Post-operative paediatric cerebellar mutism syndrome (pCMS) is a well-recognised complication of resective surgery for brain tumours of the cerebellum and fourth ventricle in children. Occurring in around 25% of infratentorial craniotomies, it is characterised by a delayed onset of mutism and emotional lability, and may comprise motoric and cognitive cerebellar deficits. Transient mutism gives way to prolonged, and often incomplete, recovery. Neuroimaging studies are beginning to reveal anatomical and functional aberrancies in the brain of children with pCMS. The cerebellar efferent pathways are likely to be implicated as a neuroanatomical substrate in the development of pCMS, as shown by a handful of diffusion tractography studies to date. However, the pathophysiology of this condition still remains unclear. Hypoperfusion of supratentorial cortical and subcortical structures may mediate the speech and behavioural deficits seen in pCMS, and is a candidate for a causal pathophysiological mechanism. This study aims to prospectively image children with pCMS using advanced MRI techniques including diffusion tractography and arterial spin labelling, and to correlate this with clinical descriptions of the syndrome. All children referred to Great Ormond Street Hospital for Children with a posterior fossa brain tumour will be imaged pre-operatively, post-operatively and at delayed follow-up. In tandem with this, clinical assessments will be made of children post-operatively to ascertain which patients develop pCMS. In addition, anonymised advanced MRI data on healthy controls will be used as a comparator group.
Study Type
OBSERVATIONAL
Enrollment
82
Structural, diffusion and perfusion MRI sequences
Great Ormond Street Hospital for Children
London, United Kingdom
Diffusion MRI tractography
To compare diffusion MRI derived tractography of the fronto-cerebellar circuitry (and associated metrics of fractional anisotropy and mean diffusivity) between patients with and without pCMS.
Time frame: 1 year
Arterial Spin Labeling Perfusion MRI
To compare cerebral blood flow (a metric derived from perfusion MRI) in frontal lobe regions between patients with and without pCMS.
Time frame: 1 year
Clinical measure of severity of pCMS
CMS Severity Score (Robertson 2006 JNS Peds 105: 444-451): Clinical diagnosis of pCMS Yes / No If yes: Time of onset 1. immediately post op 2. Days 1-2 3. Days 2-4 4. =\> Day 4 Mutism 1. Mild (\<1wk) 2. Moderate (1-4wks) 3. Severe (\>4wks) Ataxia 1. Mild (\<1wk) 2. Moderate (1-4wks) 3. Severe (\>4wks) Hypotonia 1. Mild (can sit or stand by \<1wk) 2. Moderate (can sit or stand by 1-4wks) 3. Severe (can sit or stand by \>4wks) Irritability 1. Mild (\<1wk) 2. Moderate (1-4wks) 3. Severe (\>4wks) Severe = at least 2 severe features Moderate = at least 2 moderate features or 1 moderate and 1 severe Mild = anything less than above
Time frame: 1 year
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