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Progressive bulbar paralysis in childhood: A case report

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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.

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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References

  1. Alajouanine T., Bouchet M., Pilaoux P., Lhermitte F.:La paralysie des dilatateurs de la glotte dans la sclérose latérale amyotrophique. Rev. Neurol. 89, 157–158, 1953.

    PubMed  Google Scholar 

  2. Alberca R., Montero C., Iba¯nez A., Segura D.L., Miranda-Nieves G.:Progressive bulbar paralysis associated with neural deafness. A nosological entity. Arch. Neurol. 37, 214–216, 1980

    PubMed  Google Scholar 

  3. Alexander M.P., Emery IIIE.S., Koerner F.C.:Progressive bulbar paresis in Childhood. Arch. Neurol. 33, 66–68, 1976.

    PubMed  Google Scholar 

  4. Arnould G., Tridon P., Laxenaire M., Picard L., Weber M., Brichet B.:Paralysis bulbo-pontine chronique progressive avec surdité. A propos d'une observation de syndrome de Fazio-Londe. Rev. Otoneuroophthalmol. 40, 158–161, 1968.

    Google Scholar 

  5. Boudin G., Pepin B., Vernant J.C., Gautier B., Goverov H.:Cas familial de paralysie bulbo-pontine chronique progressive avec surdité. Rev. Neurol. 124, 90–92, 1971.

    PubMed  Google Scholar 

  6. Brownell B., Oppenheimer D.R., Hughes J.T.:The central nervous system in motor neuron disease. J. Neurol. Neurosurg. Psychiat. 33, 338–357, 1970.

    PubMed  Google Scholar 

  7. Byers R.K., Banker B.Q.:Infantile muscular atrophy, Arch. Neurol. 5, 140–164, 1961.

    PubMed  Google Scholar 

  8. Della Giustina F., Ferriere G., Evrard N., Lyon G.:Progressive bulbar paralysis in childhood (Londe Syndrome). A Clinicopathological report. Acta Paediatr. Belg. 32, 129–133, 1979.

    PubMed  Google Scholar 

  9. Emery A.E.H., Hausmanowa-Petrusewicz I., Davie A.M., Holloway S., Skinner R., Borkowska J.:International collaborative study of the spinal muscular atrophies. Part I. Analysis of Clinical and Laboratory Data. J. Neurol. Sci. 28, 83–94, 1976.

    Article  PubMed  Google Scholar 

  10. Gomez M.R., Clermont V., Bernstein J.:Progressive bulbar paralysis in childhood (Fazio-Londe's disease). Arch. Neurol. 6, 317–323, 1962.

    PubMed  Google Scholar 

  11. Lombaert A., Dom R., Carton H., Brucher J.M.:Progressive pontobulbar palsy with deafness. A clinico-pathological study. Acta Neurol. Belg. 76, 309–314, 1976.

    PubMed  Google Scholar 

  12. Namba T., Aberfeld D.C., Grob D.:Chronic proximal spinal muscular atrophy. J. Neurol. Sci. 11, 401–423, 1970.

    Article  PubMed  Google Scholar 

  13. Thieffry S., Arthuis M., Bargeton E.:Quarante cas de maladie de Werdnig-Hoffman avec onze examens anatomiques. Rev. Neurol. 93, 621–644, 1955.

    PubMed  Google Scholar 

  14. Van Bogaert L.:La sclerose latérale amyotrophique et la paralyse bulbaire progressive chez l'enfant. Rev. Neurol. 1, 180–192, 1925.

    Google Scholar 

  15. Van Laere J.E.:Paralysie bulbo-pontine chronique progressive familiale avec surdité. Un cas de sundrome de Klippel-Trenaunay dans la même fratrie. Problèmes diagnostiques et génétiques. Rev. Neurol. 115, 289–295, 1966.

    PubMed  Google Scholar 

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Perticoni, G.F., Cantisani, T.A. & Fisher, H. Progressive bulbar paralysis in childhood: A case report. Ital J Neuro Sci 4, 107–111 (1983). https://doi.org/10.1007/BF02043448

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