[Möbius syndrome and an apparently life-threatening event].
Möbius syndrome is characterized by congenital facial weakness with impairment of ocular abduction. A subgroup of these patients have associated apneas because of involvement of brainstem respiratory centers located slightly lateral to the abducens nuclei. We report a 7-month old infant admitted to the pediatric intensive care unit because of an episode of cyanosis, hypotonia and unresponsiveness. The patient then became respirator dependent afterwards. On examination, facial diplegia, impairment of ocular abduction and hypotonia were evident. Magnetic resonance imaging (MRI) revealed abnormal signal intensity in brainstem tegmentum. At the age of 11 months he was discharged but required a home ventilator. He died 5 months later due to an infection. Möbius syndrome is associated with central respiratory dysfunction. The finding of abnormal signal intensity in brainstem tegmentum on MRI is a possible predictor of apnea in these patients.
López-Laso
E
E
Unidad de Neurología Pediátrica, Servicio de Pediatría, Críticos y Urgencias Pediátricas, Hospital Reina Sofía, Córdoba, España. med020053@saludalia.com
Pérez Navero
J L
JL
Marín Rodríguez
C
C
Camino León
R
R
Ibarra de la Rosa
I
I
Velasco Jabalquinto
Maria J
MJ
D001049:Apnea / Q000209:etiology*; D001933:Brain Stem / Q000002:abnormalities*; D003490:Cyanosis; D017809:Fatal Outcome; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D020331:Mobius Syndrome / Q000503:physiopathology*; D009123:Muscle Hypotonia