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|Year : 2022 | Volume
| Issue : 2 | Page : 822-823
Cerebral Cavernous Malformations Confined in the Cerebral Sulci
Satoshi Tsutsumi1, Ikuko Ogino2, Akihide Kondo2, Hisato Ishii1
1 Departments of Neurological Surgery, Juntendo University Urayasu Hospital, Urayasu, Chiba
2 Department of Neurological Surgery, Juntendo University School of Medicine, Bunkyo-Ku, Tokyo, Japan
|Date of Submission||09-Sep-2020|
|Date of Decision||04-Oct-2021|
|Date of Acceptance||11-Oct-2021|
|Date of Web Publication||3-May-2022|
Dr. Satoshi Tsutsumi
Department of Neurological Surgery, Juntendo University Urayasu Hospital, 2-1-1 Tomioka, Urayasu - 279-0021, Chiba
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Tsutsumi S, Ogino I, Kondo A, Ishii H. Cerebral Cavernous Malformations Confined in the Cerebral Sulci. Neurol India 2022;70:822-3
Cerebral cavernous malformations (CCMs) are hamartomatous vascular anomalies occurring in the central nervous system. A fraction of CCMs shows familiar forms. On neuroimages, a CCM typically exhibits a well-circumscribed, parenchymal lesion accompanying hemorrhage and iron deposition. A 29-year-old woman was transported to the Emergency Department sustaining a generalized seizure. Cerebral magnetic resonance imaging (MRI) revealed multiple lesions in the cerebral hemispheres and brainstem. They appeared isointense on gradient-echo sequence and hypointensity on T2-weighted imaging. Some of them were located in the cerebral sulci, instead of the parenchyma. They lacked perilesional hemosiderin deposits or brain edema. Besides, cerebral edema was noted in the left temporal lobe [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d, [Figure 1]e, [Figure 1]f, [Figure 1]g. Based on the radiological appearance, familial CCMs were assumed. The patient underwent genomic analysis as a previous investigation. The procedure identified a CCM1 (KRIT1) missense variant (c. 470T > C; p.Leu157Ser). Oral administration of levetiracetam was started with satisfactory control of the patient's seizure. The MRI performed 9 months later revealed stable appearance of lesions in the cerebral sulci andregression of the cerebral edema [Figure 1h]. To our knowledge, there is no study documenting CCMs confined in the cerebral sulci. The unusual locations outside the brain parenchyma can be, at least partly, explained by the nature of the CCM1 that causes abnormal angioproliferation in any part of the brain. In addition, the vascular permeability of CCMs is suggested to correlate with the disease activities. These two factors might have contributed to the unusual formation of CCMs in the cerebral sulci and its unique clinical appearance.
|Figure 1: (a–f) Axial gradient-echo (a, d) and T2-weighted (b, e) magnetic resonance imaging at presentation showing CCMs in the cerebral sulci appearing isointense on gradient-echo and hypointense on T2-weighted sequence. (c) Magnified view of the boxed area in b. (f) Magnified view of the boxed area in e. (g) Axial T2-weighted image at presentation showing cerebral edema in the left temporal lobe (asterisk) and its magnified view (inset). (h) Axial T2-weighted MRI taken 9 months later showing resolution of cerebral edema and emergence of parenchymal CCM (arrow) with their magnified view (inset).|
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We declare that the present study has been approved by the institution's guidelines for human research, and performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.
Declaration of patient consent
We declare that the presented patient gave informed consent prior to inclusion in this study.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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