1. Protection From Clinical Peripheral Sensory Neuropathy in Alström Syndrome in Contrast to Early-Onset Type 2 Diabetes
- Author
-
Mary P. Thomson, Rosamund M. Paisey, Pietro Maffei, Susan Barnett, Julian P.H. Shield, Jan D. Marshall, Richard B Paisey, and Lynne Bower
- Subjects
Adult ,medicine.medical_specialty ,Pediatrics ,Adolescent ,Endocrinology, Diabetes and Metabolism ,Population ,Type 2 diabetes ,Blindness ,Vibration perception ,Young Adult ,Insulin resistance ,Diabetes mellitus ,Sensation ,Internal Medicine ,medicine ,Humans ,education ,Pathophysiology/Complications ,Child ,Foot Ulcer ,Advanced and Specialized Nursing ,education.field_of_study ,business.industry ,Genetic Diseases, Inborn ,Peripheral Nervous System Diseases ,Syndrome ,medicine.disease ,Surgery ,Diabetes Mellitus, Type 2 ,Age of onset ,business ,Alström syndrome - Abstract
OBJECTIVE—Alström syndrome, with type 2 diabetes, and blindness could confer a high risk of foot ulceration. Clinical testing for neuropathy in Alström syndrome and matched young-onset type 2 diabetic subjects was therefore undertaken. RESEARCH DESIGN AND METHODS—Fifty-eight subjects with Alström syndrome (18 insulin-resistant nondiabetic and 40 diabetic; aged 8–43 years) and 30 young-onset diabetic subjects (aged 13–35 years) were studied. Neuropathy symptom questionnaires were administered. Graded monofilament and 128-MHz tuning fork vibration perception were assessed in both feet. RESULTS—Neuropathic symptoms, loss of monofilament, and/or vibration perception were reported by 12 of the 30 young-onset type 2 diabetic subjects (6 had neuropathic ulceration) but none of the subjects with Alström syndrome. CONCLUSIONS—The striking preservation of protective foot sensation in Alström syndrome may provide a clue to the causes of differential susceptibility to neuropathy in the wider diabetic population.
- Published
- 2009