High-grade Astrocytoma Presenting With Early-onset, Rapidly-progressive Dementia and Cerebral Hemiatrophy: A Diagnostic Chameleon
Julia Francesca Bonato Cavalcanti1, Ahmed Haydar1, Afonso Jaccoud1, Maria da Graca Morais Martin2, Yuri Reis Casal3, Adalberto Studart Neto1, Eduardo Mutarelli1, Luiz Castro1
1Department of Neurology, 2Department of Radiology, 3Department of Pathology, University of São Paulo
Objective:
Description of a hemispheric atrophy due to a high-grade astrocytoma. For years it mimicked a primary movement disorder before evolving into a rapidly progressive dementia. This case highlights the diagnostic challenges of atypical glioma presentations.
Background:
A 42-year-old woman presented with a four-year history of daily, stereotyped, large-amplitude craniofacial movements initially diagnosed as motor tics. Over time, she developed left hemiparesis, profound anosognosia, spatial disorientation, apraxias, hemispatial neglect, and marked behavioral changes consistent with frontal lobe dysfunction. The insidious, multifocal progression posed a significant diagnostic challenge, with early considerations including autoimmune, infectious, and psychiatric etiologies.
Design/Methods:
This report describes the patient's evolution through serial neurological and neuropsychological evaluations. Workup included multimodal neuroimaging (MRI, FDG-PET), continuous video-EEG, and serial CSF analyses. Definitive diagnosis was established by right insular brain biopsy with histopathological and immunohistochemical analysis.
Results:
Neuroimaging revealed marked right-hemispheric atrophy - fronto-parieto-insular, caudate head, hippocampus - with extensive T2/FLAIR hyperintensities with an area of high signal on DWI and contrast-enhancement in the posterior insula, extending to adjacent temporal and centrum semiovale regions. CT and MR angiography excluded vascular malformation or ischemic etiology for the cerebral hemiatrophy. Vessel-wall imaging showed no evidence of vasculitis. FDG-PET demonstrated profound right-hemispheric cortical hypometabolism with crossed cerebellar diaschisis. CSF revealed a mild pleocytosis and modest protein elevation without intrathecal IgG synthesis; metagenomic and autoimmune panels were negative. Right insular biopsy confirmed an IDH-mutant astrocytoma, WHO Grade 3, with high proliferative index (Ki-67 ≈ 20%). The postoperative course was complicated by focal status epilepticus.
Conclusions:
High-grade gliomas can be clinical chameleons. This case highlights that progressive, asymmetric neurological syndromes, even in the absence of an identifiable mass on initial imaging, should prompt consideration of an infiltrative neoplasm. Early advanced imaging and biopsy are critical in such atypical cases.
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