Pseudo-Graefe's sign: a manifestation of aberrant regeneration of the fourth cranial nerve?

  • Massimo Martorina
  • Eliana Porté
Clinical Investigations


The phenomena involved in paradoxical upper lid retraction have been observed during recovery from paralysis of the third cranial nerve (CN). One of these phenomena is pseudo-Graefe's sign or Fuch's sign [21], which is characterized by elevation or retraction of the upper eyelid when the eye is looking downwards and inwards. This synkinesis is caused by an aberrant regeneration of newly formed axons of the third CN that subsequently reach muscles not originally connected with them. Pseudo-Graefe's sign may occur after congenital or acquired deseases. Acquired forms occur more frequently and result from paralysis of the third CN following various intracranial diseases: aneurysms [8, 9, 12, 15, 19, 29], traumas [4, 6, 7, 9, 11, 14, 16, 18, 22, 23, 29], and tumors [1, 2, 5, 17, 25, 27–29].


Public Health Cranial Nerve Intracranial Disease Aberrant Regeneration Fourth Cranial Nerve 
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  1. 1.
    Asbury AK, Aldredge H, Hershberg R, Fisher CM (1970) Oculomotor palsy in diabetes mellitus: a clinicopathological study. Brain 93:555–566Google Scholar
  2. 2.
    Bender MB (1936) The nerve supply to orbicolaris muscle and the physiology. of movements of the upper lid: with particular reference to the Pseudo-Graefe phenomenon. Arch Ophthalmol 15:21–30Google Scholar
  3. 3.
    Bender MB, Fulton JF (1939) Factors in functional recovery following section of the oculomotor nerve in monkeys. J Neurol Neurosurg Psychiatry 2:285–292Google Scholar
  4. 4.
    Bielschowsky A (1935) Lectures on motor anomalies of the eyes. II. Paralysis of individual eye muscles. Arch Ophthalmol 13:33–59Google Scholar
  5. 5.
    Boghen D, Chartrand JP, La Flamme P (1979) Primary aberrant third nerve regeneration. Ann Neurol 6:415–418Google Scholar
  6. 6.
    Bozzoni F (1960) Su di un caso di sincinesia post-traumatica oculo-palpebrale da rigenerazione anomala del III P. NN.CR Boll Ocul 39:919–927Google Scholar
  7. 7.
    Coppez H (1931) Sur le pseudo-signe de Graefe (Signe de Fuchs). Arch Ophthalmol 48:385–389Google Scholar
  8. 8.
    Cox TA, Wurster JB, Dodfrey WA (1979) Primary aberrant oculomotor regeneration due to intracranial aneurysms. Arch Neurol 36:570–571Google Scholar
  9. 9.
    Cristini G (1947) Movimenti associati palpebrali e pupillari anomali da rigenerazione errata delle fibre nervose dell 'oculomotore comune (Descrizione di 3 casi). Riv Oto-Neuro-Oftalmol 22:323–334Google Scholar
  10. 10.
    Etzine S (1966) Congenital ptosis with paradoxic eyelid movements. Am J Ophthalmol 61:793–795Google Scholar
  11. 11.
    Ferreira Filho J (1947) Pseudo-Graefe phenomenon. Arch Ophthalmol 37:308–317Google Scholar
  12. 12.
    Forster RK, Schatz NJ, Smith JL (1969) A subtle eyelid sign in aberrant regeneration of the third nerve. Am J Ophthalmol 67:696–698Google Scholar
  13. 13.
    Gifford SR (1939) Paradoxic elevation of the lid. Arch Ophthalmol 22:252–256Google Scholar
  14. 14.
    Guy J, Engel HM, Lessner AM (1989) Acquired contralateral Oculomotor synkinesi. Arch Neurol 46:1021–1023Google Scholar
  15. 15.
    Hoang-Xuan-Man (1954) Signe de Fuchs ou pseudo-phéno-mène de Graefe. Bull Soc Ophtalmol Fr 67:299–312Google Scholar
  16. 16.
    Jain NS (1957) Synkinetic lid retraction. Br J Ophthalmol 41:247–253Google Scholar
  17. 17.
    Johnson LN, Pack WL (1988) Transient oculomotor nerve misdirection in a case of pituitary tumor with hemorrhage. Arch Ophthalmol 106:584–585Google Scholar
  18. 18.
    Keane JR (1975) Bilateral aberrant regeneration of the third cranial nerve following trauma: case report. J Neurosurg 43:95–97Google Scholar
  19. 19.
    Kernes JM, Smith DR, Janotta FS, Alper MG (1979) Oculomotor nerve regeneration after aneurysm surgery. Am J Ophthalmol 87:225–233Google Scholar
  20. 20.
    Levin PM (1952) Intracranial aneurysms. Clinicopathologic consideration of oculomotor nerve rgeneration and intracerebral hemorrhage. Arch Neurol Psychiatry 67:771–786Google Scholar
  21. 21.
    Martorina M, Porte E (1986) Ptosis palpébral congénital avec syncinésic oculo-palpébrale paradoxale. J Fr Ophtalmol 9:281–284Google Scholar
  22. 22.
    Norton E, Wetzig P (1954) Aberrant regeneration of third cranial nerve. Arch Ophthalmol 51:400–402Google Scholar
  23. 23.
    Peralta S (1966) Singolari forme di sincinesia durante la risoluzione di paralisi dell'oculomotore comme. Ann Ottalmol Clin Ocul 92:229–234Google Scholar
  24. 24.
    Ramon Y Cajal S (1928) Degeneration and regeneration of the nervous system. Oxford University Press, LondonGoogle Scholar
  25. 25.
    Schatz NJ, Savino PJ, Corbett JJ (1977) Primary aberrant oculomotor regeneration: a sign of cavernous meningioma. Arch Neurol 34:29–32Google Scholar
  26. 26.
    Sibony PA, Lessel S, Gittinger JW (1984) Acquired oculomotor synkinesis. Surv Ophthalmol 28:382–390Google Scholar
  27. 27.
    Trobe JD, Glaser JS, Post JD (1978) Meningiomas and aneurysms of the cavernous sinus: Neuro-ophthalmologic features. Arch Ophthalmol 96:457–467Google Scholar
  28. 28.
    Unsöld R, Safran AB, Safran E, Hoyt WF (1980) Metastatic infiltration of nerves in the cavernous sinus. Arch Neurol 37:59–61Google Scholar
  29. 29.
    Walsh FB (1957) Third nerve regeneration. A clinical evaluation. Br J Ophthalmol 41:577–598Google Scholar

Copyright information

© Springer-Verlag 1993

Authors and Affiliations

  • Massimo Martorina
    • 1
  • Eliana Porté
    • 1
  1. 1.U.O. Oculistica - Ospedale RegionaleAostaItaly

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