Treatment of hemorrhagic acute disseminated encephalomyelitis with cyclophosphamide.

PURPOSE: Improvements in hemorrhagic and clinical symptoms in a patient with hemorrhagic acute disseminated encephalomyelitis (ADEM) treated with i.v. cyclophosphamide are reported.

SUMMARY: A 49-year-old woman was hospitalized with progressively worsening left-sided weakness, dysphagia, diplopia, and vertigo. Shortly before hospital admission, the patient had been treated at another facility with corticosteroids and plasma exchanges. Brain magnetic resonance imaging (MRI) studies conducted at the time of admission showed demyelinating lesions of the pons consistent with ADEM; rapid progression of the patient's symptoms also suggested an autoimmune, demyelinating process, and viral studies ruled out an infectious etiology. Despite initial treatment with i.v. immune globulin and additional corticosteroid courses, the patient's condition continued to deteriorate over the next few weeks, with development of respiratory distress requiring intubation. Repeat MRI revealed a new brain lesion in the splenial region of the corpus callosum, prompting the initiation of i.v. cyclophosphamide therapy (180 mg daily). Approximately 19 days after cyclophosphamide therapy was initiated, an MRI scan revealed substantial reduction of the pontine hemorrhage and a normal splenium appearance, with no new lesion development. The use of cyclophosphamide for hemorrhagic ADEM refractory to other treatments has been previously reported.

CONCLUSION

After approximately three weeks of daily i.v. cyclophosphamide therapy, a patient with hemorrhagic ADEM was noted to have stable to improved brain MRI findings along with limited improvement of mental status and movement symptoms.