Skip to main content
SpringerLink
Log in

Multimodality evoked potentials in myotonic dystrophy

  • Original Articles
  • Published:
The Italian Journal of Neurological Sciences Aims and scope Submit manuscript

Abstract

Multimodality evoked potentials were performed in 18 patients affected by myotonic dystrophy (8 males and 10 females); the aim was to make an electrophysiological evaluation of the central nervous system involvement in this disease.

We observed brainstem, somatosensory and visual evoked potential abnormalities respectively in 53%, 62.5% and 71.4% of cases, with no apparent relationship to the severity of the disease.

Our abnormal findings provide further confirmation of CNS involvement in myotonic dystrophy and occur in various combinations suggesting an aspecific involvement at different levels, reflecting the multisystemic character of this disease.

Sommario

I potenziali evocati multimodali sono stati effettuati in 18 pazienti (8 maschi e 10 femmine), affetti da distrofia miotonica. Lo scopo era di effettuare una valutazione elettrofisiologica dell'interessamento del sistema nervoso centrale in questa malattia.

I potenziali evocati del tronco, somatosensitivi e visivi sono risultati abnormi rispettivamente nel 53%, 62.5% e 71.4% dei casi, senza apparente relazione con la gravità della malattia.

I risultati da noi ottenuti forniscono un'ulteriore conferma dell'interessamento del SNC nella distrofia miotonica che è possibile in varie combinazioni, suggerendo un aspecifico interessamento a diversi livelli, che riflette il carattere multisistemico della malattia.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Amantini A., De Scisciolo G., Scaioli V. et al:Distrofia miotonica: reperti oftalmologici ed elettrofisiologici (EOG, ERG, P-ERG, PEV). Comunic. XXIV Congr. Naz. Soc. Ital. Neurol., Pisa, 1985.

  2. Ambrosini P.J., Nurnberg H.G.:Psycopathology: a primary feature of myotonyc dystrophy. Psychosomatics, 20:393–399, 1979.

    CAS  PubMed  Google Scholar 

  3. Bartel P.R., Lotz B.P., Van der Meyden C.H.:Short-latency somato sensory evoked potentials in dystrophia myotonica. J. Neurol. Neurosurg. Psychiatry, 47:524–529, 1984.

    CAS  PubMed  Google Scholar 

  4. Barwick D.D., Osselton Y.M., Walton J.N.:EEG studies in hereditary myopathy. J. Neurol. Neurosurg. Psychiatry, 28:109–114, 1965.

    CAS  PubMed  Google Scholar 

  5. Beijersbergen R.S.H.M., Kemp A., Storm van Lecuwen W.:EEG observations in dystrophia myotonica (Curschmann-Steinert). Electroencephal. Clin. Neurophysiol., 49:143–151, 1980.

    CAS  Google Scholar 

  6. Black W.W., Ravin A.:Studies in dystrophia myotonica. VII. Autopsy observations in five cases. Arch. Pathol., 44:176–191, 1947.

    Google Scholar 

  7. Coccagna, G., Mantovani M., Parchi C., Mironi F., Lugaresi E.:Alveolar hypoventilation and hypersomnia in myotonic dystrophy. J. Neurol. Neurosurg. Psychiatry, 38:977–984, 1975.

    CAS  PubMed  Google Scholar 

  8. Culebras A., Feldman R.G., Merk F.B.:Cytoplasmic inclusion bodies within neurons of the thalamus in myotonic dystrophy. J. Neurol. Sci., 19: 319–329, 1973.

    Article  CAS  PubMed  Google Scholar 

  9. Domenici L., Rossi B., Siciliano G., Busso E., Murri L.:Brainstem acoustic evoked potentials in patients with myotonic dystrophy. Elsevier Science Publishers B.V., 169–173, 1985.

  10. Gott P.S., Karnaze D.S., Keane J.R.:Abnormal visual evoked potentials in myotonic dystrophy. Neurology, 33:1622–1625, 1983.

    CAS  PubMed  Google Scholar 

  11. Gott P.S., Karnaze D.S.:Short-latency somatosensory evoked potentials in myotonic dystrophy: evidence for a conduction disturbance. Electroencephal. Clin. Neurophysiol., 62:455–458, 1985.

    CAS  Google Scholar 

  12. Hamsotia P., Frens D.:Hypersomnia associated with alveolar hypoventilation in myotonic dystrophy. Neurology, 31:1336–1337, 1981.

