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. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. ACCEPTED MANUSCRIPT Title page Primary Multiple Intracranial Cystic Brain Lesions: A Diagnostic Dilemma Corresponding author: M AN U Contributors SC RI PT 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 TE D MD, DM (Neurology) Senior Resident Department of Neurology, IHBAS (Institute of Human Behaviour & Allied Sciences), New Delhi, India. Email: drashutosh.gupta24@gmail.com EP MBBS Scientific Researcher Clinical Epidemiology, Biost. and Bioinf. Academic Medical Center sandeepkcsingh@gmail.com AC C 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 ACCEPTED MANUSCRIPT 1 Primary Multiple Intracranial Cystic Brain Lesions: A Diagnostic Dilemma Case report RI PT 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 SC epilepticus protocol. Routine laboratory parameters were within normal limits. Magnetic resonance imaging (Figure 1) revealed multiple thin walled, varying size, cystic lesions in both M AN U 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 TE D 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 EP 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% AC C 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 ACCEPTED MANUSCRIPT 2 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. RI PT 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 SC 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 M AN U 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. AC C 1. EP References TE D rare it should always be considered in the differential diagnosis in the cases from endemic areas. ACCEPTED MANUSCRIPT TE D M AN U SC RI PT 3 AC C EP 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. ACCEPTED MANUSCRIPT M AN U SC RI PT 4 AC C EP TE D 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.