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Abstract Details

Atypical Non-Enhancing Primary Central Nervous System Lymphoma
Neuro-oncology
P3 - Poster Session 3 (5:30 PM-6:30 PM)
7-003

To report two cases of primary CNS lymphoma (PCNSL) with pure motor deficits mimicking demyelinating or neuromuscular disease

PCNSL in immunocompetent patients typically present with CNS mass-like symptoms. MRI brain often shows homogenous enhancing solitary or multifocal masses with diffusion restriction. However, presentation of PCNSL may mimic any type of CNS disease including motor neuron disease, demyelinating disease, stroke and brain MRIs can be non-enhancing in rare cases. Here we discuss two patients who presented with primarily upper motor neuron (UMN) findings which led to the diagnosis of primary central nervous system lymphoma.

Patient 1 was a 54 year old male who developed gradual onset of right leg weakness, dysarthria, dysphagia and blurred vision. MRI showed T2/FLAIR hyperintensities with scattered enhancement along bilateral corticospinal tracts radiating from the centrum semiovale into bilateral thalami. CSF was unremarkable. His symptoms were attributed to an autoimmune etiology and initially improved with plasmapharesis and corticosteroids however symptoms worsened. Biopsy was performed confirming diffuse large B-cell lymphoma. He received high-dose methotrexate with complete resolution of the corticospinal enhancement.

 

Patient 2 is a 46 year old male who presented with episodic alternating left and right sided weakness with headache and slurred speech. MRI showed mild left white matter T2/FLAIR hyperintensities. CSF showed monocytic pleocytosis with oligoclonal bands concerning for multiple sclerosis. He initially improved with steroids but declined after steroids were tapered. ESR and CRP were elevated suggesting vasculitis. Cerebral conventional angiography was unremarkable. Biopsy was then performed off steroids which confirmed diagnosis of CNS lymphoma. He improved after treatment with whole brain radiation and rituximab.

N/A
CNS lymphoma can present with pure motor deficits and atypical MRI findings. It can mimic vasculitis, neuromuscular or autoimmune disease due to responsiveness to steroids. Clinical suspicion and early biopsy is vital for definitive diagnosis and proper treatment.
Authors/Disclosures
Erik Valenti, MD (Saint Alphonsus Medical Group Neurology)
PRESENTER
Dr. Valenti has received personal compensation in the range of $0-$499 for serving as a Consultant for LVIS.
Xiao-Tang Kong, MD, PhD (UC Irvine) Dr. Kong has nothing to disclose.