Stenotic Nasopharyngeal Dysgenesis in the Dachshund: Seven Cases (2002–2004)

Kirberger, Robert M.; Steenkamp, Gerhard; Spotswood, Timothy C; Boy, Sonja C.; Miller, David B.; Zyl, Malan van

doi:10.5326/0420290

Cited by 16 publications

(5 citation statements)

References 11 publications

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“…To our knowledge, only one report has linked abnormalities in the palatopharyngeus muscle with dysphagia (Kirberger et al . ). In contrast, the effects of ageing or disease on the inferior pharyngeal constrictor muscles, particularly the cricopharyngeus muscle, have been well studied (Leese & Hopwood, ; Hyodo et al .…”

Section: Introductionmentioning

confidence: 97%

Ageing and muscular dystrophy differentially affect murine pharyngeal muscles in a region‐dependent manner

Randolph

Luo

et al. 2014

The Journal of Physiology

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Key pointsr Millions of elderly individuals have dysphagia, a debilitating and life-threatening condition in which the ability to swallow is impaired.r Several muscles surround the three regions of the pharynx, which are essential for proper swallowing, yet the effects of ageing and disease on these muscles are not well understood.r We demonstrate that the fibre size of murine pharyngeal muscles is differentially affected by ageing and muscular dystrophy depending on their location within the pharynx.r Using a mouse model of an age-associated dysphagic disease (oculopharyngeal muscular dystrophy), we show that overexpression of wild-type polyadenylate binding nuclear protein 1 in muscle tissue prevents age-related dysphagia and age-related muscle atrophy of laryngopharyngeal muscles.r These results demonstrate that mice are an excellent model for studying mechanisms of ageing and disease on pharyngeal muscle physiology, and such studies could lead to new therapies for individuals with dysphagia.Abstract The inability to swallow, or dysphagia, is a debilitating and life-threatening condition that arises with ageing or disease. Dysphagia results from neurological or muscular impairment of one or more pharyngeal muscles, which function together to ensure proper swallowing and prevent the aspiration of food or liquid into the lungs. Little is known about the effects of age or disease on pharyngeal muscles as a group. Here we show ageing affected pharyngeal muscle growth and atrophy in wild-type mice depending on the particular muscle analysed. Furthermore, wild-type mice also developed dysphagia with ageing. Additionally, we studied pharyngeal muscles in a mouse model for oculopharyngeal muscular dystrophy, a dysphagic disease caused by a polyalanine expansion in the RNA binding protein, PABPN1. We examined pharyngeal muscles of mice overexpressing either wild-type A10 or mutant A17 PABPN1. Overexpression of mutant A17 PABPN1 differentially affected growth of the palatopharyngeus muscle dependent on its location within the pharynx. Interestingly, overexpression of wild-type A10 PABPN1 was protective against age-related muscle atrophy in the laryngopharynx and prevented the development of age-related dysphagia. These results demonstrate that pharyngeal muscles are differentially affected by both ageing and muscular dystrophy in a region-dependent manner. These studies lay important groundwork for understanding the molecular and cellular mechanisms that regulate pharyngeal muscle growth and atrophy, which may lead to novel therapies for individuals with dysphagia. Abbreviations A10-WT, wild-type A10.1 PABPN1 overexpression transgenic mouse; A17-MUT, mutant A17.1 PABPN1 overexpression transgenic mouse; FVB, Friend leukaemia virus B; H&E, haematoxylin and eosin; MHC, myosin heavy chains; OPMD, oculopharyngeal muscular dystrophy; PABPN1, polyadenylate binding nuclear protein 1; type I, slow twitch oxidative myofibre; type II, fast twitch glycolytic myofibre; WT, wild-type.

