title: Severe insulin resistance in a patient with type 1 diabetes and stiff-man syndrome treated with insulin lispro.
abstract: We describe a patient with type 1 diabetes with recurrent diabetic ketoacidosis and severe insulin resistance. Extensive evaluation of the etiology of the insulin resistance did not reveal an etiology, and well over 1000 U of daily insulin did not prevent the ketoacidosis. Her blood glucose and insulin requirements were improved with glucocorticoids and octreotide, but the effects of both of these agents were short-lived. She was given a trial of insulin lispro with immediate and dramatic effects, lowering her HbA1c from 14.6 to 5.1% in 7 months with a decrease in insulin requirements of 1600-100 U per day. Besides her diabetes, she had a history of pain and stiffness affecting numerous muscle groups, and hospitalization was required for pain control. The diagnosis of stiff-man syndrome (SMS) was confirmed with high titers of glutamic acid decarboxylase 65 antibodies in both serum and cerebral spinal fluid. In summary, we describe the first patient with type 1 diabetes, SMS, and severe insulin resistance. Although the etiology of the insulin resistance is unknown, due to the efficacious response to insulin lispro, hydrocortisone, and perhaps octreotide, we propose an immune-mediated etiology. Although rare, this syndrome needs to be considered as an etiology of insulin resistance.
authors.affiliation: UWMC, Seattle, WA 98195, USA.
authors.forename: I B
authors.initials: IB
authors.lastname: Hirsch
authors.forename: D
authors.initials: D
authors.lastname: D'Alessio
authors.forename: L
authors.initials: L
authors.lastname: Eng
authors.forename: C
authors.initials: C
authors.lastname: Davis
authors.forename: A
authors.initials: A
authors.lastname: Lernmark
authors.forename: A
authors.initials: A
authors.lastname: Chait
mesh_terms: D000328:Adult; D001786:Blood Glucose / Q000032:analysis; D015992:Body Mass Index; D003922:Diabetes Mellitus, Type 1 / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology; D016883:Diabetic Ketoacidosis / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology; D005260:Female; D005968:Glutamate Decarboxylase / Q000097:blood / Q000134:cerebrospinal fluid / Q000276:immunology; D006442:Glycated Hemoglobin / Q000032:analysis; D006728:Hormones / Q000627:therapeutic use; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D007004:Hypoglycemic Agents / Q000494:pharmacology / Q000627:therapeutic use*; D007328:Insulin / Q000031:analogs & derivatives* / Q000494:pharmacology / Q000627:therapeutic use; D061268:Insulin Lispro; D007333:Insulin Resistance / Q000502:physiology*; D015282:Octreotide / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D016750:Stiff-Person Syndrome / Q000150:complications* / Q000503:physiopathology
chemical_list: D001786:Blood Glucose; D005968:Glutamate Decarboxylase; D006442:Glycated Hemoglobin; D006728:Hormones; D006854:Hydrocortisone; D007004:Hypoglycemic Agents; D007328:Insulin; D061268:Insulin Lispro; D015282:Octreotide; D011241:Prednisone