The natural history of hereditary neuralgic amyotrophy in the Dutch population: Two distinct types?

On investigation of 101 attacks in 24 patients with hereditary neuralgic amyotrophy (HNA) from nine different families, we found that HNA can run two distinct courses: a `classic' relapsing–remitting and a chronic undulating type with exacerbations. Only one type occurred per family, suggesting genetic heterogeneity. This is supported by the finding that only in a family with `classic type' HNA are data of linkage analysis compatible with linkage to the 17q24–q25 interval which harbours a locus for the disease. The average number of attacks per patient during a follow-up of 26 years was four i... Mehr ...

Verfasser: van Alfen, N.
van Engelen, B. G. M.
Reinders, J. W. C.
Kremer, H.
Gabreëls, F. J. M.
Dokumenttyp: TEXT
Erscheinungsdatum: 2000
Verlag/Hrsg.: Oxford University Press
Schlagwörter: Articles
Sprache: Englisch
Permalink: https://search.fid-benelux.de/Record/base-26632327
Datenquelle: BASE; Originalkatalog
Powered By: BASE
Link(s) : http://brain.oxfordjournals.org/cgi/content/short/123/4/718

On investigation of 101 attacks in 24 patients with hereditary neuralgic amyotrophy (HNA) from nine different families, we found that HNA can run two distinct courses: a `classic' relapsing–remitting and a chronic undulating type with exacerbations. Only one type occurred per family, suggesting genetic heterogeneity. This is supported by the finding that only in a family with `classic type' HNA are data of linkage analysis compatible with linkage to the 17q24–q25 interval which harbours a locus for the disease. The average number of attacks per patient during a follow-up of 26 years was four in the classic form of HNA and five in the chronic undulating type. All patients suffered from residual symptoms on follow-up, with a median Rankin score of 2 in both groups, showing that long-term prognosis is less favourable than previously reported.