title: [A case of recurrent paraneoplastic cerebellar ataxia with antibodies to GluR epsilon 2 causally related to ganglioneuroma].
abstract: Acute cerebellar ataxia (ACA) in childhood following viral infection is a self-limited disease. We present a boy with recurrent episodes of paraneoplastic cerebellar ataxia associated with a ganglioneuroma. A 20-month-old boy developed the first episode of cerebellar ataxia after nonspecific respiratory tract infections. During this episode he showed a wide gait and truncal ataxia with intention tremor and horizontal nystagmus. Our initial diagnosis was ACA, and gradual improvement of ataxia was observed thereafter. At 2 years and 6 months, similar cerebellar symptoms recurred after respiratory tract infections. Speech difficulty and cognitive problems developed thereafter. We suspected paraneoplastic syndrome. Computed tomography revealed a retroperitoneal tumor, and autoantibodies against GluR epsilon 2 were detected in the cerebrospinal fluid. After the tumor resection, the cerebellar symptoms did not recur and speech difficulty and cognitive problems improved gradually. Recent neuroimaging and neuropsychological studies have revealed that cerebellar function contributes to higher brain functions including cognition and learning. We will follow up this patient's long-term cognitive function and consider special educational support and programs.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Yamanashi University, Chuo, Yamanashi.
authors.forename: Sayaka
authors.initials: S
authors.lastname: Noguchi
authors.forename: Yoshimi
authors.initials: Y
authors.lastname: Kaga
authors.forename: Yukitoshi
authors.initials: Y
authors.lastname: Takahashi
authors.forename: Kakuro
authors.initials: K
authors.lastname: Aoyagi
authors.forename: Kohsuke
authors.initials: K
authors.lastname: Nakamura
authors.forename: Yuko
authors.initials: Y
authors.lastname: Kamiya
authors.forename: Takaya
authors.initials: T
authors.lastname: Nakane
authors.forename: Hideaki
authors.initials: H
authors.lastname: Kanemura
authors.forename: Kanji
authors.initials: K
authors.lastname: Sugita
authors.forename: Masao
authors.initials: M
authors.lastname: Aihara
mesh_terms: D001323:Autoantibodies / Q000032:analysis*; D002524:Cerebellar Ataxia / Q000209:etiology* / Q000276:immunology; D005729:Ganglioneuroma / Q000150:complications*; D006801:Humans; D007223:Infant; D008297:Male; D010257:Paraneoplastic Syndromes / Q000276:immunology; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology*; D012186:Retroperitoneal Neoplasms / Q000150:complications*
chemical_list: D001323:Autoantibodies; C121001:NR2B NMDA receptor; D016194:Receptors, N-Methyl-D-Aspartate