Accepted Manuscript
Primary Multiple Intracranial Cystic Brain Lesions: A Diagnostic Dilemma
Ashutosh Gupta, MD, DM (Neurology), Senior Resident, Sandeep Singh, MBBS,
Dyutima Madan, MBBS, Junior Resident
PII:

S0887-8994(18)31176-7

DOI:

https://doi.org/10.1016/j.pediatrneurol.2018.11.015

Reference:

PNU 9470

To appear in:

Pediatric Neurology

Received Date: 4 November 2018
Revised Date:

19 November 2018

Accepted Date: 21 November 2018

Please cite this article as: Gupta A, Singh S, Madan D, Primary Multiple Intracranial Cystic
Brain Lesions: A Diagnostic Dilemma, Pediatric Neurology (2018), doi: https://doi.org/10.1016/
j.pediatrneurol.2018.11.015.
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Title page
Primary Multiple Intracranial Cystic Brain Lesions: A Diagnostic Dilemma
Corresponding author:

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Contributors

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Sandeep Singh, MBBS
Clinical Epidemiology, Biost. and Bioinf.
Academic Medical Center, J1B-227
Meibergdreef 9
1105 AZ Amsterdam
Email: s.singh@amc.uva.nl, sandeepkcsingh@gmail.com, +31-685668962

1. Ashutosh Gupta

2.

Sandeep Singh

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MD, DM (Neurology)
Senior Resident
Department of Neurology,
IHBAS (Institute of Human Behaviour & Allied Sciences),
New Delhi, India.
Email: drashutosh.gupta24@gmail.com

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MBBS
Scientific Researcher
Clinical Epidemiology, Biost. and Bioinf.
Academic Medical Center
sandeepkcsingh@gmail.com

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3. Dyutima Madan

MBBS
Junior Resident
Department of Neurology,
IHBAS (Institute of Human Behaviour & Allied Sciences),
New Delhi, India.
Email: dyutima.d.m@gmail.com

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Primary Multiple Intracranial Cystic Brain Lesions: A Diagnostic Dilemma
Case report

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A 19 year old male presented in emergency in an unconscious state with refractory generalized
tonic clonic seizures (GTCS). History of fever, vomiting, immunocompromised state, any
metabolic derangement and trauma or surgery was absent. Management was started as per status

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epilepticus protocol. Routine laboratory parameters were within normal limits. Magnetic
resonance imaging (Figure 1) revealed multiple thin walled, varying size, cystic lesions in both

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cerebral hemisphere highly suggestive of hydatid cyst. Extensive workup failed to reveal primary
disease in any other organs. Finally magnetic resonance spectroscopy (MRS) was advised and
presence of pyruvate peak on the MRS leads to the final diagnosis of hydatid cystic lesions
(Figure 2). The patient was started on steroid and anticonvulsant but, due to the uncontrolled

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seizures patient was shifted on mechanical ventilation. Even after heroic management patient
landed up in super-refractory status and died after 48 hours after the admission.
Comment

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Cerebral hydatidosis represents 0.05% of all intracranial mass lesions. In endemic regions of the
world and in Indian endemic zone, intracranial hydatidosis is rare and accounts for only 1-2%

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and 0.2% respectively and usually presents as solitary lesion 1-3. Multiple intracranial hydatosis
is an extremely rare phenomenon, either developing spontaneously or owing to trauma or after
surgery 1,3,4. Absence of trauma or surgical event in the present case most probably suggest it to
be a spontaneous event. If these cystic lesions in the brain occur directly infesting the brain
parenchyma, without involving any other organ system, are classified as primary disease.
Primary intracranial hydatid disease is a rare phenomenon and the primary multiple disease is

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being considered as rarest of rare and only isolated cases documented in the literature. 3,4.
Headache, vomiting and motor weakness, features of raised intracranial tension, are being
reported as the most common and earliest symptom of presentation, lacking in our case 4.

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Although, seizure is not uncommon but has been reported as least frequent presentation.

Multiple intracranial hydatosis being considered as the lethal disease so timely diagnosis is
imperative for the better outcome. These multicystic lesions could have various presentations on

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imaging leading to differentials ranging from astrocytoma to infective lesions 2. Importance lies
in the timely differentiation of these differentials from other multiple intracranial lesions. MRI

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being superior to the CT for diagnosing such cases; however, MRI itself will not suffice.
Therefore MRS was considered for the final call regarding the confirmation. Reports suggest
that pyruvate peak in MRS has been considered as a very specific in vivo marker particularly in
the hydatid cystic lesion 5,6. Even though multiple primary hydatid intracranial cystic lesion is

2.
3.
4.
5.
6.

Ersahin Y, Mutluer S, Guzelbag E. Intracranial hydatid cysts in children. Neurosurgery.
1993;33(2):219-224; discussion 224-215.
Bartosch C, Reis C, Castro L. Large solitary cerebral hydatid cyst. Archives of Neurology.
2011;68(7):946-947.
Gupta S, Desai K, Goel A. Intracranial hydatid cyst : a report of five cases and review of
literature. Neurology India. 1999;47(3):214-217.
Özkan Ü, Kemaloğlu M, Selçuki M. Gigantic intracranial mass of hydatid cyst. Child's Nervous
System. 2001;17(10):623-625.
Kohli A, Gupta RK, Poptani H, Roy R. In vivo proton magnetic resonance spectroscopy in a case of
intracranial hydatid cyst. Neurology. 1995;45(3 Pt 1):562-564.
Jayakumar PN, Srikanth SG, Chandrashekar HS, Kovoor JM, Shankar SK, Anandh B. Pyruvate: an
in vivo marker of cestodal infestation of the human brain on proton MR spectroscopy. Journal of
magnetic resonance imaging : JMRI. 2003;18(6):675-680.

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References

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rare it should always be considered in the differential diagnosis in the cases from endemic areas.

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Figure 1. MRI brain showing axial T2 images at the (a) supra-ganglionic level and (b)
ganglionic level, having multiple thin walled cystic lesions which are variable in size from 2mm
to 7cms widely distributed over both the hemispheres. Fig 1 (c, d) MRI brain axial T1 post
contrast images of the same level, as described above, further delineating the contrast ring
enhancement of the walls of the lesions.

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Figure 2. In-vivo MR spectroscopy of the lesion over right parietal region, performed by
multivoxel chemical shift imaging technique at (a) TE-144msec and (b) TE-35msec respectively.
Figure 2(a) showing maintained N-acetyl aspartate (NAA) peak at 2 ppm, stable creatine peak at
3 ppm and mildly increased choline peak at 3.2 ppm. An inverted bifid lactate peak was noted at
1.3 ppm due to J coupling. Figure 2(b) in addition to figure 2(a) showing pyruvate peak at 2.48
ppm (encircled in green). These spectroscopic findings are in favor of intracranial hydatidosis.