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Abstract #2300

Divergent trajectory of age-related cerebellar volume change in children with ataxia telangiectasia

Rob A. Dineen1, Stefan Pszczolkowski1, Felix Raschke2, Hannah McGlashan1, Manish Prasad3, Gabriel Chow3, William Whitehouse4, and Dorothee P. Auer1

1Radiological Sciences, University of Nottingham, Nottingham, United Kingdom, 2Radiotherapy and Radiation Oncology, University Hospital Carl Gustav Carus of the Technische Universit├Ąt Dresden, Dresden, Germany, 3Paediatric Neurology, Nottingham University Hospitals NHS Trust, Nottingham, United Kingdom, 4Division of Child Health, University of Nottingham, Nottingham, United Kingdom

Cerebellar atrophy occurs in the inherited neurodegenerative syndrome Ataxia Telangiectasia (A-T) but the trajectory of cerebellar atrophy across childhood and its relationship to clinical status is unknown. We report cerebellar and fourth ventricular volumes (normalized to intracranial volume) from 24 children with A-T and 24 matched controls. Cerebellar volume declined linearly with increasing age in the A-T group with a divergent trajectory of age-related cerebellar volume change compared to normally-developing children. Fourth ventricular volume increased with age in children with A-T and correlated with clinical status, and may provide an imaging marker of neurological status in childhood A-T.

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