Skip to main content

Advertisement

SpringerLink
Log in

Late occurrence of additional ocular and intracranial pathologies in the linear naevus sebaceous (Feuerstein-Mims) syndrome

  • Special Cases
  • Published:
Acta Neurochirurgica Aims and scope Submit manuscript

Summary

The linear naevus sebaceous syndrome of Feuerstein-Mims belongs to the phacomatoses and classically is composed of a linear cutaneous lesion associated with mental retardation and epilepsy, sometimes also with ocular dystrophies.

During an unusually long follow-up of 32 years of such a case, which is reported here, the late occurrence of additional ocular and intracranial pathology are demonstrated: conjunctival and bulbar tumours with progressive deterioration of visual acuity, bilateral intracranial arachnoidal cysts, dilatation and torstuosity of the middle cerebral artery.

As a consequence of these observations the prognosis in cases with this syndrome has to be given with special caution.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Alexander GL, Norman RM (1960) The Sturge-Weber syndrome. J Wright & Sons LTD, Bristol

    Google Scholar 

  2. Baker BB, Imber RJ, Templer JW (1975) Nevus sebaceous of Jadassohn. Arch Otolaryngol 101: 515–516

    PubMed  Google Scholar 

  3. Baker RS, Ross PA, Baumann RJ (1987) Neurologic complications of the epidermal nevus syndrome. Arch Neurol 44: 227–232

    PubMed  Google Scholar 

  4. Barth RG, Valk J, Kalsbeek GL, Blom A (1977) Organoid nevus syndrome (linear nevus of Jadassohn): clinical and radiological study of a case. Neuropädiatrie 8: 418–428

    Google Scholar 

  5. Besser FS (1976) Linear sebaceous naevi with convulsions and mental retardation (Feuerstein-Mims' Syndrome), vitamin-D-resistant Rickets. Proc Roy Soc Med 69: 518–520

    PubMed  Google Scholar 

  6. Bianchine JW (1970) The nevus sebaceous of Jadassohn. A neurocutaneous syndrome and a potentially premalignant lesion. Am J Dis Child 120: 223–228

    PubMed  Google Scholar 

  7. Brihaye-van Geertruyden M (1963) Contribution à l'étude expérimentale de l'origine du mélanoblaste choroïdien chez le Mammifère. Arch Biol 74: 1–44

    Google Scholar 

  8. Brini A (1950) L'origine du mélanoblaste choroïdien. Bull Soc Ophtal (Paris) 63: 260–264

    Google Scholar 

  9. Chalhub EG, Volpe JJ, Gado MH (1975) Linear nevus sebaceous syndrome associated with porencephaly and nonfunctioning major cerebral venous sinuses. Neurology 25: 857–860

    PubMed  Google Scholar 

  10. Choi BH, Kudo M (1981) Abnormal neuronal migration and gliomatosis cerebri in epidermal nevus syndrome. Acta Neuropathol (Berlin) 53: 319–325

    Google Scholar 

  11. Chao Hsi-Chih D (1959) Congenital neurocutaneous syndromes in childhood II. Tuberous sclerosis. J Pediatr 18: 447–459

    Google Scholar 

  12. Corner AE, Bryan H (1967) Nevus sebaceous of Jadassohn. Am J Dis Child 114: 626–630

    PubMed  Google Scholar 

  13. Enjolras O, Guerin D, Hewitt J (1979) Contribution à la connaissance du syndrome du naevus epidermique de Solomon. Ann Dermatol Veneorol (Paris) 106: 673–680

    Google Scholar 

  14. Feuerstein RC, Mims LC (1962) Linear nevus sebaceous with convulsions and mental retardation. Am J Dis Child 104: 675–679

    PubMed  Google Scholar 

  15. Gellis SS, Feinhold M (1970) Picture of the month. Linear nevus sebaceous syndrome. Am J Dis Child 120: 139–140

    PubMed  Google Scholar 

  16. Gördüren S (1962) Aberrant lacrimal gland associated with other congenital abnormalities. Br J Ophthalmol 46: 277–280

