PMID- 18382985 OWN - NLM STAT- MEDLINE DCOM- 20080715 LR - 20091119 IS - 1019-5149 (Print) IS - 1019-5149 (Linking) VI - 18 IP - 1 DP - 2008 Jan TI - Hereditary neuropathy with liability to pressure palsies in a Turkish patient (HNPP): a rare cause of entrapment neuropathies in young adults. PG - 82-4 AB - Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant nerve disease usually caused by 1,5 Mb deletion on chromosome 17p11.2.2-p12, the region where the PMP-22 gene is located. The patients with HNPP usually have relapsing and remitting entrapment neuropathies due to compression. We present a 14-year-old male who had acute onset, right-sided ulnar nerve entrapment at the elbow. He had electrophysiological findings of bilateral ulnar nerve entrapments (more severe at the right side) at the elbow and bilateral median nerve entrapment at the wrist. Genetic tests of the patient demonstrated deletions in the 17p11.2 region. The patient underwent decompressive surgery for ulnar nerve entrapment at the elbow and completely recovered two months after the event. Although HNPP is extremely rare, it should be taken into consideration in young adults with entrapment neuropathies. FAU - Celik, Yahya AU - Celik Y AD - Trakya University School of Medicine Neurology, Edirne, Turkey. celikyahyatr@yahoo.com FAU - Kilincer, Cumhur AU - Kilincer C FAU - Hamamcioglu, M Kemal AU - Hamamcioglu MK FAU - Balci, Kemal AU - Balci K FAU - Birgili, Baris AU - Birgili B FAU - Cobanoglu, Sebahattin AU - Cobanoglu S FAU - Utku, Ufuk AU - Utku U LA - eng PT - Case Reports PT - Journal Article PL - Turkey TA - Turk Neurosurg JT - Turkish neurosurgery JID - 9423821 RN - 0 (Myelin Proteins) RN - 0 (PMP22 protein, human) SB - IM MH - Adolescent MH - Chromosomes, Human, Pair 17 MH - Elbow Joint/innervation MH - Gene Deletion MH - Hereditary Sensory and Motor Neuropathy/*genetics/*pathology MH - Humans MH - Male MH - Median Neuropathy/genetics/pathology MH - Myelin Proteins/*genetics MH - Turkey MH - Ulnar Nerve Compression Syndromes/*genetics/*pathology EDAT- 2008/04/03 09:00 MHDA- 2008/07/17 09:00 CRDT- 2008/04/03 09:00 PHST- 2008/04/03 09:00 [pubmed] PHST- 2008/07/17 09:00 [medline] PHST- 2008/04/03 09:00 [entrez] PST - ppublish SO - Turk Neurosurg. 2008 Jan;18(1):82-4.