Skip to main content
SpringerLink
Log in

Somatosensible Leitungsmessungen bei neurologischen Systemerkrankungen

Investigations of peripheral and central somatosensory pathways in peroneal muscular atrophy and Friedreich's heredoataxia

Neurale Muskelatrophien und spinocerebelläre Ataxien

  • Published:
Archiv für Psychiatrie und Nervenkrankheiten Aims and scope Submit manuscript

Summary

Twenty-eight patients with peroneal muscular atrophy (PMA) have been investigated. Eighteen of them were affected by the Charcot-Marie-Tooth disease and three by Dejerine-Sottas disease. In these 21 cases the nerve conduction velocity (NCV) was decreased.

Four patients presented the neuronal type of PMA, two cases showed sensory neuropathy of the neuronal type with ophthalmoplegia, and one case, PMA with ataxia, i.e., a myatrophic ataxia. In the neuronal type of PMA, including both cases with ophthalmoplegia, the amplitudes of the sensory nerve action potentials were decreased, and the NCV was normal to slightly subnormal. In myatrophic ataxia NCV was decreased. In all cases with reduced NCV, the latencies of the spinal-evoked potentials (spinEP) and somatosensory-evoked potentials (ssEP) were prolonged. In the neuronal type of PMA, these latencies were normal.

The central latencies (from thoracic and cervical level) were normal in all 28 patients with PMA of different types.

Seventeen patients with Friedreich's heredoataxia have been investigated. In all except two cases, NCV was normal. The sensory nerve-action potentials markedly decreased or disappeared in all cases. The peripheral neurographic picture, accordingly, corresponds to that of patients with the neuronal type of PMA. The latencies of spinEP were normal. All patients with Friedreich's heredoataxia, however, showed prolonged latencies of ssEP. Calculating the central latencies as the difference between ssEP and spinEP latencies (cervical and thoracic) confirms that this is due to a slowing of the conduction velocity via the spinobulbar tracts.

Zusammenfassung

28 Patienten mit neuraler Muskelatrophie (NMA) wurden untersucht. Davon hatten 18 Patienten eine vom Typ Charcot-Marie-Tooth, 3 Patienten eine vom Typ Dejerine-Sottas. Bei diesen 21 Fällen war die Nervenleitgeschwindigkeit (NLG) vermindert. Bei 4 Patienten lag die neuronale Form der NMA vor. In 2 Fällen bestand eine Neuropathie vom axonalen Typ mit Ophthalmoplegie; in einem Fall eine NMA mit Ataxie, eine sog. myatrophische Ataxie. Bei den Fällen mit neuronaler Form der NMA einschließlich der beiden Fälle mit Ophthalmoplegie waren die Amplituden der sensiblen Nervenaktionspotentiale (NAP) vermindert, die NLG normal bis leicht subnormal. Bei myatrophischer Ataxie war die NLG vermindert.

In allen Fällen mit herabgesetzter NLG fanden sich Verlängerungen der Latenzen der spinalen Evoked Potentials (spinEP) und somatosensiblen Evoked Potentials (ssEP). Bei axonaler NMA waren diese Latenzen normal. Die zentralen Leitungsverhältnisse — zentrale Latenzzeiten von thorakal und cervical nach cortical — waren in allen untersuchten 28 Fällen bei Patienten mit NMA der verschiedenen Formen normal.

17 Patienten mit Friedreichscher Erkrankung wurden untersucht. Bis auf 2 Fälle hatten sie normale NLG. Die sensiblen NAP waren in allen Fällen stark herabgesetzt oder aufgehoben. Das periphere neurographische Bild entspricht demnach dem der Patienten mit neuronaler Form der NMA. Die Latenzen der spinEP waren normal. Alle Patienten mit Friedreichscher Ataxie zeigten aber verlängerte Latenzen der ssEP. Nach Bestimmung der zentralen Latenzzeiten — als Differenz der Latenzen der ssEP und spinEP cervical und thoracal — beruht dies auf einer Herabsetzung der Erregungsleitung zentral zwischen thorakal bzw. cervical und cortical, und damit auf einer Verlangsamung der Erregungsleitung über die Tractus spino-bulbares.

