Introduction
Lhermitte–Duclos disease (LDD), also known as dys-
plastic gangliocytoma, is a rare disorder of the cerebel-
lum. Since the initial report in 1920 [1], less than
100 cases have been reported. The aetiology of this
elusive, mysterious condition is uncertain; it has been
regarded as a hamartoma, a developmental anomaly, a
manifestation of a phakomatosis and a low-grade neo-
plasm [2]. Several reports have described the MRI fea-
tures and emphasised the avascular nature of LDD, and
hence the lack of contrast enhancement [3, 4, 5, 6, 7].
Contrast enhancement has, however, been reported in a
few articles [8, 9, 10]. In two of these [9, 10] contrast
enhancement was also seen on CT. We report two new
patients with histologically proven LDD, in whom
contrast enhancement of the lesion was observed on
MRI.
L. Spaargaren
P. Cras
M.A.M. Bomhof
S.T. Lie
A.M. de Barsy
P.H. Croese
J.L.J.M. Teepen
V.H.J.M. Duwel
J.W. Van Goethem
O. Ozsarlak
L. van den Hauwe
A.M. De Schepper
P.M. Parizel
Contrast enhancement in Lhermitte–Duclos
disease of the cerebellum: correlation of
imaging with neuropathology in two cases
Received: 14 August 2002
Accepted: 21 January 2003
Published online: 16 May 2003
Ó Springer-Verlag 2003
Abstract Lhermitte–Duclos disease
(LDD), also known as dysplastic
gangliocytoma, is a rare cerebellar
lesion. It has long been regarded as
avascular. We report two patients
with surgically proven LDD in
whom contrast enhancement was
observed on MRI. Neuropatholo-
gical examination revealed prolifer-
ation of veins. We suggest that
peripheral enhancement of LDD
probably reflects vascular prolifera-
tion of the cerebellar venous drain-
ing system, and should be considered
part of the imaging features of LDD.
Keywords Lhermitte–Duclos dis-
ease Æ Dysplastic gangliocytoma Æ
Contrast enhancement
A.M. de Barsy
Department of Neurology,
Algemeen Ziekenhuis KLINA,
Augustijnslei 100,
2930 Brasschaat, Belgium
P.H. Croese
Department of Neurosurgery,
Algemeen Ziekenhuis KLINA,
Augustijnslei 100,
2930 Brasschaat, Belgium
J.L.J.M. Teepen
Department of Neuropathology,
Elisabeth Ziekenhuis Tilburg,
Hilvarenbeekseweg 60,
5022 GC, Tilburg, The Netherlands
V.H.J.M. Duwel
Department of Pathology,
Algemeen Ziekenhuis KLINA,
Augustijnslei 100,
2930 Brasschaat, Belgium
L. van den Hauwe
Department of Radiology,
Algemeen Ziekenhuis KLINA,
Augustijnslei 100,
2930 Brasschaat, Belgium
Neuroradiology (2003) 45: 381–385
DOI 10.1007/s00234-003-0984-2
DIAGNOSTIC NEURORADIOLOGY
L. Spaargaren Æ J.W. Van Goethem
O. Ozsarlak Æ A.M. De Schepper
P.M. Parizel (&)
Department of Radiology,
Universitair Ziekenhuis Antwerpen,
University of Antwerp,
Wilrijkstraat 10, 2650
Edegem, Belgium
E-mail: parizelp@uia.ua.ac.be
Tel.: +32-3-8213124
Fax: +32-3-8252026
P. Cras
Department of Neurology,
Universitair Ziekenhuis Antwerpen,
University of Antwerp, Wilrijkstraat
10, 2650 Edegem, Belgium
M.A.M. Bomhof
Department of Neurology,
Amphia Ziekenhuis Breda,
Molengracht 21, 4818 CK, Breda,
The Netherlands
S.T. Lie
Department of Neurosurgery,
Elisabeth Ziekenhuis Tilburg,
Hilvarenbeekseweg 60,
5022 GC Tilburg, The Netherlands