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Lhermitte-Duclos disease
Lhermitte-Duclos disease Patrick Fransen MD* JacquesFavre MD* Philippe Maeder MD** Heinz Fankhauser MD* Department of Neurosurgery*, and Department of Radiology** Centre Hospitalier Universitaire Vaudois, Lausanne. Switzerland.
Lhermitte-Duclos disease (dysplasticgangliocytoma of the cerebellum) is a rare pseudo-neoplastic disorder of the cerebellumwithtypicalMRI fmdings. A %-year-old man presentingwithprogressiveneck pain, dizziness,and impaired vision is reported. CT and h4RIrevealeda left cerebellarhaemisphericmass and obstructivehydrocephalm. LhermitteDuclos diseasewas histologicallyconfirmed after surgicalremoval of the lesion. The typicalMRI appearanceof a nonenhancing haemispheric cerebellar mass with preservation and exaggeration of the normal gyral pattern allows pre-operative diagnosis of this condition. The literature is reviewed and clinical presentation, radiology and histopathologyare discussed. Journal
of Clinical Neuroscience
Keywords: Lhermitte-Duclos
0 Longman
1994,1(4):274-276
disease, Dysplastic gangliocytoma,
Group
UK Ltd
Cerebellar tumour, Magnetic resonance imaging,
Diagnosis
Introduction Lhermitte-Duclos disease (LDD) is an uncommon posterior fossa lesion, first described by Lhermitte and Duclos in 1920.’ Its controversial origin has led to avariety of denominations: dysplastic gangliocytoma, cerebellar ganglioneuroma, cerebellar hamartoma, Purkinjoma, granular and granulo-molecular cell hypertrophy. Classically, it affects young adults, causing a progressive cerebellar syndrome and signs of raised intracranial pressure due to hydrocephalus. It is often associated with macrocephaly and mental retardation as well as with several congenital disorders like hemihypertrophy, polydactyly, cutaneous and visceral angiomas or leontiasis ossea.2~3~4 The uniqueness, however, resides in its typical MRI aspect, which is likely to allow definite diagnosis before operation.
papilloedema. Vision was normal on the left side; on the right side, localization of light only was possible. Cerebellar coordination was impaired in all four limbs. CT demonstrated a heterogeneous hypodense left cerebellar haemispheric mass, which displaced and obstructed the fourth ventricle, causing severe hydrocephalus. There was no abnormal contrast enhancement (Fig. 1).
Case report A 25-year-old truck driver with a 5-year history of intermittent neck and low back pain developed, in July 1992, increased neck pain associated with dizziness followed, in August 1992, by progressive visual loss. For many years the patient has been considered to present a minor personality disorder. On admission in September 1992, the general examination revealed a patient with a moderately debilitated facial expression, and a thoracic cyphoscoliosis with lumbar hyperlordosis, and, radiologically, a spina bifida Sl. On neurological examination there was bilateral
274
J. Clin. Neuroscience
Volume 1
Number 4
Fig.1
Contrast enhanced CT showing a hypodense mass in the left cerebellar hemisphere, obstructing the fourth ventricle and causing supratentorial hydrocephalus.
October 1994
Lhermitte
Duclos disease
Case reports
Fig. 2(A,B,C) Tl weighted left parasagittal, and Tl and T2 weighted transverse MRls showing the extent of the cerebellar mass. The pons is displaced forward and the fourth ventricle is shifted towards the right side. A well defined hypertrophied gyral pattern, hypointense on Tl and hyperintense on T2 weighted sequences, extends troughout the mass. Clear boundaries of the lesion are not visible.
