A 32-year-old man who had unexplained diabetes insipidus for 14 years developed hypogonadism and was found to have a pi-
Presentation of CaseA 32-year-old engineer noted tingling of the right hand and foot 5 months after uncomplicated transsphenoidal pituitary adenectomy.At age 18, in 1975, he had developed diabetes insipidus (DI) and had been treated with chlorpropamide and later desmopressin acetate (DDAVP). There had been no history of radiation therapy or head trauma and no family members had DI. At that time, visual fields and skull radiographs were normal. Measurements of growth hormone (GH), thyroid-stimulating hormone (TSH), luteinizing hormone (LH), follicle-stimulating hormone (FSH), Received May 1, 1991, and in revised form Jun 1L Accepted for publication Jun 13, 199LAddress correspondence to Dr FetelL adrenocorticotropic hormone (ACTH), and cortisol were within normal limits. The testosterone level was low normal, at 360 ngjdl (normal, 300-900 ng/dl). Seven years later the testosterone level was 184 ng/dl (normal, 300-1,200 ngjdl) but he fathered his second child. Head computed tomograms (CT) in axial and coronal planes appeared normal at this time.Difficulty fathering a third child at age 32 prompted reevaluation. He noticed less frequent shaving but no change in glove or shoe size. Findings on neurological examination, including visual fields, were normal. The testosterone level was 16 ng/dl (normal, > 300 ng/dl); GH, TSH, LH, FSH, and ACTH levels were normal. Prolactin measured 30 ngjml (normal, 0-20 ng/ml). A T1-weighted magnetic resonance image (MRI) in the sagittal plane showed a 2 X 2.5-cm sellar mass that projected upward to reach the inferior portion of the optic chiasm (Fig 1). A T1-weighted study with gadolinium enhancement demonstrated that the sellar mass was uniformly enhanced and that there was an irregular enhancing mass in the pons (Fig 2). A T2-weighted study (not pictured) showed high signal diffusely within both masses. Gadolinium-enhanced axial and coronal studies showed that the pontine mass compressed the fourth ventricle and extended rostrally to the hypothalamus but spared the optic chiasm (Figs 3, 4). There was no hydrocephalus. At the same time, an axial CT with contrast material demonstrated only faint enhancement in the pons (Fig 5). In the cerebrospinal fluid, protein content was 81 mg/dl and glucose, 56 mg/dl; there were 2 white blood cells per mm 3 and cytology showed no malignant cells. Transsphenoidal adenectomy led to the pathological diagnosis of chromophobe adenoma; stains for GH, ACTH, and prolactin were negative.Since the brainstem lesions noted on MRI were asymptomatic, they were not treated. Six months after surgery, the patient noted paresthesias of the right hand and foot and a diagnostic procedure was performed.
Differential DiagnosisDr Keith A. Sanders: This man developed central DI at age 18 and over the next 15 years hypogonadism appeared_ Ultimately, a unilateral sensory disturbance was attributed to brainstem and hypothalamic abnormalities demonstrated...