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

Literatur

  • Amick, L. D., Lemmi, H.: Electromyographic studies in peroneal muscular atrophy. Arch. Neurol. (Chic.) 9, 273–284 (1963)

    Google Scholar 

  • Barbeau, A.: Friedreich's ataxia 1976, an overview. J. Canad. Sci. Neurol. 3, 389–397 (1976)

    Google Scholar 

  • Bastiaensen, L. A. K., Jaspar, H. H. J., Stadhouders, A. M., Egberink, G. J. M., Korten, J. I.: Chronic progressive external ophthalmoplegia in heredo-ataxia, neurogenic or myogenic? Acta Neurol. Scand. 56, 483–507 (1977)

    Google Scholar 

  • Bauer, R. B., Meyer, J. S., McMorrow, K. J.: Nerve conduction times in neurological diagnosis. Am. J. Phys. Med. 42, 166–174 (1963)

    Google Scholar 

  • Behse, F., Buchthal, F.: Peroneal muscular atrophy (PMA) and related disorders. II. Histological findings in sural nerves. Brain 100, 67–85 (1977)

    Google Scholar 

  • Broughton, R.: Average evoked potentials. In: Methods, results and evaluations, E. Donchin and D. B. Lindsley (eds.), pp. 79–84. Washington: Nasa Scientific Publication 1969

    Google Scholar 

  • Buchthal, F., Behse, F.: Peroneal muscular atrophy and related disorders. Part 1 (clinical manifestations as related to biopsy findings, nerve conductions and electromyography). Brain 100, 41–66 (1977)

    Google Scholar 

  • Buchthal, F., Behse, F.: Polyneuropathy. Contemporary clinical neurophysiology. Electroenceph. Clin. Neurophysiol. Suppl. 34, 373–383 (1978)

    Google Scholar 

  • Carr, R. E.: Primary retinal degenerations. In: Clinical ophthalmology, Vol. 3. New York: Harper and Row 1976

    Google Scholar 

  • Cavanagh, J. B.: The significance of the “Dying back” process in experimental and human neurological disease. Int. Rev. Exp. Pathol. 3, 219–267 (1964)

    Google Scholar 

  • Christie, B. G. B.: Electrodiagnosis features of Charcot-Marie-Tooth disease. Proc. Roy. Soc. Med. 54, 321–326 (1961)

    Google Scholar 

  • Danta, G., Hilton, R. C., Lynch, P. G.: Chronic progressive external ophthalmoplegia. Brain 98, 473 (1975)

    Google Scholar 

  • Desmedt, J. E., Noél, P., Debecker, J., Namêche, J.: Maturation of afferent conduction velocity as studied by sensory nerve potentials and by cerebral evoked potentials. In: New developments in electromyography and clinical neurophysiology, Vol. 2, pp. 52–63. Basel: Karger 1973a

    Google Scholar 

  • Desmedt, J. E., Noél, P.: Average cerebral evoked potentials in the evaluation of lesions of the sensory nerves and of central somato-sensory pathway. In: New developments in electromyography and clinical neurophysiology, Vol. 2, pp. 352–371. Basel: Karger 1973 b

    Google Scholar 

  • Desmedt, J. E., Brunko, E., Debecker, J.: Maturation of the somatosensory evoked potentials in normal infants and children with special reference to the early N1 component. Electroenceph. Clin. Neurophysiol. 40, 43–58 (1976)

    Google Scholar 

  • Drachman, D.: Ophthalmoplegia plus. The neurodegenerative disorders associated with progressive external ophthalmoplegia. Arch. Neurol. (Chic.) 18, 654–674 (1968)

