Hereditary Necrotising Myelopathy
Hereditary Necrotising Myelopathy (ENM) is a fatal, inherited neurodegenerative disorder unique to the Nederlandse Kooikerhondje, causing progressive paralysis and early death.
Detailed Description
ENM is a severe, autosomal recessive neurodegenerative disease affecting the central nervous system. It is characterized by progressive demyelination and necrosis of the spinal cord and brainstem, leading to ataxia, paresis, and ultimately paralysis. The disease is caused by a mutation in the IBA57 gene, which is involved in mitochondrial function. Affected puppies typically show signs within the first few months of life and the disease is invariably fatal.
Common Symptoms
- Progressive ataxia
- Hind limb weakness
- Paralysis
- Loss of proprioception
- Rapid progression to euthanasia or death
Management Options
- Supportive care
- Euthanasia due to poor prognosis
Frequently Asked Questions
Is there a cure for ENM?
No, there is no cure; prevention through genetic testing is essential.
How common is ENM in the breed today?
Clinical cases are rare due to widespread genetic testing, but carrier frequency remains significant.
Can carriers show symptoms?
No, only dogs with two copies of the mutation develop the disease.
What should breeders do to prevent ENM?
Test all breeding stock and avoid mating two carriers.
How early can ENM be detected?
Genetic testing can be performed at any age, including in puppies.
Condition Details
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Typical Age of Onset
2–6 months
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Testing Available
Yes
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Diagnosing Methods
Clinical neurological examination; MRI; Genetic testing
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Preventative Measures
Genetic testing of breeding dogs; Avoiding carrier-to-carrier matings
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Related Conditions
Degenerative myelopathy, Other inherited neuropathies