Triple H syndrome: A novel autoimmune endocrinopathy characterized by dysfunction of the hippocampus, hair follicle, and hypothalamic-pituitary-adrenal axis

We describe two unrelated adult Caucasian females who developed the previou sly unreported triad of isolated ACTH deficiency, impairment of anterograde memory and alopecia areata. In one bf these women the abnormalities were s evere, with marked hypocortisolemia, alopecia universalis, and incapacitati ng, irreversible memory loss associated with radiological abnormalities in the hippocampus. In the other subject disturbances in all systems were less profound, and the alopecia and memory impairment were transient. The simil arity between these two cases, and the uncommon nature of the component abn ormalities suggest that they represent a novel syndrome, probably of autoim mune origin. Other than the very rare encephalopathy associated with Hashimoto's thyroid itis, there are few examples where the central nervous system is a target o rgan in the context of autoimmune endocrine disease. The highly specific na ture of the central nervous system involvement in this novel syndrome sugge sts the involvement of a molecular target for autoimmunity shared among the hippocampus, the hair follicle, and the hypothalamus and/or corticotroph. We suggest that the term triple Il syndrome may serve to alert clinicians t o this-previously undescribed triad.