Neurobehavioral alterations in an adolescent following posterior fossa tumor resection

Abstract

The posterior fossa syndrome (PFS) consists of a variety of symptoms, including cerebellar mutism, behavioral disturbances and personality changes. We report longitudinal clinical, neuroradiological and neurobehavioral findings in a 19-year-old left-handed patient, diagnosed with attentional deficit hyperactivity disorder (ADHD) at the age of 12, who underwent posterior fossa tumor resection. Although the patient did not develop cerebellar mutism after surgery, marked apathy and emotional indifference, urinary retention, eye-lid apraxia and visual hallucinosis became apparent after a brief interval of normal functioning. Based on these findings it is argued that the PFS might be considered a semiological heterogeneous condition with variable clinical expressions. Long-term follow-up investigations revealed subtle, but significant cognitive and affective deficits, resembling the cerebellar cognitive affective syndrome in adults. As demonstrated by functional neuroimaging studies with SPECT, symptoms were associated with perfusional deficits in the anatomoclinically suspected supratentorial regions, reflecting the distant impact of the cerebellum on cognitive and affective functions.

Memory impairment following right cerebellar infarction: a case study

“…Some functional magnetic resonance imaging (MRI) studies revealed the cerebello-cortical pathway with multiple fiber tracts that terminate within the regions of the contralateral prefrontal cortex (O'Halloran et al, 2012). According to previous reports, cognitive impairments caused by a cerebellar lesion have been often explained by the functional suppression of the contralateral frontal lobe (Baillieux et al, 2006;Cooper et al, 2012;Lagarde, Hantkie, Hajjioui, & Yelnik, 2009;Mariën et al, 2008Mariën et al, , 1996Neau, Arroyo-Anllo, Bonnaud, Ingrand, & Gil, 2000;Peterburs, Bellebaum, Koch, Schwarz, & Daum, 2010;Suenaga et al, 2008).…”

Long-term sequelae after acquired pediatric hemorrhagic cerebellar lesions

“…Symptom-lesion mapping, neuroanatomical and functional neuroimaging studies provide further indications that the cerebellum is involved in cognitive functioning [17][18][19][20][21][22][23][24]. Some studies have even found connections between lesions of the posterior lateral cerebellar hemispheres and higher order cognitive problems, whereas injuries involving the vermis of the cerebellum are accompanied by dysregulation of affect [25][26][27][28].…”

“…Despite the fact that neurocognitive problems after acquired pediatric cerebellar lesions have been described, reports about the emergence of cerebellar cognitive affective syndrome in this population are scarce [28][29][30][31]. Nevertheless, many authors stress the importance of an intact cerebellum for the development of neuropsychological functions [24,[32][33][34].…”

“…The majority of pediatric studies focusing on cognition after acquired cerebellar lesions have mainly recruited patients suffering from oncological diseases. Neoplastic pediatric cerebellar lesions have repeatedly been found to be associated with different neuropsychological problems [24,28,29,32,39].…”

Cerebellocerebral Diaschisis and Postsurgical Posterior Fossa Syndrome in Pediatric Patients

“…As consistently reflected by CCD, the distant metabolic impact of surgical damage to the cerebellum via the dentatorubrothalamic tract on the supratentorial brain regions crucially involved in language dynamics and behavioral and affective regulation was later confirmed in an adolescent patient as well as in a larger study of 5 children with PFS. 7,8 The authors infer from the correlation between PFS symptoms and perfusional changes within the frontal lobe regions that "mutism in PFS is probably a speech apraxia rather than a simple dysarthria, dysphasia, or other cerebellar speech disorder. "…”