hype- and T2 hyperintense mass lesion of‘tl~e left cerebellar hemisphere, without contrast u~hallcement. The whole mass presented a hypertrophied gyral pattern on Tl as well as T2 weighted images which extended throughout its depth. This picture was highl) suggchst ivt> of the diagnosis of LDD (Fig. 2). A postt*rior f’ossa craniotomy was performed. The superior part of the lef’t cerebollar hemisphere showed pachygyria and swelling. but no discoloration. Extensive remo\ al of the lesion was made. Clear delimitation in the depth was visllall!, not possible, and the resection was guided 1~: anatomical landmarks from the MKI. No ventrit7llai shlmting was needed. Post-of,~t-;lti~el!, the patient showed a tnodet-ate irlcrt>a\r of t~c~rebrllat~ ataxia, and transient neliropsycllological ;IhliorJll;llities. I’isual acuity had worst*ned with an actlit) of:{/ 10 on the lef’t and absent vision 011 the right. h’o particular reason could be determined despite exteii5ive ~~phthalniological workup. After a 3 month fi)llou-lip, the minor pt’rsonality disorder already found f~re-of~c~t-~ilivel~~rcmaincd prc5ent as well as the visual impairtnctrt, but cct-ebellar ataxia had almost completel) disappcat-cd. One and a half years later, the neurological N~ru-opathological examinastatus rc~maintSd ~lJlchaJl#td. tion confir~n~tl the typical pattern of LDD. The mokcr~lar layer MX ~l~icl\~~t~~l. made of large-sized myclinated axons, and the. norm;11 granulate ;uJd P~irkin,je cells were replaced by h! pc,rtrophic cells. Ml
;I Tl
Discussion Since its early description by Lhcrmitte and Duclos’, less than 100 cases of I,DD havr been reported in the literature. LDD af‘fecty F)rc,dotninaJltlp):ourlg and middle aged adults in thrir third or f’ourth decade, but it has been reported in int;trlcy, childhood,“.’ and also at an age of 73 years. Xltho~~gh ;I familial case has been reported by Ambler,” no fi~rthc~ cxviclence oi‘ inheritance has been follnd. Padhrq ~1 al Ijelieve that its occasional association with
_
(:owden disease establishes the combined occLu.rence as a single phakomatosis.” The most common symptoms art‘ r&ted tt) rhrotlicAly clrvated intracranial pressure due to progt-rssivcs h~drocephalus. such as headache, associatc*d or not with nausea and vomiting. Dccreascsd visual acuity and ocr~lotnotor nerve palsies are also li-rqucntl\~ noted. ‘.‘.“’ A mild cerchellar svndrome ic comn1on and cat1 be associated with an occasional p!,ramidal syndrome, neck stif’fnrss. mental deterioration and. rareI\. with orthostatic hypolrnsion. Illt~Jltal ;th;lc,l.nialitit’s Alld ” I11 defiricd neL~rops~chologica1 disorders C‘;~II also 1~. prest’ttt. Megalencephalv is fouiid in IJIOS~ casc5. Thtx lesion usually presents ;LSa Iulilalet-al hemispheric swelling of the cerel~ellum preserving the ~yl.;il pattrrn of thr cortex with moderate to widt*l\ enlarged f’olia. ‘I‘hrsr fijlia are described as ~ellowisli c,r’piirplish gray, but the! had a ~lormal color in our case. ‘1litxr.c is no clear demarcation fi-om the normal adjacent c~rt~bt~llmn. Extension of thr Iesion outsidr the postr‘rior f’oasa, towards the mcsencephalon and the interr~e~l~l~lcL~l~~~.cisrr~.t1. although uncommon, has brert reportcd.~~7~‘Y N~uro~~at~~~~logical findings include excessive trn~~lination of the asons, which are also larger than LlsLlal. The sllperficial molec~~~lar layet. ;is wrll 3s the titidcrl~inCg gi.all~il;~r.-c.t,ll 1a>ct- become thickned, and the normal granular cells arv rqkiced bv ~~iiks~rally large
ne~irotiitl
cells.
T’l~(w titwt~otial
cells caIi
~~11s;1nt1 show sigrls of a high lta\.el ot’tierlrofilament protrin mc~tabolisni. Pm-kinje cells \\hite I11;iltc’r is tl~crcasrd arc’ missing, and the suhcortkd arid mitoscs arc or absent. Niiclrar rlysmorph~ lx liyp~rtt~t~phiecl
absent.“‘.”
