4.4 Article

Ethmoidal meningoencephalocele in a C57BL/6J mouse

期刊

LABORATORY ANIMALS
卷 55, 期 2, 页码 181-188

出版社

SAGE PUBLICATIONS INC
DOI: 10.1177/0023677220944449

关键词

Congenital disease; neurology; MRI imaging; frontoethmoidal meningoencephalocele; mouse

资金

  1. MSKCC NCI Cancer Center Support Grant [P30 CA008748]

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An otherwise healthy two-month-old female C57BL/6J mouse presented with a left-sided head tilt, which was found to be caused by a congenital malformation of the nasal cavity and frontal aspect of the skull known as an ethmoidal meningoencephalocele through MRI and histopathologic examination. This abnormality has not been previously reported in mice, and further research is needed to understand the etiology and potential genetic factors associated with this condition.
An otherwise healthy two-month-old female C57BL/6J mouse presented with a left-sided head tilt. Differential diagnoses included idiopathic necrotizing arteritis, bacterial otitis media/interna (Pasteurella pneumotropica,Pseudomonas aeruginosa,Streptococcus sp.,Mycoplasma pulmonisandBurkholderia gladioli), encephalitis, an abscess, neoplasia, a congenital malformation and an accidental or iatrogenic head trauma. Magnetic resonance imaging (MRI) revealed a large space-occupying right olfactory lobe intra-axial lesion with severe secondary left-sided subfalcine herniation. Following imaging, the animal was euthanized due to poor prognosis. Histopathologic examination revealed a unilateral, full-thickness bone defect at the base of the cribriform plate and nasal conchae dysplasia, resulting in the herniation of the olfactory bulb into the nasal cavity. There was also a left midline-shift of the frontal cortex and moderate catarrhal sinusitis in the left mandibular sinus. The MRI and histopathologic changes are consistent with a congenital malformation of the nasal cavity and frontal aspect of the skull known as an ethmoidal meningoencephalocele. Encephaloceles are rare abnormalities caused by herniation of contents of the brain through a defect in the skull which occur due to disruption of the neural tube closure at the level anterior neuropore or secondary to trauma, surgical complications, cleft palate or increased intracranial pressure. The etiology is incompletely understood but hypotheses include genetics, vitamin deficiency, teratogens, infectious agents and environmental factors. Ethmoidal encephaloceles have been reported in multiple species including humans but have not been reported previously in mice. There are multiple models for spontaneous and induced craniofacial malformation in mice, but none described for ethmoidal encephaloceles.

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