Maria Garcia-Dominguez1, Margaret Owegi2, Wissam Deeb3, David Cachia2, Carolina Ionete3
1UMass Memorial Medical Center, 2University of Massachusetts Medical Center, 3UMass Memorial
Objective:
To present challenges in recognizing central nervous system (CNS) lymphoma involvement vs reactivation of Epstein Barr virus (EBV) infection in immunocompromised patients.
Background:
Chronic lymphocytic lymphoma (CLL) rarely presents with CNS involvement. EBV encephalitis is a difficult diagnosis based on diverse clinical manifestations most commonly presenting with altered mental status, and parkinsonism. Radiographic findings can include basal ganglia (BG) lesions.
Design/Methods:
A case report is presented on a patient who presented to a tertiary care center in 2022.
Results:
A 77-year-old male presented with cognitive decline and unsteady gait over 5 weeks. Medical history was significant for CLL genetic mutation variant (treatment naive) and essential tremor. Neurological examination was significant for severe cognitive decline with a Montreal Cognitive Assessment (MoCA ©) score of 13/30 and parkinsonism features. Brain MRI with and without gadolinium (Gd) contrast revealed no acute intracranial abnormality and routine electroencephalogram (EEG) with no electrographic seizures. Cerebrospinal fluid (CSF) analysis revealed pleocytosis WBC 30 with protein 107. Flow cytometry in CSF revealed monoclonal CD5+, CD23-, EBV+ B-cells consistent with a lymphoproliferative disorder (i.e. lymphoma, leukemia). Management included high-dose intravenous methylprednisolone (IVMP) for 5 days with an improved MoCA©score of 23/30 and resolution of cog-wheel rigidity.
Conclusions:
EBV encephalitis can involve the BG and present with reversible parkinsonism, commonly presenting as rhombencephalitis Discrepancies in CSF with monocytic predominance compared to monoclonal B cells and rapid response to steroids may be observed. This scenario represents challenges in the treatment approach for clinicians.