“These
granLrlat-
findings
mak
process linlikcly and are interprctcY1 cell migration. Koessmann stiggmts
a prolikrativc
tutnolwal
as a tlvf~~ct in granlllat~ Ihal thv gratll~lal-cells
may mature too early. become :ilt-t3tcd in the molcc~k~r Iaver- and CotltitlL~c lo gHw into IargY ;~l~noi~mal Crll.5.’ PI-~-operative radiological stllrlics art’ not specific. Skull S-rays can show signs of chronic. rksul intracranial pr<‘ss”rc’. ASwell as ;I thinning of’lhta c)cci~)i~;tl s(ltl;lln;\.i).‘l’.’ ’
. J. Clin. Neuroscience
Volume 1
Number 4
October 1994
275
Lhermitte Duclos disease
Case report
Angiography shows the mass effect, but is unable to determine the extent nor to specify the nature of the radiologically avascular lesion.2,7 CT shows an iso- or hypodense mass without contrast enhancement, occasionally containing small areas of calcification.‘~14~16,17 MRI is particularly helpful to make the diagnosis of LDD. It shows a cerebellar hemispheric hyperintense mass on T2 weighted sequences, with well defined margins. Its major interest, however, is that Tl and T2 weighted images show the preservation and accentuation of the normal gyral pattern. This clear anatomical picture characterized by successive linear bands represents hypertrophic cerebellar folia. The high sensitivity of MRI, combined with the specificity of the pathological images, makes this technique useful for both initial diagnosis and assessment of the lesion’s natural course or post-operative p~~g~~~~~~~~3~‘2~‘4~~6~17~18~~~
References
1. Lhermitte J, Duclos P. Sur un ganglioneuroma
2.
3.
4.
5.
6.
Long asymptomatic periods before clinical onset and incidental autopsy findings tend to prove a slow growth rate of the tumour. Poor outcome in non-operated patients can be explained by sudden decompensation of a longstanding hydrocephalus but it is likely that, in some patients, acute hydrocephalus never develops. However, surgery remains strongly indicated in all symptomatic patients, with the addition of ventricular shunting if necessary.3.10.13,14,‘7 Th e main surgical difficulty resides in the poor demarcation of the border zone between the lesion and the normal surrounding brain. Examination of sequential frozen sections of the transitional area could prove helpful in determining the extent of surgical resection, although we did not use this technique. Some cases of tumour regrowth have been observed as late as 11 years after the first surgical procedure,“,’ and resection should therefore be as radical as possible. There is no relevant data concerning radiation therapy in LDD.’
7.
8.
9.
10.
11.
12.
13.
Conclusion Even though the exact nature of LDD, tumoural or dysembryogenic, remains debatable, a reliable preoperative diagnosis can now be made due to the specific aspect of the disease on MRI, consisting of a cerebellar hemispheric, non-enhancing mass, retaining and exaggerating its normal gyral pattern on Tl and T2 weighted images. This enables the neurosurgeon to adequately plan and perform the surgical resection which remains the treatment of choice. MRI is also a useful tool for long term follow-up and early detection of tumour regrowth.
Correspondence and offprint requests: Dr H. Fankhauser, Department of Neurosurgery, Centre Hospitalier UniversitaireVaudois, 1011 Lausanne, Switzerland. Tel: 41 21 314 48 74 Fax: 41 21 314 4R 73
1. Clin. Neuroscience
15.
16.
17.
18.
Received 4 March 1994 Accepted for publication 24 March 1994
276
14.
Volume 1
19.
20.
Number 4
diffus du cortex du cervelet. Bull Assoc Franc Etude Cancer 1920;9:99-107. Lejeune JP, Blond S, Dupard T, Combelles G, Delandsheer JM, Christiaens JL. Maladie de LhermitteDuclos: a propos d’une observation. Neurochirurgie 1987;33:408-11. 