Specific cerebellar and cortical degeneration correlates with ataxia severity in spinocerebellar ataxia type 7.
Spinocerebellar ataxia type 7 (SCA7) is a progressive neurodegenerative disorder that is accompanied by loss of motor control and macular degeneration. Previous studies have shown cerebellar and pons atrophy as well as functional connectivity changes across the whole brain. Although different MRI modalities have been used to study the degenerative process, little is known about the relationship between the motor symptoms and cerebral atrophy. Twenty-four patients with molecular diagnosis of SCA7 where invited to participate in this study. Ataxia severity was evaluated using the scale for the assessment and rating of ataxia (SARA). Structural magnetic resonance imaging (MRI) brain images were used to obtain the grey matter volume of each participant. As expected, we found a significant negative correlation between the SARA score and the grey matter volume in distinct regions of the cerebellum in the patient group. Additionally, we found significant correlations between the ataxia degree and the degeneration of specific cortical areas in these patients. These findings provide a better understanding of the relationship between gray matter atrophy and ataxia related symptoms that result from the SCA7 mutation.
Consejo Nacional de Ciencia y Tecnología - Cátedras - Instituto de Neuroetologia, Universidad Veracruzana, Xalapa, México.
Carlos R
CR
Hernandez-Castillo
Instituto de Neuroetologia, Universidad Veracruzana, Xalapa, México.
Victor
V
Galvez
Departamento de Fisiología, Facultad de Medicina, Universidad Nacional Autónoma de México, Distrito Federal, México.
Rosalinda
R
Diaz
Instituto de Neuroetologia, Universidad Veracruzana, Xalapa, México. jfr@unam.mx.|Departamento de Fisiología, Facultad de Medicina, Universidad Nacional Autónoma de México, Distrito Federal, México. jfr@unam.mx.
Juan
J
Fernandez-Ruiz
D000328:Adult; D001284:Atrophy; D002531:Cerebellum / Q000000981:diagnostic imaging* / Q000473:pathology; D002540:Cerebral Cortex / Q000000981:diagnostic imaging* / Q000473:pathology; D005260:Female; D066128:Gray Matter / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D007091:Image Processing, Computer-Assisted / Q000379:methods; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D009929:Organ Size; D012720:Severity of Illness Index; D020754:Spinocerebellar Ataxias / Q000000981:diagnostic imaging* / Q000473:pathology
Cerebellum; Motor impairment; Precentral gyrus; Spinocerebellar ataxia; VBM