Cerebellar Mutism, Posterior Fossa Syndrome
Conditions
Brief summary
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.
Interventions
DIAGNOSTIC_TESTAdvanced MRI sequences
Structural, diffusion and perfusion MRI sequences
Sponsors
Great Ormond Street Hospital for Children NHS Foundation Trust
Study design
Observational model
CASE_CONTROL
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
No minimum to 18 Years
Healthy volunteers
Yes
Inclusion criteria
Age \<18 Referred and operated upon within study period Brain tumour of cerebellum, IVth ventricle, brainstem undergoing craniotomy for resection (including re-do surgery) No restrictions as to tumour histology or grade (embryonal tumour / glioma / ependymoma) Informed consent given by parents
Exclusion criteria
Non-neoplastic infratentorial lesions Claustrophobia Contraindication to MRI Pregnant female Scans missing at \>2 timepoints Tumour resection surgery at another hospital if no pre- or post-operative scans available
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Diffusion MRI tractography | 1 year | 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. |
| Arterial Spin Labeling Perfusion MRI | 1 year | To compare cerebral blood flow (a metric derived from perfusion MRI) in frontal lobe regions between patients with and without pCMS. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Clinical measure of severity of pCMS | 1 year | 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 |
Countries
United Kingdom
Outcome results
None listed