    Google Scholar 

  • Dunn, H. G.: Nerve conduction studies in children with Friedreich's ataxia and ataxia teleangiectasia. Develop. Med. Child Neurol. 15, 324–337 (1973)

    Google Scholar 

  • Dyck, P. J.: Inherited neuronal degeneration and atrophy affecting peripheral motor, sensory and autonomie neurons. In: Peripheral neuropathy, Vol. 2, pp. 825–867. Philadelphia: Saunders 1975

    Google Scholar 

  • Dyck, P. J., Lais, A. C.: Evidence for segmental demyelination secondary to axonal degeneration in Friedreich's ataxia. In: Clinical studies in myology, Int. Congr. Series. Amsterdam: Excerpta medica 1973

    Google Scholar 

  • Dyck, P. J., Lambert, E. H.: Lower motor and primary sensory neuron diseases with peroneal muscular atrophy. Arch. Neurol. 18, 603–618 (1968a)

    Google Scholar 

  • Dyck, P. J., Lambert, E. H.: Lower motor and primary sensory neuron diseases with peroneal muscular atrophy. II. Neurologic, genetic and electrophysiologic findings in various neuronal degenerations. Arch. Neurol. 18, 619–625 (1968b)

    Google Scholar 

  • Dyck, P. J., Lambert, E. H., Mulder, D. W.: Charcot-Marie-Tooth disease: nerve conduction and clinical studies of a large kinship. Neurology (Minneap.) 13, 1 (1963)

    Google Scholar 

  • Dyck, P. J., Lambert, E. H., Nicholls, P. C.: Quantitative, measurement of sensation related to compound action potential and number and sizes of myelinated and unmyelinated fibers of sural nerve in health, Friedreich's ataxia, hereditary sensory neuropathy and tabes dorsalis. In: Handbook of electroencephalography and clinical neurophysiology, Vol. 9, pp. 83–118. Amsterdam: Elsevier 1971

    Google Scholar 

  • Dyck, P. J., Lambert, E. H., Sanders, K., O'Brien, P. C.: Severe hypomyelination and marked abnormality of conduction in Dejerine-Sottas hypertrophie neuropathy: myelin thickness and compound action potential of sural nerve in vitro. Mayo Clin. Proc. 46, 432 (1971b)

    Google Scholar 

  • Dyck, P. J., Ohta, M.: Neuronal atrophy and degeneration predominantly affecting peripheral sensory neurons. In: Peripheral neuropathy, Vol. 2, pp. 791–824. Philadelphia: Saunders 1975

    Google Scholar 

  • Geoffroy, G., Breton, G., Barbeau, A., Lemieux, B., Aube, M., Leger, C., Bouchard, J. P.: Clinical description and roentgenologic evaluation of patients with Friedreich's ataxia. J. Canad. Sci. Neurol. 3, 279–286 (1976)

    Google Scholar 

  • Gilliatt, R. W.: Peripheral nerve conduction in neurological patients. J. Neurol. Neurosurg. Psychiat. 22, 344 (1959)

    Google Scholar 

  • Gilliatt, R. W., Thomas, P. K.: Extreme slowing of nerve conduction in peroneal muscular atrophy. Ann. Phys. Med. 4, 104 (1957)

    Google Scholar 

  • Gilliatt, R. W., Thomas, P. K.: Changes in nerve conduction with ulnar lesions at the elbow. J. Neurol. Neurosurg. Psychiat. 23, 312 (1960)

    Google Scholar 

  • Glaser, J. S.: Neuro-ophthalmology. New York: Harper and Row 1978

    Google Scholar 

  • Goff, G. D., Matsumiya, J., Allison, T., Goff, W. R.: The scalp topography of human somatosensory and auditory evoked potential. Electroenceph. Clin. Neurophysiol. 42, 57–76 (1977)

    Google Scholar 

  • Hopf, H. C., Port, F. K.: Friedreichsche Ataxie mit Beteiligung der peripheren Nerven nach Art der neuralen Muskelatrophie. Deutsch. Z. Nervenheilk. 194, 1–16 (1968)

