Friday, August 5, 2016

The Pediatric Cerebellum in Inherited Neurodegenerative Disorders: A Pattern-recognition Approach

Susan I. Blaser, Maja Steinlin, Almundher Al-Maawali, Grace Yoon, Neuroimaging Clinics of North America, Volume 26, Issue 3, August 2016, Pages 373-416, ISSN 1052-5149, doi:10.1016/j.nic.2016.03.007.

FRIEDREICH ATAXIA IS THE PROTOTYPE FOR NEURODEGENERATIVE DISORDERS WITH PREDOMINANT SPINAL CORD ATROPHY:
Assessment of the upper cervical cord is predominantly useful in the evaluation of patients with Friedreich ataxia (FRDA/FXN), in whom cord thinning caused by neuronal loss in the spinal ganglia and Clarke column may be the first imaging clue to the disorder.
Involvement of the cerebellum was initially considered a rare feature in FRDA, however, volumetric analysis of the cerebellum in FRDA confirms volume loss in the rostral vermis, dorsal medulla, the dentate nuclei, the peridentate white matter, and the associated superior cerebellar peduncle.