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 cerebellrlm). Neurosurgery 1988;23:240-45. Rousseaux M, Combelles G, Krivosik Y, Christiaens JL. Maladie de Lhermitte-Duclos: hypertrophie granulomolculaire du cervelet. Neurochirurgie 1987;33:232-35. Roessmann R, Wongmongkolrit T. Dysplastic gangliocytoma of cerebellum in a newborn. J Neurosurg 1984;60:84547. Marano SR, Spetzler RF’, Lhermitte Duclos disease (letter) J Neurosurg 1984;61:617-18. Marano SR, Johnson PC, Spetzler RF. Recurrent Lhermitte-Duclos disease in a child. Case report. J Neurosurg 1988;69:599-603. Ambler M, Pogacar S, Sidman R. Lhermitte-Duclos disease (granule cell hypertrophy of the cerebellum). Pathological analysis of the first familial cases. J Neuropathol Exp Neurol 1969;28:622-47. Padberg GW, Schot JD, Vielvoye GJ, Bots G, de Beer FG. Lhermitte Duclos disease and Cowden disease: a single phakomatosis. Ann Neurol 1991;29:517-23. Leech RW, Christoferson LA, Gilbertson RL. Dysplastic gangliocytoma of the cerebellum..J Neurosurg 1977;47:609-12. Ruchoux M, Gray F, Gherardi R, Schaeffer A, Comoy J, Poirier J. Orthostatic hypotension from a cerebellar gangliocytoma (Lhermitte-Duclos disease). J Neurosurg 1986;65:245-52. Faillot T, Sichez JP, Brault JL, Capelle L, Kujas M, Bordi L, Boukobza M. Lhermitte-Duclos disease (Dysplastic gangliocytoma of the cerebellum). Acta Neurochir 1990;105:4449. Di Lorenzo N, Lunardi P, Fortuna A. Granulomolecular hypertrophy of the cerebellum (Lhermitte-Duclos disease). J Neurosurg 1984;60:64446. Milbouw G, Born JD, Martin D, Collignon J, Hans P, Reznik M, Bonnal J. Clinical and radiological aspects of Dysplastic Gangliocytoma (Lhermitte-Duclos disease): A report of two cases with review of the litterature. Neurosurgery 1988;22:12428. Roski RA, Roessmann LJ, Spetzler RF, Kaufman B, Nulsen FE. Clinical and pathological study of dysplastic gangliocytoma..J Neurosurg 1981;55:318-21. Ashley DG, Zee CS, Chandrasoma PT, Segall HD. Lhermitte-Duclos disease: CT and MR findings. J Comput Assist Tomogr 1990;14:98487. Sabin HI, Lidov HGW, Kendall BE, Symon L. LhermitteDuclos disease (Dysplastic Gangliocytoma): a case report with CT and MRI. Acta Neurochir 1988;93:149-53. Carter JE, Merren MD, Swann KW. Pre-operative diagnosis of Lhermitte-Duclos disease by magnetic resonance imaging: case report. J Neurosurg 1989;70:135-37. Choudhury AR. Pre-operative magnetic resonance imaging in Lhermitte-Duclos disease. Br J Neurosurg 1990;4:225-30. Ganerjee AK, Gleadhill CA. Lhermitte-Duclos disease (diffuse cerebellar hypertrophy) ; prolonged postoperative survival. Ir J Med Sci 1979;148:97-99.
October 1994
Lhermitte-Duclos disease (dysplasticgangliocytoma of the cerebellum) is a rare pseudo-neoplastic disorder of the cerebellumwithtypicalMRI fmdings. A %-year-old man presentingwithprogressiveneck pain, dizziness,and impaired vision is reported. CT and h4RIrevealeda left cerebellarhaemisphericmass and obstructivehydrocephalm. LhermitteDuclos diseasewas histologicallyconfirmed after surgicalremoval of the lesion. The typicalMRI appearanceof a nonenhancing haemispheric cerebellar mass with preservation and exaggeration of the normal gyral pattern allows pre-operative diagnosis of this condition. The literature is reviewed and clinical presentation, radiology and histopathologyare discussed. Journal
of Clinical Neuroscience
Keywords: Lhermitte-Duclos
0 Longman
1994,1(4):274-276
disease, Dysplastic gangliocytoma,
Group
UK Ltd
Cerebellar tumour, Magnetic resonance imaging,
Diagnosis
Introduction Lhermitte-Duclos disease (LDD) is an uncommon posterior fossa lesion, first described by Lhermitte and Duclos in 1920.’ Its controversial origin has led to avariety of denominations: dysplastic gangliocytoma, cerebellar ganglioneuroma, cerebellar hamartoma, Purkinjoma, granular and granulo-molecular cell hypertrophy. Classically, it affects young adults, causing a progressive cerebellar syndrome and signs of raised intracranial pressure due to hydrocephalus. It is often associated with macrocephaly and mental retardation as well as with several congenital disorders like hemihypertrophy, polydactyly, cutaneous and visceral angiomas or leontiasis ossea.2~3~4 The uniqueness, however, resides in its typical MRI aspect, which is likely to allow definite diagnosis before operation.
papilloedema. Vision was normal on the left side; on the right side, localization of light only was possible. Cerebellar coordination was impaired in all four limbs. CT demonstrated a heterogeneous hypodense left cerebellar haemispheric mass, which displaced and obstructed the fourth ventricle, causing severe hydrocephalus. There was no abnormal contrast enhancement (Fig. 1).