    Google Scholar 

  17. Haim S, Mayer K, Klaus M (1969) A Segmental neurocutaneous manifestation. Report of a case of nevus unius lateralis and unilateral hypotrophy. Europ Neurol 2: 59–64

    Google Scholar 

  18. Herbst BA, Cohen ME (1971) Linea nevus sebaceous. A neurocutaneous syndrome associated with infantile spasms. Arch Neurol 24: 317–322

    PubMed  Google Scholar 

  19. Hermans EH (1963) A fifth phacomatosis, naevus epitheliomatodes multiplex. Dermatologica 127: 216–218

    PubMed  Google Scholar 

  20. Holden KR, Dekaban AS (1972) Neurological involvement in nevus unis lateris and nevus linearis sebaceus. Neurology 22: 879–887

    PubMed  Google Scholar 

  21. Hornstein OP, Knickenberg M (1974) Zur Kenntnis des Schimmelpenning-Feuerstein-Mims-Syndroms (Organoïde-Naevus-Phakomatose). Arch Derm Forsch 250: 33–50

    Google Scholar 

  22. Jancar J (1970) Naevus syringocystadenomatosus papilliferus with skull and brain lesions, hemiparesis, epilepsy and mental retardation. Br J Derm 82: 402–405

    Google Scholar 

  23. Jones EW, Heyl T (1970) Naevus sebaceous. A report of 140 cases with special regard to the development of secondary malignant tumours. Br J Derm 82: 99–117

    Google Scholar 

  24. Kurokawa T, Sasaki K, Hanai T, Goya N, Komaki S (1981) Linear nevus sebaceus syndrome. Report of a case with Lennox-Gastaut syndrome following infantile spasms. Arch Neurol 38: 375–377

    PubMed  Google Scholar 

  25. Lall K (1962) Teratoma of conjunctiva (associated with nevus systematicus and epilepsis symptomatica). Acta Ophthalmol 40: 555–558

    Google Scholar 

  26. Lambert HM, Sipperley JO, Shore JW, Dieckert JP, Evans R, Lowd DK (1987) Linear nevus sebaceous syndrome. Ophthalmology 94: 278–282

    PubMed  Google Scholar 

  27. Lansky LL, Funderburk S, Cuppage FE, Schimke RN, Diehl AM (1972) Linear sebaceous nevus syndrome. A hamartoma variant. Am J Dis Child 123: 587–590

    PubMed  Google Scholar 

  28. Lansky LL (1972) Correspondence. Reply by the authors, concerning linear sebaceous nevus syndrome. Am J Dis Child 124: 944

    Google Scholar 

  29. Lantis S, Leyden J, Thew M, Heaton C (1968) Nevus sebaceus of Jadassohn. Part of a new neurocutaneous syndrome? Arch Derm 98: 117–123

    PubMed  Google Scholar 

  30. Larregue M, Coscas G, Masclef P, Solignac J (1974) Le syndrome du naevus épidermique de Solomon. Ann Derm Syph (Paris) 101: 45–55

    Google Scholar 

  31. Lentz CL, Altman J, Mopper C (1968) Nevus sebaceous of Jadassohn. Arch Derm 97: 294–296

    PubMed  Google Scholar 

  32. Louis-Bar D (1950) Les rapports entre les angiomatoses du type Sturge-Weber et les autres dysplasies (formes de passage). Acta Neurol Psych Belg 50: 680–710

    Google Scholar 

  33. Lovejoy FH, Boyle WE (1973) Linear nevus sebaceous syndrome: report of two cases and a review of the literature. Pediatric 52: 382–387

    Google Scholar 

  34. Maloney ER (1934) Nevus sebaceus linearis. Arch Derm Syph 30: 167–168

    Google Scholar 

  35. Marchau MMB (1984) Linear cutaneous lesions and neurological deficit. A case report. Clin Neurol Neurosurg 86: 111–113

