Canis ISSN: 2398-2942

Leukoencephalomyelopathy

Synonym(s): LEMP, LEM

Contributor(s): Karen Munana, Simon Platt, Laurent Garosi

Introduction

  • Cause: unknown.
  • History: progressive ataxia involving all four limbs beginning insidiously between 1.5 and 4 years of age in both males and females.
  • Treatment: no specific treatment. Physiotherapy and hydrotherapy may help to maintain muscle mass and strength.
  • Prognosis: poor; with no specific treatment. Progression of the disease frequently leads to recumbency.

Pathogenesis

Etiology

  • Unknown.
  • Autosomal recessive mode of inheritance resulting from a 1 base pair insertion within the LEMP gene.
  • All studied Rottweiler homozygous affected (D/D) for this leukoencephalomyelopathy (LEMP) mutation have shown clinical signs of disease. However, not all Rottweilers with suspected LEMP have tested for this mutation. It is possible that these dogs may be suffering from a different form of LEMP or the other neurodegenerative disease described in this breed (neuroaxonal dystrophy Neuroaxonal dystrophy). 
  • Some dogs, related.
  • Tentatively classified as a myelinolytic disease rather than a leukodystrophy; however this issue remains unresolved.

Pathophysiology

  • Little known.
  • The signs do not begin until adulthood (mean 2.2 years in one study) and are progressive. The neuropathological picture is one of repair of prior demyelinating episode(s).
  • Predominant involvement of one segment of the spinal cord (cervical) similar to other diseases eg Afghan hound myelopathy (a leukodystrophy).
  • Lesions tend to be restricted to the lateral and dorsal funiculi of the cervical spinal cord and spinal tracts of the trigeminal nerve, pyramids, caudal cerebellar peduncles, cerebellar medulla and optic tracts.
  • Leukoencephalomyelopathy denotes a disorder affecting white matter in the brain and spinal cord; unknown as to whether it is a demyelinating disease (characterized by a process of disintegration of the myelin sheath without damage to the axons) or a leukodystrophy (inherited condition in which, myelin formation is defective and cannot be maintained).

Diagnosis

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Treatment

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Outcomes

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Further Reading

Publications

Refereed papers

  • Recent references from PubMed and VetMedResource.
  • Eagleson J S, Kent M, Platt S R, Rech R R & Howerth E W (2013) MRI Findings in a Rottweiler with Leukoencephalomyelopathy. JAAHA 49 (4), 255-261 PubMed.
  • Hirschvogel K, Matiasek K,  Flatz K et al (2013) Magnetic resonance imaging and genetic investigation of a case of rottweiler leukoencephalomyelopathy. BMC Vet Res 9, 57 PubMed.
  • Oevermann A, Bley T, Konar M, Lang J & Vandevelde M (2008) A novel leukoencephalomyelopathy of Leonberger dogs. JVIM 22 (2), 467-471 PubMed.
  • Chrisman C L (1992) Neurological diseases of Rottweilers: Neuroaxonal dystrophy and leukoencephalomalacia . J Small Anim Pract 33 (10), 500-504 VetMedResource.
  • Slocombe R F, Mitten R & Mason T A (1989) Leukoencephalomyelopathy in Australian Rottweiler dogs. Aust Vet J 66 (5), 147-150 PubMed.
  • Wouda W & van Ness J J (1986) Progressive ataxia due to central demyelination in Rottweiler dogs. Vet Q 8 (2), 89-97 PubMed.
  • Gamble D A & Chrisman C L (1984) A leukoencephalomyelopathy of Rottweiler dogs. Vet Pathol 21 (3), 274-280 PubMed.

Other sources of information


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