Case report A 25-year-old truck driver with a 5-year history of intermittent neck and low back pain developed, in July 1992, increased neck pain associated with dizziness followed, in August 1992, by progressive visual loss. For many years the patient has been considered to present a minor personality disorder. On admission in September 1992, the general examination revealed a patient with a moderately debilitated facial expression, and a thoracic cyphoscoliosis with lumbar hyperlordosis, and, radiologically, a spina bifida Sl. On neurological examination there was bilateral
274
J. Clin. Neuroscience
Volume 1
Number 4
Fig.1
Contrast enhanced CT showing a hypodense mass in the left cerebellar hemisphere, obstructing the fourth ventricle and causing supratentorial hydrocephalus.
October 1994
Lhermitte
Duclos disease
Case reports
Fig. 2(A,B,C) Tl weighted left parasagittal, and Tl and T2 weighted transverse MRls showing the extent of the cerebellar mass. The pons is displaced forward and the fourth ventricle is shifted towards the right side. A well defined hypertrophied gyral pattern, hypointense on Tl and hyperintense on T2 weighted sequences, extends troughout the mass. Clear boundaries of the lesion are not visible.
hype- and T2 hyperintense mass lesion of‘tl~e left cerebellar hemisphere, without contrast u~hallcement. The whole mass presented a hypertrophied gyral pattern on Tl as well as T2 weighted images which extended throughout its depth. This picture was highl) suggchst ivt> of the diagnosis of LDD (Fig. 2). A postt*rior f’ossa craniotomy was performed. The superior part of the lef’t cerebollar hemisphere showed pachygyria and swelling. but no discoloration. Extensive remo\ al of the lesion was made. Clear delimitation in the depth was visllall!, not possible, and the resection was guided 1~: anatomical landmarks from the MKI. No ventrit7llai shlmting was needed. Post-of,~t-;lti~el!, the patient showed a tnodet-ate irlcrt>a\r of t~c~rebrllat~ ataxia, and transient neliropsycllological ;IhliorJll;llities. I’isual acuity had worst*ned with an actlit) of:{/ 10 on the lef’t and absent vision 011 the right. h’o particular reason could be determined despite exteii5ive ~~phthalniological workup. After a 3 month fi)llou-lip, the minor pt’rsonality disorder already found f~re-of~c~t-~ilivel~~rcmaincd prc5ent as well as the visual impairtnctrt, but cct-ebellar ataxia had almost completel) disappcat-cd. One and a half years later, the neurological N~ru-opathological examinastatus rc~maintSd ~lJlchaJl#td. tion confir~n~tl the typical pattern of LDD. The mokcr~lar layer MX ~l~icl\~~t~~l. made of large-sized myclinated axons, and the. norm;11 granulate ;uJd P~irkin,je cells were replaced by h! pc,rtrophic cells. Ml
;I Tl
Discussion Since its early description by Lhcrmitte and Duclos’, less than 100 cases of I,DD havr been reported in the literature. LDD af‘fecty F)rc,dotninaJltlp):ourlg and middle aged adults in thrir third or f’ourth decade, but it has been reported in int;trlcy, childhood,“.’ and also at an age of 73 years. Xltho~~gh ;I familial case has been reported by Ambler,” no fi~rthc~ cxviclence oi‘ inheritance has been follnd. Padhrq ~1 al Ijelieve that its occasional association with
_
(:owden disease establishes the combined occLu.rence as a single phakomatosis.” The most common symptoms art‘ r&ted tt) rhrotlicAly clrvated intracranial pressure due to progt-rssivcs h~drocephalus. such as headache, associatc*d or not with nausea and vomiting. Dccreascsd visual acuity and ocr~lotnotor nerve palsies are also li-rqucntl\~ noted. ‘.‘.“’ A mild cerchellar svndrome ic comn1on and cat1 be associated with an occasional p!,ramidal syndrome, neck stif’fnrss. mental deterioration and. rareI\. with orthostatic hypolrnsion. Illt~Jltal ;th;lc,l.nialitit’s Alld ” I11 defiricd neL~rops~chologica1 disorders C‘;~II also 1~. prest’ttt. Megalencephalv is fouiid in IJIOS~ casc5. Thtx lesion usually presents ;LSa Iulilalet-al hemispheric swelling of the cerel~ellum preserving the ~yl.;il pattrrn of thr cortex with moderate to widt*l\ enlarged f’olia. ‘I‘hrsr fijlia are described as ~ellowisli c,r’piirplish gray, but the! had a ~lormal color in our case. ‘1litxr.c is no clear demarcation fi-om the normal adjacent c~rt~bt~llmn. Extension of thr Iesion outsidr the postr‘rior f’oasa, towards the mcsencephalon and the interr~e~l~l~lcL~l~~~.cisrr~.t1. although uncommon, has brert reportcd.~~7~‘Y N~uro~~at~~~~logical findings include excessive trn~~lination of the asons, which are also larger than LlsLlal. The sllperficial molec~~~lar layet. ;is wrll 3s the titidcrl~inCg gi.all~il;~r.-c.t,ll 1a>ct- become thickned, and the normal granular cells arv rqkiced bv ~~iiks~rally large
ne~irotiitl
cells.