    PubMed  Google Scholar 

  36. McAuley DL, Isenberg DA, Gooddy W (1978) Neurological involvement in the epidermal naevus syndrome. J Neurol Neurosurg Psychiat 41: 466–469

    PubMed  Google Scholar 

  37. Marden PM, Venters HD (1966) A new neurocutaneous syndrome. Am J Dis Child 112: 78–81

    Google Scholar 

  38. Mehregan AH, Pinkus H (1965) Life history of organoid nevi. Special reference to nevus sebaceous of Jadassohn. Arch Derm 91: 574–588

    PubMed  Google Scholar 

  39. Meyerson LB (1967) Nevus unius lateralis, brain tumor, and diencephalic syndrome in a newborn. Arch Derm 95: 501–504

    PubMed  Google Scholar 

  40. Mollica F, Pavone L, Nuciforo G (1974) Linear Sebaceous nevus syndrome in a newborn. Am J Dis Child 128: 868–871

    PubMed  Google Scholar 

  41. Moynahan EJ, Wolff OH (1967) A new neuro-cutaneous syndrome (skin, eye and brain) consisting of linear naevus, bilateral lipodermoid of the conjunctivae, cranial thickening, cerebral cortical atrophy and mental retardation. Br J Dermat 79: 651–652

    Google Scholar 

  42. Shapiro K, Shulman K (1976) Facial nevi associated with anomalous venous return and hydrocephalus. J Neurosurg 45: 20–25

    PubMed  Google Scholar 

  43. Sherman AR (1943) Teratoid tumor of conjunctiva and other developmental anomalies with naevus verrucosus of scalp. Report of case. Arch Ophthalmol 29: 441–445

    Google Scholar 

  44. Solomon L, Fretzin DF (1967) An unusual neurocutaneous syndrome. Arch Derm 96: 732–733

    PubMed  Google Scholar 

  45. Solomon LM, Fretzin DF, Dewald RL (1968) The epidermal nevus syndrome. Arch Derm 97: 273–285

    PubMed  Google Scholar 

  46. Sugarman GI, Reed WB (1969) Two unusual neurocutaneous disorders with facial cutaneous signs. Arch Neurol 21: 242–247

    PubMed  Google Scholar 

  47. Tishler PV (1975) A family with coexistent von Recklinghausens's neurofibromatosis and von Hippel-Lindau's disease: diseases possibly derived from a common gene. Neurology (1975) 25: 840–844

    Google Scholar 

  48. Tripp JH (1971) A new “neurocutaneous” syndrome (skin, eye, brain, and heart syndrome). Proc Roy Soc Med 64: 7–8

    Google Scholar 

  49. Van Bogaert L (1950) Pathologie des angiomatoses Acta Neurol Psych Belg 50: 525–610

    Google Scholar 

  50. Wauschkuhn J, Rhode BB (1971) Systematisierte Talgdrüsen-, Pigment- und epitheliale Naevi mit neurologischer Symptomatic: Feuerstein-Mimssches Neuroektodermales Syndrom. Der Hautarzt 22, Heft 1, 10–13

    Google Scholar 

  51. Wilkes SR, Campbell RJ, Waller RR (1981) Ocular malformation in association with ipsilateral facial nevus of Jadassohn. Am J Ophthalmol 92: 344–352

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurosurgery, Institut Bordet, Université Libre de Bruxelles, Belgium

    J. Brihaye & J. Retif

  2. Department of Ophthalmology, Akademisch Ziekenhuis, Vrije Universiteit Brussel, Belgium

    M. Brihaye-van Geertruyden & A. M. Mercier

Authors
  1. J. Brihaye
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. M. Brihaye-van Geertruyden
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. J. Retif
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. A. M. Mercier
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Brihaye, J., Brihaye-van Geertruyden, M., Retif, J. et al. Late occurrence of additional ocular and intracranial pathologies in the linear naevus sebaceous (Feuerstein-Mims) syndrome. Acta neurochir 92, 132–137 (1988). https://doi.org/10.1007/BF01401983

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01401983

Keywords

Search

Navigation