Protection From Clinical Peripheral Sensory Neuropathy in Alström Syndrome in Contrast to Early-Onset Type 2 Diabetes

  1. Richard B. Paisey, MD1,
  2. Rosamund M. Paisey, RGN1,
  3. Mary P. Thomson, PHD1,
  4. Lynne Bower1,
  5. Pietro Maffei, MD3,
  6. Julian P.H. Shield, MD2,
  7. Sue Barnett, PHD2 and
  8. Jan D. Marshall4
  1. 1Torbay Hospital, Torquay, U.K.
  2. 2Bristol Royal Hospital for Children, Bristol, U.K.
  3. 3Department of Medical and Surgical Science, University School of Medicine, Padua, Italy
  4. 4The Jackson Laboratory, Bar Harbor, Maine
  1. Corresponding author: Richard B. Paisey, richard.paisey{at}nhs.net

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.

Footnotes

  • Published ahead of print at http://care.diabetesjournals.org on 17 December 2008.

    Readers may use this article as long as the work is properly cited, the use is educational and not for profit, and the work is not altered. See http://creativecommons.org/licenses/by-nc-nd/3.0/ for details.

    The costs of publication of this article were defrayed in part by the payment of page charges. This article must therefore be hereby marked “advertisement” in accordance with 18 U.S.C Section 1734 solely to indicate this fact.

    • Accepted December 4, 2008.
    • Received August 29, 2008.
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  1. Published online before print December 17, 2008, doi: 10.2337/dc08-1584 Diabetes Care March 2009 vol. 32 no. 3 462-464
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