Cerebellomedullary compression in recessive craniometaphyseal dysplasia
| العنوان: | Cerebellomedullary compression in recessive craniometaphyseal dysplasia |
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| المؤلفون: | Yasuhiro Yonekawa, Hermann F. Sailer, Bernhard Schmitt, W. Wichmann, Anton Valavanis, Eugen Boltshauser |
| المصدر: | Neuroradiology. 38:S193-S195 |
| بيانات النشر: | Springer Science and Business Media LLC, 1996. |
| سنة النشر: | 1996 |
| مصطلحات موضوعية: | Cerebellum, Ataxia, Decompression, Basilar invagination, Constriction, Pathologic, Central nervous system disease, Cerebrospinal fluid, Developmental Neuroscience, Clivus, medicine, Humans, Radiology, Nuclear Medicine and imaging, Brain Diseases, Foramen magnum, business.industry, Skull, Infant, Anatomy, medicine.disease, Magnetic Resonance Imaging, Osteochondrodysplasia, medicine.anatomical_structure, Neurology, Cranial Fossa, Posterior, Posterior cranial fossa, Pediatrics, Perinatology and Child Health, Female, Neurology (clinical), medicine.symptom, Cardiology and Cardiovascular Medicine, business |
| الوصف: | Craniometaphyseal dysplasia (CMD) is a very rare disorder of bone remodelling characterised by sclerosis of the skull base, vault and facial bones and metaphyseal splaying of tubular bones. The recessive form appears to be more severe than the dominant. Cranial nerve deficits have been reported in infancy and early childhood in a few patients, but the long-term history of recessive CMD is not well documented. We report cerebellomedullary compression in a girl with recessive CMD recognised at 14 years because of progressive truncal ataxia. MRI revealed backward angulation of the thickened clivus, narrowing of the foramen magnum and upward deviation of the cerebellum by a markedly thickened occipital squama, tonsillar herniation and obliteration of the infratentorial cerebrospinal fluid spaces. Posterior cranial fossa decompression resulted in marked improvement of the ataxia. Compression of posterior cranial fossa structures has to be considered in the natural history and management of CMD. |
| تدمد: | 1432-1920 0028-3940 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::77a43f2ebb5f10bb63d2720d0e6e0a01Test https://doi.org/10.1007/bf02278158Test |
| حقوق: | CLOSED |
| رقم الانضمام: | edsair.doi.dedup.....77a43f2ebb5f10bb63d2720d0e6e0a01 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 14321920 00283940 |
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