Cranial imaging in autosomal recessive osteopetrosis. Part II. Skull base and brain
| Autor: | Allen D. Elster, Michael Y. M. Chen, L. Lyndon Key, E. G. Theros |
|---|---|
| Rok vydání: | 1992 |
| Předmět: |
Male
Pathology medicine.medical_specialty Sphenoid bone Synchondrosis Genes Recessive Sphenoid Bone Temporal bone medicine Humans Radiology Nuclear Medicine and imaging Child Ossification business.industry Skull Occipital bone Brain Infant Temporal Bone Osteopetrosis Anatomy medicine.disease Magnetic Resonance Imaging Facial nerve medicine.anatomical_structure Child Preschool Occipital Bone Female medicine.symptom Tomography X-Ray Computed business |
| Zdroj: | Radiology. 183:137-144 |
| ISSN: | 1527-1315 0033-8419 |
| DOI: | 10.1148/radiology.183.1.1549660 |
| Popis: | The authors reviewed cranial imaging studies (radiographs, computed tomographic scans, and magnetic resonance [MR] images) in 13 infants and children with the autosomal recessive form of osteopetrosis to characterize patterns of skull base, brain, and cranial nerve involvement at presentation and with progression of disease. Marked sclerosis and deposition of osteopetrotic bone was noted along the anterior (but not posterior) occipitomastoid suture (n = 8), at the basioccipital-exoccipital synchondrosis (n = 9), and along the sphenooccipital synchondrosis (n = 8). Endobones, presumably representing unresorbed primitive ossification centers, were seen in the sphenoidal body and basioccipital bone in 11 of the 13 patients. Marked cupping at the basioccipital-exoccipital synchondrosis was observed in three. Neurologic deficits included blindness (n = 11), conductive hearing loss (n = 11), and facial nerve palsies (n = 4). Delayed myelination was seen with MR imaging in two of five retarded infants, including one with a documented coexisting neuronal storage defect. Prominent extracerebral cerebrospinal fluid spaces were present over the frontal lobes in five of the eight developmentally normal patients, representing either subclinical parenchymal disease or a phenomenon related to discordant growth rates between skull and brain. |
| Databáze: | OpenAIRE |
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