The Cerebellum in Children with Spina Bifida and Chiari II Malformation: Quantitative Volumetrics by Region

Juranek, Jenifer; Dennis, Maureen; Cirino, Paul T.; El-Messidi, Lyla; Fletcher, Jack Μ.

doi:10.1007/s12311-010-0157-x

Cited by 47 publications

(35 citation statements)

References 52 publications

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“…Because of the Chiari II malformation, the cerebellum and its connectivity to other brain regions is impaired in SBM ( Juranek et al, 2010). Hand function also reflects the cerebellar influences on precision and timing of fine motor movements.…”

Section: Dtt Volume and Integrity Of The CCmentioning

confidence: 99%

Structure, Integrity, and Function of the Hypoplastic Corpus Callosum in Spina Bifida Myelomeningocele

et al. 2014

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Although there are many studies of people with complete or partial hypogenesis of the corpus callosum (CC), little is understood about the hypoplastic CC in which all structures are present but thinned. Spina bifida myelomeningocele (SBM) is a model organism for such studies because many have either a hypogenetic or hypoplastic CC. We used diffusion tensor tractography (DTT) to evaluate the hypoplastic CC in SBM and its relation to interhemispheric functions and intelligence quotient (IQ). Participants were individuals with SBM and an intact or hypoplastic CC (n = 28), who were compared to a typically developing comparison group (n = 32). Total and regional DTT volume and integrity measures (fractional anisotropy, axial diffusivity, and radial diffusivity) of the CC were related to measures of intelligence (IQ), bimanual motor functioning, and dichotic auditory performance. As predicted, DTT showed variations in volume and integrity that were maximized in the entire CC and the posterior CC. IQ correlated with entire CC volume, anterior and posterior regional CC volumes, and also with measures of integrity. Bimanual motor functioning correlated with the anterior and posterior volumes of the CC but not with any integrity measures. Axial diffusivity in the posterior CC was negatively correlated with right ear dichotic listening performance. The hypoplastic CC is not macrostructurally or microstructurally intact in SBM, even when it appears radiologically intact. Both volume and integrity of the posterior regions were related to reductions in IQ and to interhemispheric processing. These findings may transfer to other disorders characterized by a hypoplastic CC.

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Section: Dtt Volume and Integrity Of The CCmentioning

confidence: 99%

Structure, Integrity, and Function of the Hypoplastic Corpus Callosum in Spina Bifida Myelomeningocele

et al. 2014

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“…These changes include malformations of the hindbrain and cerebellum that are the hallmarks of spina bifida, along with decreased WM integrity in limbic regions and impaired long-association pathways as measured by diffusion tensor imaging (DTI). 24,25 A small longitudinal study of children having acute hydrocephalus indicated that 7 of 10 cases displayed elevated fractional anisotropy (FA) in the posterior limb of the internal capsule and decreased FA in the genu of the corpus callosum. However, these increases in FA normalized after insertion of a ventriculoperitoneal shunt.…”

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Postshunt lateral ventricular volume, white matter integrity, and intellectual outcomes in spina bifida and hydrocephalus

Williams

Juranek²,

Stuebing

et al. 2015

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OBJECT No previous reports exist that have evaluated the relationships of white matter (WM) integrity with the number of shunt revisions, ventricular volume after shunting, and cognition in medically stable children who have spina bifida and hydrocephalus (SBH). The authors hypothesized that enlarged ventricles and a greater number of shunt revisions decrease WM integrity in children. METHODS In total, 80 children (mean age 13.7 years) who had SBH underwent MRI and IQ testing. Probabilistic diffusion tractography was performed to determine mean diffusion tensor imaging (DTI) metrics along the frontal and parietal tectocortical pathways. The DTI metrics were evaluated for significant correlation with a composite IQ measure and with the total number of shunt revisions and the total lateral ventricular volume obtained through semiautomated parcellation of T1-weighted MRI scans. RESULTS An enlargement in total lateral ventricle volume and an increase in the number of shunt revisions were both associated with higher fractional anisotropy (FA) and with lower radial diffusivity (RD) along both frontal and parietal tectocortical pathways. Children who had not undergone a shunt revision had on average a greater lateral ventricle volume and higher FA and lower RD along frontal and parietal pathways than those who had undergone multiple shunt revisions. The mean DTI metrics along parietal pathways predicted IQ scores, but intellectual ability was not significantly correlated with ventricular volume or with the number of lifetime shunt revisions. CONCLUSIONS Significant changes in DTI metrics were observed as a function of ventricular volume. An increased lateral ventricle volume was associated with elevated FA and decreased RD. Given that the participants were medically stable at the time of the MRI examination, the results suggested that those who have enlarged ventricles show a DTI pattern consistent with axonal compression due to increased intracranial pressure (ICP) in attenuated hydrocephalus. Although limited by a cross-sectional design, the study's findings suggest that DTI metrics may serve as sensitive indicators for chronic, mild hydrocephalus in the absence of overt clinical symptoms due to increased ICP. Having enlarged ventricles and undergoing multiple shunt revisions did not affect intellectual ability in children with SBH.

