title: Acquired nystagmus as the initial presenting sign of chiasmal glioma in young children.
abstract: OBJECTIVE
  The aim of the study was to investigate the incidence of nystagmus at diagnosis in children with optic pathway glioma involving the chiasm and hypothalamus.
  
  
  METHODS
  Twenty-two patients with a measurable optic pathway/hypothalamic glioma (without neurofibromatosis-1) were followed in our center from 2001 to 2013. The medical files were retrospectively reviewed for demographic and clinical findings, and the imaging scans, for tumor characteristics.
  
  
  RESULTS
  There were 9 boys and 13 girls of mean age 3.5 ± 4.4 years at diagnosis; 15 were aged <2 years. Tumor size ranged from 10 × 6 mm to 62 × 29 mm. Mean duration of follow-up was 8.3 ± 5.4 years. Nystagmus was detected at diagnosis in 10 children (45%), all <2 years old (66.6% of the younger group); no child older than 2 years presented with nystagmus. Nystagmus, once present, did not resolve and continued throughout follow-up. There were no cases of new onset of nystagmus during follow-up in the children in whom it was not detected at diagnosis. Treatment consisted of partial resection/biopsy with/without shunting (n = 13) and chemotherapy (n = 19) with (n = 2) or without adjuvant radiation. Of the 22 children, 6 had a radiographic response to treatment, 8 remained stable, and 8 (all of whom received chemotherapy) showed disease progression despite treatment.
  
  
  CONCLUSIONS
  In conclusion, monocular nystagmus is a more common presenting sign of optic pathway/hypothalamic glioma in children <2 years old than previously estimated. Although subtle, nystagmus has a very narrow differential diagnosis, and its presence should raise suspicions of a chiasmal tumor with prompt referral for imaging. The visual prognosis is moderate to poor.
authors.affiliation: Department of Pediatric Oncology, Schneider Children's Medical Center of Israel, Petach Tikva 49202, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 6997801, Israel.
authors.forename: Helen
authors.initials: H
authors.lastname: Toledano
authors.affiliation: Department of Ophthalmology, Rabin Medical Center, Petach Tikva 49100, Israel.
authors.forename: Orkun
authors.initials: O
authors.lastname: Muhsinoglu
authors.affiliation: Department of Radiology, Rabin Medical Center, Petach Tikva 49100, Israel.
authors.forename: Judith
authors.initials: J
authors.lastname: Luckman
authors.affiliation: Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 6997801, Israel; Unit of Pediatric Ophthalmology, Schneider Children's Medical Center of Israel, Petach Tikva 49202, Israel; Krieger Eye Research Laboratory, Felsenstein Medical Research Center, Petach Tikva 49100, Israel. Electronic address: ncohen1@gmail.com.
authors.forename: Nitza
authors.initials: N
authors.lastname: Goldenberg-Cohen
authors.affiliation: Unit of Pediatric Neurosurgery, Schneider Children's Medical Center of Israel, Petach Tikva 49202, Israel.
authors.forename: Shalom
authors.initials: S
authors.lastname: Michowiz
mesh_terms: D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D005910:Glioma / Q000150:complications*; D006801:Humans; D015994:Incidence; D008297:Male; D009456:Neurofibromatosis 1 / Q000175:diagnosis; D009759:Nystagmus, Pathologic / Q000453:epidemiology* / Q000209:etiology*; D009897:Optic Chiasm / Q000473:pathology*; D019574:Optic Nerve Neoplasms / Q000150:complications*; D011379:Prognosis; D012189:Retrospective Studies
keywords: Chiasmal glioma; Monocular nystagmus; Optic pathway/hypothalamic glioma; Visual outcome