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[Progressive bulbar palsy (Fazio-Londe disease): case report].

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.

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  • Authors+Show Affiliations

    ,

    Serviço de Neurologia, Hospital São Lucas, Pontifícia Universidade Católica do Rio Grande do Sul, Porto Alegre, RS, Brasil.

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    Source

    Arquivos de neuro-psiquiatria 60:3-B 2002 Sep pg 830-4

    MeSH

    Bulbar Palsy, Progressive
    Child, Preschool
    Diagnosis, Differential
    Disease Progression
    Follow-Up Studies
    Humans
    Male

    Pub Type(s)

    Case Reports
    English Abstract
    Journal Article

    Language

    por

    PubMed ID

    12364956

    Citation

    Batista, Bianca Helena Brum, et al. "[Progressive Bulbar Palsy (Fazio-Londe Disease): Case Report]." Arquivos De Neuro-psiquiatria, vol. 60, no. 3-B, 2002, pp. 830-4.
    Batista BH, Almeida AG, Nunes ML, et al. [Progressive bulbar palsy (Fazio-Londe disease): case report]. Arq Neuropsiquiatr. 2002;60(3-B):830-4.
    Batista, B. H., Almeida, A. G., Nunes, M. L., Pitrez, P. M., & Ehlers, J. A. (2002). [Progressive bulbar palsy (Fazio-Londe disease): case report]. Arquivos De Neuro-psiquiatria, 60(3-B), pp. 830-4.
    Batista BH, et al. [Progressive Bulbar Palsy (Fazio-Londe Disease): Case Report]. Arq Neuropsiquiatr. 2002;60(3-B):830-4. PubMed PMID: 12364956.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - [Progressive bulbar palsy (Fazio-Londe disease): case report]. AU - Batista,Bianca Helena Brum, AU - Almeida,Andrea Garcia, AU - Nunes,Magda Lahorgue, AU - Pitrez,Paulo Márcio Condessa, AU - Ehlers,João Arthur, PY - 2002/10/5/pubmed PY - 2003/2/7/medline PY - 2002/10/5/entrez SP - 830 EP - 4 JF - Arquivos de neuro-psiquiatria JO - Arq Neuropsiquiatr VL - 60 IS - 3-B N2 - 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. SN - 0004-282X UR - https://www.unboundmedicine.com/medline/citation/12364956/[Progressive_bulbar_palsy__Fazio_Londe_disease_:_case_report]_ L2 - http://www.scielo.br/scielo.php?script=sci_arttext&amp;pid=S0004-282X2002000500026&amp;lng=en&amp;nrm=iso&amp;tlng=en DB - PRIME DP - Unbound Medicine ER -