title: MR imaging and spectroscopy in Lhermitte-Duclos disease.
abstract: Lhermitte-Duclos disease is a rare abnormality occurring in the cerebellum with only 130 cases reported in the literature. There is debate as to whether this abnormality is a hamartoma, a malformation or a tumour. In this case report we discuss the spectroscopy findings from two patients presenting with this disease. The patients, one 40-year-old Caucasian woman with a 6-year history of headaches, unsteady gait and falls, deterioration in vision and another 28-year-old Caucasian man with a 1-year history of headaches and a previous history of a transient stroke, were found to have this lesion in the cerebellum. Proton spectroscopic data were obtained using a single-voxel PRESS technique (TE=135 ms, TR=1600 ms), from the region of the abnormality. The results were expressed as ratios under the three prominent resonances representing choline (Cho), creatine (Cr), and N-acetyl (NA) moieties. The metabolite ratios were compared to normative data. The two cases demonstrated reduced ratios in NA/Cho and NA/Cr in relation to the controls. The ratios of Cho/Cr appeared closer to the normal mean ratio. There were peaks attributable to lactate in both cases. The low NA/Cr and NA/Cho ratios could be due to the apparent lack of neuronal architecture and the presence of embryonic neural tissue, which does not express NA, indicating more favourably towards a 'benign' hamartoma rather than a tumour.
authors.affiliation: Academic Unit of Radiology, Royal Hallamshire Hospital, Glossop Road, Sheffield S10 2JF, UK. s.nagaraja@shef.ac.uk
authors.forename: S
authors.initials: S
authors.lastname: Nagaraja
authors.forename: T
authors.initials: T
authors.lastname: Powell
authors.forename: P D
authors.initials: PD
authors.lastname: Griffiths
authors.forename: I D
authors.initials: ID
authors.lastname: Wilkinson
mesh_terms: D000328:Adult; D002528:Cerebellar Neoplasms / Q000175:diagnosis*; D002531:Cerebellum / Q000473:pathology*; D005260:Female; D005729:Ganglioneuroma / Q000175:diagnosis*; D006222:Hamartoma / Q000175:diagnosis; D006801:Humans; D008279:Magnetic Resonance Imaging; D009682:Magnetic Resonance Spectroscopy; D008297:Male