Abstract
Cerebral cavernous malformations (CCMs) are usually intraparenchymal lesions commonly associated with intraparenchymal hemorrhage. Extra-axial Lesions are uncommon and have rarely been reported as the cause of aneurysmal like subarachnoid hemorrhage (SAH). We present a 33-years-old female with a past medical history significant for hypertension who presented with acute onset head and neck pain after bending over. En route to the hospital, she had abnormal motor movements suggestive of seizures. Computed tomography (CT) of the head was significant for diffuse aneurysmal like SAH. Head CT angiography (CTA) and catheter digital subtraction angiography (DSA) were both negative for aneurysm. Magnetic resonance imaging (MRI) of the brain showed susceptibility artifact in the right parafalcine frontal lobe suspicious for underlying CCM. This lesion was in the area suspected of having an aneurysm on CT head. The patient was diagnosed with SAH secondary to cavernous malformation mimicking aneurysmal etiology. This case is important as it serves as a good reminder to consider cavernous malformations in the differential in patients presenting with angiogram negative SAH. Diagnosis of this entity has treatment implications including discontinuation of calcium channel blockers if initiated, discussion about duration of anti-seizure medication, in addition to consideration for surgical intervention in select populations.
Keywords: cavernous malformation hemorrhage, subarachnoid hemorrhage, cavernous angioma
Introduction
Cerebral cavernous malformations (CCMs) are abnormally enlarged capillary cavities without intervening brain parenchyma that are not usually visualized by angiograms due to limited blood flow.1,2 Their prevalence is estimated at .16% in the general population, occurring in both sporadic and inherited forms with several known genetic associations. 3 Cerebral cavernous malformations are commonly found incidentally but can also present with focal neurologic findings, seizures, and more commonly intraparenchymal hemorrhage with an estimated annual hemorrhage rate of 2.5% per patient-year. 4 We report a case of diffuse aneurysmal like subarachnoid hemorrhage (SAH) due to a possible CCM.
Case Description
A 33-years-old female with a history of hypertension presented with sudden onset severe holocephalic head and neck pain while bending over. The headache was accompanied by right lower extremity numbness and photophobia. En route EMS reported bilateral abnormal motor movements lasting 30 seconds followed by fatigue, suggestive of seizure. Her NIH Stroke Scale Score was 0, however, she reported increased pain with neck flexion and her blood pressure (BP) was 169/75.
Computed tomography (CT) of the head without contrast showed diffuse SAH suggestive of possible aneurysmal rupture. The patient was loaded with levetiracetam and a nicardipine infusion was initiated for strict BP control with a goal of <140/90. Computed tomography angiography of the head and neck showed no aneurysm, stenosis, or dissection. Given the extent of the hemorrhage catheter digital subtraction angiography (DSA) was performed and showed no aneurysm, stenosis, or dissection. She was placed on nimodipine due to the diffuse nature of SAH pending repeat DSA in 7 days. Overnight magnetic resonance imaging (MRI) of the brain with and without contrast was performed and showed a focal area of susceptibility artifact in the right parafalcine frontal lobe suspicious for underlying cavernoma (Figure 1).
Figure 1.
Top row A-C: Non-Contrast CT head showing diffuse SAH within the basal cistern, suprasellar cistern, sylvian fissures, anterior interhemispheric fissure, ambient cistern and hemispheric peripheral cortical sulci. Image C shows a possible focus in the Right Anterior Cerebral Artery region. Bottom Row D: Cerebral Angiogram negative for aneurysm in the ACA or Left MCA territories. E-F: Small area of susceptibility Artifact within the right para-falcine frontal lobe suggestive of a cavernoma.
Over the next few days, transcranial doppler showed no evidence of vasospasm. On day 7 repeat catheter DSA remained negative for arterial aneurysmal dilation or angiographic vasospasm leading to the final diagnosis of SAH due to cavernoma. During hospitalization resection of lesion was offered however patient declined. Nimodipine was discontinued and the patient was discharged in stable condition on levetiracetam. Unfortunately, patient was lost to follow up and therefore we were unable to obtain repeat MRI brain.
Discussion
Cerebral cavernous malformations commonly result in intraparenchymal hemorrhage and have rarely been reported as a cause of SAH mimicking aneurysmal etiology. There are only three published case reports of intracranial SAH due to CCMs in adults. In one case, a 70-years-old male presented with headache and was found to have a left cranial nerve IV palsy, imaging showed peri-mesencephalic SAH which was deemed to be due to 2 cavernous angiomas located in the left superior cerebellar peduncle and left posterior parietal lobe. 5 A second case described a 50-year-old woman who developed a severe headache and was found to have diffuse SAH in the basal cisterns and fourth ventricle which was concluded to be due to the presence of a CCM located on the surface of the left cerebellar hemisphere. 6 A third case described a 56-years-old male with a posterior fossa cavernous angioma causing SAH and persistent headache. 7
Three case series have also uncovered CCMs as a cause of SAH. One study of 32 patients with non-aneurysmal, non-traumatic SAH found 1 patient with a cavernoma. 8 Another study of 230 patients with CTA-negative SAH also found a single patient with a cavernous malformation. 9 Lastly a study containing 240 patients with SAH and angiogram negative evaluated the utility of MRI of the brain. One hundred and thirty-one patients underwent MRI of the brain, 2 patients were found to have cavernomas. 10 This study concluded that routinely performing MRI of the brain in such patients is of low diagnostic yield and likely unnecessary. Other studies have instead suggested that MRI can be useful to evaluate organic intracranial processes in patients with atypical SAH and high Hunt & Hess scores suggestive of high mortality. 8
Though a rare cause of SAH, our case serves as a good reminder that in patients presenting with angiogram negative for SAH and no pathological cause on CT, there is a need to entertain structural brain pathology such as CCMs. We suggest that MRI of the brain be considered in such cases to rule out pathologic processes such as CCMs. These findings may guide acute and chronic management including, but not limited to, discontinuation of calcium channel blockers, duration of antiseizure medication, and discussion of surgical resection in the appropriate patient population.
Footnotes
Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding: The author(s) received no financial support for the research, authorship, and/or publication of this article.
ORCID iD
Cleopatra Thurman https://orcid.org/0000-0002-9075-8602
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