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
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.
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
Alexander M.P., Emery IIIE.S., Koerner F.C.:Progressive bulbar paresis in Childhood. Arch. Neurol. 33, 66–68, 1976.
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.
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.
Brownell B., Oppenheimer D.R., Hughes J.T.:The central nervous system in motor neuron disease. J. Neurol. Neurosurg. Psychiat. 33, 338–357, 1970.
Byers R.K., Banker B.Q.:Infantile muscular atrophy, Arch. Neurol. 5, 140–164, 1961.
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.
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.
Gomez M.R., Clermont V., Bernstein J.:Progressive bulbar paralysis in childhood (Fazio-Londe's disease). Arch. Neurol. 6, 317–323, 1962.
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.
Namba T., Aberfeld D.C., Grob D.:Chronic proximal spinal muscular atrophy. J. Neurol. Sci. 11, 401–423, 1970.
Thieffry S., Arthuis M., Bargeton E.:Quarante cas de maladie de Werdnig-Hoffman avec onze examens anatomiques. Rev. Neurol. 93, 621–644, 1955.
Van Bogaert L.:La sclerose latérale amyotrophique et la paralyse bulbaire progressive chez l'enfant. Rev. Neurol. 1, 180–192, 1925.
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.
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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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DOI: https://doi.org/10.1007/BF02043448