A Case of Refractory Acute Disseminated Encephalomyelitis (ADEM) Presenting with Severe Gastrointestinal Hemorrhage

Thursday, May 25, 2017
B2 (New Orleans Convention Center)
Erica M Rivas, MD , Neurology, University of Southern California, south pasadena, CA
Erica M Rivas, MD , Neurology, University of Southern California, south pasadena, CA

Background:  ADEM is a rare demyelinating disorder. Influenza infection should be considered in the setting of ADEM and GI hemorrhage or hemorrhagic gastritis.

Objectives: To present an atypical presentation of Acute Disseminated Encephalomyelitis (ADEM)

Methods: Single case report and literature review.

Results:  A 30-year-old otherwise healthy Hispanic man was brought in by police after going missing. He was confused, disheveled and incontinent of stool and urine. Three days earlier he began acting oddly and was excessively sleepy. Two months prior he had a URI and malaise. On exam he was nonverbal and encephalopathic, with a left hemiparesis, brisk reflexes and sustained ankle clonus bilaterally. Brain MRI revealed multiple large T2 hyperintensities in the supratentorial white matter, bilateral caudate and right basal ganglia, with restricted diffusion and gadolinium (Gd) enhancement, consistent with inflammatory demyelination. Spinal cord MRIs were normal. CSF showed mild lymphocytic pleocytosis, normal protein, normal IgG index and negative OCB. Infectious CSF assays and serologic inflammatory workup were negative. He received 1g IV methylprednisolone followed by plasma exchange with persistent Gd enhancement. Despite GI prophylaxis he developed bleeding gastric ulcers requiring blood transfusions and interventions. Gd enhancement improved after cyclophosphamide induction. He was discharged to a rehabilitation facility two months after presentation. 

At 6 months follow up, the patient was alert, ambulatory but with decreased spontaneous speech, abulia, inattention, and poor insight into his deficits. His strength, sensation, and reflexes were normal. MRI showed no Gd enhancement or new lesions, and evolution of prior lesions.

Conclusions:  ADEM is a monophasic immune-mediated demyelinating disorder of the CNS commonly seen in the pediatric population. It usually follows a viral infection or vaccinations. ADEM may affect 1 in 125,000 to 250,000 individuals. Presentation in adults are less well characterized and difficult to diagnose which potentially carries a worse outcome. Treatment is with IV corticosteroids and, if refractory, plasma exchange, IVIG, and cyclophosphamide or rituximab can be used. The diagnosis fits best with ADEM given the history of a preceding viral illness, encephalopathy, lack of OCB, characteristic MRI lesions, nearly all lesions Gd enhancing, and no new lesion formation over time.  Our patient had a refractory phenotype requiring cyclophosphamide to achieve improvement. His hospitalization was complicated by bleeding gastric ulcers. Influenza virus infection, especially Influenza A N1H1, has been associated with post-infectious encephalopathy or ADEM, and several case reports of GI hemorrhage in the setting of influenza have been published. Although not tested in our patient, influenza should be considered in the setting of ADEM with concurrent GI hemorrhages.