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The posterior fossa syndrome (PFS) consists of transient cerebellar mutism, cognitive symptoms, and neurobehavioral abnormalities that typically develop in children following posterior fossa (PF) tumor resection. The pathophysiological substrate of the syndrome remains unclear. We investigated eight children of whom five presented with a variety of clinically relevant non-motor language symptoms associated with cognitive and behavioral disturbances after PF tumor resection. Four children developed transient cerebellar mutism followed by dysarthric speech. Non-motor language symptoms consisted of agrammatism, anomia, impaired verbal fluency, comprehension deficits, and a spontaneous speech. Neurocognitive deficits included executive dysfunctions, concentration deficits, and visuo-spatial disorders. In addition, all children presented with behavioral and affective disturbances. Functional neuroimaging studies during the phase of mutism by means of SPECT showed perfusional deficits in the anatomo-clinically suspected supratentorial areas subserving language dynamics, syntax, naming, executive functioning, affective regulation, and behavior. A significant improvement of frontal perfusional deficits paralleled the clinical remission of mutism. These results add to the view that the PFS might represent a cerebello-cerebral diaschisis phenomenon, reflecting the metabolic impact of the cerebellar lesion on supratentorial cognitive and affective functions.

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