Abstract Details

Title Rapidly Progressive Dementia Prompting Histopathological Confirmation: An Unusual Case of CNS Intravascular Lymphoma

Topic Neuro-oncology

Presentation(s) P3 - Poster Session 3 (12:00 PM-1:00 PM)

Poster/Presentation
Number 13-012

Objective NA

Background Intravascular lymphoma (IVL) remains a diagnostic challenge as its clinical presentation is usually nonspecific. It is a rare form of extra nodal diffuse large B cell lymphoma in which tumor cells proliferate within blood vessel lumens. Diagnosis confirmation usually requires tissue biopsy. Classically it involves the skin, however, when CNS involvement is present, it can mimic various neurological syndromes.

Design/Methods A 64-year-old male presenting with 3 weeks of progressive fatigue, physical deconditioning, decreased appetite, confusion and aphasia. Neuro exam was remarkable for akinetic mutism features without eye tracking movements, verbal output, and following commands. Patient had purposeful movements, symmetrical withdrawal to noxious stimuli in all 4 extremities, bilateral lower extremity hyperreflexia (3+), and right lower extremity clonus. Patient progressed to an obtunded state over a few days and was intubated due to hypoxemia.

Results

Patient had an MRI compatible pacemaker, but facility protocol does not allow ICD patients who are unable to verbalize needs/pain during the imaging process. EEG showed diffuse severe cerebral dysfunction with no epileptiform discharges. CTA head followed by non-contrast CT Head demonstrated delineated and circular bilateral cortical hypodensities without enhancement. CSF only revealed elevated protein. Multiple serological studies were only positive for elevated beta-2 microglobulin and kappa/lambda chains. Bone marrow biopsy demonstrated normal cellular marrow and left-shifted myeloid maturation. Brain biopsy revealed intravascular large B-cell lymphoma infiltrating the parenchyma and dura. Staging workup was not suggestive of metastasis. The patient responded with gradual improvement in neurological status with IV steroids initiation.

Conclusions IVL with CNS involvement needs to be considered in the differential diagnosis of elderly patients with rapidly progressive cognitive decline and cortical or subcortical hypodensities in the head CT imaging of unclear etiology. Histopathological confirmation should be prompted in patients with the appropriate clinical picture and progressive neurological decline who are unable to complete brain MRI.

Authors/Disclosures
Belen Lawless, MD PRESENTER	No disclosure on file
Gregory M. Blume, MD (University of Illinois College of Medicine)	No disclosure on file
Diana P. Londono, MD (Fullerton Neurology and Headache Center)	Dr. Londono has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech.
Luis G. Manrique, MD (MedStar Georgetown University Hospital)	Dr. Manrique has nothing to disclose.
	No disclosure on file

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