Rivista di Neuroradiologia 11 (Suppl 2), 1998 MRI Helps in the Early Diagnosis of Corticobasilar Degeneration M. GALLUCCFZ, M. BONAMINI 1 ,A. CATALUCCP, V. LEUZZP, C. MICHELP, M. CAULO ' ,E. SCARNATI 4 1 Dept. of Radiology; University of L'Aquila, 2 Dept. of Radiology, H. S. Raffaele; Rome, 3 Dept. of Child Neurology, University " La Sapienza"; Rome , ' Dept. of Physiology; Uni versity of L' Aquil a, Italy Key words: basal ganglia, diseases, degenerative disease, magnetic resonance SUMMARY - We describe MR findings in patients with a clinical diagnosis of corticobasal degeneration (CBD ). Five patients with different extrapyramidal symptoms were submitted to M RI. All patients were clinically followed-up for a minimum period of at least 12 months. MRI study was performed on a 0.5 T system (Philips NT 5 !!), using conventional SE, TSE and t-FLAIR sequences on axial and coronal planes. In all cases MR showed bilateral parietal cortical atrophy. t-FLAIR sequences demonstrated cortical signal alteration on both parietal and frontal cortex in four cases and on the parietal cortex alone in 1 case. During the follow-up clinical evolution gave rise to the suspicion of CBD. MRI demonstrated parietal cortex atrophy even in those cases in which symptoms at onset were not specific for CBD, thus helping the diagnosis. Moreover MRI, using t-FLAIR sequences suggested CBD diagnosis even in the earlier phases of the diseas e, in which cortical damage has not yet become evident as atrophy. Introduction Corticobasal degeneration (CBD) is a relatively rare neurodegenerative disease recently defined on the basis of the association between movement dis- orders of extrapyramidal type and cortical parietal symptoms (apraxia andlagnosia)7" 10 • Clinical features do not always enable differential diagnosis with other neurodegenerative disorders such as Parkinson disease, Multiple System Atrophy, Progressive Supranuclear Palsy and Pick's disease<, especially in the early stages of clinical manifesta- tions. Neuropathological studies demonstrated cortical atrophy, mostly involving fronto-parietal cortex, asso- ciated to swollen pale achromatic neurons, this last finding being similar to changes reported in and Pick's disease 1. 6 • This aspect often led to consider CBD as a variant of Pick's disease 7 • Neuronal loss and gliosis in the basal ganglia have been also reported 1 • MRI studies demonstrated cortical atrophy selec- tively involving fronto-parietal regions; however, no intracortical signal changes have ever been described using conventional sequences 9 • This paper describes T2-weighted signal alter- ations selectively involving the cortex, registered on t-FLAIR sequences, in 5 patients affected by CBD. Materials and Methods Five patients ( 4F, 1M, age 24 to 64 yrs) were studied. In 1 case clinical CBD diagnosis was suspected at the time of MR examination; 2 cases were affected by dif- fuse dystonia; 2 cases had a parkinsonian syndrome non responsive to L-dopa administration. All patients were clinically followed-up for a minimum period of at least 12 months. Evolution of symptoms in the last 4 patients suggested clinical diagnosis of CBD during the follow-up. MR was performed using a 0.5 T unit. Conventional SE Tl-w (550125 TRITE), TSE T2-w (30001120 TRITE), t-FLAIR (60001 15012000 TR/ TEI- TI) sequences at a 5 mm slice thickness, on axial and coronal planes were performed. Results In all patients MR showed bilateral parietal cortex atrophy. On t-FLAIR sequences cortical hyperinten- sity was found in parietal and frontal cortex in 4 pa- tients, and in parietal cortex alone in 1 (table 1). No other MR changes were found in supratento- rial or subtentorial areas. Discussion and Conclusions T-FLAIR sequences, using both a long TI and a long TE, produce strongly T2-weighted images with 13