Japanese macaque encephalomyelitis: A spontaneous multiple sclerosis–like disease in a nonhuman primate
Article first published online: 14 JUN 2011
Copyright © 2011 American Neurological Association
Annals of Neurology
Volume 70, Issue 3, pages 362–373, September 2011
How to Cite
Axthelm, M. K., Bourdette, D. N., Marracci, G. H., Su, W., Mullaney, E. T., Manoharan, M., Kohama, S. G., Pollaro, J., Witkowski, E., Wang, P., Rooney, W. D., Sherman, L. S. and Wong, S. W. (2011), Japanese macaque encephalomyelitis: A spontaneous multiple sclerosis–like disease in a nonhuman primate. Ann Neurol., 70: 362–373. doi: 10.1002/ana.22449
- Issue published online: 8 SEP 2011
- Article first published online: 14 JUN 2011
- Accepted manuscript online: 7 APR 2011 02:15PM EST
- Manuscript Accepted: 1 APR 2011
- Manuscript Revised: 23 MAR 2011
- Manuscript Received: 15 DEC 2010
To describe Japanese macaque encephalomyelitis (JME), a spontaneous inflammatory demyelinating disease occurring in the Oregon National Primate Research Center's (ONPRC) colony of Japanese macaques (JMs, Macaca fuscata).
JMs with neurologic impairment were removed from the colony, evaluated, and treated with supportive care. Animals were humanely euthanized and their central nervous systems (CNSs) were examined.
ONPRC's JM colony was established in 1965 and no cases of JME occurred until 1986. Since 1986, 57 JMs spontaneously developed a disease characterized clinically by paresis of 1 or more limbs, ataxia, or ocular motor paresis. Most animals were humanely euthanized during their initial episode. Three recovered, later relapsed, and were then euthanized. There was no gender predilection and the median age for disease was 4 years. Magnetic resonance imaging of 8 cases of JME revealed multiple gadolinium-enhancing T1-weighted hyperintensities in the white matter of the cerebral hemispheres, brainstem, cerebellum, and cervical spinal cord. The CNS of monkeys with JME contained multifocal plaque-like demyelinated lesions of varying ages, including acute and chronic, active demyelinating lesions with macrophages and lymphocytic periventricular infiltrates, and chronic, inactive demyelinated lesions. A previously undescribed gamma-herpesvirus was cultured from acute JME white matter lesions. Cases of JME continue to affect 1% to 3% of the ONPRC colony per year.
JME is a unique spontaneous disease in a nonhuman primate that has similarities with multiple sclerosis (MS) and is associated with a novel simian herpesvirus. Elucidating the pathogenesis of JME may shed new light on MS and other human demyelinating diseases. ANN NEUROL 2011;