Focal pontine leukoencephalopathy in a patient with the Shwachman-Diamond syndrome.
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.
Mah
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Department of Pathology (Neuropathology), UCLA Medical Center 90024.
Nelson
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Vinters
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HV
D000328:Adult; D000380:Agranulocytosis / Q000473:pathology*; D001369:Axons / Q000648:ultrastructure; D002531:Cerebellum / Q000473:pathology; D010188:Exocrine Pancreatic Insufficiency / Q000473:pathology*; D005260:Female; D006801:Humans; D002479:Inclusion Bodies / Q000648:ultrastructure; D007968:Leukoencephalopathy, Progressive Multifocal / Q000473:pathology*; D009434:Neural Pathways / Q000473:pathology; D009503:Neutropenia / Q000473:pathology*; D010198:Pancytopenia / Q000473:pathology*; D011149:Pons / Q000473:pathology*; D013577:Syndrome