Progressive bulbar paralysis in childhood: A case report

  • Perticoni G. F. 
  • Cantisani T. A. 
  • Fisher H. 
Case Reports

Abstract

The case of a progressive bulbar paresis in a nine and a half year old child is reported. The first symptoms were present at birth; however the subsequent evolution was very slow. Lesion of the motor nuclei of the V, VII, IX, XII, cranial nerves was evident on electromyographic investigation. Damage to the acoustic brain stem pathway was documented by the brain stem evoked potentials although audiometry was normal. No other neuronal systems or districts appeared to be damaged.

The case suggests Fazio-Londe disease, although the involvement (albeit partial) of the auditory pathways recalls Van Laere syndrome.

This supports the view that motor neuron disease in infancy is not an autonomous entity but a variant in a wide spectrum of progressive neuronal diseases.

Key-words

Fazio-Londe syndrome Van Laere syndrome motor neuron disease in childhood 

Sommario

Viene presentato un caso di paralisi bulbare progressiva osservata in una bambina di 9 anni. I primi sintomi si sono manifestati già immediatamente dopo la nascita e la successiva evoluzione è stata molto lenta. La lesione dei nuclei motori del V, VII, IX, XII nervo cranico depone per una malattia di Fazio-Londe; tuttavia le alterazioni riscontrate a carico delle vie acustiche troncoencefaliche richiamano la sindrome di Van Laere. Non appaiono essere danneggiati altri sistemi neuronali. Tali reperti appaiono essere a sostegno delle tesi di quegli Autori che considerano la malattia del neurone di moto nell'infanzia non come entità autonoma ma come ulteriore variante nell'ampio spettro delle malattie progressive del neurone.

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Copyright information

© Masson Italia Editori S.p.A. 1983

Authors and Affiliations

  • Perticoni G. F. 
    • 1
  • Cantisani T. A. 
    • 1
  • Fisher H. 
    • 1
  1. 1.Servizio di NeurofisiopatologiaOspedale Generale RegionalePerugia

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