This report adds 4 members in a kindred with diabetes insipidus, diabetes mellitus, and optic atrophy to the published descriptions (DeLawter, 1949;Fraccaro and Gastaldi, 1952;Casa, 1955;Raiti, Plotkin, and Newns, 1963;Bretz et al, 1970;Ikkos et al, 1970) left frontal area and right anterior temporal spiking. Audiometry demonstrated moderate bilateral perception deafness. Craniotomy performed at age 11 revealed arachnoiditis in the area of the optic chiasm with atrophy of both optic nerves.At age 21, the patient was 167 cm tall and weighed 66-7 kg. In addition to the primary optic atrophy, vertical nystagmus and rare microaneurysms were present. Knee and ankle tendon reflexes could not be obtained. His testis was 4 cm long on the right and 3-5 cm on the left.Except for variable hyperglycaemia, blood and serum solutes have been normal. Glucose tolerance test revealed a clearly diabetic curve with no evidence of insulin response (Morgan, 1966) to an oral glucose load. A minor growth hormone (Morgan, 1966) peak was recorded at the 5th hour of this test. Plasma ll(OH) corticosteroid levels (Mattingly, 1962) have been within the normal range. Intravenous arginine (0-41 mg/kg) had no effect upon serum insulin levels but did produce a rise in serum growth hormone to 11 ng/ml. Serum PBI (Danowski, Johnston, and Greenman, 1950) and thyroidal 13"I uptake were 3-8y% and 16%, respectively.Creatinine clearance (Peters, 1942) was 141 ml/min. The urinary 17-ketosteroids (Holtorff and Koch, 1940) were 12-2 with Porter-Silber chromogens (Porter and Silber, 1950) at 11-4 and compound S metabolites (Henke, Doe, andJacobson, 1960) at 0-88 mg/day. Case 2. (brother of case 1). This 24-year old single male was found to have diabetes mellitus at the age of 3 years following polyuria, polyphagia, and polydipsia. He was treated with an anti-diabetic diet and insulin, most recently 54 units of NPH daily. At the age of 12 years, a diagnosis of optic atrophy was also made following the discovery of optic atrophy in his sibs. At age 15, urine volumes increased to 8 1/dy. Twenty hours of water deprivation raised urine osmolarity relative to serum only slightly, ie, 455 versus 314 milliosmols, respectively, during an interval when urine sugar was zero. Chlorpropamide, 250 mg/dy, reduced urine volumes to 21/dy. The intravenous infusion of arginine (0-41 mg/kg) produced a rise in the serum growth hormone to 25 ng/ml. 408 on 11 May 2018 by guest. Protected by copyright.