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Early myoclonic epileptic encephalopathy (E.M.E.E.)

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Abstract

The authors describe the electroclinical aspects and evolution of nine cases of myoclonic epileptic encephalopathy which began between two days and ten weeks of life. at onset it is associated with: myoclonic jerks, partial fits and periodic paroxysmal EEG abnormalities. Repeated spasms coexisting with partial fits and ‘suppression-bursts’ (both appearing later) complete the electroclinical picture. The neurological status (initially normal) progressively deteriorates leading within a few months to a decerebrate posture with opisthotonos. In spite of thorough neuroradiological, biochemical, cytological, metabolic, and ultrastructural investigations, the etiology remained unknown. However, the electroclinical and evolutive patterns are similar to those of some metabolic diseases (Polyodystrophy, Non-Ketotic Hyperglycinemia, etc.). All these observations display a homogeneous electroclinical pattern for which the authors propose the name of Early Myoclonic Epileptic Encephalopathy. This type deserves to be classified as a particular electroclinical entity among the epileptic encephalopathies of the first year of life; since its course is regularly downhill in all cases there may be a familial recurence due to the possibility of a metabolic etiology.

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References

  1. 1.

    Aicardi J, Chevrie JJ, Rousselie F (1969) Le syndrome spasmes en flexion, agénesie calleuse, anomalies choriorétiniennes. Arch Franç Pédiat 26:1103–1120

  2. 2.

    Aicardi J, Chevrie JJ (1978) Les spasmes infantiles. Arch Franç Pédiat 35:1015–1023

  3. 3.

    Aicardi J, Goutierres F (1978) Encéphalopatie myoclonique néonatale. Rev EEG Neurophysiol 8:99–101

  4. 4.

    Cavazzuti GB, Nalin A, Ferrari F, Grandori L, Beghini G (1978) Encefalopatia epilettica ad insorgenza neonatale. Clin Ped 5:239–246

  5. 5.

    Chevrie JJ, Aicardi J (1977) Convulsive disorders in the first year of life. Etiologic factors. Epilepsia 18:489–498

  6. 6.

    Chevrie JJ, Aicardi J (1978) Convulsive disorders in the first year of life. Neurological and mental outcome and mortality. Epilepsia 19:67–74

  7. 7.

    Curatolo P, Allemand F, Brinciotti M, Matricardi M (1980) Le encefalopatie miocloniche ad esordio neonatale in “Neurologia Neonatale”. Edited by Ottaviano E and Allemand F, Verducci ed. Rome: 251–256

  8. 8.

    Dalla Bernardina B, Beghini G, Padovani E, Gaburro D (1977) NBon-ketotic hyperglycinemia. Electroenceph and Clin Neurophysiol 42:432

  9. 9.

    Dalla Bernardina B, Plouin P, Lerique A, Beghini G, Trevisan C (1978) Aspects electrocliniques des crises survenant dans les deus Progressi in epilettologia. Lega Italiana Contro l'Epilessia, nn 22–23:137–144

  10. 10.

    Dalla Bernardina B, Aicardi J, Goutierres F, Plouin P (1979) Glycine encephalopathy. Neuropädiatrie 10:209–225

  11. 11.

    Dalla Bernardina B, Dulac O, Bureau M, Dravet C, Del Zotti F, Roger J (1982) Encéphalopathie myoclonique précoce avec épilepsie. Rev EEG Neurophysiol 12:8–14

  12. 12.

    Gaetti MT, Varani R, Bini PL (1977) Un caso di ipsaritmia. Considerazioni etio-patogenetiche. Rlunione di aggiornamento di neuropediatria, Ancona

  13. 13.

    Grandgeorge D (1979) L'acidémie D glycerique. Anomalie héréditaire du métabolisme de la glycine. Thése Grenoble

  14. 14.

    Jeavons PM, Bower BD, Dimitrakoudi M (1973) Long-term prognosis of 150 cases of West Syndrome. Epilepsia 14:153–164

  15. 15.

    Lacy JR, Penry JK (1976) Infantile spasms. Raven Press, New York

  16. 16.

    Maheswari MC, Jeavons PM (1976) The prognostic implications of suppression-bursts activity in the EEG in infancy. Epilepsia 16:127–131

  17. 17.

    Mises G, Moussalli-Salefranque F, Plouin P, Teman G, Saudubray JM (1978) L'EEG dans les hyperglycinémies sans cétose. Rev EEG Neurophysiol 7:102–106

  18. 18.

    Ohtahara S, Ishida T, Oka E, Yamatoga Y, Inique H, Ohtsuka Y, Kanda S (1976) On the age-dependent epileptic syndrome: the early-infantile encephalopathy with suppression burs. Brain and Development 8:270–288

  19. 19.

    Ohtahara S (1978) Clinico-electrical delineation of epileptic encephalopathies in childhood. Asian Med J 21:7–17

  20. 20.

    Pampiglione G, Harden A (1968) Resuscitation after cardiocirculatory arrest. Prognostic evaluation of early electroencephalographic findings. Lancet 1:1261–1264

  21. 21.

    Piattella L, Cardinali C, Zamponi N, Cenci L, Tavoni MA, Porfiri L, Emanuelli M, Tucci E (1981) Su di un caso di encefalopatia de glicina. Cavazzutti GB (ed) Atti 3o. Convegno di Neurologia Neonatale, pp 149–154

  22. 22.

    Turcker JS, Solitare GB (1963) Infantile myoclonic spasms. Clinical, electrographic and neuropathologic observations. Epilepsia 4:45–59

  23. 23.

    Vigevano F, Cincinnati P, Bosman C, Maccagnani F, Sabetta G (1983) L'encefalopatia mioclonica precoce. Riv Ital Neurobiol (in press)

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Correspondence to B. Dalla Bernardina.

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Bernardina, B.D., Dulac, O., Fejerman, N. et al. Early myoclonic epileptic encephalopathy (E.M.E.E.). Eur J Pediatr 140, 248–252 (1983). https://doi.org/10.1007/BF00443371

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Key words

  • Epileptic encephalopathy
  • Myoclonias
  • Partial fits
  • Tonic spasms
  • Suppression-bursts
  • Metabolic disorders