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“…However, they found that posterior fossa cerebrospinal fluid (CSF) volume improves and reaches to almost normal values after posterior fossa decompression as compared to normal individuals. In a recent investigation [3], total cerebellar volume was found to be significantly reduced in spina bifida myelomeningocele group as compared to the normal individuals. The results of this investigation coincide with the observations of the group, who proposes posterior fossa decompression in early infancy.…”

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confidence: 84%

Comment to the paper “Posterior fossa decompression and the cerebellum in Chiari type II malformation: A preliminary MRI study”

Selçukı

2010

Childs Nerv Syst

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Set of Chiari malformations, per se, comprise an ensemble of interesting clinical forms, which are sometimes totally different from each other in regard to the clinical symptoms and accompanying pathological conditions of the central nervous system. Embryological background and development stages still require further explanations. There is still no single treatment mode that is widely accepted. Although most of the neurosurgeons accept early surgical correction for the symptomatic cases, treatment modality for nonsymptomatic cases is still under debate. Chiari type I and type I.5 cases are totally different story [1,2]. The authors propose posterior fossa decompression in early infancy to prevent atrophy of cerebellum and other related posterior fossa structures in Chiari type II malformations. They calculated volumes of various structures in posterior fossa and came to a conclusion that cerebellar atrophy takes place in utero, and decompressive attempts do not help preventing cerebellar atrophy. However, they found that posterior fossa cerebrospinal fluid (CSF) volume improves and reaches to almost normal values after posterior fossa decompression as compared to normal individuals. In a recent investigation [3], total cerebellar volume was found to be significantly reduced in spina bifida myelomeningocele group as compared to the normal individuals. The results of this investigation coincide with the observations of the group, who proposes posterior fossa decompression in early infancy. The main question that suddenly rises in the mind is, while this posterior fossa decompression relieves the structures in the posterior fossa and causes an increase in CSF volume in Chiari type II cases, could this approach has any help to hydrocephalus in these patients, which is commonly attributed to the jam at foramen magnum level. The authors have found no improvement in cerebellar atrophy after decompressive surgery. On the other hand, this investigation showed that CSF volume (space as well) is augmented by posterior fossa decompression. The aim of all decompressive surgical attempts at posterior fossa is to relieve the level of foramen magnum and provide appropriate circulation of CSF for improvement of syringomyelia and brain stem compression. Although this kind of surgical approaches need more accumulated data related to the similar cases, in order to prevent forthcoming syringomyelia, to establish appropriate CSF circulation at level of foramen magnum, and to prevent brain stem compression, which can lead to lethal complications, this procedure may be helpful for the future prognosis of the patients. After the myelomeningocele operation, the timing of the posterior fossa decompression for Chiari II patients is another question to be answered as well.

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The Cerebellum in Children with Spina Bifida and Chiari II Malformation: Quantitative Volumetrics by Region

Cited by 47 publications

References 52 publications

Structure, Integrity, and Function of the Hypoplastic Corpus Callosum in Spina Bifida Myelomeningocele

Structure, Integrity, and Function of the Hypoplastic Corpus Callosum in Spina Bifida Myelomeningocele

Postshunt lateral ventricular volume, white matter integrity, and intellectual outcomes in spina bifida and hydrocephalus

Comment to the paper “Posterior fossa decompression and the cerebellum in Chiari type II malformation: A preliminary MRI study”

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