Beta-Mannosidosis

Todayโ€™s path rounds are on ๐›๐ž๐ญ๐š-๐ฆ๐š๐ง๐ง๐จ๐ฌ๐ข๐๐จ๐ฌ๐ข๐ฌ!

๐–๐ก๐š๐ญ ๐ข๐ฌ ๐ข๐ญ?
๐๐ž๐ญ๐š-๐ฆ๐š๐ง๐ง๐จ๐ฌ๐ข๐๐จ๐ฌ๐ข๐ฌ is a storage disease where ๐ฆ๐š๐ง๐ง๐จ๐ฌ๐ž, a type of sugar, accumulates in the body. Mannose particularly accumulates in the neurons, impairing normal function.

๐–๐ก๐จ ๐ ๐ž๐ญ๐ฌ ๐ข๐ญ?
We most commonly see this in ๐’๐š๐ฅ๐ž๐ซ๐ฌ ๐œ๐š๐ญ๐ญ๐ฅ๐ž.

๐–๐ก๐š๐ญ ๐œ๐š๐ฎ๐ฌ๐ž๐ฌ ๐ข๐ญ?
Beta-mannosidosis is a genetic condition in Salers cattle, and is thought to have an ๐š๐ฎ๐ญ๐จ๐ฌ๐จ๐ฆ๐š๐ฅ ๐ซ๐ž๐œ๐ž๐ฌ๐ฌ๐ข๐ฏ๐ž inheritance pattern. This means that both parents must have a mutated gene in order to pass the disease onto their calves. The parents themselves are not necessarily affected by the disease.

๐–๐ก๐ฒ ๐ข๐ฌ ๐ญ๐ก๐ข๐ฌ ๐š ๐ฉ๐ซ๐จ๐›๐ฅ๐ž๐ฆ?
Accumulations of mannose affect the ability of neurons to signal properly. Ultimately, this leads to ๐ง๐ž๐ฎ๐ซ๐จ๐ฅ๐จ๐ ๐ข๐œ๐š๐ฅ ๐ฌ๐ข๐ ๐ง๐ฌ such as an inability to stand, tremors and deafness. The mannose accumulations also affect the thyroid gland, which can result in malformations of the head and permanently contracted tendons in the limbs.

๐‡๐จ๐ฐ ๐ข๐ฌ ๐ข๐ญ ๐๐ข๐š๐ ๐ง๐จ๐ฌ๐ž๐?
Diagnosis is typically made after necropsy. The tissues sampled during the necropsy are examined under the microscope, and reveal large accumulations within neurons and other cells.

๐‡๐จ๐ฐ ๐ข๐ฌ ๐ข๐ญ ๐ญ๐ซ๐ž๐š๐ญ๐ž๐?
Unfortunately, there is no treatment for this disease.

๐๐ก๐จ๐ญ๐จ๐ฌ
1-4) Examples of neurons with lots of little white dots in them. Each of these dots is mannose!

๐’๐จ๐ฎ๐ซ๐œ๐ž๐ฌ
Maxie, G. Jubb, Kennedy and Palmerโ€™s Pathology of Domestic Animals, Volume 1. Sixth Edition.

Photos 1-4 ยฉ Noahโ€™s Arkive contributor Leipold, King licensed under CC BY-SA 4.0.

#veterinarypathology #pathology #veterinarymedicine #vetstudent #pathologyandponies #pathrounds

Leave a Reply