Variant of multiple sclerosis with dementia and tumefactive demyelinating brain lesions

We describe an unusual clinical and diagnostic featureof a patient with multiple sclerosis(MS). A 25-yearold woman was admitted to the Neurology department(December 2009) with one month history of rapid cognitive deterioration. She had poor cognition, dysphasia, reduction in visual acuity and temporal pallor of the optic discs. She had prolonged latencies of P100 component of visual evoked potentials(VEPs). Magnetic resonance imaging(MRI)-brain showed multifocal large(≥ 3 cm) white-matter hypointense lesions in T1 W and hyperintense in T2 W and fluid-attenuated inversion recovery images and patchy enhancement. A diagnosis of tumefactive MS was given. She received two consecutive 5-d courses of 1 g daily intravenous methylprednisolone for 2 mo and oral prednisolone in dose of 80 mg twice/daily in between. At the 3rd month, Mini Mental State Examination and VEPs returned to normal but not the MRI. Patient continued oral steroids after hospital discharge(March 2010) for 9 mo with significant MRI improvement after which tapering of steroids started for a year. The patient refused immunomodulation therapy due to her low socioeconomic status. Neither clinical relapse nor new MRI lesions were observed throughout the next 4 years. In spite of the aggressive course of tumefactive MS variant, good prognosis may be seen in some patients.

Magnetic Resonance Imaging Characteristics of Tumefactive Demyelinating Lesions

Tumefactive demyelinating lesions (TDLs) are often misdiagnosed as brain tumors. To identify imaging characteristics of TDLs for their proper diagnosis, their differences from malignant gliomas, etc., were investigated based on 3-Tesla magnetic resonance imaging (MRI) and MR spectroscopy data that was obtained from 5 patients diagnosed with TDLs in our hospital. MfRI displayed a paraventricular lesion with a maximum diameter of ≥2 cm. Diffusion-weighted imaging (DWI) displayed a region of bright signal intensity at the margin of the lesion, and apparent diffusion coefficient (ADC) imaging displayed high signal intensity at the core of the lesion. The mass effect was mild relative to the size of the lesion, and the edema around the lesion was mild to moderate. Gadolinium-enhanced MRI displayed a characteristic open-ring sign, in which the ring of enhancement was incomplete (open towards the cortex), with the medullary vein running through the lesion, resulting in the enhancement of the surrounding area. MR spectroscopy displayed an increase in the choline peak, a decrease in the N-acetyl aspartate (NAA) peak, the presence of lactate/lipid peaks, and the presence of β, γ-glutamate-glutamine (GLX) peaks that are slightly shifted to a higher resonance frequency (2.1 - 2.4 ppm) from the NAA peak. Taken together, the following main characteristics were identified as imaging features of TDLs: bright rim on DWI, a high signal core on ADC imaging, an open-ring sign, and β, γ-GLX peaks on proton MR spectroscopy. We concluded that the key to the improvement of neurological symptoms of patients is the prompt initiation of steroid treatment following the accurate diagnosis of TDLs.

Chinese Guidelines for the Diagnosis and Management of Tumefactive Demyelinating Lesions of Central Nervous System

Tumefactive demyelinating lesions （TDLs）,[1-3] previously named as tumor-like inflammatory demyelinating disease[4,5] or demyelinating pseudotumor,[6] are relatively special type of immune-mediated inflammatory demyelinating lesions in the central nervous system （CNS）,[7] which mainly occur within cerebrum,but rarely in spinal cord.TDLs are so named because it mimics brain tumors with such characteristics as less severe symptoms,large lesions with perilesional edema,mass effect and/or enhancement on neuroimaging,and easily misdiagnosed as brain tumors.[8,9]

A clinically isolated syndrome:butterfly glioma mimic

The report explores a unique and treatable“butterfly”-glioma mimic and the neuroimaging characteristics that help to diagnose this entity.A 35-year-old patient presented with subacute-onset,progressive frontal lobe dysfunction followed by features of raised intracranial pressure.Neuroimaging features were consistent with a“butterfly”lesion that favored the possibility of a gliomatosis cerebri with significant edema and marked corpus callosum and fornix thickening.Contrast-enhanced and perfusion images revealed a confluent tumefactive lesion with a characteristic“broken-ring”pattern of enhancement,mass-effect and low perfusion;features favoring an alternative inflammatory pathology.This was peculiar as calloso-forniceal involvement of this nature has not been previously reported in inflammatory demyelinating mass lesions.This was confirmed as a tumefactive demyelination on histopathology.Following treatment,on clinical and imaging follow-up,significant resolution was evident suggesting a monophasic illness.This case highlights the stringent clinico-radiological-pathological approach required in the evaluation and management of butterfly lesions despite the striking imaging appearances.Tumefactive demyelination in this patient represents a clinically isolated syndromic presentation of an inflammatory pathology that can resemble gliomatosis cerebri.These“butterfly”-glioma mimics are scarcely reported in the literature,are eminently treatable with variable prognosis and prone for relapse.