PT  - JOURNAL ARTICLE
AU  - Albazron, Fatimah M.
AU  - Bruss, Joel
AU  - Jones, Robin M.
AU  - Yock, Torunn I.
AU  - Pulsifer, Margaret B.
AU  - Cohen, Alexander L.
AU  - Nopoulos, Peg C.
AU  - Abrams, Annah N.
AU  - Sato, Mariko
AU  - Boes, Aaron D.
TI  - Pediatric postoperative cerebellar cognitive affective syndrome follows outflow pathway lesions
AID  - 10.1212/WNL.0000000000008326
DP  - 2019 Oct 15
TA  - Neurology
PG  - e1561--e1571
VI  - 93
IP  - 16
4099  - http://n.neurology.org/content/93/16/e1561.short
4100  - http://n.neurology.org/content/93/16/e1561.full
SO  - Neurology2019 Oct 15; 93
AB  - Objective To evaluate lesion location after pediatric cerebellar tumor resection in relation to the development of severe cognitive and affective disturbances, or cerebellar cognitive affective syndrome (CCAS).Methods The postsurgical lesion location of 195 pediatric patients with cerebellar tumors was mapped onto a template brain. Individuals with CCAS were matched to 2 participants without CCAS by sex, age, and lesion volume. Lesion analyses included both a hypothesis-driven evaluation of the cerebellar outflow pathway (deep nuclei and superior cerebellar peduncles) and data-driven multivariate lesion symptom mapping. Lesion-associated networks were evaluated by comparing connectivity patterns between the lesion location of cases with and those without CCAS with resting-state functional connectivity MRI data from large normative adult and pediatric cohorts.Results CCAS was present in 48 of 195 participants (24.6%) and was strongly associated with cerebellar outflow tract lesions (p < 0.0001). Lesion symptom mapping also highlighted the cerebellar outflow pathway, with peak findings in the fastigial nuclei extending into the inferior vermis. Lesion network mapping revealed that the cerebellar region most associated with CCAS was functionally connected to the thalamic mediodorsal nucleus, among other sites, and that higher connectivity between lesion location and the mediodorsal nucleus predicts CCAS occurrence (p < 0.01). A secondary analysis of 27 participants with mutism revealed similar localization of lesions and lesion-associated networks.Conclusion Lesions of the cerebellar outflow pathway and inferior vermis are associated with major cognitive and affective disturbances after pediatric cerebellar tumor resection, and disrupted communication between the cerebellum and the thalamic mediodorsal nucleus may be important.CCAS=cerebellar cognitive affective syndrome; CMS=cerebellar mutism syndrome; MNI=Montreal Neurological Institute; ROI=region of interest; rs-fcMRI=resting-state functional connectivity MRI