    Google Scholar 

  • Hopf, H. C., Volles, E.: Spinocerebellar ataxia with neural myatrophy. Neuropädiatrie 3, 97 (1971)

    Google Scholar 

  • Hughes, J. T., Brownell, B., Hewer, R. L.: The peripheral sensory pathway in Friedreich's ataxia. Brain 91, 803–818 (1968)

    Google Scholar 

  • Hume, A. L., Cant, B. R.: Conduction time in central somatosensory pathways in man. Electroenceph. Clin. Neurophysiol. 45, 361–375 (1978)

    Google Scholar 

  • Isch, F., Isch-Treussard, C., Jesel, M.: La mesure de la vitesse de conduction des fibres nerveuses motrices dans les atrophies neurogènes chroniques et progressives. Rev. Neurol. 115, 122–129 (1966)

    Google Scholar 

  • Jampel, R. S., Okazaki, H., Bernstein, J.: Ophthalmoplegia and retinal degeneration associated with spino-cerebellar ataxia. Arch. Ophthal. 61, 247 (1961)

    Google Scholar 

  • Jones, S. J.: Short latency potentials recorded from the neck and scalp following median nerve stimulation in man. Electroenceph. Clin. Neurophysiol. 43, 853–863 (1977)

    Google Scholar 

  • Jones, S. J., Small, D. G.: Spinal and sub-cortical evoked potentials following stimulation of the posterior tibial nerve in man. Electroenceph. Clin. Neurophysiol. 44, 299–306 (1978)

    Google Scholar 

  • Kaeser, H. E.: Veränderungen der Leitgeschwindigkeit bei Neuropathien und Neuritiden. Fortschr. Neurol. Psychiat. 33, 221–250 (1965)

    Google Scholar 

  • Kaeser, H. E.: Evoked muscle and evoked sensory nerve action potentials. In: Handbook of electroencephalography and clinical neurophysiology, Vol. 16A, pp. 22–32. Amsterdam: Elsevier 1975a

    Google Scholar 

  • Kaeser, H. E.: Evoked muscle and sensory nerve action potentials in polyneuropathies. In: Handbook of electroencephalography and clinical neurophysiology, Vol. 16A, pp. 36–43. Amsterdam: Elsevier 1975b

    Google Scholar 

  • Kearns, T. P., Sayre, G. P.: Retinitis pigmentosa, external ophthalmoplegia and complete heart block. Arch. Ophthal. 60, 280–289 (1958)

    Google Scholar 

  • Kerschensteiner, M., Schenck, E.: Untersuchung der Nervenleitung bei neuraler Muskelatrophie und Friedreichscher Heredoataxie. Dtsch. Z. Nervenheilk. 195, 166–186 (1969)

    Google Scholar 

  • Lambert, E. H., Bastron, J. A., Mulder, J. W.: Conduction velocity of motor fibers of peripheral nerves in peroneal muscular atrophy. Annual meeting of the American Academy of Neurology, Philadelphia, April 1958

  • Lambert, E. H., Dyck, P. J.: Compound actions potentials of human sural nerve biopsies. Bull. Am. Ass. Electromyogr. Electrodiagn. 14, 23 (1967)

    Google Scholar 

  • Lambrior, R. A.: Un cas de maladie de Friedreich avec autopsie. Rev. Neurol. 21, 525–540 (1911)

    Google Scholar 

  • McLeod, J. G.: An electrophysiological and pathological study of peripheral nerves in Friedreich's ataxia. J. Neurol. Sci. 12, 333–349 (1971)

    Google Scholar 

  • Mott, F. W.: Case of Friedreich's disease, with autopsy and systematic microscopical examination of the nervous system. Arch. Neurol. 3, 180–200 (1907)

