Pseudotumoral demyelinating lesion in a teenager, case report

Abstract

Introduction: Pseudotumor lesions are an atypical presentation of demyelinating diseases, commonly associated with Multiple Sclerosis (MS) of different varieties (Balo, Schilder and Marburg) and occasionally linked to other demyelinating processes, such as MOGAD, ADEM and NMOSD. Estimated prevalence of three cases per million per inhabitant and occurrence of 1.4–8.2% in patients with MS. Objectives: To report a case of Pseudotumoral Demyelinating Lesion (PDL) in a teenager, attended at the General Hospital of Goiânia. Methods: Information obtained through clinical follow-up in a Neurology ward. Results: Male, 14 years old, onset of seizures 40 days ago. Ten days ago, he presented left crural monoparesis. On examination, hemiparesis of left crural predominance, left hyperreflexia, patellar and plantar clonus, left hemihypoesthesia. Normal cerebrospinal fluid (CSF), negative oligoclonal bands and oncotic cytology. Brain magnetic resonance imaging (MRI) showed an expansive cortical and subcortical lesion in the superior frontal gyrus and paracentral lobe on the right, cingulate gyrus and trunk of the corpus callosum on the right, with hypersignal on T2/FLAIR (T2-weighted-Fluid-Attenuated Inversion Recovery) and focal areas of gadolinium enhancement, predominantly subcortical. Spectroscopy increased Choline and reduced peaks of N-Acetyl Aspartate. The perfusion study showed cold perfusion. Proposed therapy with high doses of intravenous corticosteroids for five days, with improvement of the deficit. A control MRI showing partial regression of the lesion after therapy, therefore the first hypothesis is a PDL. Conclusion: PDL is a diagnostic challenge, as they mimic tumors and abscesses. Diagnosis based on the combination of clinical evolution, neuroimaging, CSF and brain biopsy, when appropriate. Initial treatment with high doses of intravenous corticosteroids, and most patients has good response. This case highlights pseudotumoral demyelinating diseases in teenage.