    Google Scholar 

  13. Lugaresi F.:Sul probabile significato di alcuni aspetti EEG presenti in malattie degenerative del sistema nervoso. Riv. Neurol., 25:659–663, 1955.

    CAS  PubMed  Google Scholar 

  14. Lundervold A., Refsum S., Jacobsen W.:The EEG in dystrophya myotonica. Europ. Neurol., 2: 279–284, 1969.

    CAS  PubMed  Google Scholar 

  15. Maas O., Paterson A.D.:Mental changes in familis affected by dystrophya myotonica. Lancet, 21–23, 1937.

  16. Mongia S.K., Lundervold A.:Electrophysiological abnormalities in cases of dystrophya myotonica. Europ. Neurol., 13:360–376, 1975.

    CAS  PubMed  Google Scholar 

  17. Muratorio A.:Reperti EEG nelle Distrofie Muscolari Progressive. Sistema Nervoso, 12–32, 1960.

  18. Refsum S., Engeset A., Lonnum A.:Pneumoencephalgraphic changes in dystrophia myotonica. Acta Psychiat. Neurol. Scand., Suppl., 137:34–98, 1959.

    Google Scholar 

  19. Refsum S., Lonnum A., Sjaastad O., Engeset A.:Dystrophya myotonica. Repeated penumoncephalographic studies in ten patients. Neurology, 17: 345–348, 1967.

    CAS  PubMed  Google Scholar 

  20. Rosman N.P., Kakulas B.A.:Mental deficiency associated with muscolar dystrophy. A neuropathological study. Brain, 89:769–787, 1966.

    CAS  PubMed  Google Scholar 

  21. Rossi B., Sartucci F., Stefanini A., Paoletti P., Perissinotto A., Murri L.:Sonno e funzionalità respiratoria nella distrofia miotonica. Riv. Ital. EEG Neurof. Clin., 3:147–152, 1980.

    Google Scholar 

  22. Savoldi F.:Steinert's disease. Electroencephal. Clin. Neurophysiol., 2:386, 1959.

    Google Scholar 

  23. Savoldi F., Martelli M., Maurelli M., Sinforiani E., Nappi G. In: Nappi G., Arrigo A., Savoldi F. (Eds.).Distrofia Miotonica EMI, Pavia, 176–183, 1981.

    Google Scholar 

  24. Steinert H.:Myopathologische Beiträge. Über das klinische und anatomische Bild des Musckelschwunds der Myotoniker. Dtsch. Z. Nervenheilk. 37: 58–104, 1909.

    Article  Google Scholar 

  25. Streib E.W., Sun S.F.:Somatosensory evoked responses (SSER) in myotonic muscular dystrophy (MYD). Electroencephal. Clin. Neurophysiol., 56: 178, 1983.

    Google Scholar 

  26. Thomasen E.:Thomsen's disease, paramyotonia and dystrophya myotonica. A clinical and heredobiological investigation. Myotonia. Munksgaard, Copenhagen 1948.

  27. Thompson D.S., Woodword J.B., Ringel P.S., Nelson L.M.:Evoked potential abnormalities in myotonic dystrophy. Electroencephal. Clin. Neurofisiol., 56:453–456, 1983.

    CAS  Google Scholar 

  28. Trelles J.O., Gutierrez C., Aranibar A.Y., Palomino L.:Estudio anatomoclinico de la enfermedad de Steinert. Revue de Neuro-Psiquiatria, 99: 139–204, 1956.

    Google Scholar 

  29. Walton J.N., Irving D., Tomlison B.E.:Spinal cord limb motor neurons in dystrophia myotonica. J. Neurol. Sci., 34:199–211, 1978.

    Google Scholar 

  30. Wisniewski H.M., Berry K., Spiro A.J.:Ultrastructure of thalamic neuronal inclusion in myotonic dystrophy. J. Neurol. Sci., 24:321–329, 1975.

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Istituto di Clinica Neurologica, Università di Pisa, Via Roma, 57, 56100, Pisa, Italy

    Sartucci F., Marconi F., Busso E., Rossi B. & Murri L.

Authors
  1. Sartucci F.
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Marconi F.
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Busso E.
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Rossi B.
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Murri L.
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Sartucci, F., Marconi, F., Busso, E. et al. Multimodality evoked potentials in myotonic dystrophy. Ital J Neuro Sci 10, 61–67 (1989). https://doi.org/10.1007/BF02333873

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02333873

Key-Words

Search

Navigation