Protection from clinical peripheral sensory neuropathy in Alström syndrome in contrast to early-onset type 2 diabetes.
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.
Paisey
Richard B
RB
Torbay Hospital, Torquay, UK. richard.paisey@nhs.net
Paisey
Rosamund M
RM
Thomson
Mary P
MP
Bower
Lynne
L
Maffei
Pietro
P
Shield
Julian P H
JP
Barnett
Sue
S
Marshall
Jan D
JD
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