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.
Background: The cerebellum was traditionally considered to be exclusively involved in the coordination of voluntary movement, gait, posture, balance and motor speech. However, this view was challenged by recent neuroanatomical, neuroimaging and clinical findings, providing preliminary evidence of a cerebellar contribution to linguistic functioning. Aim: To discuss the role of the cerebellum in a variety of linguistic functions and to explore the underlying mechanisms. Methods: A literature search was conducted via electronic databases. Exclusion criteria were: disorders following congenital cerebellar lesions, motor speech disorders, cognitive deficits outside the language sphere, neuropsychiatric disorders and insufficient information on the cerebellar role in language. Abstracts were not included. In addition, only adult subjects were taken into consideration. Results: A variety of linguistic disorders were found to occur following acquired cerebellar lesions: (1) impaired phonological and semantic fluency; (2) agrammatism (at morphological and sentence level); (3) naming and word finding difficulties; (4) cerebellar-induced aphasia; (5) reading difficulties; (6) writing problems, and (7) higher-level language deficits, including disturbed listening comprehension, impaired language proficiency and metalinguistic ability. Several hypotheses have been suggested to explain the nature of the cerebellar contribution to language. However, findings are not univocal. Conclusion: The cerebellum appears to be involved in a variety of linguistic functions. However, the precise nature of this contribution is not clear yet. Linguistic, neuroimaging, neuroanatomical and neuropsychological studies should be combined in order to disentangle the specific contribution of the cerebellum to linguistic processing.
The posterior fossa syndrome (PFS) is a well-known clinical consequence of posterior fossa surgery that has only been reported in a limited number of cases with a nontumoral etiology. It consists of transient cerebellar mutism, behavioral abnormalities and personality changes. We describe a 12-year-old child who developed transient cerebellar mutism associated with behavioral and emotional symptoms following rupture of a vermis arteriovenous malformation (AVM). Following the stroke, the girl experienced a 24-hour symptom-free interval. After that, she became mute and her emotional state was characterized by severe anxiety, irritability and withdrawal. After 3 days, mutism resolved and dysarthria became apparent. Two weeks after stroke, the AVM was surgically removed and the postoperative course was uneventful. This case is the first reported in which the PFS occurred after focal nonsurgically induced cerebellar damage.
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