Transtentorial herniation from tumefactive multiple sclerosis mimicking primary brain tumor

Abstract

Background: Multiple sclerosis (MS) is a chronic central nervous system inflammatory demyelinating disease characterized by multiple lesions disseminated in time and space. The lesions often have characteristic imaging findings on magnetic resonance (MR) imaging and cerebrospinal fluid findings that lead to their diagnosis. At times, these lesions may resemble tumors due to their large size (>2 cm), significant vasogenic edema, and ring-enhancing MR imaging findings. Such lesions are described as tumefactive demyelinating lesions or tumefactive MS, and they are generally seen in aggressive forms of MS associated with rapid progression.

Case description: We report an uncommon but clinically significant case of transtentorial brain herniation secondary to malignant cerebral edema from tumefactive MS in a 50-year-old woman. After the initial diagnosis of MS, the patient continued to have progression of her white matter lesions suggesting evolution of her MS despite treatment with intravenous (IV) steroids, IV immunoglobulin, and plasmapheresis. She was admitted to the hospital with a new, large, ring-enhancing lesion that displayed significant mass effect from vasogenic edema and progressed, necessitating a decompressive hemicraniectomy.

Conclusion: Tumefactive MS presents a unique pathology that can often mimic primary brain tumors. Although these lesions affect white matter and infrequently cause a significant amount of mass effect, they can act like a tumor, causing edema that generates sufficient intracranial pressure to cause transtentorial herniation.

Keywords: Decompressive hemicraniectomy; transtentorial herniation; tumefactive demyelinating lesion; tumefactive multiple sclerosis.

Figure 1

Axial T1-weighted contrast-enhanced magnetic resonance image demonstrating a right temporoparietal, partial ring-enhancing lesion in the white matter

Figure 2

Axial computed tomographic image obtained at the time of admission, before high-dose steroid therapy was started, showing subfalcine herniation

Figure 3

Axial computed tomographic image showing aggressive development of edema causing transtentorial herniation