Unusual presentation of more common disease/injury

Intracranial venous sinus thrombosis mimicking Lhermitte
Duclos disease
Heramba Narayan Praharaj, Maneesh Kumar Singh, Ravindra Kumar Garg, Tushar Premraj Raut
Department of Neurology, Chhatrapati Shahuji Maharaj Medical University, Lucknow, Uttar Pradesh, India
Correspondence to Dr Heramba Narayan Praharaj, babipraharaj@gmail.com

Summary
A middle-aged man presented with 1-month history of headache, dizziness, staggering of gait and progressive deafness. Examination
revealed decreased visual acuity, papilloedema, gaze evoked nystagmus, bilateral sensorineural deafness and gait ataxia. MRI showed TI iso
and T2 hyperintensity of cerebellum, prominence of folia with gyral enhancement in a tigroid pattern making impression of the rare entity,
Lhermitte Duclos disease (LDD) or dysplastic gangliocytoma of cerebellum. Conservative management for the raised intra cranial pressure did
not have any impact on patient’s general condition for which an MR venogram was planned and it surprisingly showed non-visualisation of
left transverse sinus and internal jugular vein, suggestive of thrombosis. Patient was treated with anticoagulants but he succumbed to his
illness soon after. We present this case to convey that a high index of suspicion should always be there in mind for this commoner condition
(venous sinus thrombosis) before diagnosing the rarer ones (here LDD disease) to prevent the disastrous consequences of the former.

BACKGROUND
Cerebral venous sinus thrombosis (CVST) is a relatively
common condition resulting from various infective, inflammatory, neoplastic and thrombophilic conditions.1 Though
the classical presentation is with headache, vomiting,
seizure, focal deficits, vision loss and alteration of sensorium, unusual forms of presentation may be seen. Though
MR venogram clinches the diagnosis, plain MRI may not
show the classical signs of venous thrombosis and hence
sometimes creates diagnostic confusion especially when
the presentation mimics other rare diseases.2 We here
present a similar case whose MRI resembled Lhermitte
Duclos disease (LDD), a rare neuronal tumour of cerebellum but MR venogram revealed intracranial venous sinus
thrombosis. Our message is that, a high index of suspicion
should be there regarding CVSTeven if plain MRI misleading and we should go for a venogram to diagnose at the
earliest and prevent the potential mortality.

CASE PRESENTATION
A 42-year-old man, pharmacist by profession, presented to
our department with 1 month history of dizziness and
staggering of gait. There was headache associated with dizziness but no vomiting. After 15 days he complained of
decreased hearing which gradually increased in intensity so
that patient at admission was not able to hear even when
spoken close to his ears. There was no history of fever, ear
discharge, seizures, unconsciousness, drug intake, any
other comorbid illness and family history was not contributory. There were no symptoms suggestive of any
other cranial nerve dysfunction. Neurological examination
showed apparently normal higher function. Cranial nerve
examination showed visual acuity of 6/12, early papilloedema, normal occular movements, bilateral gaze evoked
nystagmus and gross bilateral sensorineural hearing loss.
Motor and sensory examinations were unremarkable but
BMJ Case Reports 2012; doi:10.1136/bcr-2012-007058

there was ataxia of gait. Skull and spine were normal and
there were no meningeal signs.

INVESTIGATIONS
Biochemical and haematological investigations including
homocystine and vitamin B12 were unremarkable. BAER
(Brainstem Auditory Evoked Response) showed no recordable wave forms. Cerebrospinal fluid (CSF) showed an
increase in protein with pleocytosis but work-up for
tubercular or viral aetiology was negative. MRI of brain
showed T1 isointense (figure 1) and T2 hyperintense
(figure 2) diffusely involving whole of the grey matter of
cerebellum, thickening of cortical layer, prominent folia
with contrast enhancement producing a tigroid pattern
(figure 3).

DIFFERENTIAL DIAGNOSIS
The aforementioned MRI features are characteristic of a
dysplastic neuronal tumour of cerebellum called LDD.
Cerebellar infarct sometimes produces similar picture of
prominent folia despite mass effect.2

TREATMENT
He was initially treated with intravenous steroids, mannitol and diuretics.

