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NEUROIMAGE
Year : 2023 | Volume
: 71
| Issue : 5 | Page : 1090-1091
Sturge–Weber Syndrome and Hippocampal Sclerosis: Two Epileptogenic Conditions in One Patient
João Vitor Gerdulli Tamanini1, Yuri Inoue2, Tânia Aparecida Marchiori de Oliveira Cardoso1, Fabiano Reis2
1 Department of Neurology, Faculty of Medical Sciences, Campinas State University, Campinas, Brazil
2 Department of Radiology, Faculty of Medical Sciences, Campinas State University, Campinas, Brazil
Correspondence Address:
Fabiano Reis
Department of Radiology, Faculty of Medical Sciences, Campinas State University, Campinas
Brazil
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/0028-3886.388109
How to cite this article:Sturge–Weber syndrome (SWS) is a rare genetic neurocutaneous disorder caused by activating mutations in the GNAQ gene. It usually presents with port wine stains on the skin, with ipsilateral capillary-venous leptomeningeal malformation, and seizures. Specifically, seizures would be a consequence of cortical hypoxia, ischemia, and gliosis due to vascular malformations and usually present as focal aware or focal impaired awareness seizures or even as focal to bilateral tonic–clonic.[1]
On the other hand, a more frequent cause of epilepsy is hippocampal sclerosis (HS). Particularly, HS presents with neuronal loss and gliosis that might worsen due to seizures. Therefore, HS has been discussed as both cause and consequence of epilepsy.[2]
The association of two epileptogenic lesions—one hippocampal and the other extrahippocampal—has been described as dual pathology.[3] However, dual pathology with SWS and HS has not been frequently reported in literature. Therefore, we describe a case of an individual with SWS findings in the left cerebral hemisphere and ipsilateral HS. The patient is a 34-year-old woman who presented with Todd's paralysis of the right upper and lower limbs after a bilateral tonic–clonic seizure. The patient presented no other forms of seizure. The findings on neuroimaging are shown in [Figure 1]. The patient was discharged after the resolution of symptoms with optimization of anti-seizure therapy that consisted of carbamazepine and clobazam. She has not presented with any further seizure episodes.
Figure 1: Brain computed tomography (CT) demonstrates tram-track sign of cortical and subcortical calcification (circle) and associated parenchymal volume loss (a). Brain magnetic resonance imaging on T1 after gadolinium (b) reveals an enlarged and enhancing left glomus angioma (white arrow) and prominent transmedullary vein (black arrow), a temporo-occipital pial angioma (dotted black line), and dural enhancement (white dotted arrow). Coronal T2-weighted image (c) shows hypointensity in the subcortical white matter (black arrow), volumetric reduction, and also calvarial thickening (white arrow). Coronal T2-weighted image (d) demonstrates volumetric reduction and hyperintensity in the left hippocampus (black arrow), consistent with hippocampal sclerosisThis case highlights a rare syndromic cause of epilepsy with one of the most common epilepsy-associated lesions in one single patient as well as related neuroimaging findings.
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 initials 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
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