Dziedziczna polineuropatia leonbergerów

Choroba nerwów obwodowych o charakterze genetycznym, będąca konsekwencją mutacji w genie AHGEF10.

Dziedziczona jest w sposób autosomalny recesywny. Chorują osobniki homozygotyczne, posiadające 2 nieprawidłowe kopie genu. Heterozygoty nie mają objawów. Skrzyżowanie psy i suki będących nosicielami choroby statystycznie skutkuje urodzeniem się 25% chorych szczeniąt, 50% nosicieli i 25% zdrowych.

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Objawy:

The age on LPN onset is variable, from less than 1 year of age to 11 years of age. Dogs affected with an early onset of the disorder usually develop more severe symptoms and stages of the disorder.

Since Leonberger Polyneuropathy (LPN) is a progressive disorder, owners in its beginning stages may not even notice the first symptoms, such as unbalanced walk. It is common that veterinarians are not able to recognize the disorder before the already progressed stages.

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Two main symptoms of Leonberger Polyneuropathy (LPN) are laryngeal paralysis and rear weakness characterized also by lack of coordination. These two symptoms usually do not develop simultaneously. Laryngeal paralysis can be recognized by specific coughing after eating or drinking, or in a change of the dog’s bark which will appear like hoarse. Dog will have also problems with breathing which will seem louder and heavier than usual even after a light exercise. Affected dog will tend to easily get tired after exercising and will require a long rest. In the end stages of Leonberger Polyneuropathy (LPN) stridor develops. Stridor is a high-pitched sound made during dog’s breathing as a result of turbulent air flow in the larynx. This high-pitched breathing sound is always an indicator of a serious problem.

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Neurological examinations reveal atrophy of the distal limb muscles, depressed spinal and cranial nerve reflexed, and weak or missing movements of laryngeal muscles. Electrophysiological examination reveals lack of muscle action potentials and slowed motor nerve conduction velocity. Biopsy shows muscle atrophy. Axonal degeneration in LPN affected dogs causes decreased myelinated fibre density. This examination results can be recognized by following symptoms: high-stepping pelvic limb gait, decreased or absent tendon reflexes, and changes associated with degeneration of the recurrent laryngeal nerve, including inspiratory stridor resulting from laryngeal paralysis.

Typowe objawy:

• obrz

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Diagnostyka

Dia

 

Leczenie

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Rokowanie

Dobre

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dr n.wet. Diana Stęgierska