EPIDEMIOLOGY OF HEREDITARY NEUROPATHY WITH LIABILITY TO PRESSURE PALSIES (HNPP) IN SOUTH WESTERN FINLAND

An epidemiological study of hereditary neuropathy with liability to pr essure palsies (HNPP) was carried out in south western Finland, with a population of 435 000. The diagnosis was established in 69 patients f rom 23 unrelated families through family and medical history, clinical neurological and neurophysiological examinations and with documentati on of the deletion at gene locus 17p11.2 in at least one member of eac h family. This gave a prevalence of at least 16/100 000, which is rema rkably high. However, due to the insidious nature of HNPP, most probab ly it is still an underestimation. This is the first population-based prevalence figure reported for HNPP. The prevalence is somewhat lower than that obtained for CMT in the same population, which agrees with t he proposal that HNPP and CMT 1A are reciprocal products of the same u nequal crossing-over. The clinical pictures of our patients were, in g eneral, similar to those previously described in HNPP. (C) 1997 Elsevi er Science B.V.