TY - JOUR
T1 - A case of childhood stiff-person syndrome with striatal lesions
T2 - A possible entity distinct from the classical adult form
AU - Sanefuji, Masafumi
AU - Torisu, Hiroyuki
AU - Kira, Ryutaro
AU - Yamashita, Hiroshi
AU - Ejima, Kazuna
AU - Shigeto, Hiroshi
AU - Takada, Yui
AU - Yoshida, Keiko
AU - Hara, Toshiro
N1 - Funding Information:
We thank Dr. Kimiyoshi Arimura (Kagoshima University, Graduate School of Medical and Dental Sciences) for valuable advice on the diagnosis. This work was supported in part by a Grant-in-Aid for Scientific Research to Masafumi Sanefuji from the Ministry of Education, Culture, Sports, Science and Technology of Japan .
PY - 2013/6
Y1 - 2013/6
N2 - Parainfectious or autoimmune striatal lesions have been repeatedly described in children. We report a 7-year-old girl with painful muscle spasms, leading to the diagnosis of childhood stiff-person syndrome (SPS). Striatal lesions were demonstrated by diffusion-weighted magnetic resonance imaging (MRI) and single-photon emission computed tomography but not by conventional MRI. Autoantibodies against glutamic acid decarboxylase (GAD) were absent. Steroid pulse therapy and high-dose intravenous immunoglobulin resolved all the symptoms with slight sequelae. Childhood SPS may be characterized by absent anti-GAD antibodies and a transient benign clinical course, and it may have a pathomechanism distinct from that in adult SPS.
AB - Parainfectious or autoimmune striatal lesions have been repeatedly described in children. We report a 7-year-old girl with painful muscle spasms, leading to the diagnosis of childhood stiff-person syndrome (SPS). Striatal lesions were demonstrated by diffusion-weighted magnetic resonance imaging (MRI) and single-photon emission computed tomography but not by conventional MRI. Autoantibodies against glutamic acid decarboxylase (GAD) were absent. Steroid pulse therapy and high-dose intravenous immunoglobulin resolved all the symptoms with slight sequelae. Childhood SPS may be characterized by absent anti-GAD antibodies and a transient benign clinical course, and it may have a pathomechanism distinct from that in adult SPS.
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U2 - 10.1016/j.braindev.2012.08.003
DO - 10.1016/j.braindev.2012.08.003
M3 - Article
C2 - 22944247
AN - SCOPUS:84876782805
SN - 0387-7604
VL - 35
SP - 575
EP - 578
JO - Brain and Development
JF - Brain and Development
IS - 6
ER -