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

Diabetes Care. 2009 Mar;32(3):462-4. doi: 10.2337/dc08-1584. Epub 2008 Dec 17.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Blindness / genetics
  • Child
  • Diabetes Mellitus, Type 2 / complications*
  • Diabetes Mellitus, Type 2 / physiopathology
  • Foot Ulcer / diagnosis
  • Foot Ulcer / etiology
  • Genetic Diseases, Inborn / complications*
  • Humans
  • Peripheral Nervous System Diseases / diagnosis
  • Peripheral Nervous System Diseases / etiology*
  • Syndrome
  • Young Adult