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EEG and evoked potential findings in mitochondrial myopathies

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Abstract

Electroencephalograms (EEGs) and evoked potentials (EPs) were studied in 43 patients with mitochondrial myopathies. Abnormalities were found most frequently in patients who presented predominantly or exclusively with central nervous system (CNS) dysfunction (abnormal EEGs in 18 of 21 patients, abnormal EPs in 9 of 11 patients). However, of patients presenting with ocular myopathy or proximal muscle weakness who had little or no CNS involvement clinically, 8 of 22 had abnormal EEGs and 5 of 10 had abnormal EPs, suggesting that electrophysiological tests are of value in demonstrating subclinical CNS disease in mitochondrial myopathy, although the abnormalities are not specific.

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References

  1. Battaglia A (1981) Electroencephalogram, electroretinogram and visual evoked potential studies in opthalmoplegia plus. Acta Neurol Ital 3:721–727

    Google Scholar 

  2. Berkovic SF, Andermann F, Carpenter S, Wolfe LS (1986) Progressive myoclonus epilepsies: specific causes and diagnosis. N Engl J Med 315:296–305

    Google Scholar 

  3. Dawson GD (1947) Investigations on a patient subject to myoclonic seizures after sensory stimulation. J Neurol Neurosurg Psychiatry 10:141–162

    Google Scholar 

  4. DiMauro S, Bonilla E, Zeviani M, Nakagawa M, De Vivo DC (1985) Mitochondrial myopathies. Ann Neurol 17:521–538

    Google Scholar 

  5. Direke M, Campbell G (1975) Further studies on progressive external ophthalmoplegia. Acta Neurol Scand 51:245–248

    Google Scholar 

  6. Egger J, Lake BD, Wilson J (1981) Mitochondrial cytopathy: a multisystem disorder with ragged red fibres on muscle biopsy. Arch Dis Child 56:741–752

    Google Scholar 

  7. Fukuhara N, Tokiguchi S, Shirakawa K, Tsubaki T (1980) Myoclonus epilepsy associated with ragged-red fibres (mitochondrial abnormalities): disease entity or syndrome? Lightand electron-microscopic studies of two cases and review of literature. J Neurol Sci 47:117–133

    Google Scholar 

  8. Hammans SR, Sweeney MG, Brockington M, Morgan Hughes JA, Harding AE (1991) Mitochondrial encephalopathies: molecular genetic diagnosis from blood samples. Lancet 337:1311–1313

    Google Scholar 

  9. Harden A, Pamplighone G, Battaglia A (1982) Mitochondrial myopathy or mitochondrial disease? EEG, ERG, VEP studies in 13 children. J Neurol Neurosurg Psychiatry 45:627–633

    Google Scholar 

  10. Holt IJ, Harding AE, Cooper JM, et al (1989) Mitochondrial myopathies: clinical and biochemical features of 30 patients with major deletions of muscle and mitochondrial DNA. Ann Neurol 26:699–709

    Google Scholar 

  11. Jasper HH (1958) Report of the committee on methods of clinical examination in electroencephalography. Electroencephalogr Clin Neurophysiology 10:370–375

    Google Scholar 

  12. Kiruyama M, Umezaki H, Fukuda Y, Osame M, Koike K, Tateishi J, Igata A (1984) Mitochondrial encephalomyopathy with lactate-pyruvate elevation and brain infarctions. Neurology 34:72–77

    Google Scholar 

  13. Morgan-Hughes JA (1986) Mitochondrial disease. Trends Neurosci 9:15–19

    Google Scholar 

  14. Morgan-Hughes JA, Hayes DJ, Clark JB, Landon DN, Swash M, Stark RJ, Rudge P (1982) Mitochondrial encephalomyopathies: biochemical studies in two cases revealing defects in the respiratory chain. Brain 105:553–582

    Google Scholar 

  15. Obeso JA, Rothwell JC, Marsden CD (1985) The spectrum of cortical myoclonus. Brain 108:193–224

    Google Scholar 

  16. Pavlakis SG, Phillips PC, DiMauro S, De Vivo DC, Rowland LP (1984) Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes: a distinctive clinical syndrome. Ann Neurol 3:455–458

    Google Scholar 

  17. Petty RKH, Harding AE, Morgan-Hughes JA (1986) The clinical features of mitochondrial myopathy. Brain 109:915–938

    Google Scholar 

  18. Rosing HS, Hopkins LC, Wallace DC, Epstein CM, Weidenheim K (1985) Maternally inherited mitochondrial myopathy and myoclonic epilepsy. Ann Neurol 17:228–237

    Google Scholar 

  19. Rowland LP, Hays AP, DiMauro S, De Vivo DC, Behrens M (1983) Diverse clinical disorders associated with morphological abnormalities of mitochondria. In: Scarlato G, Cerri C (eds) Mitochondrial pathology in muscle disease. Piccin, Padua, pp 142–158

    Google Scholar 

  20. Shapira Y, Harel S, Russell A (1977) Mitochondrial encephalomyopathies: a group of neuromuscular disorders with defects in oxidative metabolism. Isr J Med Sci 13:161–164

    Google Scholar 

  21. Shibasaki H, Yamashita Y, Kuroiwa Y (1978) Electroencephalographic studies of myoclonus. Myoclonus-related cortical spikes and high amplitude somatosensory evoked potentials. Brain 101:447–460

    Google Scholar 

  22. Shoffner JM, Lott MT, Lezza AMS, Seibel P, Ballinger SW, Wallace DC (1990) Myoclonic epilepsy and ragged-red fibre disease (MERRF) is associated with a mitochondrial DNA tRNAlys mutation. Cell 61:931–937

    Google Scholar 

  23. So N, Berkovic S, Andermann F, Kuzniecky R, Gendron D, Quesney LF (1989) Myoclonus epilepsy and ragged red fibre (MERRF). 2. Electrophysiological studies and comparison with other progressive myoclonus epilepsies. Brain 112:1261–1270

    Google Scholar 

  24. Tartara A, Manni R, Scelsi R, Piccolo G, Cosi V (1983) Electroencephalographic findings in patients with chronic progressive external opthalmoplegia. Ital J Neurol Sci 4:459–462

    Google Scholar 

  25. Truong DD, Harding AE, Scaravilli F, Smith SJM, MorganHughes JA, Marsden CD (1990) Movement disorders in mitochondrial myopathies: a study of nine cases with two autopsy studies. Mov Disord 5:109–117

    Google Scholar 

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Smith, S.J.M., Harding, A.E. EEG and evoked potential findings in mitochondrial myopathies. J Neurol 240, 367–372 (1993). https://doi.org/10.1007/BF00839969

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  • DOI: https://doi.org/10.1007/BF00839969

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