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October 2004: a 49-year-old man with progressive dementia

P Widdess-Walsh, A Nair, S M Staugaitis

    Brain Pathology (Zurich, Switzerland)
    |May 27, 2005
    PubMed

    Insights

    This case study highlights a rare instance of diffuse large B-cell lymphoma presenting as a rapidly progressive dementia. Early diagnosis through advanced imaging and biopsy is crucial for managing this aggressive brain lymphoma.

    Area of Science:

    • Neurology
    • Oncology
    • Neuroimaging

    Background:

    • A 49-year-old male presented with a 4-month history of progressive cognitive decline, including memory, calculation, and language impairments.
    • Associated symptoms included fatigue, weight loss, gait imbalance, and urinary incontinence, suggesting a diffuse neurological process.
    • The patient had a history of transfusion-associated Hepatitis C, a potential factor in immune-related conditions.

    Observation:

    • Neurological examination revealed dysarthria, dysnomia, left-sided neglect, bilateral Babinski signs, and a grasp reflex, indicating bilateral hemispheric dysfunction.
    • Electroencephalogram (EEG) showed diffuse slowing and right hemisphere background decrease, suggestive of encephalopathy and a structural lesion.
    • Magnetic Resonance Imaging (MRI) demonstrated multifocal FLAIR hyperintensities and patchy enhancement, while Fluorodeoxyglucose-Positron Emission Tomography (FDG-PET) revealed focal increased uptake correlating with MRI findings.

    Findings:

    • Cerebral angiography was unremarkable, ruling out vascular abnormalities as the primary cause.
    • Brain biopsy confirmed diffuse infiltrates of malignant cells immunoreactive for CD20, establishing a diagnosis of diffuse large B-cell lymphoma (DLBCL).
    • The combination of clinical presentation and neuroimaging findings supported bilateral hemispheric involvement, with DLBCL being the final diagnosis.

    Implications:

    • This case underscores the importance of considering infiltrating neoplasms, particularly lymphoma, in the differential diagnosis of rapidly progressive dementia.
    • Advanced neuroimaging techniques (MRI, FDG-PET) play a critical role in identifying structural and metabolic abnormalities suggestive of CNS lymphoma.
    • Morphological and immunohistochemical analysis of brain biopsy remains the gold standard for definitive diagnosis of primary CNS lymphoma, guiding appropriate oncological treatment.

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