T’l~(w titwt~otial
cells caIi
~~11s;1nt1 show sigrls of a high lta\.el ot’tierlrofilament protrin mc~tabolisni. Pm-kinje cells \\hite I11;iltc’r is tl~crcasrd arc’ missing, and the suhcortkd arid mitoscs arc or absent. Niiclrar rlysmorph~ lx liyp~rtt~t~phiecl
absent.“‘.”
“These
granLrlat-
findings
mak
process linlikcly and are interprctcY1 cell migration. Koessmann stiggmts
a prolikrativc
tutnolwal
as a tlvf~~ct in granlllat~ Ihal thv gratll~lal-cells
may mature too early. become :ilt-t3tcd in the molcc~k~r Iaver- and CotltitlL~c lo gHw into IargY ;~l~noi~mal Crll.5.’ PI-~-operative radiological stllrlics art’ not specific. Skull S-rays can show signs of chronic. rksul intracranial pr<‘ss”rc’. ASwell as ;I thinning of’lhta c)cci~)i~;tl s(ltl;lln;\.i).‘l’.’ ’
. J. Clin. Neuroscience
Volume 1
Number 4
October 1994
275
Lhermitte Duclos disease
Case report
Angiography shows the mass effect, but is unable to determine the extent nor to specify the nature of the radiologically avascular lesion.2,7 CT shows an iso- or hypodense mass without contrast enhancement, occasionally containing small areas of calcification.‘~14~16,17 MRI is particularly helpful to make the diagnosis of LDD. It shows a cerebellar hemispheric hyperintense mass on T2 weighted sequences, with well defined margins. Its major interest, however, is that Tl and T2 weighted images show the preservation and accentuation of the normal gyral pattern. This clear anatomical picture characterized by successive linear bands represents hypertrophic cerebellar folia. The high sensitivity of MRI, combined with the specificity of the pathological images, makes this technique useful for both initial diagnosis and assessment of the lesion’s natural course or post-operative p~~g~~~~~~~~3~‘2~‘4~~6~17~18~~~
References
1. Lhermitte J, Duclos P. Sur un ganglioneuroma
2.
3.
4.
5.
6.
Long asymptomatic periods before clinical onset and incidental autopsy findings tend to prove a slow growth rate of the tumour. Poor outcome in non-operated patients can be explained by sudden decompensation of a longstanding hydrocephalus but it is likely that, in some patients, acute hydrocephalus never develops. However, surgery remains strongly indicated in all symptomatic patients, with the addition of ventricular shunting if necessary.3.10.13,14,‘7 Th e main surgical difficulty resides in the poor demarcation of the border zone between the lesion and the normal surrounding brain. Examination of sequential frozen sections of the transitional area could prove helpful in determining the extent of surgical resection, although we did not use this technique. Some cases of tumour regrowth have been observed as late as 11 years after the first surgical procedure,“,’ and resection should therefore be as radical as possible. There is no relevant data concerning radiation therapy in LDD.’
7.
8.
9.
10.
11.
12.
13.
Conclusion Even though the exact nature of LDD, tumoural or dysembryogenic, remains debatable, a reliable preoperative diagnosis can now be made due to the specific aspect of the disease on MRI, consisting of a cerebellar hemispheric, non-enhancing mass, retaining and exaggerating its normal gyral pattern on Tl and T2 weighted images. This enables the neurosurgeon to adequately plan and perform the surgical resection which remains the treatment of choice. MRI is also a useful tool for long term follow-up and early detection of tumour regrowth.
