Abstract
A 23-year-old man presented with a 2-week history of intracranial hypertension. CT showed a large, nonenhancing cerebellar mass with surrounding calcification and displacement of the fourth ventricle. MRI revealed a septate lesion, with low signal on T1-weighted and high signal on T2-weighted images. The cerebellar tonsils were displaced below the foramen magnum and there was associated syringomyelia. The MRI features were characteristic of Lhermitte-Duclos (LD) disease (dysplastic gangliocytoma) and the diagnosis was confirmed following surgery. In this case, we emphasise the usefulness of MRI in the diagnosis of LD disease and consider the possible pathogenesis of the associated syringomyelia.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Lhermitte J, Duclos P (1920) Sur un ganglioneurome diffus du cortex du cervelet. Bull Assoc Fr Etude Cancer 9: 99–107
Ambler M, Pogacar S, Sidman R (1969) Lhermitte-Duclos disease (granule cell hyperfrophy of the cerebellum): pathological analysis of the first familial cases. J Neuropathol Exp Neurol 28: 622–647
Reeder RF, Saunders RL, Roberts DW, Fratkin JD, Cromwell LD (1988) Magnetic resonance imaging in the diagnosis and treatment of Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum) Neurosurgery 23: 240–245
Ashley DG, Zee CS, Chandrasona PI, Segall HD (1990) Lhermitte-Duclos disease: CT and MR findings. J Comput Assist Tomogr 14: 984–987
Smith RR, Grossman RI, Goldberg HI, Hackney DB, Bilaniuk LT, Zimmerman RA (1989) MR imaging of Lhermitte-Duclos disease: a case report. AJNR 10: 187–189
Meltzer CC, Smirniotopoulos JG, Jones RV (1995) The striated cerebellum: an MR imaging sign in Lhermitte-Duclos disease (dysplastic gangliocytoma) Radiology 194: 699–703
Milbouw G, Born JD, Martin D, Collignon J Hans P, Reznik M, Bonnal J (1988) Clinical and radiological aspects of dysplastic gangliocytoma (Lhermitte-Duclos disease): a report of two cases with review of the literature. Neurosurgery 22: 124–128
Sabin HI, Lidov HGW, Kendall BE, Symon L (1988) Lhermitte-Duclos disease (dysplastic gangliocytoma): a case report with CT and MRI. Acta Neurochir (Wien) 93: 149–153
Williams DW, Elster AD, Ginsberg LE, Stanton C (1992) Recurrent Lhermitte-Duclos disease: report of two cases and association with Cowden's disease. AJNR 13: 287–290
Marano SR, Johnson PC, Spetzler RF (1988) Recurrent Lhermitte-Duclos in a child: case report. J Neurosurg 69: 599–603
Shiurba RA, Gessaga EC, Eng LF, Sternberger LA, Sternberger NH, Urich H (1988) Lhermitte-Duclos discase: an immunohistochemical study of cerebellar cortex. Acta Neuropathol (Berl) 75: 474–480
Albrecht S, Haber RM, Goodman JC, Duvic M (1992) Cowden syndrome and Lhermitte-Duclos disease. Cancer 70: 869–876
Mihorat TH, Miller JI, Johnson WD, Adler DE, Heger IM (1993) Anatomical basis of syringomyelia occuring with hindbrain lesions. Neurosurgery 32: 748–754
Aboulker J (1979) La syringomyélie et les liquides intra-rachidiens. Neurochirurgie 25 [Suppl 1]: 1–144
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Marcus, C.D., Galeon, M., Peruzzi, P. et al. Lhermitte-Duclos disease associated with syringomyelia. Neuroradiology 38, 529–531 (1996). https://doi.org/10.1007/BF00626089
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00626089