    Google Scholar 

  • Myrianthopoulos, N. C., Lane, H. M., Silberberg, D. H., Vincent, B. L.: Nerve conduction and other studies in families with Charcot-Marie-Tooth disease. Brain 87, 589–608 (1964)

    Google Scholar 

  • Myrianthopoulos, N. C., Smith, J. K.: Amyotrophic lateral sclerosis with progressive dementia. Neurology (Minneap.) 12, 603–610 (1962)

    Google Scholar 

  • Namerow, N. S.: Somatosensory evoked responses in multiple sclerosis. Bull. Los Angeles Neurol. Soc. 33, 74–81 (1968)

    Google Scholar 

  • Oh, S. Y., Halsley, J. M.: Abnormality in nerve potentials in Friedreich's ataxia. Neurology 23, 52–54 (1973)

    Google Scholar 

  • Peyronnard, J. M., Lapointe, L., Bouchard, J. P., Lamontagne, A., Lemieux, B., Barbeau, A.: Nerve conductions studies and electromyography in Friedreich's ataxia. J. Canad. Sci. Neurol. 3, 313–317 (1976)

    Google Scholar 

  • Preswick, G.: The peripheral neuropathy of Friedreich's ataxia. Intern. Meeting EMG, Glasgow (1967). Bull. Amer. Ass. Electromyogr. Electrodiagn. 14, 23 (1967)

    Google Scholar 

  • Richter, H.: Somatosensible kortikale Antworten auf elektrische Reizung peripherer Nerven bei gesunden Versuchspersonen und bei neurologischen Patienten. Dissertation, Freiburg i. Br. 1971

  • Salisachs, P., Codina, M., Pradas, J.: Motor conduction velocity in patient with Friedreich's ataxia. J. Neurol. Sci. 24, 331–337 (1975)

    Google Scholar 

  • Sauer, M., Schenck, E.: Vergleichende Untersuchung somatosensibler spinaler und kortikaler Evoked Potentials bei Kindern. Arch. Psychiat. Nervenkr. 223, 295–308 (1977a)

    Google Scholar 

  • Sauer, M., Schenck, E., Richter, H.: Electrophysiologic investigations in Friedreich's heredoataxia and in hereditary motor and sensory neuropathy. Electroenceph. clin. Neurophysiol. 43, 623 (1977b)

    Google Scholar 

  • Spiller, W. G.: Friedreich's ataxia. J. Nerv. Ment. Dis. 37, 411–435 (1910)

    Google Scholar 

  • Stephens, J., Hoover, M. L., Denst, J.: On familial ataxia, neural amyotrophy, and their association with progressive external ophthalmoplegia. Brain 81, 556 (1958)

    Google Scholar 

  • Thomas, P. K.: Symptomatology and differential diagnosis of peripheral neuropathy. In: Peripheral neuropathy, Vol. I, pp. 495–512. Philadelphia: Saunders 1975

    Google Scholar 

  • Werner, W.: Zur Frage der myatrophischen Ataxie. Nervenarzt 41, 350 (1970)

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Kinderklinik, Psychiatrische Klinik, Universität Freiburg, Freiburg i. Br., Bundesrepublik Deutschland

    Manfred Sauer

  2. EMG-Labor, Psychiatrische Klinik, Universität Freiburg, Freiburg i. Br., Bundesrepublik Deutschland

    Manfred Sauer

Authors
  1. Manfred Sauer
    View author publications

    You can also search for this author in PubMed Google Scholar

Additional information

Mit Unterstützung durch die Deutsche Forschungsgemeinschaft über Sonderforschungsbereich Hirnforschung und Sinnesphysiologie (SFB 70)

Rights and permissions

Reprints and permissions

About this article

Cite this article

Sauer, M. Somatosensible Leitungsmessungen bei neurologischen Systemerkrankungen. Arch. Psychiat. Nervenkr. 228, 223–242 (1980). https://doi.org/10.1007/BF00342348

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00342348

Key words

Schlüsselwörter

Search

Navigation