Skip to main content Accessibility help
Hostname: page-component-559fc8cf4f-8sgpw Total loading time: 0.337 Render date: 2021-03-05T05:13:52.913Z Has data issue: true Feature Flags: { "shouldUseShareProductTool": true, "shouldUseHypothesis": true, "isUnsiloEnabled": true, "metricsAbstractViews": false, "figures": false, "newCiteModal": false, "newCitedByModal": true }

Lhermitte-Duclos Disease: Literature Review and Novel Treatment Strategy

Published online by Cambridge University Press:  18 September 2015

Sagun Tuli
Division of Neurosurgery, The Toronto Hospital, University of Toronto, Toronto.
John P. Provias
Division of Neurosurgery, The Toronto Hospital, University of Toronto, Toronto.
Mark Bernstein
Division of Neurosurgery, The Toronto Hospital, University of Toronto, Toronto.
Rights & Permissions[Opens in a new window]



Lhermitte-Duclos disease (LDD) is a rare pathologic entity involving the cerebellum. The fundamental nature of the entity and its pathogenesis remain unknown, and considerable debate has centered on whether it represents a neoplastic, malformative or hamartomatous lesion. The cell or cells of origin remain incompletely defined. Previous reports of cases in the English literature have dealt predominantly with the clinical and pathological aspects yet few address issues of treatment.


A case of Lhermitte-Duclos disease (LDD) in a 54-year-old female leading to local compressive symptoms and obstructive hydrocephalus is presented. A craniectomy, in addition to a CI laminectomy followed by a decompressive duroplasty (using autologous fascia lata graft) was performed.


The patient clinically improved and follow-up MRI 11 months post-operatively revealed improvement in hydrocephalus.


The histological and immunohistochemical features of the lesion are described, emphasizing the role of an abnormal dysplastic granule cell layer. The evidence in favor of each of the major theories of pathogenesis, malformative and neoplastic is discussed. Based on these facts a form of surgical intervention involving decompressive duroplasty is proposed.

