First episode of seizure in young adult :cerebral caverous malformation

Clinical History

The patient presented in emergency department after a single seizure episode few hours back. There was no history of fever, headache, weakness, head injury, recent medication or any previous history of seizure. Patient has no history of smoking, alcohol or illicit drug intake. Patient was conscious, oriented, with normal systemic examination. Blood investigations where normal.

Imaging Findings

Head NCCT showed a well-delineated heterogeneous predominantly hyperdense lesion with scattered intralesional calcification, without evidence of mass effect in left frontal region (Fig 1 and 2).
CE-MRI showed a well-delineated, variegated altered signal intensity lesion in the left frontal region which appears heterogeneous predominantly hyperintense on T2WI, FLAIR and hyperintense on T1WI with focal areas of signal drop, with peripheral rim on T2WI (haemosiderin rim) demonstrating mulberry appearance. The lesion showed blooming on SWI sequences (calcification/bleed) with evidence of non-restriction on DWI. The lesion showed mild enhancement on post contrast scans (atypical finding).

Discussion

The first seizure in young adults can have many causes. Intracranial space occupying a lesion of infective aetiology (tuberculoma /neurocysticercosis ) is one of the common causes in South Asia, however malformative or neoplastic causes such as gangliogliomas or gangliocytomas and DNET are less common, although seizure is a common presentation of these lesions [1].

Cerebral cavernous malformation (CCM) are benign malfomative hamartomas, and the third most common cerebral vascular malformation after developmental venous anamoly and capillary telengiectasia with an incidence of 0.5- 0.9% in the general population [2, 3]. Cerebral cavernous malformations are also the most common components in mixed vascular malformations. The lesions have acquired (rare) and genetic causes, at least half of the cases are familial, inherited as an autosomal dominant disease with variable penetrance [4].
Seizures are the most common manifestation, both in adults and children, followed by headache and focal symptoms [5].

Cantu et al. noted a two-fold increased rate of haemorrhage per patient and per year for Cavernous angioma (CCA) located in brainstem and cerebellum, and deep hemispheric CCA [6].
MRI is the best radiological technique to explore cavernomas. In the emergency setting, the first exam is often a CT for patients presenting acute neurological sign(s) and/or with a clinical suspicion of haemorrhagic stroke. Angiography is generally not contributive; nevertheless, this exam is often necessary when an associated vascular abnormality is suspected.

Management of cerebral cavernous malformations is expectant follow-up in asymptomatic patients or lesions in inaccessible sites, microsrgical resection of symptomatic and accessible lesions, sterotactic radiosurgery of symptomatic lesions at inoperable sites .

Take home message:

Intracranial space occupying lesions is a common cause of seizures in young patients, warranting evaluation by cross-sectional imaging.

Some cerebral vascular malformations (e.g. capillary malformations ) are almost clinically silent and found incidentally, others such as cavernous malformations may haemorrhage unexpectedly.

Differential Diagnosis List

Final Diagnosis

Cerebral cavernous malformation ( Zabrasmski type 2 ).

URL:	https://www.eurorad.org/case/13113
DOI:	10.1594/EURORAD/CASE.13113
ISSN:	1563-4086