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Dissociation of plasma and spinal fluid ACTH in Nelson syndrome.

Nine years after a bilateral adrenalectomy for Cushing syndrome and three years after a craniotomy for a chromophobe adenoma, a 51-year-old white woman with marked hyperpigmentation was reexamined because of recurrent visual field losses. Serum and cerebrospinal fluid adrenocorticotropic hormone (ACTH) levels were elevated. Following intravenous administration of hydrocortisone, the serum ACTH levels were only partially suppressed. Unexpectedly, the cerebral spinal fluid ACTH level increased and remained elevated. ( JAMA 228:491-492, 1974)