Dr. Foram Gala, Dr. Swapnil MoharkarPatient
4 years, male
A four-year-old boy was brought to the hospital with recurrent episodes of focal left seizures since one and half years of age. Antiepileptic medications were started for the same. He had breakthrough seizures on medications, and hence MRI was advised.
On MRI, there was a large diffuse ill-defined lesion showing hypointense signal on T1 and T2W images involving the right high frontoparietal region, centrum semiovale and corona radiata (Figures 1,2). Few well-defined T1/T2 hyperintense ovoid/ round areas were seen in the lesion suggestive of late subacute hematomas. (Figures 1b,2)
On post-contrast scans, there was gyriform enhancement involving cortex and subcortical white matter. (Figures 3a)
Extensive blooming was seen on SWI (susceptibility weighted imaging) images. (Figures 3b)
On the FLAIR sequence, the lesion was hypointense due to calcifications and haemorrhage. (Figures 4)
The lesion show gyriform hyperdensity on CT images with areas of calcification.(Figure 5 a, b). No perilesional oedema or mass effect on adjacent ventricle was seen.
Meningioangiomatosis is a rare, benign, hamartomatous lesion of the leptomeninges that was initially described by Worcester-Drought et al. in 1937 as an atypical, incomplete manifestation of neurofibromatosis type 2 . Since then, multiple cases have been reported in patients who did not have neurofibromatosis.
Headaches and seizures are the most common clinical presentation, with seizures being the predominant clinical problem in most patients [2,3,4]. The majority of those affected are children and young adults [2,3,4].
The lesions are typically cortical based, and the frontal and the temporal lobes are most commonly involved .
Meningioangiomatosis is a benign slow-growing tumour. Surgical resection is the typical treatment strategy[3,4]. Main goal of surgical resection is to achieve seizure control for the patient.
Take-Home Message / Teaching Points
There are variable imaging features of meningioangiomatosis and it is a difficult diagnosis on imaging. It should be one of the differentials in a young patient with seizures and headaches.
 Meningioangiomatosis: clinical, radiologic, and histopathologic correlation.
 R N Aizpuru, R M Quencer, M Norenberg, N Altman, and J Smirniotopoulos
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 Osama N. Kashlan, David V. LaBorde, LaKesha Davison, Amit M. Saindane, Daniel Brat, Patricia A. Hudgins, Robert E. Gross, "Meningioangiomatosis: A Case Report and Literature Review Emphasizing Diverse Appearance on Different Imaging Modalities",Case Reports in Neurological Medicine, vol. 2011, Article ID 361203, 8 pages, 2011.https://doi.org/10.1155/2011/361203
 Makary MS, Kobalka P, Giglio P, Slone HW. Meningioangiomatosis: Clinical, Imaging, and Histopathologic Characteristics. J Clin Imaging Sci. 2020 Jun 12;10:36. doi: 10.25259/JCIS_39_2020. PMID: 32637227; PMCID: PMC7332468.
 Sun, Z., Jin, F., Zhang, J. et al. Three cases of sporadic meningioangiomatosis with different imaging appearances: case report and review of the literature. World J Surg Onc13, 89 (2015). https://doi.org/10.1186/s12957-015-0477-x
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