NEURORADIOLOGIC CORRELATION IN MÖBIUS SYNDROME AND CENTRAL RESPIRATORY DISFUNCTION
| Autor: | L. Cáceres, L. Caramuta, G. Agosta, A. Dericco, E. Muñoz, S. Frenkel, C. Maxit, E. Vaucheret, J. Muntadas |
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| Rok vydání: | 2006 |
| Předmět: |
Mechanical ventilation
medicine.medical_specialty Möbius syndrome business.industry medicine.medical_treatment Cranial nerves General Medicine medicine.disease Surgery Atrophy Neuroimaging Pediatrics Perinatology and Child Health Paralysis Medicine Neurology (clinical) Radiology Brainstem medicine.symptom Respiratory system business |
| Zdroj: | Neuropediatrics. 37 |
| ISSN: | 1439-1899 0174-304X |
| DOI: | 10.1055/s-2006-945618 |
| Popis: | Objectives: To describe neuroradiologic findings in six patients with Mobius syndrome and associated central respiratory dysfunction. Methods: Clinical, and neuroimaging (MRI) data were reviewed, including Brain CT, MRI 3 D. Results: We present six newborns, four premature neonates who suffered from bilateral VI and VII nerves paralysis, without respiratory effort from birth, who required prolonged mechanical ventilation until their death, and two term infants with the same symptoms, actually live with home care. Brain CT scans revealed bilateral brainstem calcifications in all the cases. Cerebral MRI showed typical findings; in the two term infants we perform a cerebral MRI with 3D reconstruction of the emergency of the VII cranial nerve, without finding the nerve. Conclusion: The anatomical studies show cranial nerves nuclei atrophy, secondary to periphery nerve lesion. Recent reports referred the clinical variability of this entity, a subgroup of patients showing risk of apnoea, and lack of respiratory impulse from birth in some of them. These central respiratory dysfunction patients had tegmental brainstem calcifications in brain CT scan. Brain MRI 3D of Mobius patients exhibit brainstem hypoplasia and absence of 7th cranial nerve. Diagnostic in the neonatal period may have relevance to take as soon as possible the appropriate decisions and interventions in the respiratory airway. |
| Databáze: | OpenAIRE |
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