OMIA:001470-9615 : Necrotising meningoencephalitis in Canis lupus familiaris (dog)

Categories: Nervous system phene

Mendelian trait/disorder: no

Mode of inheritance: Multifactorial

Considered a defect: yes

Key variant known: no

Cross-species summary: Necrotizing/necrotising meningoencephalitis is one of several non-infectious inflammatory disorders of the central nervous system (CNS) without a histopathological diagnosis (meningoencephalitis of unknown etiology (MUE)) 

Species-specific name: includes Pug dog myelopathy (PDM), Pug dog encephalitis (PDE)

Species-specific symbol: NME

Mapping: By conducting a GWAS on 28 affected and 66 control Pugs, each genotyped with the Illumina CanineHD Genotyping BeadChip (yielding 86,692 informative SNPs for analysis), Barber et al. (2011) highlighted two regions, one being the DLA class-II region of the MHC on chromosome CFA12, and the other being within the STYX gene on CFA8. Windsor et al. (2022) summarised findings from other studies: "Genetic risk for NME has been linked to chromosome 12 within the dog leukocyte antigen (DLA) major histocompatibility (MHC) II complex [Greer et al., 2009; Pedersen et al., 2022; Safra et al., 2011] with additional association to chromosome 15 in the pug and chromosome 4 in the maltese [Schrauwen et al., 2014].

Breeds: Brussels Griffon (Dog) (VBO_0200241), Chihuahua (Dog) (VBO_0200338), Coton de Tulear (Dog) (VBO_0200389), Maltese (Dog) (VBO_0200856), Papillon (Dog) (VBO_0200985), Pekingese (Dog) (VBO_0200994), Pug (Dog) (VBO_0201089), Shih Tzu (Dog) (VBO_0201223), Yorkshire Terrier (Dog) (VBO_0201448).
Breeds in which the phene has been documented. (If a likely causal variant has been documented for the phene, see the variant table breeds in which the variant has been reported).

Cite this entry

Nicholas, F. W., Tammen, I., & Sydney Informatics Hub. (2024). OMIA:001470-9615: Online Mendelian Inheritance in Animals (OMIA) [dataset].


Note: the references are listed in reverse chronological order (from the most recent year to the earliest year), and alphabetically by first author within a year.

