2011
DOI: 10.3174/ajnr.a2830
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Imaging Features and Progression of Hyperostosis Cranialis Interna

Abstract: BACKGROUND AND PURPOSE:HCI is a unique autosomal-dominant sclerosing bone dysplasia affecting the skull base and the calvaria, characterized by cranial nerve deficits due to stenosis of neuroforamina, whereby the mandible is affected to a lesser extent. The aim of this study is to describe the specific radiologic characteristics and course of the disorder.

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Cited by 13 publications
(15 citation statements)
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“…While hyperostosis has been characterized as benign and not causal to clinical disease [2], excessive growth can present with clinical symptoms depending on extent and location. Overgrowth could potentially cause intracranial hypertension [1], localized headache [10], and cranial nerve entrapment [3].…”
Section: Discussionmentioning
confidence: 99%
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“…While hyperostosis has been characterized as benign and not causal to clinical disease [2], excessive growth can present with clinical symptoms depending on extent and location. Overgrowth could potentially cause intracranial hypertension [1], localized headache [10], and cranial nerve entrapment [3].…”
Section: Discussionmentioning
confidence: 99%
“…An affected frontal bone, termed hyperostosis frontalis interna (HFI), comprises a majority of cases with particular prevalence in post menopausal females. HFI is usually an incidental finding and is not known to cause clinical disease [2], although excessive growth can be symptomatic upon brain tissue compression and/or cranial nerve entrapment [3]. Hyperostosis of the sphenoid bone or other bones comprising the floor of the cranium are rare and are generally seen unilaterally in association with intraosseous meningiomas [4,5].…”
Section: Introductionmentioning
confidence: 99%
“…The presence of bilateral lesions, however, may suggest a more diffuse process, such as an underlying bone remodeling abnormality similar to the abnormalities observed in hyperostosis cranialis interna (HCI), hyperostosis frontalis interna, fibrous dysplasia, or Paget's disease. 6,17,18 HCI is an especially intriguing candidate to explain bilateral calvarial and skull base hyperostosis that is isolated to the cranium. Patients with HCI present with significant thickening of the inner table of the frontal, parietal, occipital, and temporal bones.…”
Section: Pathophysiologymentioning
confidence: 99%
“…Radiographically, the hyperostotic lesions of the IAC found in HCI resemble exostoses. 17,18 Thus, in patients with diffuse thickening of the inner table across the calvarium there is likely a genetic predisposition to IAC hyperostosis that is compounded by phenotypical factors, such as local inflammation, and aberrant suture closure.…”
Section: Pathophysiologymentioning
confidence: 99%
“…Moreover, the differential diagnosis of extensive osseous thickening in the ISFB ranges from such focal masses as meningioma and endosteal osteoma, subdural and dural calcification and to the diffuse skull process including Paget's disease, acromegaly and fibrous dyplasia (14,17,18,24).…”
Section: Location In Frontal Bone (A: Anterior Part Of the Frontal Sqmentioning
confidence: 99%