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Focal Pontine Leukoencephalopathy in a Patient with the Shwachman-Diamond Syndrome

Published online by Cambridge University Press:  18 September 2015

Vei Mah ,
Lee Nelson  and
Harry V. Vinters
Vei Mah*
Affiliation:
Department of Pathology (Neuropathology), UCLA Medical Center, Los Angeles
Lee Nelson
Affiliation:
Department of Pathology (Neuropathology), UCLA Medical Center, Los Angeles
Harry V. Vinters
Affiliation:
Department of Pathology (Neuropathology), UCLA Medical Center, Los Angeles
*
Department of Pathology (Neuropathology), CHS 18-170, UCLA Medical Center, Los Angeles, CA. U.S.A. 90024
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Abstract:

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A 35-year-old woman who died after a long history of an illness consistent with Shwachman-Diamond Syndrome, was found to have extensive calcified necrotizing lesions confined to the pontocerebellar fibers of the basis pontis. The possible relationship of this recently described lesion to the patient's immunosuppressed state and/or other systemic metabolic disturbances is discussed.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 14 , Issue 4 , November 1987 , pp. 608 - 610
Copyright
Copyright © Canadian Neurological Sciences Federation 1987

References

1.Anders, KH, Guerra, WF, Tomiyasu, U. et al. The neuropathology of AIDS. UCLA experience and review. Am J Pathol 1986: 124: 537–558.Google ScholarPubMed
2.Breuer, AC. Blank, NK. Schoene, WC. Multifocal pontine lesions in cancer patients treated with chemotheraphy and CNS radiotherapy. Cancer 1978:41: 2112–2120.3.0.CO;2-W>CrossRefGoogle Scholar
3.Rubinstein, LJ. Herman, MM. Long, TF, Wilbur, JR. Disseminated necrotizing leukoencephalopathy: a complication of treated central nervous system leukemia and lymphoma. Cancer 1975: 35: 291–305.3.0.CO;2-V>CrossRefGoogle ScholarPubMed
4.Vinters, HV, Anders, KH. Barach, P. Focal pontine leukoencephalopathy in immunosuppressed patients. Arch Pathol Lab Med 1987: 111: 192–196.Google ScholarPubMed
5.Watanabe, I, Hodges, GR. Dworzack, DL. et al. Neurotoxicity of intrathecal gentamicin: a case report and experimental study. Ann Neurol 1978: 4: 564–572.CrossRefGoogle ScholarPubMed
6.Ang, LC, Heathcote, JG, Gilbert, JJ. Multifocal pontine leukoencephalopathy. A clinicopathologic study of three cases. J Neuropathol Exp Neurol 1986; 45: 350 (abstract).CrossRefGoogle Scholar
7.Aggett, PJ, Cavanagh, NPC, Matthew, DJ, et al. Shwachman’s Syndrome. A review of 21 cases. Arch Dis Child 1980; 55: 331–347.CrossRefGoogle ScholarPubMed
8.Shwachman, H, Diamond, LK, Oski, FA, Khaw, K-T. The syndrome of pancreatic insufficiency and bone marrow dysfunction. J Pediatr 1964; 65: 645–663.CrossRefGoogle ScholarPubMed
9.Kleinschmidt-Demasters, BK. Norenberg, MD. Rapid correction of hyponatremia causes demyelination: relation to central pontine myelinolysis. Science 1981:211: 1068–1070.CrossRefGoogle ScholarPubMed
10. Idem. Neuropathologic observations in electrolyte-induced myelinolysis in the rat. J Neuropathol Exp Neurol 1982; 41: 67–80.CrossRefGoogle Scholar
11.Laureno, R. Central pontine myelinolysis following rapid correction of hyponatremia. Ann Neurol 1983: 13: 232–242.CrossRefGoogle ScholarPubMed
12.Sterns, RH, Riggs, JE. Schochet, SS Jr. Osmotic demyelination syndrome following correction of hyponatremia. N Engl J Med 1986: 314: 1535–1542.CrossRefGoogle ScholarPubMed
13.Steinsapir, KD. Vinters, HV. Central pontine myelinolysis in a child with the Shwachman-Diamond Syndrome. Hum Pathol 1985: 16: 741–743.CrossRefGoogle Scholar