OUTCOME AND FOLLOW-UP
Patient did not have any significant improvement in
symptoms. An MR venogram was planned and it surprisingly showed non-visualisation of left transverse sinus
and left internal jugular vein suggesting thrombosis
(figures 4 and 5). He was immediately started on
low-molecular-weight heparin but he did not show a satisfactory response and went on deteriorating and succumbed to his illness on the 20th day of admission.
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Figure 3 T1 contrast coronal MRI of brain showing
enhancement of cerebellum in a tigroid pattern.

Figure 1 T1 weighted axial MRI of brain showing isointense
cerebellum.

DISCUSSION
CVST commonly presents with headache, vomiting, seizures, acute onset focal deficits and disorientation.

Figure 2 T2 weighted coronal MRI of brain showing hyperintense
cerbellar cortex with thickening and prominence of folia.
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Sometimes the presentation may be that of a subacute
onset craniopathy like vestibular neuropathy, deafness and
facial weakness.3 Presenting with bilateral deafness is
extremely unusual in venous sinus thrombosis and was
misleading in our case.
Classical MRI features of CVST on plain MRI include
absence of flow void, bilateral or multifocal infarcts
beyond distribution of arterial occlusion, a haemorrhagic
infarct or visualisation of enhancing thrombus.1 When
these features are not distinct, it may sometimes be

Figure 4 Sagital MR venogram of brain showing non
visualisation of left transverse sinus.
BMJ Case Reports 2012; doi:10.1136/bcr-2012-007058

MRI shows T1 hypointensity and T2 hyperintensity
involving one or both lobes of cerebellum2 with prominence of folia producing a characteristic tigroid pattern
(resembling our case). The lesion may or may not
enhance with contrast.4 There may be hydrocephalous
due to the whole mass obstructing the fourth ventricle.
As mentioned earlier, the similar may be found only in
infarct of cerebellum (venous or arterial).
CSF examination in CVST is usually normal but may
show increased protein, and when associated with an
increased cell count3 may create confusion with tubercular
meningitis as happened in the present case. We did not
get any evidence of tuberculosis in CSF in form of AFB or
PCR positivity.
Treatment also varies for the two conditions. Where
surgery is curative for LDD, prompt treatment with anticoagulant is necessary in CVST.2 Mortality in untreated
cases of the latter may range from 5.5% to 18% for which
an early MR venogram in appropriate clinical setting is
inevitable even if MRI is atypical.

Learning points

Figure 5 Coronal MR venogram of brain showing non
visualisation of left transverse sinus and left internal jugular vein.
difficult to diagnose CVST on plain MRI. MRI in our case
showed T1 isointensity and T2 hyperintensity of the
whole of cerebellar cortex with contrast enhancement and
prominence of folia in a tigroid pattern which mimics a
neuronal tumour of cerebellum called the dysplastic cerebellar ganglionocytoma or LDD (mentioned below).
LDD is one of the neuronal tumours of brain which
presents with sub-acute onset cerebellar signs and symptoms of raised intra cranial pressure in form of headache,
vomiting, ataxia, visual blurring and deafness. The
tumour consists of a hamartomatous proliferation of ganglion cells which replaces the inner granular cell and
middle Purkinje cell layer of cerebellum.3

▸ Intracranial venous sinus thrombosis is a potentially
treatable condition if diagnosed early.
▸ MRI may not always show the classical features and
may mimic other rare diseases like Lhermitte Duclos
disease in the present case.
▸ An early MR venogram in appropriate clinical setting is
lifesaving even if plain MRI is atypical.

Competing interests None.
Patient consent Obtained.

REFERENCES
1. Allroggen H, Sarawan J. Cerebral venous sinus thrombosis. J Postgrad Med
2000;76:12–15.
2. Bayat MRH, Sarawan J. CT and MRI findings in Lhermitte Duclos disease.
SA J Radiol 2005;Feb:30–1.
3. Bozbuga M, Gulec I, Suslu HT, et al. Bilateral Lhermitte Duclos disease.
Neurol India 2010;58:309–11.
4. Spaargaren L, Cras P, Bomhof MA, et al. Contrast enhancement in
Lhermitte-Duclos disease of the cerebellum: correlationof imaging and
neuropathology in two cases. Neuroradiology 2003;45:381–5.

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Praharaj HN, Singh MK, Garg RK, Raut TP. Intracranial venous sinus thrombosis mimicking Lhermitte Duclos disease.
BMJ Case Reports 2012;10.1136/bcr-2012-007058, Published XXX
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