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The Cornell veterinarian1990; 80(4); 357-379;

Equine motor neuron disease; a preliminary report.

Abstract: A spontaneous motor neuron disease or neuronopathy was identified in 10 horses from the northeastern United States. Signs of generalized weakness, muscle fasciculations, muscle atrophy and weight loss progressed over 1 to several months in young and old horses of various breeds. Pathologic studies revealed that degeneration and loss of motor neurons in the spinal cord and brain stem resulted in axonal degeneration in the ventral roots and peripheral and cranial nerves and denervation atrophy of skeletal muscle. Many spinal neurons were swollen, chromatolytic and contained neurofilamentous accumulations. Other cell bodies were shrunken and undergoing neuronophagia and some were lost and replaced by glia. This fatal equine motor neuron disease has not been reported previously and its cause has not been determined. The progressive weakness and wasting and the neuronal degenerative changes in these horses were similar to those described in people with sporadic amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease.
Publication Date: 1990-10-01 PubMed ID: 2209016
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  • Journal Article
  • Research Support
  • Non-U.S. Gov't

Summary

This research summary has been generated with artificial intelligence and may contain errors and omissions. Refer to the original study to confirm details provided. Submit correction.

This research paper discusses the identification of a new neurodegenerative disease affecting horses in the northeastern United States, named Equine Motor Neuron Disease (EMND), which shows symptoms and progresses similar to human Amyotrophic Lateral Sclerosis (ALS).

Identification and Symptoms of EMND in Horses

  • The research identified Equine Motor Neuron Disease (EMND) over a period of several months in 10 horses of different ages and breeds, all located in the northeastern United States.
  • An array of symptoms were observed, starting from generalized weakness, muscle twitching (fasciculations), muscle atrophy (wasting), to noticeable weight loss. These signs were progressive, worsening over time.

Pathological Studies and Findings

  • In the horses affected with EMND, the pathological studies revealed that the disease caused degeneration and eventual loss of motor neurons, which are essential components of the nervous system controlling muscle movement, in the spinal cord and brain stem.
  • This loss of motor neurons resulted in axonal degeneration – the breakdown of the axon part of the nerve cell – in parts of the nervous system, namely the ventral roots and peripheral and cranial nerves.
  • This cascading damage led to denervation atrophy of skeletal muscles i.e., the muscles started shrinking due to lack of nerve stimulation.
  • On examination, many of the spinal neurons appeared swollen, chromatolytic (indicating cellular stress or damage) and contained neurofilamentous accumulations which are unusual protein structures indicating a pathological condition.
  • Others neurons were found to be shrunken and undergoing neuronophagia, where damaged or dying neurons are devoured by other cells. Some neurons had completely disappeared and were replaced by glial cells – supportive cells of the nervous system.

Comparison to ALS in Humans

  • EMND in horses graphically resembles Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s disease in humans. Both are neurodegenerative diseases progressing over time and characterized by muscle weakness, wasting, and degeneration of motor neurons.
  • The research asserts that notwithstanding EMND in horses has never been reported before, the clinical picture it paints bears close resemblance to human ALS. However, the exact cause of EMND is yet to be determined.

Cite This Article

APA
Cummings JF, de Lahunta A, George C, Fuhrer L, Valentine BA, Cooper BJ, Summers BA, Huxtable CR, Mohammed HO. (1990). Equine motor neuron disease; a preliminary report. Cornell Vet, 80(4), 357-379.

Publication

ISSN: 0010-8901
NlmUniqueID: 0074245
Country: United States
Language: English
Volume: 80
Issue: 4
Pages: 357-379

Researcher Affiliations

Cummings, J F
  • Department of Anatomy, New York State College of Veterinary Medicine, Cornell University, Ithaca 14853.
de Lahunta, A
    George, C
      Fuhrer, L
        Valentine, B A
          Cooper, B J
            Summers, B A
              Huxtable, C R
                Mohammed, H O

                  MeSH Terms

                  • Animals
                  • Atrophy
                  • Axons / pathology
                  • Axons / ultrastructure
                  • Brain Stem / pathology
                  • Female
                  • Horse Diseases / pathology
                  • Horses
                  • Male
                  • Microscopy, Electron
                  • Motor Neurons / pathology
                  • Motor Neurons / ultrastructure
                  • Muscles / pathology
                  • Neuromuscular Diseases / pathology
                  • Neuromuscular Diseases / veterinary
                  • Spinal Cord / pathology

