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The Chédiak-Higashi syndrome: CT and MR findings

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Abstract

Chédiak-Higashi syndrome (CHS) is a rare autosomal recessive disorder postulated to result from lack of regulation of fusion of the primary lysosomes. In this report we present the MR and CT features of the brain in a patient with known CHS. These findings include diffuse atrophy of the brain with diffuse periventricular decreased density identified with CT, as well as increased signal on the T2-weighted images and lack of enhancement on the T1-weighted images in the periventricular and corona radiata regions.

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References

  1. Blume RS, Wolff SM (1972) The Chédiak-Higashi syndrome: studies in four patients and a review of the literature. Medicine (Baltimore) 51: 247–280

    Google Scholar 

  2. Sheramata W, Kott S, Cyr DP (1971) The Chédiak-Higashi-Steinbrinck syndrome: presentation of three cases with features resembling spinocerebellar degeneration. Arch Neurol 25: 289–294

    PubMed  Google Scholar 

  3. Sung JH, Meyers JP, Stadlan EM, Cowen D, Wolf A (1969) Neuropathological changes in Chédiak-Higashi disease. J Neuropathol Exp Neurol 28: 86–118

    PubMed  Google Scholar 

  4. Fix JD (1992) Lesions of the spinal cord. In: Fix JD (ed) Neuroanatomy. Williams and Wilkins, Baltimore, p 105

    Google Scholar 

  5. Stolz W, Graubner U, Gerstmeier J, Burg G, Belohradsky B (1989) Chédiak-Higashi sydnrome: approaches in diagnosis and treatment. Curr Probl Dermatol 18: 93–100

    PubMed  Google Scholar 

  6. Barak Y, Nir E (1987) Chédiak-Higashi syndrome. Am J Pediatr Hematol Oncol 9: 42–55

    PubMed  Google Scholar 

  7. Maeda K, Sueishi K, Lida M (1989) A case report of Chédiak-Higashi syndrome complicated with systemic amyloidosis and olivo-cerebellar degeneration. Pathol Res Pract 185: 231–237

    PubMed  Google Scholar 

  8. Argyle JC, Kmeldsberg CR, Marty J, Shigeoka AO, Hill R (1982) T-cell lymphoma and the Chédiak-Higashi syndrome. Blood 60: 672–676

    PubMed  Google Scholar 

  9. Dent PB, Fish LA, White JG, Good RA (1966) Chédiak-Higashi syndrome: observations on the nature of the associated malignancy. Lab Invest 15: 1634–1642

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Radiology, Neuroradiology Section, Duke University Medical Center, Box 3808, 27710, Durham, NC, USA

    R. Ballard & R. D. Tien

  2. Department of Neurology, Duke University Medical Center, 27710, Durham, NC, USA

    V. Nohria & V. Juel

Authors
  1. R. Ballard
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  2. R. D. Tien
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  3. V. Nohria
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  4. V. Juel
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Ballard, R., Tien, R.D., Nohria, V. et al. The Chédiak-Higashi syndrome: CT and MR findings. Pediatr Radiol 24, 266–267 (1994). https://doi.org/10.1007/BF02015453

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  • DOI: https://doi.org/10.1007/BF02015453

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