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European Journal of Pediatrics

, Volume 152, Issue 5, pp 402–405 | Cite as

A kindred with Griscelli disease: Spectrum of neurological involvement

  • H. Hurvitz
  • R. Gillis
  • S. Klaus
  • A. Klar
  • F. Gross-Kieselstein
  • E. Okon
Immunology/Allergology

Abstract

We report four members of a highly consanguineous family with silver-grey pigmentation of hair, two of whom had skin histology compatible with Griscelli disease. Unlike previously reported patients, they did not suffer from recurrent infections. In addition, there was a spectrum of neurological involvement varying from mild cognitive delay with a convulsive disorder in one patient, to a fatal degenerative course in three others. One patient developed a prolonged febrile illness with histological evidence of florid lymphoid hyperplasia.

Key words

Griscelli Silver hair Cerebral involvement Lymphoid hyperplasia 

Abbreviation

EBV

Epstein-Barr virus

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Copyright information

© Springer-Verlag 1993

Authors and Affiliations

  • H. Hurvitz
    • 1
  • R. Gillis
    • 1
  • S. Klaus
    • 2
  • A. Klar
    • 1
  • F. Gross-Kieselstein
    • 1
  • E. Okon
    • 3
  1. 1.Department of PaediatricsBikur Cholim General HospitalJerusalemIsrael
  2. 2.Department of DermatologyHaddasah Medical CentreJerusalemIsrael
  3. 3.Department of PathologyHadassah Medical CentreJerusalemIsrael

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