                  Citations

                  This article has been cited 14 times.
                  1. Hales EN, Aleman M, Marquardt SA, Katzman SA, Woolard KD, Miller AD, Finno CJ. Postmortem diagnoses of spinal ataxia in 316 horses in California. J Am Vet Med Assoc 2021 Jun 15;258(12):1386-1393.
                    doi: 10.2460/javma.258.12.1386pubmed: 34061609google scholar: lookup
                  2. Cappai MG, Dimauro C, Biggio GP, Cherchi R, Accioni F, Pudda F, Boatto G, Pinna W. The metabolic profile of Asinara (albino) and Sardo donkeys (pigmented) (Equus asinus L., 1758) points to unequivocal breed assignment of individuals. PeerJ 2020;8:e9297.
                    doi: 10.7717/peerj.9297pubmed: 32714654google scholar: lookup
                  3. Finno CJ, Kaese HJ, Miller AD, Gianino G, Divers T, Valberg SJ. Pigment retinopathy in warmblood horses with equine degenerative myeloencephalopathy and equine motor neuron disease. Vet Ophthalmol 2017 Jul;20(4):304-309.
                    doi: 10.1111/vop.12417pubmed: 27491953google scholar: lookup
                  4. Husulak ML, Lohmann KL, Gabadage K, Wojnarowicz C, Marqués FJ. Equine motor neuron disease in 2 horses from Saskatchewan. Can Vet J 2016 Jul;57(7):771-6.
                    pubmed: 27429468
                  5. Finno CJ, Miller AD, Sisó S, Divers T, Gianino G, Barro MV, Valberg SJ. Concurrent Equine Degenerative Myeloencephalopathy and Equine Motor Neuron Disease in Three Young Horses. J Vet Intern Med 2016 Jul;30(4):1344-50.
                    doi: 10.1111/jvim.13977pubmed: 27298214google scholar: lookup
                  6. El-Assaad I, Di Bari JA, Yasuda K, Divers TJ, Summers BA, de Lahunta A, Mohammed H. Differential expression of TAR DNA-binding protein (TDP-43) in the central nervous system of horses afflicted with equine motor neuron disease (EMND): a preliminary study of a potential pathologic marker. Vet Res Commun 2012 Dec;36(4):221-6.
                    doi: 10.1007/s11259-012-9533-ypubmed: 22918699google scholar: lookup
                  7. Tovar-Y-Romo LB, Santa-Cruz LD, Tapia R. Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis. Mol Neurodegener 2009 Jul 20;4:31.
                    doi: 10.1186/1750-1326-4-31pubmed: 19619317google scholar: lookup
                  8. Mohammed HO, Starkey SR, Stipetic K, Divers TJ, Summers BA, de Lahunta A. The role of dietary antioxidant insufficiency on the permeability of the blood-brain barrier. J Neuropathol Exp Neurol 2008 Dec;67(12):1187-93.
                    doi: 10.1097/NEN.0b013e31818f8f51pubmed: 19018244google scholar: lookup
                  9. Mohammed HO, Divers TJ, Summers BA, de Lahunta A. Vitamin E deficiency and risk of equine motor neuron disease. Acta Vet Scand 2007 Jul 2;49(1):17.
                    doi: 10.1186/1751-0147-49-17pubmed: 17605810google scholar: lookup
                  10. Ludolph AC. Treatment of amyotrophic lateral sclerosis--what is the next step?. J Neurol 2000 Dec;247:13-8.
                    doi: 10.1007/BF03161074pubmed: 11200700google scholar: lookup
                  11. Davies PC. Shivering in a thoroughbred mare. Can Vet J 2000 Feb;41(2):128-9.
                    pubmed: 10723600
                  12. Cummings JF, de Lahunta A, Mohammed HO, Divers TJ, Summers BA, Valentine BA, Jackson CA. Endothelial lipopigment as an indicator of alpha-tocopherol deficiency in two equine neurodegenerative diseases. Acta Neuropathol 1995;90(3):266-72.
                    doi: 10.1007/BF00296510pubmed: 8525800google scholar: lookup
                  13. Cummings JF, de Lahunta A, Summers BA, Mohammed HO, Divers TJ, Valentine BA, Trembicki-Graves K. Eosinophilic cytoplasmic inclusions in sporadic equine motor neuron disease: an electron microscopic study. Acta Neuropathol 1993;85(3):291-7.
                    doi: 10.1007/BF00227725pubmed: 8384774google scholar: lookup
                  14. Lofstedt J, Ikede BO. Motor neuron disease in a quarter horse from Nova Scotia. Can Vet J 1994 Aug;35(8):507-9.
                    pubmed: 7954225