Progressive cerebellar atrophy: hereditary ataxias and disorders with spinocerebellar degeneration

Progressive cerebellar atrophy: hereditary ataxias and disorders with spinocerebellar degeneration. Nicole I. Wolf, Michel Koenig; Handbook of Clinical Neurology, Volume 113, 2013, Pages 1869–1878; Pediatric Neurology Part III. http://dx.doi.org/10.1016/B978-0-444-59565-2.00057-5

The hereditary ataxias with onset in childhood are a group of heterogeneous disorders, usually with autosomal recessive inheritance. In many of them, magnetic resonance imaging (MRI) shows cerebellar atrophy. The most prominent exception to this is Friedreich's ataxia, where MRI shows normal cerebellar volume, but sometimes spinal cord atrophy.