Imaging of corticobasal degeneration syndrome

Introduction Diagnosing corticobasal degeneration is often difficult on the basis of clinical symptoms and radiological images. We aimed to clarify the imaging findings of corticobasal degeneration syndrome (CBDS). Methods Included in the study were 16 patients (8 men, 8 women, 46-75 years old) with clinically diagnosed CBDS. We evaluated the patients' symptoms and signs, and MR and single-photon emission CT (SPECT) imaging findings. Results All the patients had cerebral atrophy. Asymmetric cerebral atrophy was observed in 13 patients (81%) predominantly contralateral to the side clinically more affected. Atrophy in the cerebral peduncle was observed in seven patients. FLAIR images showed hyperintensity in the subcortical white matter in the frontoparietal lobes in the clinically more affected side in 14 patients, and in the rolandic region in 13 patients. Asymmetric hypoperfusion in the frontoparietal lobes on SPECT images was observed in all of the patients, and in the basal ganglia in 11 patients. Conclusion CBDS might be unique in showing hyperintensity in the subcortical white matter in the rolandic region on FLAIR images with asymmetric atrophy predominantly contralateral to the side clinically more severely affected. Asymmetric atrophy in the cerebral peduncle without signal abnormalities was also characteristic of CBDS. Atrophy in the midbrain tegmentum was also seen in patients with CBDS.