The long-term effects on neurologic, neuropsychological, and behavioral functioning in a consecutive series of 23 children treated surgically for cerebellar pilocytic astrocytoma without additional radio- and chemotherapy are determined in a study at Sophia Children’s Hospital, Rotterdam, The Netherlands, and other medical centers. Follow-up ranged from 1 year to 8 years after tumor resection. Age at testing ranged from 6 to 22 years (mean, 12 years). Shunting of a preoperative hydrocephalus was required in 11 children (48%). Neurologic status at time of psychological assessment was normal to mildly impaired; 7 had mild ataxia and 2 a mild intention tremor. Orofacial apraxia occurred in 1 and upper limb neglect in 2. Dysarthria was present in 5 (22%), and language problems in 7 (30%). Two had suffered mutism and MSD syndrome immediately postoperative, with persistence of dysarthria. Neuropsychological tests showing significantly weaker performances in comparison with norms included sustained attention, executive functioning, visual-spatial function and memory. Severe hydrocephalus before surgery was associated with lower scores on visual-spatial skills and complex figure drawing. Behavioral disturbances were observed in 15 children (65%), including overanxious disorder, Asperger syndrome, ADHD, and posttraumatic stress disorder. Flattened affect, disinhibited or clingy behavior, loss of speech spontaneity, and verbal hyperspontaneity were also seen. The cerebellar cognitive affective syndrome (CCAS) following cerebellar resection in children is a loose complex with variable composition. The high percentage requiring special education reflects the severity and persistence of the cognitive deficits. [1]

COMMENT. The authors stress the need to inform parents and teachers about behavioral and cognitive sequelae following cerebellar surgery, so that timely social and educational intervention is made available. The cerebellum is now known to be involved in cognitive and emotional responses. A posterior fossa syndrome (PFS) is a neurobehavioral syndrome consisting of transient short-term deficits arising 1 to 6 days postoperatively and gradually resolving over months. Mutism and subsequent dysarthria (MSD) is the most common example of the PFS. The cerebellar cognitive affective syndrome (CCAS) as seen in adults consists of 4 major symptoms: disturbances of executive function, impaired spatial cognition, linguistic difficulties, and personality changes. In children following cerebellar tumor resection, CCAS is atypical but apparently more prevalent than previously reported.