Disclosures: Daniel T. Probst, MD: Nothing to disclose

Case Description: A 23-year-old female presented with the acute onset of right-sided hemiplegia, global aphasia, incontinence of urine and stool, and fevers. Head CT and brain MRI were concerning for left frontal lobe mass or infection with surrounding vasogenic edema. CT of the patient’s chest, abdomen, and pelvis revealed multiple liver lesions.

Setting: Academic medical center.

Patient: Young female with fever, hemiplegia, global aphasia, and bowel/bladder incontinence.

Assessment/Results: Due to mental status decline and concern for herniation, the patient underwent a decompressive hemicraniectomy and brain biopsy. Left frontal lobe pathology was consistent with acute disseminated encephalomyelitis (ADEM) without evidence of infection or neoplasm. CSF cytology and flow cytometry were notable for marked acute inflammation without evidence of malignancy. Oligoclonal bands were absent. An autoimmune encephalopathy panel and paraneoplastic panel were negative. Outside hospital records revealed that 10 months prior, the patient was admitted for emesis and diarrhea with an abdominal CT demonstrating multiple hepatic lesions. A liver biopsy demonstrated epitheliod hemangioendothelioma (EHE), a rare malignant vascular neoplasm. She was lost to follow up until her current presentation. She was admitted to acute inpatient rehabilitation and experienced a remarkable recovery of her aphasia and hemiplegia, functionally improving from requiring moderate assistance to complete independence.

Discussion: Despite the negative paraneoplastic panel, it was felt that the patient’s ADEM represented paraneoplastic syndrome secondary to EHE. The paraneoplastic panel was negative as no specific antibody associated with paraneoplastic ADEM has been isolated. Thus, a negative paraneoplastic panel would not rule out paraneoplastic syndrome, but rather fail to identify paraneoplastic syndrome associated with the tested antibodies. ADEM presenting as a paraneoplastic syndrome has been reported in the literature, but never in association with EHE.

Conclusion: ADEM presented as a paraneoplastic syndrome secondary to EHE. Despite the patient’s marked aphasia and hemiplegia on admission to inpatient rehabilitation, she made a remarkable recovery.

Level of Evidence: Level V

« Back to AAPM&R Annual Assembly 2019

PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/acute-disseminated-encephalomyelitis-as-a-result-of-a-paraneoplastic-process-from-epithelioid-hemangioendothelioma-a-case-report/