European Journal of Radiology Extra 64 (2007) 83–86
Preoperative diagnosis of Lhermitte-Duclos disease:
MRI and
1
H-MR spectroscopy findings
Esra
¨
Ozkavukcu
a,*
, Pelin Seher
¨
Oztekin
b
,
˙
Ilhan Erden
a
a
Ankara University School of Medicine, Turkey
b
Ankara Training and Research Hospital, Turkey
Received 3 May 2007; received in revised form 9 August 2007; accepted 22 August 2007
Abstract
Lhermitte-Duclos disease (LDD), also known as dysplastic gangliocytoma of the cerebellum, is a rare cerebellar disorder of uncertain
etiology. LDD presents with highly typical findings on MRI. Furthermore,
1
H-MR spectroscopy can provide additional information supporting
the diagnosis. Herein, we present the MRI and
1
H-MR spectroscopy findings of a 46-year-old LDD patient with a brief review of the relevant
literature. The patient had a unilateral, cerebellar hemispheric mass with a layered appearance, resembling “tiger stripes”. On single-voxel
1
H-MR spectroscopy, N-acetyl aspartate (NAA), cholin (Cho), creatinine (Cr) levels and NAA/Cr, Cho/Cr ratios were found to be within
reference values, however, lactate level was elevated. In conclusion, although conventional MRI characteristics of LDD are typical,
1
H-MR
spectroscopy can provide additional and supportive information, revealing the benign nature of this rare lesion. Hence, preoperative diagnosis
of LDD can be made with high accuracy using MRI and
1
H-MR spectroscopy.
© 2007 Elsevier Ireland Ltd. All rights reserved.
Keywords: Lhermitte-Duclos disease; Magnetic resonance imaging; Magnetic resonance spectroscopy
1. Introduction
Lhermitte-Duclos disease (LDD), also known as dysplas-
tic gangliocytoma of the cerebellum, is a rare cerebellar
disorder of uncertain etiology. LDD presents with highly
typical findings on magnetic resonance imaging (MRI).
Furthermore, magnetic resonance spectroscopy (MRS) can
provide additional information supporting the diagnosis of
the lesion. Herein, we present the MRI and
1
H-MRS findings
of a LDD patient, which has been confirmed histologically,
with a brief review of the relevant literature.
2. Case report
A 46-year-old male patient referred to our neurology
department with complaints of progressively increasing
*
Corresponding author at: Ankara
¨
Universitesi Tıp Fak¨ ultesi Cebeci Has-
tanesi, Radyoloji B ¨ ol¨ um¨ u, 06590 Cebeci, Ankara, Turkey.
Tel.: +90 532 3476156.
E-mail address: eozkavukcu@gmail.com (E.
¨
Ozkavukcu).
occipital headache lasting for 3 years, blurred vision,
and unbalanced gait. Neurological examination revealed
ataxia, moderate dysmetria in the right cerebellar tests, and
dysdiadokinesia. Romberg test was positive. In the ophtal-
mological examination, slight bilateral papillary edema was
observed.
Initially, cranial MRI examination was planned with a pre-
diagnosis of intracranial mass. MRI, which was performed
with a 1.5 T system (Signa Advantage, GE Medical Systems,
Milwaukee, WI, USA) revealed a striated lesion in the right
side of cerebellum, almost occupying the whole hemisphere.
The mass lesion was compressing the fourth ventricle and
cerebellar aquaduct, displacing the aquaduct slightly to the
left. The lesion had layers of hypo- and isointense signals on
T1-weighted (T1W) images (Fig. 1a), hyper- and isointense
signals on T2-weighted (T2W) images (Fig. 1b). No edema
accompanied the lesion. Hydrocephaly was also noted due to
the compression of the fourth ventricle and cerebral aquaduct.
Afterwards, MRS examination was performed using a
Proton Brain Exam/Single Voxel (PROBE/SV) software (GE
Medical Systems) added to the Signa MRI system (Signa
Advantage, GE Medical Systems, Milwaukee, WI, USA).
1571-4675/$ – see front matter © 2007 Elsevier Ireland Ltd. All rights reserved.
doi:10.1016/j.ejrex.2007.08.005