title: Protection from clinical peripheral sensory neuropathy in Alström syndrome in contrast to early-onset type 2 diabetes.
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.
  
  
  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.
authors.affiliation: Torbay Hospital, Torquay, UK. richard.paisey@nhs.net
authors.forename: Richard B
authors.initials: RB
authors.lastname: Paisey
authors.forename: Rosamund M
authors.initials: RM
authors.lastname: Paisey
authors.forename: Mary P
authors.initials: MP
authors.lastname: Thomson
authors.forename: Lynne
authors.initials: L
authors.lastname: Bower
authors.forename: Pietro
authors.initials: P
authors.lastname: Maffei
authors.forename: Julian P H
authors.initials: JP
authors.lastname: Shield
authors.forename: Sue
authors.initials: S
authors.lastname: Barnett
authors.forename: Jan D
authors.initials: JD
authors.lastname: Marshall
mesh_terms: D000293:Adolescent; D000328:Adult; D001766:Blindness / Q000235:genetics; D002648:Child; D003924:Diabetes Mellitus, Type 2 / Q000150:complications* / Q000503:physiopathology; D016523:Foot Ulcer / Q000175:diagnosis / Q000209:etiology; D030342:Genetic Diseases, Inborn / Q000150:complications*; D006801:Humans; D010523:Peripheral Nervous System Diseases / Q000175:diagnosis / Q000209:etiology*; D013577:Syndrome; D055815:Young Adult