Multiple sporadic cerebral cavernous malformations

Múltiplos cavernomas cerebrais esporádicos

A 32-year-old previously healthy man presented with headache that progressively worsened during the day. He denied any previous history of headache and trauma. There was no family history of neurological diseases. Examination revealed nuchal rigidity. Cranial computed tomography disclosed a left frontal hemorrhage. Brain magnetic resonance imaging revealed multiple cerebral cavernous malformations (CCM). The patient received conservative treatment. Cerebral cavernous malformations are commonly described in the familial form and are frequently asymptomatic. When symptoms do occur, seizures are the most common followed by focal deficits and headache¹. Several mutations in CCM genes have already been identified in patients with sporadic disease².

Figure 1
Axial cranial CT scan: an oval-shaped hyperdense lesion in the left frontal lobe with perilesional vasogenic edema and a small focus of blood in the contralateral frontal lobe.

Figure 2
Brain MRI in axial T1 (A), Axial-susceptibility-weighted (B) and multiplanar reconstruction (C) showing an heterogeneous lesion with a hyperintense signal associated with perilesional vasogenic edema suggestive of acute hematoma. Additionally, there are multiple nodules with hypointense signal throughout the parenchyma and a subarachnoid hemorrhage in the left Sylvian fissure.

References

• ¹
  Chahine LM, Berg MJ. Clinical reasoning: cerebral cavernous malformations. Neurology. 2009;73(9):e44-9. https://doi.org/10.1212/WNL.0b013e3181b59a5b
  » https://doi.org/10.1212/WNL.0b013e3181b59a5b
• ²
  Scimone C, Bramanti P, Alafaci C, Granata F, Piva F, Rinaldi C et al. Update on novel CCM gene mutations in patients with cerebral cavernous malformations. J Mol Neurosci. 2016;61(2):189-98. https://doi.org/10.1007/s12031-016-0863-z
  » https://doi.org/10.1007/s12031-016-0863-z

Publication Dates

• Publication in this collection
  Dec 2017

History

• Received
  25 Jan 2017
• Reviewed
  28 Aug 2017
• Accepted
  05 Sept 2017