title: Clinical and neuroanatomical predictors of cerebellar mutism syndrome.
abstract: Cerebellar mutism syndrome (CMS) is an important medical challenge in the management of pediatric posterior fossa brain tumors, because it occurs in a subset of children following tumor resection. A definitive clinical profile and neuroanatomical substrate associated with CMS remains unclear. We investigated the relationship between presurgical and clinical variables and the incidence of CMS, along with diffusion tensor imaging, to characterize the integrity of cerebello-thalamo-cerebral white matter pathways. Seventeen children with posterior fossa tumors and CMS, 34 children with posterior fossa tumors without CMS, and 28 healthy children were enrolled in this study. Bilateral cerebello-thalamo-cerebral pathways were delineated and segmented into anatomical regions. Mean integrity measures for each region were compared among children with CMS, children without CMS, and healthy children. Left-handedness, medulloblastoma histology, and larger tumor size distinguished between patients with CMS and patients without CMS (P < .04). Right cerebellar white matter within the cerebello-thalamo-cerebral pathway was compromised in children with CMS relative to children without CMS and healthy children (P < .02). We provide a potential schema for CMS risk among children treated for posterior fossa tumors. Left-handed children treated for medulloblastoma may be the most at risk for CMS, and unilateral, localized damage within the cerebello-thalamo-cerebral pathway at the level of the right cerebellum is implicated in the presentation of CMS. This disruption in communication between the right cerebellum and left frontal cortex may contribute to speech-language problems observed in children with CMS. Our findings may be relevant for surgical planning and speech-language therapy to mitigate symptoms of CMS.
authors.affiliation: Program in Neuroscience and Mental Health, Hospital for Sick Children, 555 University Ave, Toronto, ON, M5G 1X8, Canada.
authors.forename: Nicole
authors.initials: N
authors.lastname: Law
authors.forename: Mark
authors.initials: M
authors.lastname: Greenberg
authors.forename: Eric
authors.initials: E
authors.lastname: Bouffet
authors.forename: Michael D
authors.initials: MD
authors.lastname: Taylor
authors.forename: Suzanne
authors.initials: S
authors.lastname: Laughlin
authors.forename: Douglas
authors.initials: D
authors.lastname: Strother
authors.forename: Christopher
authors.initials: C
authors.lastname: Fryer
authors.forename: Dina
authors.initials: D
authors.lastname: McConnell
authors.forename: Juliette
authors.initials: J
authors.lastname: Hukin
authors.forename: Caelyn
authors.initials: C
authors.lastname: Kaise
authors.forename: Frank
authors.initials: F
authors.lastname: Wang
authors.forename: Donald J
authors.initials: DJ
authors.lastname: Mabbott
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002526:Cerebellar Diseases / Q000150:complications / Q000175:diagnosis* / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D002658:Developmental Disabilities / Q000175:diagnosis* / Q000209:etiology / Q000401:mortality; D056324:Diffusion Tensor Imaging; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D015192:Infratentorial Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000401:mortality; D008297:Male; D009155:Mutism / Q000175:diagnosis* / Q000209:etiology / Q000401:mortality; D009445:Neuroanatomy; D009483:Neuropsychological Tests; D011379:Prognosis; D015996:Survival Rate