Original Articles
Copyright © Canadian Neurological Sciences Federation 1997


1.Bielschowsky, M, Simons, A. Uber diffuse hamartome (ganglioneurome) des Kleinhirns und ihre Genese. J F Psychol Neurol 1963; 40: 395.Google Scholar
2.Foerster, O, Gagel, O. Ein Fall von Gangliocytoma Dysplasticum des Kleinhirns. Ztschr Nervenh 1959; 146: 792.Google Scholar
3.Christensen, E. Uber Ganglienzellgeschwulste im Gehirn. Virchows Arch Path Anat 1937; 300: 567.CrossRefGoogle Scholar
4.Bignami, JC, De Matteis, A. La Malattia di Lhermitte e Duclos del Cervelletto (Ganglioneuroma dysplastico). Riv Anat Pat 1956; 11: 523.Google Scholar
5.Courville, CB. Gangliocytoma myelinicum diffusum of the cerebellar cortex. Bull Los Angeles Neurol Society 1958; 23: 72.Google ScholarPubMed
6.Fischer, W, Busch, KT. Gangliozytome. Klinische und Morphologische Betrachtungen Zbl. Neurochir 1963; 23: 286.Google Scholar
7.Dilorenzo, N, Lunardi, P, Fortuna, A. Granulomolecular hypertrophy of the cerebellum (Lermitte-Duclos disease). Case report. J Neurosurg 1984; 60(3): 644646.CrossRefGoogle Scholar
8.Lhermitte, J, Duclos, P. Sur un Ganglioneurome Diffus du cortex du cervelet. Bull Assoc Franc Cancer 1920; 9: 99.Google Scholar
9.Albrecht, S, Haber, RM, Goodman, JC, Duvic, M. Cowden syndrome and Lhermitte-Duclos disease. Cancer 1992; 70(4): 869876.3.0.CO;2-E>CrossRefGoogle ScholarPubMed
10.Ashley, DG, Zee, C, Chandrasoma, PT, Segall, HD. Lhermitte-Duclos disease: CT and MR findings. J Comput Assist Tomogr 1990; 14(6): 984987.CrossRefGoogle ScholarPubMed
11.Banerjee, AK, Gleadhill, CA. Lhermitte-Duclos disease (diffuse cerebellar hypertophy); prolonged post-operative survival. Irish J Med Sci 1979; 148(3): 9799.CrossRefGoogle Scholar
12.Bellamy, JC, D’Amato, NA, Szakacs, JE. Diffuse cerebellar hypertrophy. Report of a case. Am J Clin Pathol 1963; 395404.CrossRefGoogle ScholarPubMed
13.Beuche, W, Wickboldt, J, Friede, RL. Lermitte-Duclos disease — its minimal lesion in electron microscope data and CT findings. Clini Neuropathol 1983; 2(4): 163170.Google Scholar
14.Brown, WR, Angelo, JN, Kelly, DL. Lhermitte-Duclos disease: case report with computerized tomographic. Neurosurg 1980; 6(2): 189191.CrossRefGoogle ScholarPubMed
15.Carter, JE, Merren, MD, Swann, KW. Preoperative diagnosis of Lhermitte-Duclos disease by magnetic resonance imaging: case report. J Neurosurg 1989; 70(1): 135137.CrossRefGoogle ScholarPubMed
16.Choudhaury, AR. Pre-operative magnetic resonance imaging in Lhermitte-Duclos disease. Br J Neurosurg 1990; 4: 225230.CrossRefGoogle Scholar
17.Christian, HJ. So-called “gangliocytoma” of the cerebellum. J Neurosurg 1961; 18: 125128.CrossRefGoogle ScholarPubMed
18.Cook, T, Holt, S, Yates, PO. Diffuse hypertrophy of the cerebellum. J Neurol Neurosurg and Psychiatry 1962; 25: 218221.CrossRefGoogle ScholarPubMed
19.Dietlein, M, Schroder, R, Widemann, B, Benz-Bohm, G. Dysplastic gangliocytoma of cerebellum in a newborn. Diagnosis by ultrasonography and MRI. Pediatr Radiol 1992; 22(2): 131133.CrossRefGoogle Scholar
20.Faillot, T, Sichez, JP, Brault, JL, et al. Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum). Report of a case and review of the literature. Acta Neurochir 1990; 105: 4449.CrossRefGoogle ScholarPubMed
21.Ferrer, I, Isamat, F, Lopez-Obarrio, L, et al. Parvalbumin and calbindin D-28K immunoreactivity in central ganglioglioma and dysplastic gangliocytoma of the cerebellum. Report of two cases. J Neurosurg 1993; 78(1): 133137.CrossRefGoogle ScholarPubMed
22.Frerbeau, P, Marty-Double, C, Castan-Tarbouriech, E, Privat, JM, Benezech, J. Cerebellar gangliocytoma dysplasticum in an infant. Neurochirurgia 1978; 21(6): 212220.Google Scholar
23.Hair, LS, Symmans, F, Powers, JM, Carmel, P. Immunohistochemistry and proliferative activity in Lhermitte-Duclos disease. Acta Neuropathol 1992; 84(5): 570573.CrossRefGoogle ScholarPubMed
24.Hashimoto, M, Fujimoto, K, Shinoda, S, Masuzawa, T. Magnetic resonance imaging of ganglion cell tumors. Neuroradiology 1993; 35(3): 181184.CrossRefGoogle Scholar
25.Heitkamp, JW, Rose, JE. Neck pain following migration of a ventriculocervical shunt. Surg Neurol 1983; 19: 285287.CrossRefGoogle ScholarPubMed
26.Hulcelle, P, Dooms, G, Vermonden, J. Lhermitte-Duclos disease. A case report. J Neuroradiol 1994; 21(1): 4045.Google ScholarPubMed
