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, aged 8-43 years (18 insulin resistant, non-diabetic and 40 diabetic) and thirty young onset diabetic subjects aged 13-35 years were studied. Neuropathy symptom questionnaires were administered. Graded monofilament and 128MHz 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 type 2 diabetic subjects (six 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.