HEREDITARY NEURALGIC AMYOTROPHY (HNA) - C LINICAL FINDINGS AND MOLECULAR-GENETICS

Hereditary neuralgic amyotrophy (HNA) and hereditary neuropathy with l iability to pressure palsies (HNPP) are hereditary focal neuropathies. In this study we describe three families suffering from HNA. These fa milies were examined clinically and electrophysiologically. Linkage an alysis with markers from distal chromosome 17 was performed in a three -generation family. HNA could be separated from HNPP in all three fami lies based on clinical and electrophysiological findings. HNA was char acterised by recurrent episodes of painful brachial plexus lesions. In contrast to HNPP, no evidence for generalised neuropathy was found in the HNA families. Linkage analysis confirmed the HNA locus on distal chromosome 17. Additionally, we were able to refine the HNA locus to a 16 cM region on chromosome 17q24-q25.