Acral Mutilation Syndrome

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The acral Mutilationssyndrom (. Altgriech of ἄκρος , Limb tip 'and mutilation , mutilation') is an autosomal - recessive inherited nerve disease in domestic dogs , with the self-mutilation accompanied the paws. English pointer , German shorthaired pointer , English springer spaniel and miniature pinscher are particularly affected . The clinical picture arises as a result of an insufficient development of sensitive nerve fibers and further fiber loss after birth. Despite some similarities, the disease differs in clinical and / or pathological details from the forms of hereditary sensory neuropathies in humans .

Symptoms and Diagnosis

The disease typically occurs around 3 to 5 months of age. It begins with profuse licking and gnawing of the paws to self-mutilation and even self- amputation . Often the tips of the limbs are completely free of pain. The motor skills, reflexes and proprioception are undisturbed.

The diagnosis is made on the basis of the clinical picture and a histopathological examination. The dorsal root ganglia are smaller and have only about half the number of nerve cells compared to normal dorsal root ganglia. Sometimes signs of nerve cell death can be detected. The dorsal root of the spinal cord and the peripheral nerves are characterized by degenerate nerve fibers . Fiber density and myelination are also reduced in the anterior tract of the spinal cord, which conducts pain and temperature stimuli .

treatment

There is no such thing as an effective treatment. The administration of gabapentin or amitriptyline , the donning of boots, bandages or neck collars can reduce self-destruction. In severe tissue destruction is a euthanasia should be considered. Affected animals and their parents should be excluded from breeding.

literature

  • M. Bardagí, P. Montoliu, L. Ferrer, D. Fondevila, M. Pumarola: Acral mutilation syndrome in a miniature pinscher. In: Journal of comparative pathology. Volume 144, Number 2-3, 2011 Feb-Apr, pp. 235-238, doi : 10.1016 / j.jcpa.2010.08.014 , PMID 20961556 .
  • JF Cummings, A. de Lahunta, KG Braund, WJ Mitchell: Hereditary sensory neuropathy. Nociceptive loss and acral mutilation in pointer dogs: canine hereditary sensory neuropathy. In: The American journal of pathology. Volume 112, Number 1, July 1983, pp. 136-138, PMID 6574711 , PMC 1916311 (free full text).
  • Dominik Faissler et al .: Sensory axonopathies . In: André Jaggy (Ed.): Atlas and textbook of small animal neurology . Schlütersche, Hannover 2005, p. 252-253 .
  • Ariane Neuber: Neurogenic Dermatoses . In: 10th Annual Meeting of the German Society for Veterinary Dermatology , 2009, proceedings , p. 34