Tongue atrophy as a neurological finding in hereditary polyneuropathy in Alaskan malamutes
Abstract Background Tongue atrophy with wrinkling as a clinical sign of inherited polyneuropathies has not been reported in dogs. Objectives Clinically describe tongue atrophy as well as morphology of the tongue and hypoglossal nerve in Alaskan malamute polyneuropathy (AMPN). Animals Six client‐owne...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2022-03-01
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| Series: | Journal of Veterinary Internal Medicine |
| Subjects: | |
| Online Access: | https://doi.org/10.1111/jvim.16351 |
| _version_ | 1818759143547207680 |
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| author | Josefin Hultman Karin H. Jäderlund Lars Moe Arild Espenes Fredrik S. Skedsmo |
| author_facet | Josefin Hultman Karin H. Jäderlund Lars Moe Arild Espenes Fredrik S. Skedsmo |
| author_sort | Josefin Hultman |
| collection | DOAJ |
| description | Abstract Background Tongue atrophy with wrinkling as a clinical sign of inherited polyneuropathies has not been reported in dogs. Objectives Clinically describe tongue atrophy as well as morphology of the tongue and hypoglossal nerve in Alaskan malamute polyneuropathy (AMPN). Animals Six client‐owned Alaskan malamute dogs diagnosed with AMPN, all homozygous for the causative mutation in the N‐myc downstream‐regulated gene 1 (NDRG1) and 1 neurologically normal control Alaskan malamute. Methods Prospective case study. Clinical and neurological examinations were performed on affected dogs. Necropsy samples from the tongue muscle and hypoglossal nerve were examined by light and electron microscopy. Results All affected dogs had abnormal wrinkles and grooves on the dorsal surface of the tongue, a clinical sign not described previously in dogs with AMPN. Electromyography of the tongue performed in 2 dogs showed spontaneous activity. Five affected dogs underwent necropsy studies. Histopathology of the tongue showed groups of angular atrophic myofibers and changes in the hypoglossal nerve included thinly myelinated fibers, small onion bulbs, folded myelin, and axonal degeneration. Conclusion and Clinical Importance Histopathologic changes in the tongue and hypoglossal nerve were consistent with previously reported changes in skeletal muscle and other nerves from dogs with AMPN. Therefore, we conclude that macroscopic tongue atrophy is part of the disease phenotype of AMPN and should be considered a potential clinical sign in dogs with polyneuropathies. |
| first_indexed | 2024-12-18T06:38:02Z |
| format | Article |
| id | doaj.art-afae23d51c484c12af91d81cca4edf82 |
| institution | Directory Open Access Journal |
| issn | 0891-6640 1939-1676 |
| language | English |
| last_indexed | 2024-12-18T06:38:02Z |
| publishDate | 2022-03-01 |
| publisher | Wiley |
| record_format | Article |
| series | Journal of Veterinary Internal Medicine |
| spelling | doaj.art-afae23d51c484c12af91d81cca4edf822022-12-21T21:17:43ZengWileyJournal of Veterinary Internal Medicine0891-66401939-16762022-03-0136267267810.1111/jvim.16351Tongue atrophy as a neurological finding in hereditary polyneuropathy in Alaskan malamutesJosefin Hultman0Karin H. Jäderlund1Lars Moe2Arild Espenes3Fredrik S. Skedsmo4Department of Companion Animal Clinical Sciences, Faculty of Veterinary Medicine Norwegian University of Life Sciences Ås NorwayDepartment of Companion Animal Clinical Sciences, Faculty of Veterinary Medicine Norwegian University of Life Sciences Ås NorwayDepartment of Companion Animal Clinical Sciences, Faculty of Veterinary Medicine Norwegian University of Life Sciences Ås NorwayDepartment of Preclinical Sciences and Pathology, Faculty of Veterinary Medicine Norwegian University of Life Sciences Ås NorwayDepartment of Companion Animal Clinical Sciences, Faculty of Veterinary Medicine Norwegian University of Life Sciences Ås NorwayAbstract Background Tongue atrophy with wrinkling as a clinical sign of inherited polyneuropathies has not been reported in dogs. Objectives Clinically describe tongue atrophy as well as morphology of the tongue and hypoglossal nerve in Alaskan malamute polyneuropathy (AMPN). Animals Six client‐owned Alaskan malamute dogs diagnosed with AMPN, all homozygous for the causative mutation in the N‐myc downstream‐regulated gene 1 (NDRG1) and 1 neurologically normal control Alaskan malamute. Methods Prospective case study. Clinical and neurological examinations were performed on affected dogs. Necropsy samples from the tongue muscle and hypoglossal nerve were examined by light and electron microscopy. Results All affected dogs had abnormal wrinkles and grooves on the dorsal surface of the tongue, a clinical sign not described previously in dogs with AMPN. Electromyography of the tongue performed in 2 dogs showed spontaneous activity. Five affected dogs underwent necropsy studies. Histopathology of the tongue showed groups of angular atrophic myofibers and changes in the hypoglossal nerve included thinly myelinated fibers, small onion bulbs, folded myelin, and axonal degeneration. Conclusion and Clinical Importance Histopathologic changes in the tongue and hypoglossal nerve were consistent with previously reported changes in skeletal muscle and other nerves from dogs with AMPN. Therefore, we conclude that macroscopic tongue atrophy is part of the disease phenotype of AMPN and should be considered a potential clinical sign in dogs with polyneuropathies.https://doi.org/10.1111/jvim.16351Alaskan malamute polyneuropathyCharcot‐Marie‐Tooth type 4Dhypoglossal nerveNDRG1 mutation |
| spellingShingle | Josefin Hultman Karin H. Jäderlund Lars Moe Arild Espenes Fredrik S. Skedsmo Tongue atrophy as a neurological finding in hereditary polyneuropathy in Alaskan malamutes Journal of Veterinary Internal Medicine Alaskan malamute polyneuropathy Charcot‐Marie‐Tooth type 4D hypoglossal nerve NDRG1 mutation |
| title | Tongue atrophy as a neurological finding in hereditary polyneuropathy in Alaskan malamutes |
| title_full | Tongue atrophy as a neurological finding in hereditary polyneuropathy in Alaskan malamutes |
| title_fullStr | Tongue atrophy as a neurological finding in hereditary polyneuropathy in Alaskan malamutes |
| title_full_unstemmed | Tongue atrophy as a neurological finding in hereditary polyneuropathy in Alaskan malamutes |
| title_short | Tongue atrophy as a neurological finding in hereditary polyneuropathy in Alaskan malamutes |
| title_sort | tongue atrophy as a neurological finding in hereditary polyneuropathy in alaskan malamutes |
| topic | Alaskan malamute polyneuropathy Charcot‐Marie‐Tooth type 4D hypoglossal nerve NDRG1 mutation |
| url | https://doi.org/10.1111/jvim.16351 |
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