Clinical Review & Education
Images in Neurology
Fulminant Acute Disseminated Encephalomyelitis Presenting in an Adult Catherine E. Otten, MD; Claire J. Creutzfeldt, MD
A 55-year-old man was slowly recovering from a flulike illness but stopped responding to his family’s telephone calls. Paramedics found him confused and naked in his home. On arrival at the local hospital, he was aphasic and noncooperative. He declined neurologically over the following week. He had 2 seizures, required intubation, and was transferred to our intensive care unit. His physical examination was notable for coma, extensor posturing, and diffuse hyperreflexia. Moderate pleocytosis was found in his cerebrospinal fluid with 50 white blood cells, all lymphocytes. Few oligoclonal bands were present. Test results for viral and bacterial infections, including human immunodeficiency virus, were negative. Brain magnetic resonance imaging demonstrated numerous T2 hyperintense and ringenhancing lesions without diffusion restriction (Figure). Lesions were extensive but predominantly restricted to the white matter with limited brainstem involvement. A brain biopsy demonstrated an inflammatory infiltrate with a perivenular pattern. Loss of myelin was evident but nerve axons were preserved. Overall, the patient’s history, imaging, and pathology results were consistent with acute disseminated encephalomyelitis (ADEM). He was treated with intravenous methylprednisolone followed by intravenous immunoglobulins. His neurologic status recovered gradually. Two months later, he was walking with assistance and joking with hospital staff. Within 5 months, he was performing daily tasks independently while living
at home with his family. Follow-up imaging revealed no further evidence of active demyelination.
Discussion Acute disseminated encephalomyelitis is typically a monophasic demyelinating disease that occurs in children following an infection or vaccination. Cerebrospinal fluid analysis may show an elevated protein level, lymphocytic pleocytosis, and transient oligoclonal bands.1 Symptoms are generally multifocal and include encephalopathy.2,3 Acute disseminated encephalomyelitis is rare in adults and the differential diagnosis includes malignancy, infection, and other demyelinating diseases.1,2 Prognosis for ADEM is favorable, although 20% of patients develop recurring demyelination and are eventually diagnosed with multiple sclerosis.2 Magnetic resonance imaging can help differentiate ADEM from multiple sclerosis but there can be overlap in features, as in our patient. In ADEM, lesions are typically extensive but poorly defined. They are mainly found in the white matter but can extend to the deep gray matter. Multiple sclerosis lesions are commonly found in the periventricular white matter and corpus callosum and are less likely to involve the gray matter.1-3 Patients with monophasic ADEM should not have new lesions on follow-up imaging, whereas MS may be a relapsing disease. Diagnosis of ADEM is best done by reviewing the clinical presentation in combination with laboratory and neuroimaging data.
Figure. Magnetic Resonance Imaging A
B
C
A, Fluid-attenuated inversion recovery images showed multiple confluent and globular areas of increased signal. B, Peripheral enhancement was seen in the
ARTICLE INFORMATION Author Affiliations: Department of Pediatric Neurology, University of Washington, Seattle 648
postcontrast images. C, Sagittal views suggested a perivascular distribution perpendicular to the ventricles.
(Otten); Department of Neurology, University of Washington, Seattle (Creutzfeldt).
Corresponding Author: Catherine E. Otten, MD, Department of Pediatric Neurology, University of
JAMA Neurology May 2014 Volume 71, Number 5
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archneur.jamanetwork.com/ by a Florida International University Medical Library User on 05/25/2015
jamaneurology.com
Images in Neurology Clinical Review & Education
Washington, 4800 Sand Point Way NE, Seattle, WA 98105 ([email protected]). Conflict of Interest Disclosures: None reported.
2. Tenembaum SN. Acute disseminated encephalomyelitis. Handb Clin Neurol. 2013;112:1253-1262.
REFERENCES
3. Young NP, Weinshenker BG, Lucchinetti CF. Acute disseminated encephalomyelitis: current
understanding and controversies. Semin Neurol. 2008;28(1):84-94.
1. Menge T, Hemmer B, Nessler S, et al. Acute disseminated encephalomyelitis: an update. Arch Neurol. 2005;62(11):1673-1680.
jamaneurology.com
JAMA Neurology May 2014 Volume 71, Number 5
Copyright 2014 American Medical Association. All rights reserved.
