred and the patient developed additional systemic abnormalities, including mild pancytopenia and hepatic fibrosis. Our patient represents a novel association of acquired lipodystrophy, craniopharyngioma, and CIDP, possibly due to an as yet unidentified paraneoplastic autoantibody.
KW - chronic inflammatory demyelinating polyneuropathy
KW - craniopharyngioma
KW - lipodystrophy
UR - http://www.scopus.com/inward/record.url?scp=85042690501&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85042690501&partnerID=8YFLogxK
U2 - 10.1515/jpem-2017-0222
DO - 10.1515/jpem-2017-0222
M3 - Article
C2 - 29455190
AN - SCOPUS:85042690501
SN - 0334-018X
VL - 31
SP - 479
EP - 483
JO - Journal of Pediatric Endocrinology and Metabolism
JF - Journal of Pediatric Endocrinology and Metabolism
IS - 4
ER -