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Somatosensory evoked potentials following median and tibial nerve stimulation in patients with Friedreich's ataxia

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Summary

The changes in evoked potentials following median and tibial nerve stimulation in nine patients with clinically defined Friedreich's ataxia are reported and discussed. The response originating in the brachial plexus (Erb's point potential) was absent or reduced in amplitude with no prolongation of peak latency, and the response generated in the cauda equina (N18) was absent in all cases. Conduction time from the brachial plexus to cervical spine and medulla oblongata was normal, whereas the central conduction time (N13a/N20, N13b/N20) was delayed. There was moderate to marked attenuation of the primary cortical response to median nerve stimulation. In one patient N20 disappeared during the course of the disease as opposed to the persisting subsequent negative wave, the latter thus simulating a very marked delay in the primary cortical response. Accordingly the cortical response to tibial nerve stimulation, which was only present in two patients and was markedly delayed, might represent a later potential with the primary response absent. The findings are consistent with neuropathological descriptions of a dying back neuropathy with primary axonal degeneration concerning the 1st order sensory neuron. In addition there is evidence either of delayed conduction in 2nd and 3rd order sensory neurones or of abnormal synaptic transmission.

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References

  1. Allison T, Hume AL (1981) A comparative analysis of short latency somatosensory evoked potentials in man, monkey, cat and rat. Exp Neurol 72:592–661

    Google Scholar 

  2. Barbeau F (1978) Friedreich's ataxia 1978 — an overview. Can J Neurol Sci 5:161

    Google Scholar 

  3. Bergamini L, Bergamasco B, Fra L, Gandiglio G, Mombelli AM, Mutani R (1966) Responses cortcales et périphéricues évoquées par stimulation du nerf dans la pathologie des cordons postérieurs. Rev Neurol (Paris) 115:99

    Google Scholar 

  4. Blackwood W, McEnemy WH, Meyer A, Norman RM, Russell DS (1963) Greenfield's neuropathology. Arnold, London, pp 585–595

    Google Scholar 

  5. Brown JR (1962) Diseases of the cerebellum. Clin Neurol 1406:55

    Google Scholar 

  6. Cavanagh JB (1964) The significance of the “dying back process” in experimental and human neurologic disease. Int Rev Exp Pathol 3:219–267

    Google Scholar 

  7. Chiappa KH, Choi SK, Young RR (1980) Short latency somatosensory evoked potentials following median nerve stimulation in patients with neurological lesions. In: Desmedt JE (ed) Clinical uses of cerebral, brainstem and spinal somatosensory evoked potentials, vol 7. Karker, Basel, pp 264–281

    Google Scholar 

  8. Delbeke J, Mc-Comas AJ, Kopex SJ (1978) Analysis of evoked lumbosacral potentials in man. J Neurol Neurosurg Psychiatr 41:293–302

    Google Scholar 

  9. Desmedt JE, Noel P (1973) Average cerebral evoked potentials in the evaluation of lesions of the sensory nerves and of the central somatosensory pathway. In: Desmedt JE (ed) New developments in electromyography and clinical neurophysiology, vol 2. Karger, Basel, pp 352–371

    Google Scholar 

  10. Dimitrijevic MR, Larsson LE, Lehmkuhl D, Sherwood A (1978) Evoked spinal cord and nerve root potentials in humans using a non-invasive recording technique. Electroencephalogr Clin Neurophysiol 45:331–340

    Google Scholar 

  11. Dyck PJ, Lambert EH, Nichols PC (1971) Quantitative measurement of sensation related to compound action potential and number and sizes of myelinated and unmyelinated fibres of sural nerve in health, Friedreich's ataxia, hereditary sensory neuropathy and tabes dorsalis. In: Cobb (ed) Handbook of electroencephalography clinical neurophysiologie, vol 9. Somatic sensation. Elsevier, Amsterdam, pp 83–118

    Google Scholar 

  12. Dyck PJ, Lais AC (1973) Evidence for segmental demyelination secondary to axonal degeneration in Friedreich's ataxia. In: Kalkulas (ed) Clinical studies in myology. Excerpta Medica, Amsterdam, pp 253–263

    Google Scholar 

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

    Google Scholar 

  14. Greenfield JG (1954) The spino-cerebellar degenerations. Blackwell, Oxford, pp 21–34

    Google Scholar 

  15. Harding AE (1981) Early onset cerebellar ataxia with retained tendon reflexes: a clinical and genetic study of a disorder distinct from Friedreich's ataxia. J Neurol Neurosurg Psychiatr 53:285–291