2024 Rohdin, C., Ljungvall, I., Jäderlund, K.H., Svensson, A., Lindblad-Toh, K., Häggström, J. :
Assessment of glial fibrillary acidic protein and anti-glial fibrillary acidic protein autoantibody concentrations and necrotising meningoencephalitis risk genotype in dogs with pug dog myelopathy. Vet Rec :e3895, 2024. Pubmed reference: 38704817. DOI: 10.1002/vetr.3895.
2023 Son, M., Chae, Y., Kim, S., Yun, T., Koo, Y., Lee, D., Kim, H., Yang, M.P., Kang, B.T., Kim, S. :
Abnormal hyperphosphorylation of Tau in canine immune-mediated meningoencephalitis. In Vivo 37:1065-1076, 2023. Pubmed reference: 37103078. DOI: 10.21873/invivo.13181.
Windsor, R., Stewart, S., Huentelman, M., Keller, S., Khanna, C. :
An early clinical phenotype of necrotizing meningoencephalitis in the Pug reveals similarities to multiple sclerosis in humans. Am J Vet Res :1-9, 2023. Pubmed reference: 37913632. DOI: 10.2460/ajvr.23.07.0164.
2022 Windsor, R., Stewart, S., Schmidt, J., Mosqueda, M., Piras, I., Keller, S.M., Steinmetz, B., Borjesson, D.L., Huentelman, M., Khanna, C. :
A potential early clinical phenotype of necrotizing meningoencephalitis in genetically at-risk pug dogs. J Vet Intern Med 36:1382-1389, 2022. Pubmed reference: 35621070. DOI: 10.1111/jvim.16444.
2014 Cooper, J.J., Schatzberg, S.J., Vernau, K.M., Summers, B.A., Porter, B.F., Siso, S., Young, B.D., Levine, J.M. :
Necrotizing meningoencephalitis in atypical dog breeds: a case series and literature review. J Vet Intern Med 28:198-203, 2014. Pubmed reference: 24428322. DOI: 10.1111/jvim.12233.
Schrauwen, I., Barber, R.M., Schatzberg, S.J., Siniard, A.L., Corneveaux, J.J., Porter, B.F., Vernau, K.M., Keesler, R.I., Matiasek, K., Flegel, T., Miller, A.D., Southard, T., Mariani, C.L., Johnson, G.C., Huentelman, M.J. :
Identification of novel genetic risk loci in Maltese dogs with necrotizing meningoencephalitis and evidence of a shared genetic risk across toy dog breeds. PLoS One 9:e112755, 2014. Pubmed reference: 25393235. DOI: 10.1371/journal.pone.0112755.
2011 Barber, R.M., Schatzberg, S.J., Corneveaux, J.J., Allen, A.N., Porter, B.F., Pruzin, J.J., Platt, S.R., Kent, M., Huentelman, M.J. :
Identification of risk Loci for necrotizing meningoencephalitis in pug dogs. J Hered :S40-6, 2011. Pubmed reference: 21846746. DOI: 10.1093/jhered/esr048.
Pedersen, N., Liu, H., Millon, L., Greer, K. :
Dog leukocyte antigen class II-associated genetic risk testing for immune disorders of dogs: simplified approaches using Pug dog necrotizing meningoencephalitis as a model. J Vet Diagn Invest 23:68-76, 2011. Pubmed reference: 21217030.
Safra, N., Pedersen, N.C., Wolf, Z., Johnson, E.G., Liu, H.W., Hughes, A.M., Young, A., Bannasch, D.L. :
Expanded dog leukocyte antigen (DLA) single nucleotide polymorphism (SNP) genotyping reveals spurious class II associations. Vet J 189:220-6, 2011. Pubmed reference: 21741283. DOI: 10.1016/j.tvjl.2011.06.023.
2010 Greer, KA., Wong, AK., Liu, H., Famula, TR., Pedersen, NC., Ruhe, A., Wallace, M., Neff, MW. :
Necrotizing meningoencephalitis of Pug dogs associates with dog leukocyte antigen class II and resembles acute variant forms of multiple sclerosis. Tissue Antigens 76:110-8, 2010. Pubmed reference: 20403140. DOI: 10.1111/j.1399-0039.2010.01484.x.
2009 Greer, KA., Schatzberg, SJ., Porter, BF., Jones, KA., Famula, TR., Murphy, KE. :
Heritability and transmission analysis of necrotizing meningoencephalitis in the Pug. Res Vet Sci 86:438-42, 2009. Pubmed reference: 19014875. DOI: 10.1016/j.rvsc.2008.10.002.
Young, BD., Levine, JM., Fosgate, GT., de Lahunta, A., Flegel, T., Matiasek, K., Miller, A., Silver, G., Sharp, N., Greer, K., Schatzberg, SJ. :
Magnetic resonance imaging characteristics of necrotizing meningoencephalitis in Pug dogs. J Vet Intern Med 23:527-35, 2009. Pubmed reference: 19645838. DOI: 10.1111/j.1939-1676.2009.0306.x.
2008 Eom, KD., Lim, CY., Gu, SH., Kang, BT., Kim, YB., Jang, DP., Woo, EJ., Kim, D., Cho, ZH., Park, HM. :
Positron emission tomography features of canine necrotizing meningoencephalitis. Vet Radiol Ultrasound 49:595-9, 2008. Pubmed reference: 19051653.
Flegel, T., Henke, D., Boettcher, I.C., Aupperle, H., Oechtering, G., Matiasek, K. :
Magnetic resonance imaging findings in histologically confirmed Pug dog encephalitis. Vet Radiol Ultrasound 49:419-24, 2008. Pubmed reference: 18833947. DOI: 10.1111/j.1740-8261.2008.00400.x.
Higgins, RJ., Dickinson, PJ., Kube, SA., Moore, PF., Couto, SS., Vernau, KM., Sturges, BK., LeCouteur, RA. :
Necrotizing meningoencephalitis in five Chihuahua dogs. Vet Pathol 45:336-46, 2008. Pubmed reference: 18487490. DOI: 10.1354/vp.45-3-336.
Levine, JM., Fosgate, GT., Porter, B., Schatzberg, SJ., Greer, K. :
Epidemiology of necrotizing meningoencephalitis in Pug dogs. J Vet Intern Med 22:961-8, 2008. Pubmed reference: 18647157. DOI: 10.1111/j.1939-1676.2008.0137.x.
2004 Fearnside, SM., Kessell, AE., Powe, JR. :
Cervical hyperaesthesia in a Maltese Terrier with necrotising meningoencephalitis. Aust Vet J 82:550-2, 2004. Pubmed reference: 15478726.
2001 Cantile, C., Chianini, F., Arispici, M., Fatzer, R. :
Necrotizing meningoencephalitis associated with cortical hippocampal hamartia in a Pekingese dog. Vet Pathol 38:119-22, 2001. Pubmed reference: 11199160. DOI: 10.1354/vp.38-1-119.
1999 Ducoté, J.M., Johnson, K.E., Dewey, C.W., Walker, M.A., Coates, J.R., Berridge, B.R. :
Computed tomography of necrotizing meningoencephalitis in 3 Yorkshire Terriers. Vet Radiol Ultrasound 40:617-21, 1999. Pubmed reference: 10608689.
Uchida, K., Hasegawa, T., Ikeda, M., Yamaguchi, R., Tateyama, S. :
Detection of an autoantibody from Pug dogs with necrotizing encephalitis (Pug dog encephalitis). Vet Pathol 36:301-7, 1999. Pubmed reference: 10421096. DOI: 10.1354/vp.36-4-301.
1995 Stalis, IH., Chadwick, B., Dayrell-Hart, B., Summers, BA., Van Winkle, TJ. :
Necrotizing meningoencephalitis of Maltese dogs. Vet Pathol 32:230-5, 1995. Pubmed reference: 7604489.
1994 Kobayashi, Y., Ochiai, K., Umemura, T., Goto, N., Ishida, T., Itakura, C. :
Necrotizing meningoencephalitis in pug dogs in Japan. J Comp Pathol 110:129-36, 1994. Pubmed reference: 8040380.
1989 Cordy, DR., Holliday, TA. :
A necrotizing meningoencephalitis of pug dogs. Vet Pathol 26:191-4, 1989. Pubmed reference: 2763409.

Edit History

  • Created by Frank Nicholas on 03 Jul 2009
  • Changed by Frank Nicholas on 22 May 2013
  • Changed by Imke Tammen2 on 23 Oct 2022
  • Changed by Imke Tammen2 on 16 Nov 2022
  • Changed by Imke Tammen2 on 29 Apr 2023
  • Changed by Imke Tammen2 on 07 May 2024