27.King, MA, Terence, JC, Spearritt, DJ, Boyle, RS. Lhermitte-Duclos disease and Cowden disease: a third case. Ann Neurol 1992; 32(1): 112113.CrossRefGoogle ScholarPubMed
28.Leech, RW, Christoferson, LA, Gilbertson, RL. Dysplastic gangliocytoma (Lhermitte-Duclos disease of the cerebellum). Case report. J Neurosurg 1977; 47(4): 609612.CrossRefGoogle ScholarPubMed
29.Marano, SR, Johnson, PC, Spetzler, RF. Recurrent Lhermitte-Duclos disease in a child. Case report. J Neurosurg 1988; 69(4): 599603.CrossRefGoogle Scholar
30.Milbouw, G, Born, JD, Martin, D, et al. Clinical and radiological aspects of dysplastic gangliocytoma (Lhermitte-Duclos disease): a report of two cases with review of the literature. (Review). Neurosurgery 1988; 22: 124128.Google Scholar
31.Oppenheimer, DR. A benign “tumour” of the cerebellum. Report on two cases of diffuse hypertrophy of the cerbellar cortex with a review of nine previously reported cases. J Neurol Neurosurg Psychiatry 1955; 18: 199213.CrossRefGoogle Scholar
32.Padberg, GW, Schot, JD, Vielvoye, GJ, Bots, GT, de Beer, FC. Lhermitte-Duclos disease and Cowden disease: a single phakomatosis. Ann Neurol 1991; 29(5): 517523.CrossRefGoogle ScholarPubMed
33.Radhakrishnan, VV, Sandhyamani, S, Sharma, R, Rout, D. Dysplasic gangliocytoma of the cerebellum (LDD) — a case report with review of the literature. Indian J Cancer 1992; 29(2): 8689.Google Scholar
34.Reeder, RF, Saunders, RL, Roberts, DW, Fratkin, JD, Cromwell, LD. Magnetic resonance imaging in the diagnosis and treatment of Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum). Neurosurgery 1988; 23(2): 240245.CrossRefGoogle Scholar
35.Reznick, M, Schoenen, J. Lhermitte-Duclos disease. Acta Neuropathol 1983; 59(2): 8894.CrossRefGoogle Scholar
36.Roski, RA, Roessmann, U, Spetzler, RF, Kaufman, B, Nulsen, FE. Clinical and pathological study of dysplastic gangliocytoma. Case report. J Neurosurg 1981; 55(2): 318321.CrossRefGoogle ScholarPubMed
37.Ruchoux, MM, Gray, F, Gherardi, R, et al. Orthostatic hypotension from a cerebellar gangliocytoma (Lhermitte-Duclos disease). Case report. J Neurosurg 1986; 65(2): 245248.CrossRefGoogle ScholarPubMed
38.Sabin, HI, Lidov, HG, Kendall, BE, Symon, L. Lhermitte-Duclos disease (dysplastic gangliocytoma): a case report with CT and MRI. Acta Neurochir 1988; 93(3–4): 149153.CrossRefGoogle ScholarPubMed
39.Shanley, DJ, Vassallo, CJ. Atypical presentations of Lhermitte-Duclos disease: preoperative diagnosis with MRI. Neuroradiology 1992; 34: 103104.CrossRefGoogle Scholar
40.Siddiqi, SN, Fehlings, MG. Lhermitte-Duclos disease mimicking adult-onset aqueductal stenosis. Case report. J Neurosurg 1994; 80(6): 10951098.CrossRefGoogle ScholarPubMed
41.Smith, RR, Grossman, RI, Goldberg, HI, et al. MR imaging of Lhermitte-Duclos disease: a case report. Am J Neuroradiol 1989; 10(1): 187189.Google ScholarPubMed
42.Sonier, CB, Feve, JR, Kersaint-Gilly, DE, et al. E. Lhermitte-Duclos disease. A rare cause of intracranial hypertension in adult. J Neuroradiol 1992; 19: 133138.Google Scholar
43.Stapelton, SR, Wilkins, PR, Bell, BA. Recurrent dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease) presenting with subarachnoid haemorrhage. Br J Neurosurg 1992; 6(2): 153156.CrossRefGoogle Scholar
44.ViecoPT, Del Carpio-O’Donovan PT, Del Carpio-O’Donovan, Melanson, D, et al. Dysplastic gangliocytoma (Lhermitte-Duclos disease): CT and MR imaging. Pediatr Radiolo 1992; 22: 366369.CrossRefGoogle ScholarPubMed
45.Vinchon, M, Blond, S, Lejeune, JP, et al. Association of Lhermitte-Duclos and Cowden disease: report of a new case and review of the literature. J Neurol Neurosurg Psychiatry 1994; 57(6): 699704.CrossRefGoogle ScholarPubMed
46.Wells, GB, Todd, ML, David, NY, Zager, EL. Lhermitte-Duclos disease and Cowden’s syndrome in an adolescent patient. Case report. J Neurosurg 1994; 81: 133136.CrossRefGoogle Scholar
47.Williams, DW, Elster, AD, Ginsberg, LE, Stanton, C. Recurrent Lhermitte-Duclos disease: report of two cases and association with Cowden’s disease. Am J Neuroradiol 1992; 13(1): 287290.Google ScholarPubMed
48.Yachnis, AT, Trojanowski, JQ, Memmo, M, Schlaepfer, WW. Expression of neurofilament proteins in the hypertrophic granule cells of Lhermitte-Duclos disease: an explanation for the mass effect and the myelination of parallel fibres in the disease state. J Neuropathol Exp Neurol 1988; 47(3): 206216.CrossRefGoogle Scholar
