Journal of Clinical Apheresis 29:339–340 (2014)

Letter to the Editor Treatment of Acute Disseminated Encephalomyelitis with Plasmapheresis in a 16-Year-Old Female, A Case Report and Literature Review Chun-Hui Yi,1,2 Maite La Vega-Talbott,1,3 and Mark T. Friedman1,4* 1

Blood Bank and Transfusion Services, Mount Sinai Health System, St. Luke’s-Roosevelt Hospital and Beth Israel Medical Centers, New York, New York 2 Department of Pathology, Mount Sinai Health System, St. Luke’s-Roosevelt Hospital and Beth Israel Medical Center, New York, New York 3 Pediatric Neurology, Comprehensive Pediatric Epilepsy Center, Mount Sinai Health System, St. Luke’s-Roosevelt Hospital and Beth Israel Medical Centers, New York, New York 4 Pathology Residency Program, Mount Sinai Health System, St. Luke’s-Roosevelt Hospital and Beth Israel Medical Centers, New York, New York

TO THE EDITOR: Acute disseminated encephalomyelitis (ADEM) is a rare acute central nervous system (CNS) disorder usually seen in young children. According to the current guideline of American Society for Apheresis, it belongs to the category II of indications for therapeutic apheresis: “Disorders for which apheresis is accepted as second-line therapy, either as a standalone treatment or in conjunction with other modes of treatment” [1]. We share our experience of one patient who had a rapid favorable response to plasmapheresis. Our patient was a 16-year-old girl who presented to the emergency department with bilateral progressive worsening of vision, lower left leg weakness, and gait disturbance for 3 days. One week prior to admission, she had severe and constant frontal and occipital headache with nausea. Head computed tomography showed regions of abnormal vasogenic edema within the right frontal lobe and right temporal white matter. magnetic resonance imaging (MRI) of the brain and spine also showed edema of the right parietal lobe, frontal lobe, temporal lobe, and optic chiasm; edema around the thoracic spinal cord and the periventricular area. Cerebrospinal fluid and blood serological testing and culture ruled out an infectious disease etiology. She was initially treated with high-dose IV methylprednisolone 30 mg kg21/day for 5 days followed by taper. Starting on the second day of admission, she was also given IVIG (1 g kg21/day) for 2 days. From Day 5 to Day 10, the patient began to slowly improve. However, her symptoms waxed and waned. From Day 16, six treatments of plasmapheresis (2.5 L 5% albumin replacement, C 2014 Wiley Periodicals, Inc. V

plasma volume 1.1) every other day was started. On Day 17, the patient reported improvement of visual and motor function. She could see shapes and certain colors. She continued to improve steadily. After the sixth treatment, she could see big letters, color within the lines, and eat and use the bathroom without assistance. The patient was discharged to rehabilitation on Day 29. She was doing well until 5 months, when she had a relapse with blurry vision, right side numbness, and new demyelinating lesions on MRI. This time, the symptoms responded well to corticosteroids. She was discharged home in 5 days. ADEM is a multifocal inflammatory demyelinating disorder usually affecting children less than 10 years old [2]. It has an annual incidence of 0.2–0.8 per 100,000 persons, and 70–77% of patients are reported to have viral infection or vaccination 2–4 weeks before onset [2]. The diagnosis of ADEM is based on clinical manifestations and radiology findings [3,4]. MRI is the most important method to diagnose and follow-up ADEM, typically demonstrating widespread bilateral patchy subcortical white matter involvement and complete resolution afterward [3,5]. ADEM has an *Correspondence to: Mark T. Friedman, Department of Pathology, Mount Sinai Health System, St. Luke’s-Roosevelt Hospital and Beth Israel Medical Centers, 1000 Tenth Avenue, New York, NY 10019. E-mail: [email protected]. Received 23 March 2014; Accepted 14 April 2014 Published online 13 May 2014 in Wiley Online Library (wileyonlinelibrary.com). DOI: 10.1002/jca.21327

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immunologic etiology; the structural similarity between the pathogen and host molecules induces T-cell and humoral responses targeting CNS myelin glycoproteins [6]. The first-line treatment of choice for ADEM is pulse high-dose corticosteroids intravenously followed by oral taper to reduce CNS inflammation [7]. Intravenous immunoglobulin (IVIG) therapy has been used in severe steroid-resistant cases or in cases where steroids are contraindicated [8]. The effectiveness of plasmapheresis in ADEM has not been well established. It has been reserved for the most treatment-resistant cases [9]. Plasmapheresis has been considered as an effective treatment for immune-modulated neurologic diseases by rapidly removing the offending autoimmune antibodies. This patient did not respond well to both methylprednisolone and IVIG, but rapidly responded to plasmapheresis. An early start to plasmapheresis could be considered that may rapidly restore function, reduce the hospital stay, and have a more favorable prognosis. ADEM is a rare disease; the role of plasmapheresis in the treatment of ADEM has not been well established. More studies should be done to help clinicians in making their treatment decisions.

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Journal of Clinical Apheresis DOI 10.1002/jca

Y, Shaz BH. Guidelines on the use of therapeutic apheresis in clinical practice-evidence-based approach from the Writing Committee of the American Society for Apheresis: the sixth special issue. J Clin Apher 2013;28:145–284. Tenembaum S, Chitnis T, Ness J, Hahn JS. Acute disseminated encephalomyelitis. Neurology 2007;68(16 Suppl 2):S23–S36. Alper G. Acute disseminated encephalomyelitis. J Child Neurol 2012;27:1408–1425. E. Fridinger S, Alper G. Defining encephalopathy in acute disseminated encephalomyelitis. J Child Neurol 2013 Jun 12. [Epub ahead of print] Parrish JB, Weinstock-Guttman B, Yeh EA. Cerebellar mutism in pediatric acute disseminated encephalomyelitis. Pediatr Neurol 2010;42:259–266. Pohl D. Epidemiology, immunopathogenesis and management of pediatric central nervous system inflammatory demyelinating conditions. Curr Opin Neurol 2008;21:366–372. Waldman AT, Tardieu M. Treatment and prognosis of acute disseminated encephalomyelitis. In: Dorothee Chabas ELW, editor. Demyelinating Disorders of the Central Nervous System in Childhood. New York: Cambridge University Press. 2011. pp. 212–222. Ravaglia S, Piccolo G, Ceroni M, Franciotta D, Pichiecchio A, Bastianello S, Tavazzi E, Minoli L, Marchioni E. Severe steroid-resistant post-infectious encephalomyelitis: general features and effects of IVIg. J Neurol 2007;254:1518–1523. Khurana DS, Melvin JJ, Kothare SV, Valencia I, Hardison HH, Yum S, Faerber EN, Legido A. Acute disseminated encephalomyelitis in children: discordant neurologic and neuroimaging abnormalities and response to plasmapheresis. Pediatrics 2005; 116:431–436.

Treatment of acute disseminated encephalomyelitis with plasmapheresis in a 16-year-old female, a case report and literature review.

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