| Literature DB >> 12364956 |
Bianca Helena Brum Batista1, Andrea Garcia Almeida, Magda Lahorgue Nunes, Paulo Márcio Condessa Pitrez, João Arthur Ehlers.
Abstract
Progressive bulbar palsy, also called Fazio -Londe disease, is characterized by progressive impairment of cranial nerves in children. It was first reported by Fazio in 1892 and until now only 30 cases have been published in the literature. Both sexes can be affected and clinical course can be divided on early (< 6 years age, predominance of respiratory symptoms) and late course (6-20 years of age, predominance of motor symptoms on superior limbs). We report a 4 years old boy that started with intense stridor and respiratory distress, initially being diagnosed as an acute asthma attack. Clinical signs worsened and 12 months latter he already had impairment of cranial nerves V, VII, VIII, IX and X confirmed by clinical examination and neurophysiological evaluation.Entities:
Mesh:
Year: 2002 PMID: 12364956
Source DB: PubMed Journal: Arq Neuropsiquiatr ISSN: 0004-282X Impact factor: 1.420