• Peter Mariën, Lieven Verslegers, Maarten Moens, Guido Dua, Piet Herregods, and Jo Verhoeven.
• Department of Neurology and Memory Clinic, ZNA Middelheim General Hospital, Antwerp, Belgium. peter.marien5@telenet.be
• Cerebellum. 2013 Oct 1; 12 (5): 686-91.

AbstractPosterior fossa syndrome (PFS) due to vascular etiology is rare in children and adults. To the best of our knowledge, PFS due to cerebellar stroke has only been reported in patients who also underwent surgical treatment of the underlying vascular cause. We report longitudinal clinical, neurocognitive and neuroradiological findings in a 71-year-old right-handed patient who developed PFS following a right cerebellar haemorrhage that was not surgically evacuated. During follow-up, functional neuroimaging was conducted by means of quantified Tc-99m-ECD SPECT studies. After a 10-day period of akinetic mutism, the clinical picture developed into cerebellar cognitive affective syndrome (CCAS) with reversion to a previously learnt accent, consistent with neurogenic foreign accent syndrome (FAS). No psychometric evidence for dementia was found. Quantified Tc-99m-ECD SPECT studies consistently disclosed perfusional deficits in the anatomoclinically suspected but structurally intact bilateral prefrontal brain regions. Since no surgical treatment of the cerebellar haematoma was performed, this case report is presumably the first description of pure, "non-surgical vascular PFS". In addition, reversion to a previously learnt accent which represents a subtype of FAS has never been reported after cerebellar damage. The combination of this unique constellation of poststroke neurobehavioural changes reflected on SPECT shows that the cerebellum is crucially implicated in the modulation of neurocognitive and affective processes. A decrease of excitatory impulses from the lesioned cerebellum to the structurally intact supratentorial network subserving cognitive, behavioural and affective processes constitutes the likely pathophysiological mechanism underlying PFS and CCAS in this patient.

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