49.Ambler, M, Pogacal, S, Sidman, R. Lhermitte-Duclos disease (granule cell hypertrophy of the cerebellum). Pathological analysis of the first familial cases. J Neuropathol Exp Neurol 1969; 28(4): 622647.CrossRefGoogle ScholarPubMed
50.Harff, EG, Vinken, PJ. A case of diffuse cerebellar hypertrophy. Acta Neuropathol 1966; 6(106).CrossRefGoogle ScholarPubMed
51.Richieri-Costa, A, Frederigue, U, Guion-Almeida, ML. Holoprosencephaly, hamartomatous growth of the cerebrum, dysplatic gangliocytoma of the cerebellum, unique brain anomalies, and renal agenesis in a Brazilian infant born to a diabetic mother: a clinical and pathologic study. Birth Defects 1993; 29(1): 389394.Google Scholar
52.RoessmanU, Wongmongkolrit U, Wongmongkolrit. Dysplastic gangliocytoma of cerebellum in a newborn. Case report. J Neurosurg 1984; 60: 845847.CrossRefGoogle Scholar
53.Schiurba, RA, Gessaga, EC, Eng, LF, et al. Lhermitte-Duclos disease. An immunohistochemical study of the cerebellar cortex (Review). Acta Neuropathol 1988; 75(5): 474480.Google Scholar
54.Prichett, PS, King, TI. Dysplastic gangliocytoma of the cerebellum: an ultrastructural study. Acta Neuropathol 1978; 42: 15.CrossRefGoogle Scholar
55.DeLeon, GA, Grant, JA, Darling, CF. Monstrous, crablike hypertrophy of the cerebellar vermis and its relationship with Lhermitte-Duclos disease. J Neurosurg 1996; 85: 157#x2013;162.CrossRefGoogle Scholar
56.Meyer, JS, Marchosky, JA, Hickey, WF. Cell kinetic classification of tumors of the nervous system by DNA precursor labeling invito. Human Pathol 1993; 24(12): 13571364.CrossRefGoogle Scholar
57.Russell, JR, O’Brien, M, Wells, RS. Cowden’s syndrome. Br J Dermatol 1981; 105(19): 5758.Google Scholar
58.Eng, C, Murday, V, Seal, S, et al. Eeles, RA. Cowden syndrome and Lhermitte-Duclos disease in a family: a single genetic syndrome with pleiotrophy? J Med Genet 1993; 31(6): 458461.CrossRefGoogle Scholar
59.Aljouanine, T, Bertrand, I. Dysplasia Laminaire Hypertrophique du cervelet associee a une malformation renale, avec gigantisme tissulaire. Rev Neurol 1952; 8: 289.Google Scholar
60.Barten, H. Eine seltene fehlbildung des kleinhirns. Beitr Path Anat 1934; 93: 219.Google Scholar
61.Duncan, D, Snodgrass, SR. Diffuse hypertrophy of the cerebellar cortex (myelinated neurocytoma). Arch Neurol Psychiatry 1943; 50: 677.CrossRefGoogle Scholar
62.Heinlen, H, Falkenberg, G. Beirag zur kasuistik der ganglioneurome des Kleinhirns. Ztschr f d ges Neurol u Psychiat 1939; 166: 128.CrossRefGoogle Scholar
63.Schmidt, MB. Uber halbseiigen riesenwuchs des schadels und seine beiziehung zu leontiasis und ostits fibrosa. Beitr Anat des Ohres 1926; 23: 594.Google Scholar
64.Spiegel, E. Hyperplasia des kleinhirns. Beitr path Anat 1920; 67: 539.Google Scholar
65.Penfield, W, Cobur, DF. Arnold-Chiari malformation and its operative treatment. Arch Neurol Psychiatry 1938; 40: 328.CrossRefGoogle Scholar

Full text views

Full text views reflects PDF downloads, PDFs sent to Google Drive, Dropbox and Kindle and HTML full text views.

Total number of HTML views: 0
Total number of PDF views: 215 *
View data table for this chart

* Views captured on Cambridge Core between September 2016 - 5th March 2021. This data will be updated every 24 hours.


Send article to Kindle

To send this article to your Kindle, first ensure is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about sending to your Kindle. Find out more about sending to your Kindle.

Note you can select to send to either the or variations. ‘’ emails are free but can only be sent to your device when it is connected to wi-fi. ‘’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Lhermitte-Duclos Disease: Literature Review and Novel Treatment Strategy
Available formats

Send article to Dropbox

To send this article to your Dropbox account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your <service> account. Find out more about sending content to Dropbox.

Lhermitte-Duclos Disease: Literature Review and Novel Treatment Strategy
Available formats

Send article to Google Drive

To send this article to your Google Drive account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your <service> account. Find out more about sending content to Google Drive.

Lhermitte-Duclos Disease: Literature Review and Novel Treatment Strategy
Available formats

Reply to: Submit a response

Your details

Conflicting interests

Do you have any conflicting interests? *