Protection from clinical peripheral sensory neuropathy in Alstrom syndrome in contrast to early-onset type 2 diabetes.

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Adult, Blindness, Child, Diabetes-Mellitus-Type-2, Foot-Ulcer, Genetic-Diseases-Inborn, Humans, Peripheral-Nervous-System-Diseases, Syndrome, Young-Adult

JAX Source

Diabetes Care 2009 Mar; 32(3):462-4.


OBJECTIVE: Alstrom syndrome, with type 2 diabetes, and blindness could confer a high risk of foot ulceration. Clinical testing for neuropathy in Alstrom syndrome and matched young-onset type 2 diabetic subjects was therefore undertaken. RESEARCH DESIGN AND METHODS: Fifty-eight subjects with Alstrom 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 Alstrom syndrome. CONCLUSIONS: The striking preservation of protective foot sensation in Alstrom syndrome may provide a clue to the causes of differential susceptibility to neuropathy in the wider diabetic population.