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Section: Introductionmentioning

confidence: 97%

Ageing and muscular dystrophy differentially affect murine pharyngeal muscles in a region‐dependent manner

Randolph

Luo

et al. 2014

The Journal of Physiology

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“…Lateral view of the pharyngeal area of GRippet dog, Derwood , illustrating glossal hypertrophy. The tongue extends well ventral to the line demarcating the mandible and ventral aspect of the oral cavity [ 76 ] …”

Section: Resultsmentioning

confidence: 99%

Dystrophin-deficient dogs with reduced myostatin have unequal muscle growth and greater joint contractures

Kornegay

Bogan

et al. 2016

Skeletal Muscle

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BackgroundMyostatin (Mstn) is a negative regulator of muscle growth whose inhibition promotes muscle growth and regeneration. Dystrophin-deficient mdx mice in which myostatin is knocked out or inhibited postnatally have a less severe phenotype with greater total mass and strength and less fibrosis and fatty replacement of muscles than mdx mice with wild-type myostatin expression. Dogs with golden retriever muscular dystrophy (GRMD) have previously been noted to have increased muscle mass and reduced fibrosis after systemic postnatal myostatin inhibition. Based partly on these results, myostatin inhibitors are in development for use in human muscular dystrophies. However, persisting concerns regarding the effects of long-term and profound myostatin inhibition will not be easily or imminently answered in clinical trials.MethodsTo address these concerns, we developed a canine (GRippet) model by crossbreeding dystrophin-deficient GRMD dogs with Mstn-heterozygous (Mstn+/−) whippets. A total of four GRippets (dystrophic and Mstn+/−), three GRMD (dystrophic and Mstn wild-type) dogs, and three non-dystrophic controls from two litters were evaluated.ResultsMyostatin messenger ribonucleic acid (mRNA) and protein levels were downregulated in both GRMD and GRippet dogs. GRippets had more severe postural changes and larger (more restricted) maximal joint flexion angles, apparently due to further exaggeration of disproportionate effects on muscle size. Flexors such as the cranial sartorius were more hypertrophied on magnetic resonance imaging (MRI) in the GRippets, while extensors, including the quadriceps femoris, underwent greater atrophy. Myostatin protein levels negatively correlated with relative cranial sartorius muscle cross-sectional area on MRI, supporting a role in disproportionate muscle size. Activin receptor type IIB (ActRIIB) expression was higher in dystrophic versus control dogs, consistent with physiologic feedback between myostatin and ActRIIB. However, there was no differential expression between GRMD and GRippet dogs. Satellite cell exhaustion was not observed in GRippets up to 3 years of age.ConclusionsPartial myostatin loss may exaggerate selective muscle hypertrophy or atrophy/hypoplasia in GRMD dogs and worsen contractures. While muscle imbalance is not a feature of myostatin inhibition in mdx mice, findings in a larger animal model could translate to human experience with myostatin inhibitors.Electronic supplementary materialThe online version of this article (doi:10.1186/s13395-016-0085-7) contains supplementary material, which is available to authorized users.

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“…Obstructive airway syndrome has been well described in both brachycephalic and non‐brachycephalic breeds 1–9. Nasopharyngeal dysgenesis, described in seven dachshunds,10 and redundant mucosa originating from or near the laryngeal inlet6 11 12 are rare causes of upper airway obstruction in dogs. Displacement of glossoepiglottic mucosa was first described in two dogs by Bedford6 in 1983.…”

Section: Introductionmentioning

confidence: 99%

Laryngopharyngeal mucosal fold causing upper airway obstruction in a dog

Penfold

Zee

Hartman

2019

Vet Record Case Reports

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A 2.4-kg, six-year-old, sterilised, male Pomeranian presented with dyspnoea that had begun two years prior and slowly progressed. Pharyngoscopy identified a redundant laryngopharyngeal mucosal fold that was being aspirated cranially into the laryngeal opening causing upper airway obstruction. A diode laser was used to resect the fold. Postoperative laryngeal oedema necessitated the use of a tracheostomy tube for just over two days. Otherwise, the dog made an uneventful recovery. To the authors’ knowledge, this is the first report of a redundant laryngopharyngeal mucosal fold resulting in upper airway obstruction in a dog.

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Stenotic Nasopharyngeal Dysgenesis in the Dachshund: Seven Cases (2002–2004)

Cited by 16 publications

References 11 publications

Ageing and muscular dystrophy differentially affect murine pharyngeal muscles in a region‐dependent manner

Ageing and muscular dystrophy differentially affect murine pharyngeal muscles in a region‐dependent manner

Dystrophin-deficient dogs with reduced myostatin have unequal muscle growth and greater joint contractures

Laryngopharyngeal mucosal fold causing upper airway obstruction in a dog

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