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Longitudinal Evoked Potential Studies in Hereditary Ataxias

Published online by Cambridge University Press:  18 September 2015

M.J. Taylor*
Affiliation:
Division of Neurology, Departments of Paediatrics and Medicine, The Hospital for Sick Children. University of Toronto, Toronto, Canada
W.Y. Chan-Lui
Affiliation:
Division of Neurology, Departments of Paediatrics and Medicine, The Hospital for Sick Children. University of Toronto, Toronto, Canada
W.J. Logan
Affiliation:
Division of Neurology, Departments of Paediatrics and Medicine, The Hospital for Sick Children. University of Toronto, Toronto, Canada
*
Division of Neurology, Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8
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Abstract:

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We studied multimodal evoked potentials (EPs) longitudinally in a series of children with Friedreich’s ataxia and ataxia telangiectasia to determine both their diagnostic utility and their correlation with clinical regression.

The auditory brainstem responses (ABRs) were abnormal only in the children with Friedreich’s ataxia. The abnormality seen in these patients was a rostral-caudal loss of the ABR waves. The visual EPs (VEPs) were abnormal in many of the patients; those with ataxia telangiectasia had unusually low amplitude or absent VEPs, occasionally with increased latencies, whereas those with Friedreich’s ataxia had normal amplitude VEPs, often at increased latencies. The somatosensory EPs were usually of increased latency or absent in these patients. Unlike the ABR and VEPs, they did not serve to differentiate the groups.

Changes in the EPs appeared to reflect clinical deterioration; patients with little change in their EPs over several years were regressing very slowly, whereas others had rapid deterioration in both EPs and clinical status. We suggest that the EPs are diagnostically of value in degenerative ataxias and may be of value in monitoring these patients and their response to therapy.

Type
Original Articles
Copyright
Copyright © Canadian Neurological Sciences Federation 1985

