Hostname: page-component-76fb5796d-22dnz Total loading time: 0 Render date: 2024-04-26T12:55:44.123Z Has data issue: false hasContentIssue false
  • English
  • Français

Bulbo-pontine Paralysis with Deafness: the Vialetto-Van Laere Syndrome

Published online by Cambridge University Press:  18 September 2015

J.M. Abarbanel ,
P. Ashby ,
A. Marquez-Julio  and
K.R. Chapman
J.M. Abarbanel
Affiliation:
Electromyography Department, The Toronto Hospital, Toronto and the Neurology Department, Soroka Medical Center, Ben Gurion University, Faculty of Health Sciences, Beer-Sheva, Israel
P. Ashby*
Affiliation:
Electromyography Department, The Toronto Hospital, Toronto and the Neurology Department, Soroka Medical Center, Ben Gurion University, Faculty of Health Sciences, Beer-Sheva, Israel
A. Marquez-Julio
Affiliation:
Electromyography Department, The Toronto Hospital, Toronto and the Neurology Department, Soroka Medical Center, Ben Gurion University, Faculty of Health Sciences, Beer-Sheva, Israel
K.R. Chapman
Affiliation:
Electromyography Department, The Toronto Hospital, Toronto and the Neurology Department, Soroka Medical Center, Ben Gurion University, Faculty of Health Sciences, Beer-Sheva, Israel
*
EMG Department, The Toronto Hospital, 399 Bathurst Street, Toronto, Ontario, Canada M5T 2S8
Rights & Permissions [Opens in a new window]

Abstract:

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.

A Caucasian girl developed slowly progressive sensory neural deafness and bulbar and spinal muscle weakness typical of the Vialetto-Van Laere syndrome. As the condition progressed the major disabilities became dysphagia, respiratory muscle weakness and postural hypotension. Treatment with gastrostomy feedings, oxygen and fludrocortisone acetate produced worthwhile functional improvement.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 18 , Issue 3 , August 1991 , pp. 349 - 351
Copyright
Copyright © Canadian Neurological Sciences Federation 1991

References

REFERENCES

1.Brown, CH. Infantile amytrophic lateral sclerosis of the family type. J Nerv Ment Dis 1894; 21: 707–716.CrossRefGoogle Scholar
2.Vialetto, E. Contribute alia forma ereditaria della paralisi bulbare progressiva. Riv sper Freniat 1936; 40: 1–24.Google Scholar
3.Van Laere, J. Paralysie bulbo-pontine chronique progressive familiale avec surdité. Un case de syndrome de Klippel-Trenaunay dans la meme fratrie: Problèmes diagnostiques et génétiques. Rev Neurol 1966; 115:289–295.Google Scholar
4.Van Laere, J. Un nouveau cas de paralysie bulbo-pontine chronique progressive avec surdité. Rev Neurol 1977; 133: 119–124.Google Scholar
5.Arnuold, G, Tirdon, P, Laxenaire, M, et al. Paralysie bulbo-pontine chronique progressive avec surdité. À propose d’une observation de de Fazio-Lonnde. Rev Oto-neurol-ophthal 1968; 40: 158–161.Google Scholar
6.Trillet, M, Girard, PF, Schott, B, et al. La paralysie bulbo pontine progressive avec surdité. À propos d’une observation clinique. Lyon Med 1970; 223: 145–153.Google Scholar
7.Boudin, G, Pepin, B, Vernant, JC, et al. Cas familial de paralysie bulbo-pontine chronique progressive avec surdité. Rev Neurol 1971; 124:90–92.Google Scholar
8.Serratrice, G, Gastaut, JL. Amyotrophies dégénératives et lesions du neurone moteur (à propos de 32 observations). Marseille Med 1972; 109:821–840.Google Scholar
9.Lombaert, A, Dom, R, Carton, H, et al. Progressive ponto bulbar palsy with deafness. A clinico-pathological study. Acta Neurol Belg 1976;76:309–314.Google ScholarPubMed
10.Alberca, R, Montero, C, Ibanez, et al. Progressive bulbar paralysis associated with neural deafness. A nosological entity. Arch Neurol (Chic) 1980) 37: 214–216.CrossRefGoogle ScholarPubMed
11.Gallai, V, Hockaday, JM, Hughes, JT, et al. Ponto bulbar palsy with deafness (Brown-Vialetto-Van Laere syndrome). A report on three cases. J Neurol Sci 1981; 50: 259–275.CrossRefGoogle Scholar
12.Ben Hamida, M, Hentati, F. Maladie de Charcot et sclérose lateral amyotrophique juvenile. Rev Neurol 1984; 140: 202–206.Google ScholarPubMed
13.Tavares, CCA, De Mattos, JP, De Amorim, AC. Données cochléovestibulaires dans la sclérose laterale amyotrophique (forme de Van Laere). Rev Laryngol Otol Rhinol 1985; 106: 375–378.Google Scholar
14.Summers, BA, Swash, M, Schwartz, MS, et al. Juvenile onset bulbospinal muscular atrophy with deafness: Vialetto-Van Laere syndrome or Madras-type motor neuron disease? J Neurol 1987; 234: 440–442.CrossRefGoogle ScholarPubMed
15.Sayeed, ZA. Velmurugendran, CU, Arjundas, G, et al. Anterior horn cell disease seen in south India. J Neurol Sci 1975; 26: 489–498.CrossRefGoogle ScholarPubMed
16.Meenakshisundaram, E, Jaganathan, K, Ramamurthi, B. Clinical patterns of motor neurone disease seen in young age group in Madras. Neurology (India). Neurology 1970; 18: 109–112.Google Scholar
17.Medical Research Council Memorandum No. 45. Aids to the examination of the peripheral nervous system. British Stationery Office. 1976.Google Scholar
18.Johnson, EG. Mendelian and non-Mendelian inheritance. In: Rowland, LP, Woods, DS, Scon, EA, DiMauro, S. eds. Molecular genetics in diseases of brain and nerve and muscle. Oxford University Press. New York and Oxford, 1989: 24–35.Google Scholar
19.McKusick, VA. Mendelian inheritance in man. 7th Edition. Baltimore, The John Hopkins University Press, 1986.Google Scholar