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双侧纤维发育异常累及咀嚼肌并导致一只家养短毛猫出现不可逆的牙关紧闭。

Bilateral fibrodysplasia ossificans affecting the masticatory muscles and causing irreversible trismus in a domestic shorthair cat.

作者信息

Guzu Michel, Gaillot Hugues A, Rosati Marco, Nicolier Alexandra, Hennet Philippe R

机构信息

ADVETIA Specialists Referral Hospital, Vélizy-Villacoublay, France.

Section of Clinical and Comparative Neuropathology, Centre for Clinical Veterinary Medicine, Ludwig-Maximilians-Universität, Munich, Germany.

出版信息

JFMS Open Rep. 2019 Apr 3;5(1):2055116919839857. doi: 10.1177/2055116919839857. eCollection 2019 Jan-Jun.

Abstract

CASE SUMMARY

An 8-year-old spayed female domestic shorthair cat was referred for trismus of progressive onset, which had started at least 1 month previously. The patient presented with weakness, anorexia, chronic bilateral purulent nasal discharge and concurrent reduced nasal airflow. Upon physical examination, painful mouth opening, bilateral swelling of the temporal areas, with an inability to open and close the mouth completely, were apparent. A vertical mandibular range of motion (vmROM) of 22 mm was noted. Complete blood count, biochemistry, electrolytes and various serology tests were unremarkable. CT revealed multiple mineralised lesions within the masticatory muscles. Histopathological features were consistent with those seen in the human disease fibrodysplasia ossificans progressiva (FOP). Supportive treatment did not improve the vmROM, eventually resulting in a 13 mm open bite, and total inability to close and open the mouth. Cardiac arrest occurred at the induction of an anaesthesia procedure aiming to perform tracheostomy and nasal lavage. Despite emergency tracheotomy and cardiac resuscitation, humane euthanasia was elected by the owners. Post-mortem molecular investigations highlighted a heterozygous deletion, compatible with a splicing site mutation in , which is also associated with FOP in humans.

RELEVANCE AND NOVEL INFORMATION

This is the first report in the veterinary literature of FOP-like disease selectively affecting the masticatory muscles. This condition is associated with a poor prognosis, as no medical or surgical treatment has currently proven to be of any prophylactic or curative benefit. Although rare, FOP-like disease should be included in the differential diagnosis of trismus in the cat. Any further muscle injury should be avoided.

摘要

病例摘要

一只8岁已绝育的雌性家养短毛猫因渐进性牙关紧闭前来就诊,该症状至少在1个月前就已开始。患者表现为虚弱、厌食、双侧慢性脓性鼻分泌物以及同时出现的鼻气流减少。体格检查时,明显可见张口疼痛、双侧颞部肿胀,无法完全张口和闭口。记录到垂直下颌活动范围(vmROM)为22毫米。全血细胞计数、生化检查、电解质检查和各种血清学检查均无异常。CT显示咀嚼肌内有多个矿化病变。组织病理学特征与人类进行性骨化性纤维发育不良(FOP)所见一致。支持性治疗并未改善vmROM,最终导致13毫米的开颌,且完全无法闭口和张口。在旨在进行气管切开术和鼻腔灌洗的麻醉过程中发生心脏骤停。尽管进行了紧急气管切开术和心脏复苏,但主人还是选择了实施安乐死。尸检分子研究发现了一个杂合缺失,与 中的剪接位点突变相符,这也与人类的FOP相关。

相关性和新信息

这是兽医文献中首次报道选择性影响咀嚼肌的类FOP疾病。这种情况预后不良,因为目前尚无医学或手术治疗被证明具有任何预防或治疗益处。尽管罕见,但类FOP疾病应纳入猫牙关紧闭的鉴别诊断中。应避免任何进一步的肌肉损伤。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cad5/6448120/3d34089619e6/10.1177_2055116919839857-fig1.jpg

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