References

Aguilar, MJ, Kamoshita, S, Landing, BHet al. (1968) Pathological observations in ataxia-telangiectasia. J. Exp. Neurol. 27: 659676.Google ScholarPubMed
Baloh, RW, Yee, RD, Boder, E (1978) Eye movements in ataxia telangiectasia. Neurology 28: 10991104.CrossRefGoogle ScholarPubMed
Boder, E, Sedgwick, RP (1957) A familial syndrome of progressive cerebellar ataxia oculocutaneous telangiectasia, and frequent pulmonary infection. A preliminary report on 7 children, and autopsy, and a case history. University of Southern California Medical Bulletin 9: 1522.Google Scholar
Boder, E, Sedgewick, RP (1958) Ataxia telangiectasia, afamilial syndrome of progressive cerebellar ataxia, oculocutaneous telangiectasia, and frequent pulmonary infection. Pediatrics 21: 526554.CrossRefGoogle Scholar
Bouchard, JP, Barbeau, A, Bouchard, R, Bouchard, RW (1979) Electromyography and nerve conduction studies in Friedreich’s ataxia and autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). Can. J. Neurol. Sci. 6: 185189.CrossRefGoogle ScholarPubMed
Carroll, WM, Kriss, A, Baraitser, M, Barrett, G, Halliday, AM (1980) The incidence and nature of visual pathway involvement in Friedreich’s ataxia. A clinical and visual evoked potential study of 22 patients. Brain 103: 413434.Google ScholarPubMed
Chiappa, KH, Harrison, JL, Brooks, EB, Young, RR (1980) Brainstem auditory evoked responses in 200 patients with multiple sclerosis. Ann. Neurol. 7: 135143.CrossRefGoogle ScholarPubMed
Dunn, HG (1973) Nerve conduction studies in children with Friedreich’s ataxia and ataxia-telangiectasia. Devel. Med. Child Neurol. 15: 324337.CrossRefGoogle ScholarPubMed
Dunn, HG, Meuwissen, H, Livingstone, CS, Pump, KK (1964) Ataxia telangiectasia. Canad. Med. Assoc. J. 91: 11061118.Google ScholarPubMed
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: Remond, A. (ed.), Handbook of electroencephalography and clinical neurophysiology, 9: 83118.Google Scholar
Hinton, DS, Becker, LE (1984) Personal communication regarding data in preparation for publication.Google Scholar
Hughes, JT, Brownell, B, Hewer, RL (1968) The peripheral sensory pathway in Friedreich’s ataxia. Brain 91: 803818.CrossRefGoogle ScholarPubMed
Jones, SJ, Baraitser, M, Halliday, AM (1980) Peripheral and central somatosensory nerve conduction defects in Friedreich’s ataxia. J. Neurol. Neurosurg. Psychiat. 43: 495503.CrossRefGoogle ScholarPubMed
Jason, JM, Gelfand, EW (1979) Diagnostic consideration in ataxia telangiectasia. Arch. Dis. Child. 54: 682686.Google Scholar
Kirkham, TH, Coupland, SG (1981) Abnormal pattern electroretingrams with macular cherry-red spots: evidence for selective ganglion cell damage. Current Eye Res. I: 367372.CrossRefGoogle Scholar
Maffei, L, Fiorentini, A (1981) Electroretinographic responses to alternate gratings before and after section of the optic nerve. Science 211: 953955.CrossRefGoogle Scholar
Martinez, A-Cruz, Barrio, M, Gutierrez, AM, Lopez, E (1977) Abnormalities in sensory and mixed evoked potentials in ataxia telangiectasia. J. Neurol. Neurosurg. Psychiat. 40: 4449.CrossRefGoogle ScholarPubMed
McLeod, JG (1971) An electrophysiological and pathological study of peripheral nerves in Friedreich’s ataxia. J. Neurol. Sci. 12: 333349.CrossRefGoogle ScholarPubMed
Noel, P, Desmedt, JE (1980) Cerebral and far-field somatosensory evoked potentials in neurological disorders involving the cervical spinal cord, brainstem, thalamus and cortex. Prog. Clin. Neurophysiol. 7: 205230.Google Scholar
Nuwer, MR, Perlman, SL, Packwood, JW, Kark, RAP (1983) Evoked potential abnormalities in the various inherited ataxias. Ann. Neurol. 13: 2027.CrossRefGoogle ScholarPubMed
Ochs, R, Markand, ON, DeMeyer, WE (1979) Brainstem auditory evoked responses in leukodystrophies. Neurology 29: 10891093.CrossRefGoogle ScholarPubMed
Oh, SJ, Halsey, JH (Jr.) (1973) Abnormality in nerve potentials in Friedreich’s ataxia. Neurology 23: 5254.CrossRefGoogle Scholar
Oppenheimer, DR (1979) Brain lesions in Friedreich’s ataxia. Can. J. Neurol. Sci. 6: 173176.CrossRefGoogle ScholarPubMed
Ouvrier, RA, McLeod, JG, Conchin, TE (1982) Friedreich’s ataxia. Early detection and progression of peripheral nerve abnormalities. J. Neurol. Sci. 55: 137145.CrossRefGoogle ScholarPubMed
Pedersen, L.Trojaborg, W (1981) Visual, auditory and somatosensory pathway involvement in hereditary cerebellar ataxia, Friedreich’s ataxia and familial spastic paraplegia. Electroenceph. Clin. Neurophys. 52: 283297.CrossRefGoogle ScholarPubMed
Perry, TL, Becker, LEet al. (1984) Neuropathological and neurochemical-abnormalities in ataxia telangiectasia. Neurol 34: 187191.CrossRefGoogle ScholarPubMed
Shanon, E, Himelfarb, MZ, Gold, S (1981) Auditory function in Friedreich’s ataxia. Electrophysiologic study of a family. Arch. Otolaryngol. 107: 254256.CrossRefGoogle ScholarPubMed
Solitaire, GB, Lopez, VF (1967) Louis-Bar’s syndrome (ataxia telangiectasia). Neuropathologic observations. Neurology 17: 2331.Google Scholar
Spoendlin, H (1974) Optic and cochleovestibular degenerations in hereditary ataxias. Brain 97: 4148.CrossRefGoogle ScholarPubMed
Starr, A, Hamilton, AE (1976) Correlation between confirmed sites of neurological lesions and abnormalities of far-field auditory brainstem Electroenceph Clin. Neurophys. 41: 595608.Google Scholar
Strich, SJ (1966) Pathological findings in three cases of ataxia-telangiectasia. J. Neurol. Neurosurg. Psychiat. 29: 489499.CrossRefGoogle Scholar
Taylor, MJ, Logan, WJ (1983) Multimodal electrophysiological assessment of ataxia telangiectasia. Can. J. Neurol. Sci. 10: 261265.CrossRefGoogle ScholarPubMed
Taylor, MJ, McMenamin, JB, Andermann, E, Watters, GV (1982) Electrophysiological investigation of the auditory system in Friedreich’s ataxia. Can. J. Neurol. Sci. 9: 131135.CrossRefGoogle ScholarPubMed
Young, RR, Austen, KF, Moser, HW (1964) Abnormalities of serum gamma 1A globulin and ataxia telangiectasia. Medicine 43:423433.CrossRefGoogle Scholar