    Google Scholar 

  16. Hughes JT, Bronwell B, Hewer RL (1968) The peripheral sensory pathway in Friedreich's ataxia. An examination by light and electron microscopy of the posterior nerve roots, posterior root ganglia and peripheral sensory nerves in cases of Friedreich's ataxia. Brain 91:803–818

    Google Scholar 

  17. Hume AL, Cant BR (1978) Conduction time in central somatosensory pathways in man. Electroencephalogr Clin Neurophysiol 45:361–375

    Google Scholar 

  18. Jones EG (1977) Short latency potentials recorded from the neck and scalp following median nerve stimulation in man. Electroencephalogr Clin Neurophysiol 43:853–863

    Google Scholar 

  19. Jones EG, Baraitser M, Halliday AM (1980) Peripheral and central somatosensory nerve conduction defects in Friedreich's ataxia. J Neurol Neurosurg Psychiatr 43:495–503

    Google Scholar 

  20. Kinnier Wilson SA (1954) Neurology. London, Butterworth, pp 1078–1091

    Google Scholar 

  21. Lubozynski MF, Roelofs RI (1975) Friedreich's ataxia. Southern Med J 68:757

    Google Scholar 

  22. McLeod JG (1971) An electrophysiological and pathological study of peripheral nerve in Friedreich's ataxia. J Neurol Sci 12:333–349

    Google Scholar 

  23. Noel P, Desmedt JE (1976) The somatosensory pathway in Friedreich's ataxia. Acta Neurol Belg 76:271

    Google Scholar 

  24. Oh SJ, Halsey JH (1973) Abnormality in nerve potentials. Neurology (Minneap) 23:52–54

    Google Scholar 

  25. Pedersen L, Trojaborg W (1981) Visual, auditory and somatosensory pathway involvement in hereditary cerebellar ataxia, Friedreich's ataxia and familial spastic paraplegia. Electroencephalogr Clin Neurophysiol 52:283–297

    Google Scholar 

  26. Pelosi L, Fels A, Petrillo A (1984) Friedreich's ataxia: Clinical involvement and evoked potentials. J Neurol Sci 5:165–171

    Google Scholar 

  27. Peters G (1970) Klinische Neuropathologie. Spezielle Pathologie der Krankheiten des zentralen und peripheren Nervensystems. Thieme, Stuttgart

    Google Scholar 

  28. Peyronnard JM, Lapointe L, Bluchard JP, Lamontagne A, Lemieux B, Barbeau A (1976) Nerve conduction studies and electromyography in Friedreich's ataxia. Can J Neurol Sci 3:313–317

    Google Scholar 

  29. Riffel B, Stöhr M, Körner S (1984) Spinal and cortical evoked potentials following stimulation of the posterior tibial nerve in the diagnosis and localization of spinal cord diseases. Electroencephalogr Clin Neurol 58:400–407

    Google Scholar 

  30. Sauer M (1980) Investigations of peripheral and central somatosensory pathways in peroneal muscular atrophy and Friedreich's heredo-ataxia. Arch Psychiatr Nervenkr 228–242

  31. Stöhr M, Riffel B (1982) Short-latency somatosensory evoked potentials to median nerve stimulation. Components N 13-P 13, N 14-P 14, P 15, P 16 and P 18 with different recording methods. J Neurol 228:39–47

    Google Scholar 

  32. Stöhr M, Dichgans J, Diener HC, Buettner UW (1982) Evozierte Potentiale. Springer, Berlin Heidelberg New York

    Google Scholar 

  33. Taylor MJ, Chan-Liu WY, Logan WY (1985) Longitudinal evoked potential studies in hereditary ataxias. Can J Neurol Sci 12:100–105

    Google Scholar 

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Authors and Affiliations

  1. Neurologische Klinik mit klinischer Neurophysiologie, Zentralklinikum, Stenglinstrasse 1, D-8900, Augsburg, Germany

    A. Beltinger, B. Riffel & M. Stöhr

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  1. A. Beltinger
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  2. B. Riffel
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  3. M. Stöhr
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Beltinger, A., Riffel, B. & Stöhr, M. Somatosensory evoked potentials following median and tibial nerve stimulation in patients with Friedreich's ataxia. Eur Arch Psychiatr Neurol Sci 236, 358–363 (1987). https://doi.org/10.1007/BF00377425

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  • DOI: https://doi.org/10.1007/BF00377425

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