Posterior fossa syndrome after a vermian stroke: a new case and review of the literature.
ABSTRACT 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.
- SourceAvailable from: onlinelibrary.wiley.com[Show abstract] [Hide abstract]
ABSTRACT: Posterior fossa strokes account for about 10% of ischaemic strokes in children. Although motor and dysautonomic symptoms are common, to our knowledge cognitive and affective deficits have not been described in the paediatric literature. Our aim, therefore, was to describe these symptoms and deficits. In a retrospective study, we included all cases of posterior fossa strokes in children occurring at a single centre between 2005 and 2007, and investigated cognitive and affective deficits. Five males aged 3 to 14 years met the inclusion criteria. They all presented very early with mood disturbances: outbursts of laughter and/or crying and alternating agitation or prostration that disappeared spontaneously within a few days. Persistent cognitive deficits were also diagnosed in all five: initial mutism, then anomia, followed by comprehension deficiency and deficiencies of planning ability, visual-spatial organization, and attention. Despite early and intensive rehabilitation, recovery from these cognitive deficits was slow and sometimes incomplete, and on follow-up they proved to be more disabling than the motor symptoms. These findings are similar to the cerebellar cognitive affective syndrome described in adults, and quite similar to the language and affective deficits observed in children after surgery for posterior fossa tumour. This is consistent with the role of the cerebellum and brainstem in affective and cognitive processes from early development.Developmental Medicine & Child Neurology 03/2010; 52(7):626-31. · 2.68 Impact Factor
- [Show abstract] [Hide abstract]
ABSTRACT: Although the posterior fossa syndrome (PFS) can be considered as an aetiologically heterogeneous condition affecting children and adults, it most often occurs in paediatric patients after cerebellar tumour surgery. In patients with a tumoural aetiology, the syndrome is typically characterised by a short symptom-free postoperative period followed by mutism of variable duration and behavioural and affective changes. More than 200 paediatric cases have been described but reports of adult patients are extremely rare. This paper discusses PFS in adults on the basis of a comprehensive literature survey and describes the pre- and postoperative findings in a new adult patient. In the preoperative phase, cognitive, behavioural and affective abnormalities were identified, matching a diagnosis of cerebellar cognitive affective syndrome (CCAS) (Schmahmann and Sherman, 1998; Schmahmann, 2004). The immediate postoperative course was characterised by prefrontal-like behavioural and affective abnormalities, peduncular hallucinations and confusion evolving to psychosis. Akinetic mutism subsequently developed, lasted for 12 days and then alternated with episodes of diminished responsiveness in which pathological laughing and crying (PLC) occurred. Akinetic mutism resolved after treatment with a non-ergoline dopamine-agonist but CCAS persisted during longitudinal follow-up. From a semiological point of view "relapsing-remitting akinetic mutism" and PLC in our patient might add relevant information to current insights in the clinical expression of the PFS. As evidenced by a close parallelism between single photon emission computed tomography (SPECT) and clinical findings, CCAS as well as PFS seem to reflect functional disruption of the cerebello-cerebral network involved in cognitive, behavioural and affective functions. These findings may indicate that both syndromes share overt semiological resemblances and a common pathophysiological substrate. Consequently, CCAS and PFS may both be regarded as cerebellar-induced clinical conditions showing different aspects of a spectrum that range in degree of severity and symptom duration.Cortex 07/2011; · 6.16 Impact Factor
Article: Cerebellar mutism.[Show abstract] [Hide abstract]
ABSTRACT: Cerebellar mutism occurs in about 25% of children following posterior fossa tumor surgery. It is usually accompanied by other neurological and behavioral disturbances. Mutism is transient in nature lasting several days to months and is frequently followed by dysarthria. In addition, impairment of language and other neuropsychological functions can be found after long term follow up in the majority of patients. The pathophysiological background of mutism may be higher speech dysfunction mediated by crossed cerebello-cerebral diaschisis which is frequently found during the mute period. Foremost injury to the bilateral dentatothalamocortical tract appears to be critical for the development of cerebello-cerebral diaschisis and subsequent mutism. Direct cerebellar injury is the likely reason for persisting deficits after the mute period. Minimization of injury to the dentatothalamocortical tract during surgery may be promising in the prevention of mutism. While there is no established treatment of mutism, early speech and rehabilitation therapy is recommended.Brain and Language 02/2013; · 3.39 Impact Factor