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IMAGES IN NEUROLOGY
Year : 2021  |  Volume : 24  |  Issue : 4  |  Page : 592
 

Tram track sign in sturge-weber syndrome Type 3


1 Department of Neurology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Neuroradiology, All India Institute of Medical Sciences, New Delhi, India

Date of Submission22-Jan-2021
Date of Acceptance14-Feb-2021
Date of Web Publication21-May-2021

Correspondence Address:
Dr. Manjari Tripathi
Department of Neurology, Room No. 705, C.N. Centre, All India Institute of Medical Sciences, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aian.AIAN_69_21

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How to cite this article:
Mishra B, Singh RK, Garg A, Vibha D, Bhargavi R, Tripathi M. Tram track sign in sturge-weber syndrome Type 3. Ann Indian Acad Neurol 2021;24:592

How to cite this URL:
Mishra B, Singh RK, Garg A, Vibha D, Bhargavi R, Tripathi M. Tram track sign in sturge-weber syndrome Type 3. Ann Indian Acad Neurol [serial online] 2021 [cited 2021 Oct 25];24:592. Available from: https://www.annalsofian.org/text.asp?2021/24/4/592/316506





   Clinical Summary Top


A 36-year-old male, without any previous comorbidities, presented with new-onset recurrent left focal Seizure with secondary generalization for 48 h. He developed refractory status epilepticus, which was controlled after infusion of anesthetic agents. The MRI brain revealed volume loss in the right parieto-occipital lobe with gyriform cortical hypointensity in T2-WI [arrow in [Figure 1]a] and susceptibility-WI [arrow in [Figure 1]b] hyperintensity in PHASE images suggestive of calcification (tram-track calcifications). Postgadolinium T1-WI showed gyriform enhancement [arrow in [Figure 1]c] in the right parieto-occipital lobe suggesting pial angiomas and an enlarged enhancing right choroid plexus [arrow in [Figure 1]d]. The imaging features were suggestive of Sturge-Weber syndrome (SWS).[1] In the absence of facial angiomas, and glaucoma on ocular examination, we made the diagnosis of SWS Type 3. Type 3 is the rarest variety of SWS spectrum disorders, which can present in adults for the first time, usually with seizures[2] or migraine.[3] Despite achieving adequate seizure control, the present case succumbed to in-hospital respiratory complications. As there are no neurocutaneous manifestations, and the clinical presentation can be highly variable, from migraine-like attacks with visual aura,[4] to hemiparesis and status epilepticus, a high index of suspicion is required to diagnose SWS type 3, and the diagnosis is usually clinched with the help of characteristic imaging findings.
Figure 1: The MRI brain revealed volume loss in the right parieto-occipital lobe with gyriform cortical hypointensity in T2-WI (arrow in a) and susceptibility-WI (arrow in b); hyperintensity in PHASE images (not shown) suggestive of calcification (tram-track calcifications). Postgadolinium T1-WI showed gyriform enhancement (arrow in c) in the right parieto-occipital lobe suggesting pial angiomas and an enlarged enhancing right choroid plexus (arrow in d). The imaging features were suggestive of Sturge-Weber syndrome (SWS)
Commentary or discussion on the imaging findings


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Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Hu J, Yu Y, Juhasz C, Kou Z, Xuan Y, Latif Z, et al. MR susceptibility weighted imaging (SWI) complements conventional contrast enhanced T1 weighted MRI in characterizing brain abnormalities of Sturge-Weber Syndrome. J Magn Reson Imaging 2008;28:300-7.  Back to cited text no. 1
    
2.
Ishikawa H, Ii Y, Niwa A, Matsuura K, Maeda M, Tomimoto H. A case of 55-year-old man with first-ever generalized seizure diagnosed with Sturge-Weber syndrome type III by characteristic MRI findings. Rinsho Shinkeigaku 2017;57:214-19.  Back to cited text no. 2
    
3.
Jordan PR, Iqbal M, Prasad M. Sturge-Weber syndrome type 3 manifesting as 'Status migrainosus'. BMJ Case Rep 2016;2016:bcr2016216842.  Back to cited text no. 3
    
4.
Huang HY, Lin K-H, Chen J-C, Hsu Y-T. Type III Sturge-Weber syndrome with migraine-like attacks associated with prolonged visual aura. Headache 2013;53:845-9.  Back to cited text no. 4
    


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