Correspondence and offprint requests: Dr H. Fankhauser, Department of Neurosurgery, Centre Hospitalier UniversitaireVaudois, 1011 Lausanne, Switzerland. Tel: 41 21 314 48 74 Fax: 41 21 314 4R 73
1. Clin. Neuroscience
15.
16.
17.
18.
Received 4 March 1994 Accepted for publication 24 March 1994
276
14.
Volume 1
19.
20.
Number 4
diffus du cortex du cervelet. Bull Assoc Franc Etude Cancer 1920;9:99-107. Lejeune JP, Blond S, Dupard T, Combelles G, Delandsheer JM, Christiaens JL. Maladie de LhermitteDuclos: a propos d’une observation. Neurochirurgie 1987;33:408-11. 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 cerebellrlm). Neurosurgery 1988;23:240-45. Rousseaux M, Combelles G, Krivosik Y, Christiaens JL. Maladie de Lhermitte-Duclos: hypertrophie granulomolculaire du cervelet. Neurochirurgie 1987;33:232-35. Roessmann R, Wongmongkolrit T. Dysplastic gangliocytoma of cerebellum in a newborn. J Neurosurg 1984;60:84547. Marano SR, Spetzler RF’, Lhermitte Duclos disease (letter) J Neurosurg 1984;61:617-18. Marano SR, Johnson PC, Spetzler RF. Recurrent Lhermitte-Duclos disease in a child. Case report. J Neurosurg 1988;69:599-603. Ambler M, Pogacar S, Sidman R. Lhermitte-Duclos disease (granule cell hypertrophy of the cerebellum). Pathological analysis of the first familial cases. J Neuropathol Exp Neurol 1969;28:622-47. Padberg GW, Schot JD, Vielvoye GJ, Bots G, de Beer FG. Lhermitte Duclos disease and Cowden disease: a single phakomatosis. Ann Neurol 1991;29:517-23. Leech RW, Christoferson LA, Gilbertson RL. Dysplastic gangliocytoma of the cerebellum..J Neurosurg 1977;47:609-12. Ruchoux M, Gray F, Gherardi R, Schaeffer A, Comoy J, Poirier J. Orthostatic hypotension from a cerebellar gangliocytoma (Lhermitte-Duclos disease). J Neurosurg 1986;65:245-52. Faillot T, Sichez JP, Brault JL, Capelle L, Kujas M, Bordi L, Boukobza M. Lhermitte-Duclos disease (Dysplastic gangliocytoma of the cerebellum). Acta Neurochir 1990;105:4449. Di Lorenzo N, Lunardi P, Fortuna A. Granulomolecular hypertrophy of the cerebellum (Lhermitte-Duclos disease). J Neurosurg 1984;60:64446. Milbouw G, Born JD, Martin D, Collignon J, Hans P, Reznik M, Bonnal J. Clinical and radiological aspects of Dysplastic Gangliocytoma (Lhermitte-Duclos disease): A report of two cases with review of the litterature. Neurosurgery 1988;22:12428. Roski RA, Roessmann LJ, Spetzler RF, Kaufman B, Nulsen FE. Clinical and pathological study of dysplastic gangliocytoma..J Neurosurg 1981;55:318-21. Ashley DG, Zee CS, Chandrasoma PT, Segall HD. Lhermitte-Duclos disease: CT and MR findings. J Comput Assist Tomogr 1990;14:98487. Sabin HI, Lidov HGW, Kendall BE, Symon L. LhermitteDuclos disease (Dysplastic Gangliocytoma): a case report with CT and MRI. Acta Neurochir 1988;93:149-53. Carter JE, Merren MD, Swann KW. Pre-operative diagnosis of Lhermitte-Duclos disease by magnetic resonance imaging: case report. J Neurosurg 1989;70:135-37. Choudhury AR. Pre-operative magnetic resonance imaging in Lhermitte-Duclos disease. Br J Neurosurg 1990;4:225-30. Ganerjee AK, Gleadhill CA. Lhermitte-Duclos disease (diffuse cerebellar hypertrophy) ; prolonged postoperative survival. Ir J Med Sci 1979;148:97-99.
October 1994