Froin’s Syndrome and Bilateral Choroid Plexitis Preceding the Diagnosis of Diffuse Large B-cell Lymphoma
Melanie Li1, Michael McAree1, Jennifer Frontera1, Ting Zhou1
1Department of Neurology, NYU Grossman School of Medicine
Background:
Diffuse large B-cell lymphoma (DLBCL) is an aggressive Non-Hodgkin lymphoma. Though symptoms typically involve a rapidly growing mass and accompanying B-symptoms, up to 40% of DBLCL develop extranodally and can have myriad presentations.
Results:
An 81-year-old woman sought ambulatory care for a month-long history of abdominal bloating and early satiety. Workup revealed an incidental right adrenal mass and hyponatremia (126 mmol/L) suspected to be secondary to poor oral intake. Due to progressive lethargy, she presented to the emergency department where she was noted to have a fever of 39.0°C and left periorbital erythematous swelling. MRI of the brain and sinuses revealed left maxillary sinus inflammation with concern for osteomyelitis, and she was discharged with oral antibiotics. Given lack of improvement, she returned to the emergency department and was admitted for intravenous antibiotics after CT of the chest showed possible lobar pneumonia. As she remained persistently encephalopathic, MRI of the brain and sinuses was repeated, which demonstrated new thalamic, hippocampal, ventricular signal abnormalities concerning for meningoencephalitis. A lumbar puncture showed viscous, xanthochromic cerebrospinal fluid (CSF), lymphocytic (83%) pleocytosis, markedly elevated protein (1,898 mg/dL) and hypoglycorrhachia (3 mg/dL). She was empirically treated for bacterial, viral, mycobacterial, and fungal meningoencephalitis. She developed respiratory distress and continued to deteriorate. A third MRI of the brain and spine demonstrated avid enhancement of bilateral choroid plexuses, bilateral Meckel’s caves, and cauda equina nerve roots. Lumbar puncture was repeated two more times with persistence of highly proteinaceous CSF. Extensive infectious, inflammatory and paraneoplastic panels sent from her CSF and serum remained negative. Flow cytometry and cytology from three independent CSF samples did not reveal evidence for malignancy. The decision was made to biopsy the adrenal mass and pathology was consistent with DLBCL.
Conclusions:
This case highlights the protean clinical presentations of DLBCL and its myriad extranodal involvements.
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