The Cerebellum in Chiari Type II Malformation

Salman, Michael S.

doi:10.1159/000116728

Cited by 3 publications

(9 citation statements)

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“…Figure 1 summarizes findings from this investigation and other studies [17, 18, 22, 27–29] in relationship to normal cerebellar and posterior fossa development (left column). Since the rapid cerebellar growth starts after posterior fossa rapid growth, its development is restricted and adversely affected in CII by the already small size of the posterior fossa (see box with the bold and italics text on the right in Fig.…”

Section: Should Surgical Expansion Of the Posterior Fossa Be Done In mentioning

confidence: 62%

“…The most likely explanation for the midsagittal expansion of the vermis is tissue compression occurring within a small posterior fossa, which generates pressure that squeezes the vermis from either side towards the midline [17, 18]. Expansion of the anterior vermis lobules may also be related to the enlarged anterior cerebellar lobes reported recently in CII [13].…”

Section: Structural Changes In Chiari Type II Malformationmentioning

confidence: 99%

“…Early surgical expansion of the PF ( box with the star symbol ) is suggested to prevent or reduce the cerebellar atrophy in CII patients and facilitate a more physiological and unrestricted cerebellar growth. Information shown in this figure (not drawn to scale) is based on findings from this study and on information provided in references [17, 18, 22, 27–29]…”

Section: Figure and Tablementioning

confidence: 99%

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Posterior fossa decompression and the cerebellum in Chiari type II malformation: a preliminary MRI study

Salman

2011

Childs Nerv Syst

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Objectives-Chiari type II malformation (CII) is a congenital deformity of the hindbrain. The posterior fossa and cerebellum are small in CII. The cerebellar atrophy is associated with cognitive and motor deficits. Brainstem compression occurs in some patients with CII for whom posterior fossa decompression may be life saving. The aim was to determine whether posterior fossa decompression can prevent or reduce the cerebellar atrophy in CII.Methods-Cerebellar volumes and their tissue types (gray matter, white matter, and CSF volumes) from brain MRI were compared among four CII patients, aged 9.5 to 16.5 years, who had had posterior fossa decompression in infancy, 28 CII patients who had not had posterior fossa decompression, and ten age-matched normal controls. Parametric and non-parametric tests investigated group differences.Results-Compared to controls, mean cerebellar volume was significantly smaller in CII patients (p<0.0001). Mean CSF volume within the cerebellar fissures and fourth ventricle was significantly smaller in patients without posterior fossa decompression compared to the CII patients who had the decompression, p=0.043. Mean CSF volume of the latter group was similar to the controls. Other cerebellar volumetric measurements did not differ between the CII groups.Conclusions-Posterior fossa decompression normalizes CSF spaces within the posterior fossa in CII but does not prevent the cerebellar atrophy. The author proposes that surgical expansion of the posterior fossa should be considered in infants with CII who have a significantly small posterior fossa, to prevent or reduce the deficits associated with the cerebellar atrophy.

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Section: Should Surgical Expansion Of the Posterior Fossa Be Done In mentioning

confidence: 62%

Section: Structural Changes In Chiari Type II Malformationmentioning

confidence: 99%

Section: Figure and Tablementioning

confidence: 99%

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Posterior fossa decompression and the cerebellum in Chiari type II malformation: a preliminary MRI study

Salman

2011

Childs Nerv Syst

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“…However, this reduction is not uniform and appears to involve the cerebellar hemispheres more than the vermis. 57,58 In C2M, the sagittal surface of the cerebellar vermis is enlarged, not reduced compared to controls despite a reduction in volume of the posterior fossa. 57 This finding is best explained by the caudal herniation of the cerebellar vermis and tonsils and upward herniation of the superior vermis (Fig.…”

Section: Clinical Presentationmentioning

confidence: 96%

“…8). 57,58 The cerebellum completely fills the small posterior fossa with the cerebellar hemispheres wrapping or embracing the brainstem (Fig. 9).…”

Section: Clinical Presentationmentioning

confidence: 99%

Chiari Malformations and Syringohydromyelia in Children

Poretti¹,

Boltshauser

Huisman³

2016

Seminars in Ultrasound, CT and MRI

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The Cerebellar Dysplasia of Chiari II Malformation as Revealed by Eye Movements

Salman

Dennis

Sharpe

2009

Can. j. neurol. sci.

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Chiari type II malformation (CII) is one of the most prevalent congenital deformities of the hindbrain (brainstem and cerebellum). There is early mechanical compression on the developing hindbrain in CII, as well as additional effects caused by the accompanying hydrocephalus.1 One prevailing theory is that the deformity of CII occurs secondary to mechanical compression of hindbrain structures caused by a small posterior fossa.2 The small size of the posterior fossa in CII is proposed to be caused by cerebrospinal fluid (CSF) leak through the spinal defect in spina bifida meningomyelocele, which always accompanies CII.2 This leak prevents distension of the ABSTRACT: Introduction: Chiari type II malformation (CII) is a developmental deformity of the hindbrain. We have previously reported that many patients with CII have impaired smooth pursuit, while few make inaccurate saccades or have an abnormal vestibuloocular reflex. In contrast, saccadic adaptation and visual fixation are normal. In this report, we correlate results from several eye movement studies with neuroimaging in CII. We present a model for structural changes within the cerebellum in CII. Methods: Saccades, smooth pursuit, the vestibulo-ocular reflex, and visual fixation were recorded in 21 patients with CII, aged 8-19 years and 39 age-matched controls, using an infrared eye tracker. Qualitative and quantitative MRI data were correlated with eye movements in 19 CII patients and 28 controls. Results: Nine patients with CII had abnormal eye movements. Smooth pursuit gain was subnormal in eight, saccadic accuracy abnormal in four, and vestibulo-ocular reflex gain abnormal in three. None had fixation instability. Patients with CII had a significantly smaller cerebellar volume than controls, and those with normal eye motion had an expanded midsagittal vermis compared to controls. However, patients with abnormal eye movements had a smaller (non-expanded) midsagittal vermis area, posterior fossa area and medial cerebellar volumes than CII patients with normal eye movements. Conclusions: The deformity of CII affects the structure and function of the cerebellum selectively and differently in those with abnormal eye movements. We propose that the vermis can expand when compressed within a small posterior fossa in some CII patients, thus sparing its ocular motor functions. RÉSUMÉ: Mouvements oculaires révélant une dysplasie cérébelleuse dans la malformation de Chiari de type II.Contexte : La malformation de Chiari de type II (CII) est un défaut du développement du cerveau postérieur. Nous avons déjà rapporté que les patients porteurs de CII ont une atteinte de la poursuite oculaire lente et qu'un petit nombre ont des saccades imprécises ou un réflexe vestibulo-oculaire anormal. Par contre, l'adaptation saccadique et la fixation visuelle sont normales. Dans cet article, nous établissons la corrélation entre les résultats des études de mouvements oculaires et la neuroimagerie dans le CII. Nous présentons également un modèle de changements structurels dans le cerve...

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The Cerebellum in Chiari Type II Malformation

Cited by 3 publications

References 98 publications

Posterior fossa decompression and the cerebellum in Chiari type II malformation: a preliminary MRI study

Posterior fossa decompression and the cerebellum in Chiari type II malformation: a preliminary MRI study

Chiari Malformations and Syringohydromyelia in Children

The Cerebellar Dysplasia of Chiari II Malformation as Revealed by Eye Movements

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