Article

Posterior fossa syndrome after a vermian stroke: a new case and review of the literature.

Faculty of Linguistics, Vrije Universiteit Brussel, Brussels, Belgium.
Pediatric Neurosurgery (impact factor: 0.7). 02/2007; 43(5):386-95. DOI:10.1159/000106388 pp.386-95
Source: PubMed

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.

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    Article: Cerebellar mutism
    Thora Gudrunardottir, Astrid Sehested, Marianne Juhler, Kjeld Schmiegelow
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    ABSTRACT: PurposeCerebellar mutism is a common complication of posterior fossa surgery in children. This article reviews current status with respect to incidence, anatomical substrate, pathophysiology, risk factors, surgical considerations, treatment options, prognosis and prevention. MethodsWe reviewed all peer-reviewed English publications on cerebellar mutism between the years of 1985 and 2009. The majority were found by searching for ‘cerebellar mutism’ and ‘posterior fossa syndrome’ in PubMed. Additional cases were identified by cross-checking reference lists. ResultsThe overall incidence of postoperative cerebellar mutism is 11–29%, and patients with medulloblastomas and/or brainstem invasion are at a greater risk of developing it than those with other kinds of tumors and/or without brainstem invasion. Permanent sequelae in the form of both motor- and non-motor-related speech deficits are common, especially when the right cerebellar hemisphere is involved. The mutism is caused by bilateral pertubation of the dentate nuclei and their efferent pathways, which emphasizes the need to explore surgical methods that spare these structures. The pathophysiological mechanisms of delayed onset and resolution of cerebellar mutism are not clear, but axonal damage, edema, perfusional defects and metabolic disturbances may be involved. ConclusionThe incidence of cerebellar mutism is well documented in children with medulloblastoma, but precise figures for those with astrocytoma and ependymoma are lacking. Further anatomical, functional imaging and neuropsychological studies are needed to clarify the pathophysiological mechanisms in order to define preventive measures during surgery. Randomized, controlled trials of the effects of different medication and post-operative speech therapy are necessary for improving treatment. KeywordsCerebellar mutism–Brain tumor–Posterior fossa surgery–Children–Speech disorder–Review
    Child s Nervous System 04/2012; 27(3):355-363. · 1.54 Impact Factor
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    Article: Cerebellar mutism: review of the literature.
    Thora Gudrunardottir, Astrid Sehested, Marianne Juhler, Kjeld Schmiegelow
    [show abstract] [hide abstract]
    ABSTRACT: Cerebellar mutism is a common complication of posterior fossa surgery in children. This article reviews current status with respect to incidence, anatomical substrate, pathophysiology, risk factors, surgical considerations, treatment options, prognosis and prevention. We reviewed all peer-reviewed English publications on cerebellar mutism between the years of 1985 and 2009. The majority were found by searching for ‘cerebellar mutism’ and ‘posterior fossa syndrome’ in PubMed. Additional cases were identified by cross-checking reference lists. The overall incidence of postoperative cerebellar mutism is 11-29%, and patients with medulloblastomas and/or brainstem invasion are at a greater risk of developing it than those with other kinds of tumors and/or without brainstem invasion. Permanent sequelae in the form of both motor- and non-motor-related speech deficits are common, especially when the right cerebellar hemisphere is involved. The mutism is caused by bilateral pertubation of the dentate nuclei and their efferent pathways, which emphasizes the need to explore surgical methods that spare these structures. The pathophysiological mechanisms of delayed onset and resolution of cerebellar mutism are not clear, but axonal damage, edema, perfusional defects and metabolic disturbances may be involved. The incidence of cerebellar mutism is well documented in children with medulloblastoma, but precise figures for those with astrocytoma and ependymoma are lacking. Further anatomical, functional imaging and neuropsychological studies are needed to clarify the pathophysiological mechanisms in order to define preventive measures during surgery. Randomized, controlled trials of the effects of different medication and post-operative speech therapy are necessary for improving treatment.
    Child s Nervous System 11/2010; 27(3):355-63. · 1.54 Impact Factor
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    Article: Posterior fossa syndrome after posterior fossa surgery in children with brain tumors.
    Serhan Küpeli, Bilgehan Yalçın, Burçak Bilginer, Nejat Akalan, Pınar Haksal, Münevver Büyükpamukçu
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    ABSTRACT: Posterior fossa syndrome (PFS) is defined as the temporary and complete loss of speech after posterior fossa surgery. The goal of this study was to identify incidence and risk factors for PFS and to determine accompanying neurobehavioral and psychologic problems. Between May 2007 and April 2009, children with brain tumors having posterior fossa surgery were evaluated neurologically and psychologically in preoperative and postoperative period. PFS developed in 9 patients among 36 (25%) included in the study. Mutism continued for 120 days in one patient. Histopathological diagnosis (P = 0.05), location of the tumor (P = 0.05) and socioeconomic level of the family (P = 0.06) gave the significant results in relation with the PFS by univariate analyses. In multivariate analysis the risk of developing PFS was found 7.2 times higher in patients with medulloblastoma, 6.7 times higher in tumors located at the midline, 5.7 times higher in families with low socioecnomic level. Intelligence quotients of the patients in PFS and other group (P = 0.85) with Wechsler Intelligence Scale for Children and the results of the Denver II Developmental Screening Test were not significant statistically (P = 0.5). The diagnosis of medulloblastoma, midline location of the tumor and low socioeconomic level of the families are important risk factors for the development of PFS. These findings support the hypothesis that temporary ischemia and edema due to retracted and manipulated dentate nuclei and superior cerebellar pedincles may be the cause of mutism.
    Pediatric Blood & Cancer 02/2011; 56(2):206-10. · 1.89 Impact Factor
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Keywords

apparent
 
AVM
 
emotional state
 
emotional symptoms
 
focal nonsurgically induced cerebellar damage
 
irritability
 
limited number
 
nontumoral etiology
 
personality changes
 
PFS
 
posterior fossa surgery
 
posterior fossa syndrome
 
postoperative course
 
rupture
 
transient cerebellar mutism
 
well-known clinical consequence