Pontine atrophy precedes cerebellar degeneration in spinocerebellar ataxia 7: MRI-based volumetric analysis

Journal of Neurology, Neurosurgery, and Psychiatry
O Y BangKyoon Huh

Abstract

Spinocerebellar ataxia 7 (SCA7) is characterised by cerebellar ataxia and visual loss. The aim of the present study was to elucidate the magnetic resonance imaging (MRI) findings characteristic of patients with SCA7. Twenty patients with SCA (eight SCA3, three SCA6, and nine SCA7) and 20 control subjects underwent an MRI-based volumetric analysis. The pontine volume in patients with SCA7 was decreased by a greater amount than in patients with other types of SCA (p<0.01), whereas the cerebellar volume was not different from that in other types of SCA (p>0.05). Pontine atrophy was a consistent finding in all patients with SCA7 regardless of the degree of cerebellar atrophy or the severity or duration of illness. In contrast, cerebellar atrophy was not found in those with a short duration of illness or mild ataxia, but became prominent as the severity and duration of illness progressed. Our study suggests that neurodegeneration is ongoing during the life of individuals with SCA7, and that the primary pathology in these individuals involves the brainstem rather than the cerebellum. In addition, pontine atrophy is a prominent, consistent finding in SCA7, and may help in establishing the clinical diagnosis of SCA7.

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Citations

Apr 18, 2008·The Cerebellum·Susanne DöhlingerJörg B Schulz
Aug 19, 2011·The Cerebellum·Brian C JungSarah H Ying
Aug 9, 2013·Movement Disorders : Official Journal of the Movement Disorder Society·Carlos R Hernandez-CastilloJuan Fernandez-Ruiz
Jun 13, 2015·Neurology·Roderick P P W M MaasBart P C van de Warrenburg
Apr 29, 2015·Brain Imaging and Behavior·Carlos R Hernandez-CastilloJuan Fernandez-Ruiz
Jun 1, 2011·Arquivos de neuro-psiquiatria·Sarah Teixeira CamargosAntonio Carlos dos Santos
Aug 15, 2017·Expert Review of Neurotherapeutics·Lidia SarroCaterina Mariotti
Jan 1, 2014·Cerebellum & Ataxias·Carlos R Hernandez-CastilloJuan Fernandez-Ruiz
Jul 14, 2020·Medicinal Research Reviews·Epaminondas Doxakis
Mar 19, 2021·Neuroradiology·Sirio CocozzaArturo Brunetti
Jul 3, 2021·AJNR. American Journal of Neuroradiology·C R Hernandez-CastilloJ Fernandez-Ruiz

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