{"id":218038,"date":"2024-09-12T07:23:47","date_gmt":"2024-09-12T05:23:47","guid":{"rendered":"http:\/\/staging.laboklin.com\/labogen\/labogen_wp\/akrales-mutilationssyndrom-ams"},"modified":"2024-09-12T07:23:47","modified_gmt":"2024-09-12T05:23:47","slug":"akrales-mutilationssyndrom-ams","status":"publish","type":"page","link":"https:\/\/labogen.com\/fi\/erbkrankheiten-hund\/akrales-mutilationssyndrom-ams\/","title":{"rendered":"Akrales Mutilationssyndrom (AMS)"},"content":{"rendered":"

Acral mutilation syndrome (AMS)<\/h1>

General description<\/strong><\/h4>

Acral Mutilation Syndrome (AMS) is an inherited neurological disorder in dogs that causes a congenital loss of pain sensation in the limbs. Symptoms typically appear between 4 and 9 months of age and include repeated licking, biting, or self-injury to the paws and toes.<\/p>

Breeds<\/span><\/strong><\/h4>

Cockapoo, English Cocker Spaniel, English Pointer, English Springer Spaniel, French Spaniel, German Short-haired Pointing Dog, German Spitz<\/p>

Detailed description<\/summary>

Acral Mutilation Syndrome<\/strong> (AMS) is a hereditary disease of the peripheral nervous system<\/strong> in dogs, leading to a congenital insensitivity to pain. It belongs to a group of conditions known as hereditary sensory and autonomic neuropathies (HSAN), which also occur in humans. Affected dogs suffer from progressive loss of pain sensation in their limbs<\/strong>, which can result in self-mutilating behavior<\/strong>: they lick, bite, or injure their paws and toes, often so severely that it leads to non-healing wounds, infections, or even self-amputation of the toes<\/strong>.<\/p><\/p><\/details><\/div><\/div>

<\/div><\/div>

Acral mutilation syndrome - German Spitz<\/h4>

In the German Spitz<\/strong> (especially the Pomeranian and Miniature Spitz varieties), AMS usually manifests between six and nine months of age<\/strong>. Early signs may seem mild—such as persistent licking of a paw—but can quickly progress to severe issues including lack of pain response in the distal limbs (paws and toes), chronic skin injuries, self-mutilation by chewing or biting affected areas, and even toe amputations (either spontaneous or surgical)<\/strong>. In later stages, gait abnormalities<\/strong> (e.g., walking heel-first) and proprioceptive deficits<\/strong> may also develop.<\/p>

The disease often worsens over time and can significantly impact the dog's quality of life. In some cases, progression may be slowed with protective measures such as collars or dog shoes.<\/p>

In Spitz dogs, a genetic variant in the RETREG1<\/em> gene<\/strong> has been linked to AMS. This mutation leads to the degeneration of nerve cells, explaining the pain insensitivity and subsequent injuries.<\/p><\/p><\/div>

Order details<\/summary>
Test number<\/strong><\/span><\/td>8038<\/td><\/tr>
Sample material<\/strong><\/td>0.5 ml EDTA blood, 2x cheek swab, 1x special swab (eNAT)<\/td><\/tr>
Test duration<\/strong><\/td>7-14 working days<\/td><\/tr><\/table><\/details>
Test specifications<\/summary>
Symptom complex<\/strong><\/td>neurological<\/td><\/tr>
Inheritance<\/strong><\/td>autosomal recessive<\/td><\/tr>
Age of onset<\/strong><\/td>6-9 months<\/td><\/tr>
Gene<\/strong><\/td>RETREG1<\/td><\/tr><\/table><\/details><\/div>
Order now ...<\/a><\/strong><\/div><\/div><\/div>
<\/div><\/div>

Acral mutilation syndrome - English Cocker Spaniel, English Pointer, English Springer Spaniel, French Spaniel, German Shorthaired Pointer<\/h4>

In breeds such as German Shorthaired Pointer, English Pointer, English Cocker Spaniel, English Springer Spaniel, and French Spaniel,<\/strong> symptoms typically appear around four months of age<\/strong>, when puppies begin licking, biting, or injuring their paws and toes. Despite this, proprioception, motor function, and spinal reflexes remain unaffected. Some affected puppies only show pain insensitivity in the lower limbs without self-injury, making diagnosis more challenging.<\/p><\/p><\/div>

Order details<\/summary>
Test number<\/strong><\/span><\/td>8038<\/td><\/tr>
Sample material<\/strong><\/td>0.5 ml EDTA blood, 2x cheek swab, 1x special swab (eNAT)<\/td><\/tr>
Test duration<\/strong><\/td>7-14 working days<\/td><\/tr><\/table><\/details>
Test specifications<\/summary>
Symptom complex<\/strong><\/td>neurological<\/td><\/tr>
Inheritance<\/strong><\/td>autosomal recessive<\/td><\/tr>
Age of onset<\/strong><\/td>4 months <\/td><\/tr>
Causality<\/strong><\/td>causally<\/td><\/tr>
Gene<\/strong><\/td>GDNF<\/td><\/tr>
Mutation<\/strong><\/td>C-T<\/td><\/tr>
Literature<\/strong><\/td>OMIA:001514-9615<\/a><\/td><\/tr><\/table><\/details><\/div>
Order now ...<\/a><\/strong><\/div><\/div><\/div>
<\/div><\/div>
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