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649
Images in Neurology
Fulminant Acute Disseminated Encephalomyelitis Presenting in an Adult Catherine E. Otten, MD; Claire J. Creutzfeldt, MD
A 55-year-old man was slowly recovering from a flulike illness but stopped responding to his family’s telephone calls. Paramedics found him confused and naked in his home. On arrival at the local hospital, he was aphasic and noncooperative. He declined neurologically over the following week. He had 2 seizures, required intubation, and was transferred to our intensive care unit. His physical examination was notable for coma, extensor posturing, and diffuse hyperreflexia. Moderate pleocytosis was found in his cerebrospinal fluid with 50 white blood cells, all lymphocytes. Few oligoclonal bands were present. Test results for viral and bacterial infections, including human immunodeficiency virus, were negative. Brain magnetic resonance imaging demonstrated numerous T2 hyperintense and ringenhancing lesions without diffusion restriction (Figure). Lesions were extensive but predominantly restricted to the white matter with limited brainstem involvement. A brain biopsy demonstrated an inflammatory infiltrate with a perivenular pattern. Loss of myelin was evident but nerve axons were preserved. Overall, the patient’s history, imaging, and pathology results were consistent with acute disseminated encephalomyelitis (ADEM). He was treated with intravenous methylprednisolone followed by intravenous immunoglobulins. His neurologic status recovered gradually. Two months later, he was walking with assistance and joking with hospital staff. Within 5 months, he was performing daily tasks independently while living
at home with his family. Follow-up imaging revealed no further evidence of active demyelination.
Discussion Acute disseminated encephalomyelitis is typically a monophasic demyelinating disease that occurs in children following an infection or vaccination. Cerebrospinal fluid analysis may show an elevated protein level, lymphocytic pleocytosis, and transient oligoclonal bands.1 Symptoms are generally multifocal and include encephalopathy.2,3 Acute disseminated encephalomyelitis is rare in adults and the differential diagnosis includes malignancy, infection, and other demyelinating diseases.1,2 Prognosis for ADEM is favorable, although 20% of patients develop recurring demyelination and are eventually diagnosed with multiple sclerosis.2 Magnetic resonance imaging can help differentiate ADEM from multiple sclerosis but there can be overlap in features, as in our patient. In ADEM, lesions are typically extensive but poorly defined. They are mainly found in the white matter but can extend to the deep gray matter. Multiple sclerosis lesions are commonly found in the periventricular white matter and corpus callosum and are less likely to involve the gray matter.1-3 Patients with monophasic ADEM should not have new lesions on follow-up imaging, whereas MS may be a relapsing disease. Diagnosis of ADEM is best done by reviewing the clinical presentation in combination with laboratory and neuroimaging data.
Figure. Magnetic Resonance Imaging A
B
C
A, Fluid-attenuated inversion recovery images showed multiple confluent and globular areas of increased signal. B, Peripheral enhancement was seen in the
ARTICLE INFORMATION Author Affiliations: Department of Pediatric Neurology, University of Washington, Seattle 648
postcontrast images. C, Sagittal views suggested a perivascular distribution perpendicular to the ventricles.
(Otten); Department of Neurology, University of Washington, Seattle (Creutzfeldt).
Corresponding Author: Catherine E. Otten, MD, Department of Pediatric Neurology, University of
JAMA Neurology May 2014 Volume 71, Number 5
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archneur.jamanetwork.com/ by a Florida International University Medical Library User on 05/25/2015
jamaneurology.com
Images in Neurology Clinical Review & Education
Washington, 4800 Sand Point Way NE, Seattle, WA 98105 ([email protected]). Conflict of Interest Disclosures: None reported.
2. Tenembaum SN. Acute disseminated encephalomyelitis. Handb Clin Neurol. 2013;112:1253-1262.
REFERENCES
3. Young NP, Weinshenker BG, Lucchinetti CF. Acute disseminated encephalomyelitis: current
understanding and controversies. Semin Neurol. 2008;28(1):84-94.
1. Menge T, Hemmer B, Nessler S, et al. Acute disseminated encephalomyelitis: an update. Arch Neurol. 2005;62(11):1673-1680.
jamaneurology.com
JAMA Neurology May 2014 Volume 71, Number 5
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archneur.jamanetwork.com/ by a Florida International University Medical Library User on 05/25/2015
649
