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Cerebral cavernoma

Cavernomas or cavernous malformations of the brain are lesions of the cerebral capillary blood vessels, which, when dilated and irregular, allow blood components to "leak" into neighboring brain tissue. It is a common disease (0.5% of the population) but little known since most carriers are asymptomatic. It is estimated that the reserve of patients with cavernous brain lesions without symptoms is greater than the number already diagnosed. Most symptomatic patients have epilepsy, headache or neurological deficiency, usually due to bleeding from the lesions. Magnetic resonance imaging is the most effective imaging method for detecting and analyzing a cerebral cavernous malformation. Pop-corn imaging is typical, demonstrating a heterogeneous lesion of different intralesional signal intensities, which represent small thrombi within the cavernoma secondary to its low flow, and a halo of hypodensity due to extravasation of blood elements, mainly hemosiderin. In the case above, we have a female patient with sudden headache who underwent brain MRI, where an oval image in the popcorn-shaped left frontal lobe was identified, with hypersignal on its peripheries and central hyposignal on FLAIR sequences. , T2, T1 and hyposignal in the SWI sequence and with post GD contrast reaction in the T1 sequence, compatible with cerebral cavernoma.

Interesting Cases

Axial FS T2-weighted MR image through the abdomen of this patient shows innumerable round small to moderate sized hepatic lesions (white arrows) which are moderately high in signal intensity. A few of the lesions have prominent intralesional/perilesional flow voids (black arrows). These US and MRI appearances are typical of infantile hemangiomas in the liver, which are most commonly found in infants with 5 or more cutaneous infantile hemangiomas. Multifocal or diffuse hepatic IHs can cause liver failure, heart failure, hypothyroidism, and compartment syndrome. As with cutaneous IHs, propranolol is typically the first-line therapy. (Note that these lesions are in contradistinction to the congenital large solitary hypervascular liver lesions classically causing heart failure in newborns; such lesions are congenital hemangiomas, a different type of benign vascular tumor.)

Axial T1 FLAIR (image 1), T2 (image 2), FLAIR (image 3), and T1 post-contrast (image 4) MRI show a cavernous malformation (arrow) in the right temporal lobe. The lesion exhibits heterogeneous T1 signal and minimal enhancement, with a characteristic hypointense rim on T2 and FLAIR due to mild hemorrhage. Most patients with a cavernous malformation are asymptomatic but can present with headaches, seizures, or focal deficits if hemorrhage occurs.

Sagittal T1 (image 1), coronal T2 (image 2), and axial susceptibility-weighted MRI (image 3) reveal a cavernous malformation (arrow) within the cervical spinal cord. Cavernous malformations are predominantly found in the brain, with spinal cord lesions accounting for approximately 5% of malformations. Spinal cavernous malformations most commonly occur in the thoracic region. Unlike brain lesions, spinal cord cavernous malformations are usually asymptomatic but may present with acute, stepwise neurological decline or a gradual progression of symptoms.

Interesting case I saw today at Epilepsy clinic..... A 24-year-old female referred as "query seizures" had a 9 year history of stereotypical events with aphasia, staring off, zoning out for a few seconds, with awareness impaired during them, followed by confusion and sometimes tiredness. MRI, SWi sequence is shown, as you can see on the right temporal region there is a hypointense lesion "popcorn like", classic for cavernoma. Cavernomas are sinusoidal venous vessels with low flow, hence, many times they are not seen on FLAIR, nor seen on CT. (In our example, you could see a small lesion on FLAIR, which is rare). They can bleed. In this case, genetic testing was done since she had a relative affected and it did confirm KRIT 1 mutation was found, in keeping with familial CCM (cerebral Cavernoma malformation). Her EEG however, shows a sharp wave on the LEFT temporal region (confirmed by average montage). After a prolonged, 19-day EMU admission, we saw 2 PNEEs, when she described having a seizure, without EEG correlate. After a good amount of education and counseling, she remained Szx free. We DC her ASMs. Her cavernoma on the right side is asymptomatic for now. They can grow with time and eventually burst, but that would be a different story to tell. The point here was, EMU admission, sometimes you need to push hard, especially if you suspect a structural lesion such as this. In this case, this translated ruling out epileptic seizures, but could well be the other way around since the lesion is in the right place to cause focal unaware seizures. #loveneurology.